Tumori, 92: 188-190, 2006
REPORT ON A CASE OF BREAST SARCOMA METASTATIC TO THE AXILLARY LYMPH NODES Jack Antonio Diaz Brito1, Giovanna Gatti1,2, Anna Rita Vento1, Viviana Galimberti1, Mattia Intra1, Rafaela Cecilio Sahium De Almeida1, Giancarlo Pruneri3, and Alberto Luini1,4 1 Division of Breast Surgery, European Institute of Oncology, Milan; 2Postgraduate School of General Surgery, Perugia; 3Division of Pathology, European Institute of Oncology, Milan; 4University of Milan School of Medicine, Milan, Italy
Breast sarcoma is a rare entity that accounts for less than 1% of all breast malignancies; it may spread by direct invasion and/or through the blood. Axillary lymph node involvement is extremely rare and usually associated with advanced-stage disease. In the surgical treatment of this neoplasm, complete
axillary lymphadenectomy is important for the local clearance of the clinically involved lymph nodes. We report a case of a 65-year-old woman affected by follicular dendritic cell sarcoma of the left breast. Six months after breast surgery she developed an axillary metastasis from the same disease.
Key words: axillary lymph nodes, breast sarcoma.
Introduction
Primary sarcoma of the breast is an unusual condition that accounts for less than 1% of all breast malignancies and less than 5% of all soft tissue sarcomas1,2. The most significant factors influencing treatment and prognosis are tumor grade, disease stage and previous history of breast malignancy or radiotherapy3-8. Surgical resection is the primary treatment for breast sarcoma; the role of adjuvant radiotherapy and chemotherapy is less defined. Sarcoma is resistant to standard doses of radiation and does not seem sensitive to the majority of chemotherapy agents. Breast sarcoma may spread into the body by direct invasion and/or via the blood. Axillary lymph node involvement is extremely unusual and is associated with advanced-stage disease9. Case report
A 65-year-old post-menopausal woman sought medical attention for a left breast lump. The patient was married, a non-smoker and an occasional drinker; she had two children and no family history of breast malignancy. Physical examination showed a hard, mobile left breast mass in the internal quadrants on the equatorial line. The mass had become palpable five months before the first clinical check: it was hard, irregular in clinical shape, fast-growing, with uncertain margins, 2 cm in maximum diameter. Mammogram revealed the presence of a parenchymal distortion, while ultrasound showed a round mass with inhomogeneous features.
The patient underwent a fine-needle aspiration biopsy (FNAB), which resulted in a diagnosis of “malignant mesenchymal tumor cells”. In April 2004 the patient underwent surgical resection of the left breast mass in another center. The pathology report described a follicular dendritic cell sarcoma specimen with positive surgical margins. A complete surgical pathology slide consultation report performed at the European Institute of Oncology confirmed the diagnosis of follicular dendritic cell sarcoma. In May 2004 a bilateral mammogram and breast ultrasound at our center (European Institute of Oncology, EIO) revealed a distortion in the surgical area in the left breast and a small nodule in the upper outer quadrant of the same breast. Internal quadrantectomy and upper outer partial resection of the left breast were performed. The pathology report showed no evidence of malignancy in both areas. Complete staging with bone scintigraphy, liver ultrasound and chest X-ray was negative. In October 2004 a clinical examination performed for follow-up revealed a palpