Leaf tumor. Phylloidal or leaf-shaped fibroadenoma of the mammary gland: how to treat the pathological growth of the glandular and connective tissue of the milk ducts

Over the past 30 years of operation of the oncological center, only 168 patients with this tumor pathology have been observed, which is 1.2% of all tumor diseases of the mammary glands. We have not identified men with this tumor pathology. The presence of a palpable node in the mammary gland in 166 patients (98.8%) was the main reason for visiting a doctor.

At the same time, only two women (1.2%) complained of pain in the affected mammary gland. Discharge from the nipple of the breast was observed in 2 patients (1.2%). In 2 women, the tumor was detected during a preventive examination. The age of patients with leaf-shaped tumor ranged from 11 to 74 years. The mean age of the patients was 39.9 years. Women between the ages of 30 and 50 are most susceptible to this disease.

The average age of patients with a benign leaf-shaped tumor was significantly lower (p Leaf-shaped tumors of the mammary glands were localized in the right gland in 83 cases (49.4%), in the left mammary gland - in 80 (47.6%), in both mammary glands - in 5 (2.97%) In 16 patients (9.5%) with a leaf-shaped tumor, more than one node was detected, while in 5 cases (2.97%) the tumors were localized in both mammary glands and in 11 cases (6.5% ) - in one of the glands (5 - in the right, 6 - in the left).

Synchronous occurrence of a leaf-shaped tumor and fibroadenoma in the other mammary gland was detected in 5 patients (2.97%). The presence of more than one node in the mammary gland reliably indicates a benign variant of the leaf-shaped tumor (p
The study of the anamnesis of the disease made it possible to identify the following options for the growth rate of leaf-shaped tumors: tumors characterized by slow, rapid or two-phase growth (a period of long-term stable existence is replaced by a stage of rapid growth).

In 63 cases (37.5%), rapid growth was detected, in 52 cases (30.9%), a slow increase in the tumor from the moment of its increase was noted, and in 53 cases (31.5%), a two-phase course of the process, when a long-term formation suddenly began to rise sharply.
However, this criterion does not allow differentiating different variants of leaf-shaped tumor.

When examining women with leaf-shaped tumors, in most cases, the skin over the neoplasm was not changed - 118 cases (70.2%). Such skin symptoms as its fixation over the tumor, the "platform" symptom, are extremely rare and are not typical for leaf-shaped tumors - 5 patients (2.97%). More often in patients with a leaf-shaped tumor, skin symptoms such as cyanosis, thinning of the skin over the formation, and a pronounced venous pattern occur. They reflect the rapid, expansive growth of the tumor and the violation of the trophism of the skin of the mammary gland, but by no means invasion of it by the tumor. The result of increasing trophic changes in the skin is its ulceration.

A leaf-shaped tumor on palpation was a well-defined neoplasm delimited from the surrounding breast tissue.
Clear contours were detected in 140 cases (83.3%), indistinct contours - in 28 cases (16.6%). Tuberosity and smoothness of the contours of the neoplasm were noted in almost equal proportions (75 (44.6%) and 93 (55.4%) cases, respectively).

Symptoms such as the heterogeneous consistency of the tumor and the tuberosity of its contours, detected by palpation, are a reflection of the characteristic macroscopic picture. When examining the removed tumors in such cases, cavities were found filled with a mucoid mass and polypoid growths in them.

Changes in the nipple, so typical of breast cancer, are not characteristic of a leaf-shaped tumor. We encountered nipple retraction in 3 patients (1.8%), nipple edema was found in 14 cases (8.3%) of leaf-shaped tumor. Palpable lymph nodes of elastic consistency on the side of the lesion were found in 26 patients (15.5%), lymph node enlargement was always reactive and was more common in women with trophic skin changes.

The size of leaf-shaped breast tumors varied from 1 to 35 cm. The average size in the total group of leaf-shaped tumors was 7.46 cm. However, interesting data were obtained when determining the average size of leaf-shaped tumors of various histological variants. It turned out that the minimum size of the tumor was detected in the benign variant of leaf-shaped tumors - 6.87 cm, while in the malignant variant - 14.09 cm (with the intermediate - 11.56 cm).

On this basis, benign leaf-shaped tumors with a size of up to 5 cm significantly differ from the intermediate and malignant variants of tumors (p
In the analysis of clinical diagnoses established in the clinic of the Russian Cancer Research Center. N.N. Blokhin of the Russian Academy of Medical Sciences, out of 168 patients with leaf-shaped tumors, 13 cases (7.7%) were diagnosed with a leaf-shaped tumor without specifying the degree of malignancy, and in 28 cases (16.7%) - a diagnosis of sarcoma. Breast cancer was diagnosed in 59 cases (35.1%), fibroadenoma in 58 cases (34.5%), and cyst and nodular mastopathy in 6 (3.6%) and 4 (2.4%) cases, respectively.

At the same time, in all cases with tumors less than 5 cm, an incorrect diagnosis was made ("fibroadenoma", "cancer", "cyst", "nodular mastopathy"). With tumors of large and giant sizes, clinicians in most cases diagnosed breast sarcoma - 28 cases (16.7%).

Thus, when the tumor size is less than 5 cm, the clinical diagnosis of a leaf-shaped tumor is extremely difficult. In the vast majority of such observations, the leaf-shaped tumor was represented by a well-demarcated, solid formation of a dense consistency without any skin symptoms and changes in the nipple-areolar complex, which led to the establishment of a clinical diagnosis of fibroadenoma in 58 cases (34.5%). The presence of a small seal of elastic consistency against the background of diffuse mastopathy without clear contours was the reason for the diagnosis of nodular mastopathy in 4 cases (2.4%).

Identification of skin symptoms (fixation of the skin over the tumor, "platform", etc.) in combination with a palpable tumor of a dense consistency with tuberous contours served as the basis for the diagnosis of breast cancer in 59 patients (35.1%). Cyst - in 6 cases (3.6%), diagnosed in those cases where clinically the formation had an elastic consistency, smooth, even contours (macroscopically it was represented by a single-chamber cavity with mucus-like contents and polypoid growths that did not fill its entire lumen). In 28 cases (16.7%), the basis for the diagnosis of breast sarcoma was a number of clinical and anamnestic data (rapid tumor growth with reaching large sizes; characteristic changes in the skin over the tumor in the form of thinning, hyperemia, cyanosis, increased venous pattern; heterogeneous consistency neoplasms, tuberosity of contours).

Thus, for the most part, the diagnosis of "leaf-like tumor" turns out to be a diagnosis established at the histological level. Thus, only 41% of preoperative diagnoses corresponded to the histological diagnosis.

Analyzing therapeutic approaches for benign and intermediate variants of leaf-shaped tumors, it can be stated that all variants of surgical interventions used in diseases of the mammary glands were used. The main option for surgical treatment is sectoral resection of the mammary gland (81.2% of cases). The use of various types of mastectomies and radical resections is due either to the large size of the tumor or to diagnostic errors.

The data in the table show that an increase in the volume of surgical intervention leads to a decrease in the likelihood of developing a local recurrence of the disease. So, in all cases of tumor enucleation, local recurrences occurred, with sectoral resections in 19.7% of cases, and after mastectomy - only in 1 case (4.8%). Relapses develop on average after 17 months (from 3 to 4 years). However, the time for the development of tumor recurrence after surgery is longer with a benign variant of a leaf-shaped tumor than with an intermediate one (45.5 and 26.3 months; p>0.05). Comparison of various options for performing mastectomy with the course of the disease did not reveal the presence of correlations between them.

The situation is similar with sectoral and radical resections of the mammary glands. There were no significant differences in the tendency to recurrence depending on age, neoplasm growth rate, morphological criteria. When comparing the histological variant of the tumor and the development of recurrence, it was revealed that intermediate leaf-shaped tumors recur more often than benign ones (23.8% and 17.4%, respectively, p > 0.05). Patients with relapses were re-operated: mastectomy was performed in 4 cases, sectoral resection was performed in the rest. It should be noted that the tendency to recurrence is a characteristic feature of leaf-shaped tumors, and sometimes it becomes persistent (15 relapses were noted in one patient)

Unjustified tightening of therapeutic measures (carrying out chemotherapy, radiation therapy) is due to errors in the diagnosis of the disease.

There were no distant metastases and deaths associated with these histological forms. A completely different picture is observed when analyzing the course of malignant leaf-shaped tumors (23 patients), where, along with local recurrence, there is also distant metastasis (malignancy is due to the development of sarcoma against the background of a leaf-shaped tumor). As mentioned earlier, the average size of malignant leaf-shaped tumors (11.6 cm) significantly predominates over that in other histological variants of this disease. A characteristic clinical picture is represented by an increase in the volume of the affected mammary gland. The skin of the gland is thinned, of a purple-bluish hue, with an expanded subcutaneous venous network. The tumor is mobile relative to the chest wall.

A malignant leaf-shaped tumor occurs significantly at a later age than a benign one (43.8 and 37.5 years, respectively; p
The data in the table indicate that recurrence is a characteristic feature of this tumor process and develops both after sectoral resections and after radical mastectomy. At the same time, after sectoral resections, local recurrences occurred almost twice as often as after mastectomy (40% and 22.2%, respectively; p>0.05). Relapses in a malignant variant of a leaf-shaped tumor develop significantly earlier than in a benign variant (14.25 and 45.5 months; p 0.05). No other correlations (including the fact of adjuvant treatment) affecting the likelihood of relapse were found.

Relapses that occurred in 5 patients were promptly removed. Two of them relapsed (in one case - after radiation therapy), which, in turn, required additional surgical intervention (in one patient, the pectoralis major muscle was removed with resection of the anterior rib segments - she is alive in the subsequent 8 years).

The presence of malignancy of the stromal component predetermined the features of the course of the disease. We did not reveal metastases of leaf-shaped tumors in regional lymph nodes. Hematogenous metastases were noted in 4 patients (lungs, liver, bones), which led to death.

In one case (liver metastases) occurred simultaneously with a recurrence in the area of ​​operation (after mastectomies) after 4 years, in the other - for 2 years, also after mastectomies. Attempts to conduct chemotherapy in all cases were unsuccessful. A significant relationship was found between the development of metastases and the size of the primary tumor node: for example, in the presence of metastases, the average size of the latter was 20 cm, while in the case of a favorable course of the disease, it was 6.37 cm (p

Breast sarcomas:

During the same period of time, from 1965 to 1999, 54 patients with a histologically confirmed diagnosis of breast sarcoma were treated in the clinics of the Russian Cancer Research Center of the Russian Academy of Medical Sciences, which is 0.34% of all tumor diseases of the mammary glands. In this group of tumor pathology, 1 man was noted.

The average age of patients is 44.1 years (16-69 years) and practically does not differ from that in malignant leaf-shaped tumors of the mammary glands. The advantage of the side of the lesion was not revealed: the process in the left mammary gland was detected in 26 cases, in the right - 28. Multicentricity, synchrony of the lesion in this group of patients was not noted. The size of the tumor node varied from 7 to 35 cm, averaging 14.09 cm.

Describing their disease, most patients note the rapid, sometimes rapid growth of the tumor, which is the main reason for visiting a doctor.

The clinical picture of mammary sarcomas does not fundamentally differ from that of a malignant leaf-shaped tumor: the affected mammary gland, as a rule, is significantly enlarged in volume, with purple-cyanotic skin and a pronounced subcutaneous venous network. Diagnostic criteria are more informative than for leaf-shaped tumors. More than half of patients (74%) have a short history of the disease (less than a year), which is due to the rapid, sometimes rapid growth of the tumor.

When assessing the growth rate of breast neoplasms, a history of rapid and two-phase growth rates was noted both in leaf-shaped tumors and in sarcomas. A slow growth rate was noted mainly by patients with leaf-shaped tumors. A slow growth rate is not characteristic of breast sarcomas (only 1.8%). Thus, the presence of a slow growth rate is more indicative of the presence of a leaf-shaped breast tumor than a sarcoma (p
With an increase in the size of the tumor node, the percentage of mammary sarcomas increases. Thus, when the size of the tumor node is more than 15 cm, sarcoma was detected in 71% of cases. At the same time, with a neoplasm size of up to 3 cm, not a single case of a malignant leaf-shaped tumor and sarcoma was detected.

According to the microscopic picture, the following types of soft tissue sarcomas were identified: osteogenic sarcoma - 1, angiosarcomas - 15, liposarcoma - 4, neurogenic - 5, leiomyosarcoma - 5, rhabdomyosarcoma - 0, malignant fibrous histiocytoma - 11. Review of histological preparations due to their absence in the pathoanatomical archive in 13 cases was not performed (it was treated as a polymorphic cell sarcoma without regard to histogenetic affiliation).

The large size of the tumor node, the rapid growth of the neoplasm and the threat of its ulceration predetermined the surgical stage of treatment in the vast majority of cases. Surgical intervention was an integral component of treatment in 92.6% of patients (50 patients). As an independent type of primary treatment in 33 patients (61.1%). In other cases, the operation was supplemented with radiation therapy - in 8 cases, chemotherapy - in 6 cases, and their combination - in 3 patients. 4 patients attempted chemotherapy due to the initial generalization of the process. In addition to surgery, radiation therapy (standard radiation therapy ROD 2 Gy, SOD 40-46 Gy, radiation therapy with large fractions ROD5Gy, SOD20Gy) and chemotherapy were used mainly for the malignant variant of leaf-shaped tumors and sarcomas.

As a postoperative effect, radiation therapy was used in 12 cases, in the treatment of relapses and (or) metastases - in 11. The use of various therapy regimens reflects the stages in the development of chemotherapeutic approaches in oncology: from Thio-Tef monotherapy to regimens using drugs from the group of anthracycline antibiotics and platinum preparations. As an adjuvant treatment, chemotherapy was performed in 9 cases, in 18 - as a therapy for the metastatic process. The most frequently used regimens included vincristine, adriamycin and cyclophosphamide (14 cases). Hormone therapy in the complex treatment of leaf-shaped tumors and breast sarcomas was carried out in two cases of steady progression of the metastatic process. The volume of surgical intervention varied from sectoral resection to radical Halsted mastectomy (radical resection was not performed).

There was no correlation between different types of mastectomies and the course of the disease, so all types of mastectomies are combined into one group. The table data eloquently show that the volume of surgical intervention in the form of sectoral resection is clearly insufficient - in 71% local recurrence of the disease, while with mastectomy - 22% (p
At the same time, additional therapeutic measures (radiotherapy, chemotherapy, or their combination) do not significantly affect the nature of the course of the disease. At the same time, if we do not detail adjuvant treatment by type, but divide patients with developed relapses according to the presence or absence of adjuvant therapy, then adjuvant treatment was accompanied by the development of relapse in 5 patients, and in the absence of treatment, relapse occurred in 12 patients (in 3 out of 8 after radiotherapy; in 1 out of 6 after chemotherapy and 1 out of 3 after chemoradiotherapy). And, although there is no significant difference in these groups (probably due to the small number of observations), these data should be taken into account.

Interesting results were obtained by comparing the course of the disease with the histological form of sarcoma. It turned out that in 12 (66.7%) of 18 patients with local recurrence of the disease, angiosarcoma of the breast was detected, which is characterized by persistent recurrence and an extremely unfavorable prognosis. No recurrence was found in lipo- and neurogenic breast sarcoma. Thus, the course of the disease, apparently, depends more on the histological form of the disease than on the volume of therapeutic measures.

Concerning the choice of the volume of surgical intervention, in our opinion, one should dwell on mastectomy. Lymphadenectomy has no grounds for its performance: lymphogenous metastasis is not typical for sarcomas. According to our data, histological examination of sarcoma metastases in regional lymph nodes were not detected. Metastasis was noted mainly in the lungs. The fact of local recurrence is an unfavorable prognostic factor for the development of distant metastases (in 11 out of 18 patients with local recurrence, distant metastases were detected; p
Patient survival is low. During the 1st year, 9 patients (16.6%) died, 5-year survival was 37.8%, 10 years survived 28.0%.

Treatment of distant metastases (lungs, bones, liver) is ineffective. Regardless of the type of chemotherapy, the effect was either absent or short-lived. Only 2 cases of success were noted: excision of a solitary metastasis in the lung (liposarcoma), the patient is alive for 22 years later, and 1 case of effective chemotherapy in lung metastases (malignant fibrous histiocytoma, 9 courses of chemotherapy with vincristine, carminomycin and interferon), death of this The patient came 5 years after the end of chemotherapy from the generalization of another malignant disease - gallbladder cancer.

Leaf-shaped fibroadenoma of the mammary gland or myxomatous is an uncommon disease that is a two-component formation. It is dominated by growths in the epithelium and in the connective tissue, and the latter is many times larger. Among all breast neoplasms, myxomatous fibroadenoma can be detected only in 3-5% of cases. The disease is rarely diagnosed on time, so the risk of malignancy is very high. In one case out of ten, fibroadenoma degenerates into sarcoma. In addition, the tumor is rapidly growing, often recurs, and surgery is indispensable.

Fibroepithelial dense in structure formation that does not have capsular limitation. The structure is lobulated, not involving the skin. However, with giant forms, ingrowth into the pectoral muscle can occur. Histological examination of the tissue reveals the presence of viscous mucus, which is contained in the cystic cavity. Slit-like cysts can be either in a single instance or represented by many polypoid inclusions. The color of the tumor is gray-white or pink with coarse-grained components and lobules. Also, the neoplasm can be in one breast or in both mammary glands. Cases of leaf-shaped formation have also been registered in men, despite the difference in the structure of tissues with the female breast.

The classification of a leaf-shaped tumor depends on the size, growth rate and forms of damage to the stroma of the mammary gland.

Small, up to 5 cm in diameter, leaf-shaped neoplasms have a structure of slit-like depressions, clearly localized. If the tumor is larger than 5 cm, histology shows cystic "clustered" growths that consist of more than one node.

The classification of leaf-shaped neoplasm forms is approved by an international degree. Distinguish:

  • benign
  • Intermediate or borderline
  • Malignant

The growth rate does not depend on the malignancy of the process. Even a small education can be a threat to life. Phylloid fibroadenoma is characterized by a rapid pace of development.

How dangerous is she

The danger of a tumor is the possibility of developing proliferative processes that carry a carcinogenic threat. The risk of progression of cancer cells in phyllodes fibroadenoma is several times higher than in ordinary fibromastopathy.

Also, this formation is characterized by a rapidly growing tumor. The rapid replacement of healthy tissues with tumor-like growths contributes to the deformation of the shape of the breast. A leaf-shaped neoplasm grows to a gigantic size if there is no adequate therapy.

Metastasis of tumor cells in phyllodes fibroadenoma occurs along the intracanalicular pathway - through the channels and ducts of the mammary gland. It is very difficult to treat advanced cases.

Even after surgical treatment, the disease is capable of recurrence, therefore, a woman who has undergone leaf-shaped fibroadenoma should be monitored all her life.

The diagnosability of the disease is very low compared to other breast tumors. It is determined quite by chance during an examination using ultrasound.

Reasons for development

One of the main signs that contribute to the proliferation of pathological tissues of the leaf-like type is the hormonal factor. Active production of hormones falls on the reproductive age - from 20 to 40 years. It is during this period that the possibility of the formation of the progression of phyllodes fibroadenoma appears. Also, malfunctions in the hormonal system, and precisely estrogen and progesterone, are observed in the premenopausal period of a woman's life. There is a high risk of developing the disease from 50 years of age.

Provocateurs of pathology are considered:

  • Frequent aborted cases.
  • The lactation period and its violations (for example, abruptly interrupted breastfeeding).
  • Disturbed metabolic processes, including failures of metabolic processes and obesity.
  • Cancers of other organs, including the ovaries and uterus.
  • Chronic diseases (diabetes mellitus type I and II, hepatitis and others).
  • Possible disturbances in the activity of the endocrine system.
  • Reduced protective functions of the immune system.

A particular cause of leaf-shaped fibroadenoma is a genetic predisposition to the formation of mutagenic cells and heredity.

Symptoms of manifestation

Leaf growths in the breast of a woman are of a two-stage nature: a long-term disease may not make itself felt for many years, but with a certain push (hormonal, immune, and others), progressive tumor growth is provoked. It has a rapid development and is called myxomatous giant fibroadenoma.

The initial period is characterized by the absence of symptoms of leaf-shaped fibroadenoma. Often, a breast lump is identified as a small movable ball to the touch, or with the help of diagnostic procedures: ultrasound or mammography.

The second stage is the growth of cells, which can take oncological form. This is manifested by the following symptomatic triad:

  • Allocations
  • Burning

Pain in the chest with leaf-shaped fibroadenoma can be constant, aching, or occur after physical exertion or stress and a sharp change in the emotional background. Discharge from the nipples is an interlobular effusion of cancer cells, it can be yellow, light gray or milky in color. With large tumors, the discharge has a reddish tint or with impurities of blood streaks, since the pectoral muscle is affected.

Burning is present in the chest, where a small nodule of fibroadenoma is located. At the same time, the skin above it begins to thin and turn blue. Dilated venous ducts are clearly visible, which also extend to the armpit.

In giant or advanced cases of leaf-shaped fibroadenoma, the formation of ulcers on the skin is considered a rare occurrence.

The general condition of the body also suffers. Among the symptoms of well-being, there is an increase to subfebrile temperature, weakness, malaise, loss of appetite or its absence at all. With further malignancy of the tumor, anemia develops. Medicines to relieve pain and other symptoms do not work.

When leaf-shaped fibroadenoma metastasizes, neighboring organs are affected, then the symptoms will be associated with them - pain in the liver, lungs, and so on.

Diagnostics

Diagnosis of leaf-shaped fibroadenoma is difficult, since it has been in "sleep mode" for many years. If a seal of any size is determined in the chest, you should immediately seek medical help by visiting a mammologist.

The doctor will prescribe an extended diagnostic examination, which includes:

  • Ultrasound examination of both mammary glands.
  • X-ray mammography (it is carried out depending on age - nulliparous women are often not done).
  • Doppler ultrasound to study the blood flow of the mammary glands (in the presence of a tumor, the blood flow will be disturbed).
  • Magnetic resonance imaging scans the breast tissue in layers.

Examination of the mammary glands with the help of an ultrasound machine allows you to determine the boundaries of the leaf-shaped fibroadenoma. The photo clearly shows a heterogeneous structure with numerous cavities and cystic gaps. In appearance, phyllodes fibroadenoma can be compared with a head of cabbage. Ultrasound also allows you to identify the structure of the neoplasm and the exact size and localization.

A symmetrical examination of the breast is also characteristic of mammography, which will very accurately characterize changes in the tissues of the mammary glands, will reveal the smallest formations at the earliest stages of progression, and will also give a complete description of the tumor about its structure. The structure can be coarse-grained, lobulated, or in the form of various cystic inclusions. The results are described by a competent specialist, it depends on him what the result will be:

  • Positive
  • Negative
  • false positive
  • false negative

To eliminate errors, mammography is always supplemented by other non-invasive examination methods.

MRI of the mammary glands is a unique study, which, according to doctors, is the most informative. It can be done with or without a contrast agent. In the first case, you can check for the presence of cystic elements, tissue density, and expansion of the milk ducts. When using contrast, MRI helps to find out what character the tumor has - benign or malignant, reveals enlarged and enlarged nearby lymph nodes, assesses the size and location of overgrown pathological tissues.

In fact, MRI is the most accurate method for identifying pathological cells in places where other diagnostics have been uninformative.

For optimal therapy, a progressive tumor should be clearly differentiated from a malignant process. In this case, a biopsy is taken of the tumor in different places using a puncture. The resulting tissue sample is then sent for cytological laboratory testing.

Treatment Method

The only and most effective way to treat leaf-shaped fibroadenoma is surgery. Only by excising pathological tissues can the development and growth of a tumor, as well as its degeneration into a malignant process, be prevented.

The volume of surgical intervention directly depends on the size of the tumor formation. If the process is of a benign form, then the following is performed:

  1. Sectoral resection - the organ is preserved, performed under general anesthesia or with the help of local anesthesia. Has a faster recovery period. Among the advantages is an inconspicuous scar along the edge of the areola or a substernal suture.
  2. Quadrantectomy - excision of the quadrant of the breast where the tumor is located. It is an organ-preserving operation.
  3. Enucleation - the operation looks like the process of exfoliation of the tumor, is carried out through a small incision, applicable to small sizes of leaf-shaped fibroadenoma.

Complete excision of the mammary gland with leaf-shaped fibroadenoma occurs in the following cases:

  • With a malignant process, which is dangerous in terms of rapid metastasis.
  • With a gigantic size of education.
  • With necrotic skin, which is often overstretched.

Upon receipt of data on the malignancy of the process, it is necessary to treat phyllodes fibroadenoma according to the principle of cancer therapy. After surgical treatment, chemotherapeutic drugs are applied to the body to increase the prognosis of survival and avoid recurrence of the disease.

The prognosis for leaf-shaped fibroadenoma is varied and depends on the type of tumor. With a benign course of the disease, the prognosis will be positive if measures are taken in time to remove tumor cells and further monitor the woman. With a malignant phenomenon after a radical mastetomy, fibroadenoma can recur in another breast, so regular visits to a mammologist-oncologist and the necessary diagnostics are strictly required.

Leaf-shaped tumors and sarcomas of the mammary glands: clinic, diagnosis, treatment.

Nonepithelial and fibroepithelial tumors mammary glands are quite rare (1.54%) and therefore little studied. All these tumors are characterized as neoplasms having a two-component structure with a predominant development of the connective tissue component, which is absolute in sarcomas, and in the group of fibroepithelial tumors it is combined with the parallel development of epithelial tissue. The rarity of these neoplasms, the peculiarity of the clinical course, and the polymorphism of the morphological structure explain the limited awareness of doctors about them and the heterogeneity of their views both on the nature of these processes and on the principles of treatment approaches.

In order to assess the modern possibilities of diagnosing and optimizing therapeutic approaches for leaf-shaped tumors and sarcomas of the mammary glands, we have summarized more than 25 years of experience of the Oncological Center in the treatment of these tumors; We also tried to analyze the receptor status of tumors and study the proliferative characteristics of tumors using laser flow cytofluorometry.

During this period, we identified 168 (1.2%) patients with leaf-shaped tumors and 54 (0.34%) with breast sarcomas (one of the largest observations in the world practice). During the year, no more than 10 patients with this tumor pathology receive complex treatment at the Oncological Center.

The clinical picture is not specific and varies from small tumors with clear contours to neoplasms occupying the entire mammary gland (Fig. 1). In the latter case, the skin is purple-bluish in color, thinner, with sharply dilated subcutaneous vessels. Often there is ulceration of the skin, which, however, does not always indicate the malignancy of the process.

Rice. 1. Breast sarcoma

Fig.2. Distribution of patients depending on the histological type of tumor

There are 3 histological variants of leaf shaped tumors that differ in the ratio of stromal and epithelial components, clarity of tumor contours, cellularity, nuclear polymorphism, the number of mitotic figures, and the presence of heterogeneous elements. As can be seen from fig. 2, the benign variant of the tumor predominates. The presence of various histological types of leaf-shaped tumors, which determine the characteristics of the clinical course, contributed to the emergence of numerous variants of clinical terminology for designating these neoplasms. The most common term is phyllodes cystosarcoma, indicating an aggressive course of the tumor. Of the histological variants of sarcomas, angiosarcomas and malignant fibrous histiocytomas predominate (49%). These neoplasms are detected at almost any age (from 11 to 74 years), but the peak incidence occurs at 40-50 years of age. We found benign leaf-shaped tumors significantly more often at a younger age - 38 years (Fig. 3).

Fig.3. Distribution of patients with different histological types of tumor by age (in %)

As the malignancy of the process increases, the average size of neoplasms increases: with a benign leaf-shaped tumor - 6.9 cm, with an intermediate variant - 11.6 cm, with a malignant variant and sarcomas - 14.1 cm. When analyzing the possibilities of various methods the study found no reliable diagnostic criteria. Thus, the primary conclusions of mammographic examination coincided with the histological diagnosis only in 29% of cases with leaf-shaped tumors (n=147) and in 24% with sarcomas (n=39). The so-called depletion zone was revealed by us only in 21% of cases. The greatest difficulties arise in neoplasms with a diameter of less than 5 cm. Radiological criteria have not been established to distinguish the malignant variant of leaf-like tumors from breast sarcoma (Fig. 4, 5).

Fig.4. Benign leaf-shaped tumor in patient B., 39 years old. In the right mammary gland in the lower outer quadrant, a lobular nodular formation of a homogeneous structure with clear contours, 6.5 * 5.0 cm in size, is determined. The skin, nipple and areola are not changed.

Fig.5. X-ray of the right mammary gland of the craniocaudal projection of patient A., 20 years old. Neurogenic sarcoma of the right breast. In the upper quadrant, a lobular nodular formation sized 7*6 cm is determined, the contours are clear, a strip of enlightenment along the perimeter of the tumor node.

We tried to find out the possibilities of ultrasound of the mammary glands (21 patients with leaf-shaped tumors and 3 with sarcoma). A small number of observations has not yet made it possible to identify clear diagnostic criteria for distinguishing histological variants of leaf-shaped tumors (Fig. 6, 7). The only sign that drew attention to itself was the low blood flow velocity (2.4-6.4 cm/sec), including the peak one.

Fig.6. Benign leaf-shaped tumor (patient K., 21 years old). Hypoecogenic formation with clear even contours, heterogeneous structure, slit-like cavities inside the formation.

Fig.7. Breast sarcoma (patient M., 49 years old). Hypoecogenic formation of a heterogeneous structure, with uneven fuzzy contours, infiltration rim.

An analysis of the possibilities of cytological examination of tumor punctures showed that the primary conclusions in 29% of cases with leaf-shaped tumors and in 29% with sarcomas corresponded to the actual diagnosis. Failures, in our opinion, are due to the peculiarities of the histological structure of tumors and polymorphism (a combination of epithelial and stromal components, the presence of cystic cavities). The analysis of preoperative diagnoses showed that the latter corresponded to the histological conclusion only in 42% of cases. Thus, in most cases, the diagnosis of a non-epithelial or fibroepithelial tumor of the breast was a histological diagnosis. When analyzing treatment approaches for benign and intermediate leaf-shaped tumors in 144 patients (Table 1), it can be seen that all variants of surgical interventions were used. More often performed sectoral resection of the mammary glands. The use of mastectomy or radical resection is due either to the large size of the tumors or to errors in diagnosis. An increase in the volume of surgical intervention significantly leads to a decrease in the likelihood of local recurrence. So, if after sectoral resection recurrence occurred in 19.7% of cases, after mastectomy - in 4.8%. In general, recurrence was noted in 19.4% of cases. Tumor enucleation in 100% leads to the development of local recurrence. Distant metastasis in the indicated histological forms was not noted. With these histological variants, we consider a sectoral resection to be a sufficient volume; in the case of a total lesion of the mammary gland - a mastectomy.

Table 1. Treatment of patients with benign and intermediate leaf tumors

The course of malignant leaf-shaped tumors (23 patients) was due to malignancy of the stromal component (development of sarcoma against the background of a leaf-shaped tumor). The analysis showed that the structure of surgical interventions differed significantly from that in benign tumors. Various types of mastectomy accounted for 76% (with a higher recurrence rate of 26%). Recurrence after sectoral resection was observed 2 times more often than after mastectomy (Table 2). Metastasis - hematogenous (lungs, bones, liver). Metastases to regional lymph nodes were not noted. Sufficient amount of surgical intervention - mastectomy. There is no need for a lymphadenectomy.

Table 2 Recurrence of malignant leaf tumors by treatment options

Treatment of metastases has been unsuccessful; The 5-year survival rate was 58.5%. Adjuvant treatment resulted in non-significant improvement in outcomes. The most unfavorable prognostically are breast sarcomas (53 women and 1 man). The large size of the tumor node, the rapid growth of the neoplasm, and the threat of ulceration in most cases predetermined the need for surgical treatment. Surgical intervention in the volume of sectoral resection is clearly insufficient - after it, the development of relapses was noted in 71% of cases, while after mastectomy - in 22%. At the same time, in 12 out of 18 patients with recurrence, the tumor turned out to be angiosarcoma. Necessary and sufficient amount of surgical intervention for breast sarcomas is mastectomy. There is no need to perform lymphadenectomy (metastases to regional lymph nodes have never been detected). Distant metastasis was noted in 41% of cases. Adjuvant therapy does not improve long-term outcomes; during its implementation, some deterioration in the results of treatment was noted, which, in our opinion, is due to a more pronounced initial prevalence of the process (Table 3).

Table 3. Features of the clinical course of breast sarcomas depending on the primary treatment options

Postoperative radiation therapy was performed in 12 cases, chemotherapy - in 9 (including a combination of these regimens - in 5), in which various schemes were used: from TIOTEF monochemotherapy to the use of platinum preparations and anthracycline antibiotics. Treatment of sample metastases is lematic. Radiation therapy was performed in 11 cases, chemotherapy - in 18 cases, including 9 combined treatment. Treatment was successful in 2 cases: excision of solitary lung metastasis (liposarcoma) and full effect after 9 courses of chemotherapy for malignant fibrohistiocytoma (carminomycin, vincristine, interferon); The 5-year survival was 37.8%. Data on the survival of patients with various morphological variants of the tumor is presented in Fig. 1. 8.

Fig.8. Survival of patients (in %) with different morphological variants of tumors.

We do not have our own experience with hormone therapy. Tamoxifen was used as a step of desperation in 2 cases with steady progression of the process. The receptor status was analyzed in 48 patients (30 patients with leaf shaped tumor and 18 with sarcoma). It has been established that as the process becomes malignant, the content of steroid hormone receptors decreases, including estrogens (ER) - at the level of a trend, and progesterones (PR) - with significant differences.

Comparison of the level of receptors and the course of the disease in benign and intermediate leaf-shaped tumors showed an inversely proportional relationship between ER and PR (differences are not significant), while in malignant primary neoplasms in the case of development of local recurrences of receptor-positive tumors was not observed. In breast sarcomas, there were no differences in the content of receptors in primary tumors and in local relapses, while in the case of distant metastases in the primary tumor, a higher level of both ER and PR was noted.

Another equally important criterion characterizing the tumor process is the proliferative activity of the tumor, which is detected by flow cytofluorometry. As the process becomes more malignant, the frequency of aneuploid tumors (103 paraffin blocks) increases: with malignant leaf-shaped tumors, aneuploidy is 20%, with sarcomas - more than 92%. It should be noted that with a favorable course of leaf-shaped tumors, there were no aneuploid formations. An analysis of the distribution of cells by phases of the cell cycle showed that, in addition to significant differences in the content of cells in different phases of the cycle, there were significant differences between the primary and recurrent tumors in each of the histological variants of leaf-shaped tumors. The proliferation index in benign and intermediate leaf-shaped tumors in the case of recurrence was significantly higher than in tumors with a favorable course, and in malignant leaf-shaped tumors it corresponded to that in breast sarcomas. The development of the metastatic process in sarcomas was accompanied by a significantly higher proliferation index in primary tumors.

Thus, based on the study, the following conclusions can be drawn:

  1. Existing research methods (X-ray, ultrasound of the mammary glands, routine cytological examination with Leishman staining), lacking reliable criteria for diagnosing non-epithelial and fibroepithelial tumors of the mammary glands, do not allow differentiating different histological variants of these neoplasms.
  2. Necessary and sufficient volume of surgical intervention for benign and intermediate forms of leaf-shaped tumor - sectoral resection; with a total lesion of the mammary gland, with a malignant variant of leaf-shaped tumors and sarcomas of the mammary glands - mastectomy; there are no grounds for performing lymphadenectomy.
  3. Adjuvant therapy for malignant leaf-shaped tumors and sarcomas of the mammary glands does not lead to a significant improvement in treatment outcomes: relapse-free 5-year survival rate for malignant leaf-shaped tumors in the case of adjuvant treatment - 81.8 ± 16.4%, without it - 53.4± 17.0% (p>0.05); with sarcomas - 33.73±12.5% ​​and 49.0±10.8%, respectively (p>0.05). The overall 5-year survival rate for malignant leaf-shaped tumors is 58.5 ± 15.0%, for sarcomas - 37.8 ± 8.5%.
  4. Different morphological variants of leaf-shaped tumors significantly differ in proliferative characteristics: the proliferation index for benign leaf-shaped tumors is 20.08±1.35%, for intermediate ones - 25.33±2.02%, for malignant ones - 31.23±2.71 % (p<0,05). Индекс пролиферации при саркомах молочных желез соответствует таковому при злокачественных листовидных опухолях - 31,88±2,43%.
  5. The high proliferative activity of the primary tumor in benign and intermediate leaf shaped tumors was significantly (p<0,05) ассоциируется с развитием местного рецидива. Так, индекс пролиферации при развитии местных рецидивов достоверно превышал та ковой при благоприятном течении заболевания (соответственно 26,78 ± 1,41 и 15,82±1,31%; 32,85±2,72 и 22,39±1,37%).
  6. Metastatic process in breast sarcomas significantly more often (p<0,05) развивается в случае высоких значений индекса пролиферации первичной опухоли (34,46±2,77%), при отсутствии отдаленных метастазов - в 26,35±0,69%.
  7. The morphological variant of the tumor is interrelated with the degree of aneuploidy of the neoplasm. In benign and intermediate leaf-shaped tumors, aneuploid neoplasms were not observed, while in its malignant variants and sarcomas of the mammary glands, aneuploidy was detected in 20 and 92.3% of cases, respectively (p<0,05).
  8. As the malignancy of neoplasms increases (from benign leaf-like tumors to breast sarcomas), the level of PR decreases (44.46±8.75 and 9.05±2.57 fmol/mg protein, respectively; p<0,05). Различия в уровне ЭР недостоверны.
  9. The development of recurrence in benign and intermediate variants of leaf-shaped tumors is associated with a higher level of ER compared with that in a favorable course of the disease (51.71±8.35 and 24.53±7.34 fmol/mg, respectively; p>0.05) ; changes in PR have the opposite direction, reaching maximum values ​​in the primary tumor with a favorable course of the disease (48.97±8.64 and 32.7±8.32 fmol/mg protein; p>0.05).
  10. In breast sarcomas, the level of steroid hormone receptors in the primary tumor in the case of the development of a metastatic process is higher than in its absence (ER - 24±14.92 and 10.02±3.56 fmol/mg protein, respectively; PR - 15, 9±5.24 and 5.13±1.81 fmol/mg protein, p>0.05).

PHYLLODES TUMORS AND SARCOMAS OF THE BREAST: CLINICAL PICTURE, DIAGNOSIS, TREATMENT

I.K. Vorotnikov, V.N. Bogatyrev, G.P. Korzhenkova N.N. Blokhin Russian Cancer Research Center, Russian Academy of Medical Sciences

The material is taken from the journal "Mammology", No. 1, 2006

Many women perceive any neoplasm in the breast as malignant. However, in the diagnosis, in 80% of cases, benign changes are detected - fibroadenomas. They may have a different shape. Phylloid fibroadenoma (leaf-shaped) is often detected. In most cases, it is treatable and does not turn into cancer.

Types of benign neoplasms

Fibrous adenoma of the breast is a collection of glandular and fibrous tissues. When probing the chest, it is possible to detect tissue compaction in the form of a round or oval nodule. It can cause discomfort to a woman when pain occurs. However, such a neoplasm does not pose a serious danger, since it belongs to the class of non-cancerous ones.

There are several types of fibroadenomas. They differ in localization, shape and structure:


Increased attention should be paid to the last type of fibrous adenomas. To determine the nature of changes in the mammary gland, it is necessary to know what properties a leaf-shaped fibroadenoma has.

Characteristics of the phyllodes neoplasm

Despite the fact that the tumor is benign, there is an increased risk of its transition to sarcoma. Therefore, it is important to know what properties distinguish it from other forms of formations.

Leaf-shaped tumor is most often diagnosed in women experiencing a period of hormonal surge. This is usually the time of puberty (11-20 years) or the onset of menopause (45-55 years).

The occurrence of this type of fibroadenoma is influenced by many factors, among which are noted:


When a leaf-shaped fibroadenoma occurs, a seal is observed in the mammary gland, which has a limited localization. It is characterized by a lobed structure. When probing, you can detect the connection of several nodes into a single whole.

During the growth of the neoplasm, the appearance of the breast changes. The skin above it is stretched, has a cyanotic, sometimes purple color. A vascular and venous network is visible through it.

If there is a rapid growth of the neoplasm within 3-4 months, then doctors are inclined to make a diagnosis of "phylloid type fibroma". However, it can be confirmed only with the help of various instrumental studies.

Diagnostic methods

If you suspect a phyllodes fibroadenoma, you must definitely visit a mammologist. She will prescribe the necessary examinations to confirm or refute the diagnosis. Before the appointment, the doctor will conduct a complete examination of the breast, palpation, and also collect anamnesis data. In the future, the patient will need to undergo research using laboratory and instrumental diagnostics.


Only after the diagnosis, the doctor can prescribe treatment for the neoplasm.

Method of treatment of phyllodes fibroadenoma

If there is a formation in the breast less than 1 cm in size, doctors prescribe dynamic observation. In this case, a woman must visit a mammologist, repeat ultrasound and mammography after a while to identify the condition of phyllodes fibroadenoma.

If the neoplasm is large, then surgery is prescribed. It is shown when:

  • rapid growth of neoplasm;
  • the presence of a visible breast defect;
  • an extensive neoplasm, the size of which exceeds 5 cm;
  • planned pregnancy.

The operation is carried out in two
persons:

  • enucleation method;
  • sectoral resection.

During enucleation, the neoplasm is husked through a small incision made in the chest. In this case, there are practically no scars, they are insignificant.

Sectoral resection is distinguished by the removal of the neoplasm. The elimination of the tumor itself can be shown directly. In more severe cases, it is necessary to remove the tissue that surrounds it (3 cm from the edge of the nodes). The disadvantage of the method is the possible recurrence of fibroadenoma. In this case, amputation of the breast will be indicated.

Sometimes doctors resort to prescribing conservative treatment. It is indicated for small tumors, the size of which does not exceed 8 mm. Therapy is aimed at resorption of education. However, it does not always lead to a positive result.

After any medical manipulations, a woman needs to undergo a control ultrasound. Indeed, with complications and the absence of positive dynamics, the neoplasm can turn into a malignant one for no apparent reason. Therefore, with changes in the mammary gland, a woman should definitely consult a doctor.

Fibroadenoma of the breast - video

Fibroadenoma foliaceus is a rare breast tumor that usually develops in women in their 40s. These tumors are also called phyllodes, from the Greek word phyllodes, which means leaf-like. We can say that the more correct name is “leaf-shaped tumors”, since this is a group of neoplasms, whose representatives can have very different behavior.

This name is due to the fact that tumor cells have a leaf-shaped growth pattern. Fibroadenoma foliaceus tends to grow rapidly but rarely spreads beyond the breast.

Phylloid fibroadenoma is observed in approximately 0.5% of all breast tumors, it is formed from a combination of stromal and epithelial cellular elements. A neoplasm can develop both in the right and in the left breast.

There are three main types of phyllodes tumors:

  • Benign (non-cancerous) - make up approximately 50-60% of phyllodes tumors.
  • Borderline tumors are not yet malignant, but they can turn into them.
  • Malignant - make up approximately 20-25% of all leaf-shaped tumors.

In their least aggressive form, phyllodes tumors are similar to benign fibroadenomas, which is how they got their name, leaf-shaped breast fibroadenoma. On the other hand, malignant leaf-shaped neoplasms can metastasize with the bloodstream to distant organs, sometimes turning into sarcomatous lesions.

How do phyllodes tumors develop in the breast?

Unlike breast cancer called carcinoma, which develops inside the ducts or lobules of the breast (intracanalicular tumor), leaf-shaped tumors start growing outside of them (like pericanalicular fibroadenoma). Phylloidal tumors develop in the connective tissue (stroma) of the breast, which includes fatty tissue and ligaments surrounding the ducts, lobules, blood and lymph vessels in the breast. In addition to stromal cells, they may also contain cells from the ducts and lobules of the mammary gland.

Symptoms and signs of leaf-shaped fibroadenoma

The most common symptom of phyllodes tumors is a nodule in the breast, which the patient or physician may find on self-examination or breast examination. These neoplasms can grow rapidly over several weeks or months to a size of 2-3 cm, and sometimes more. Such rapid cell proliferation does not mean that a phyllodes tumor is malignant, because benign tumors can also grow rapidly.

The nodule is usually painless. If left untreated, the nodule can create a visible bulge. In more advanced cases, a leaf-shaped tumor can lead to the formation of an ulcer or an open sore on the skin of the breast.

Diagnostics

Like other, rare, types of breast tumors, leaf-shaped fibroadenoma is difficult to diagnose, since doctors almost never encounter it. Phylloid tumors may also look similar to the more common benign fibroadenomas.

The two key differences between fibroadenomas and leaf-shaped tumors are that the latter grow more rapidly and develop about 10 years later in age (after 40 as opposed to 30). These differences can help doctors distinguish between these growths.

Establishing a diagnosis is usually carried out in several steps:

  • Physical examination of the mammary glands;
  • Mammography;
  • Ultrasound procedure;
  • Magnetic resonance imaging.

Biopsy and histology is the only way to accurately establish the diagnosis of a leaf-shaped tumor. In addition, it is possible to determine the type of neoplasm (benign, borderline or malignant) and the degree of cell proliferation.

The term "benign tumor" often leads people to think that the disease is not dangerous and does not require treatment. But benign phyllodes tumors, like malignant tumors, can grow to large sizes, create visible nodules on the breast, and even break through the skin, causing pain and discomfort. Therefore, any type of these neoplasms requires treatment.

Treatment

Whether a leaf tumor is benign, malignant, or borderline, the treatment is the same - surgery to remove the tumor along with at least 1 cm of surrounding healthy breast tissue. Some doctors believe that even more healthy tissue needs to be removed.

Wide excision is important because, when it is not done, phyllodes tend to recur in the same area of ​​the breast. This applies to both malignant and benign neoplasms.

Possible surgeries:

  1. Lumpectomy - The surgeon removes the tumor and at least 1 cm of normal tissue around it.
  2. If the mass is very large or the breast is small, it can be very difficult to perform a wide excision and retain enough healthy tissue to provide a natural looking breast. In this case, the doctor may recommend carrying out:
    • Partial or segmental mastectomy - the surgeon removes the part of the breast that contains the tumor.
    • Total or simple mastectomy - the surgeon removes the entire breast, but nothing else.

Phylloidal tumors rarely spread to the axillary lymph nodes, so in most cases they do not need to be removed.

Malignant leaf-shaped tumors are rare. If they have not spread beyond the breast, radiation therapy may be used to stop cell proliferation. If they have metastasized to other parts of the body, treatment should include chemotherapy.

Care after treatment

The doctor should observe the patient after treatment. Phylloidal tumors can sometimes recur. Relapse usually develops within a year or two after surgery. Malignant leaf-shaped tumors may reappear faster than benign ones.

Physician and patient should cooperate by scheduling visits and examinations, which may include:

  • Physical examination of the breast by a doctor within 4-6 months;
  • Mammography and ultrasound examination 6 months after treatment;
  • Magnetic resonance or computed tomography - as prescribed by the doctor, if he suspects the risk of distant metastases.

If malignant leaf-shaped tumors reappear in the breast, treatment includes wide excision or mastectomy. Some doctors also recommend radiation therapy.

Less than 5% of phyllodes tumors recur in other areas of the body (distant metastases). Possible treatments include surgical removal, radiation therapy, and chemotherapy.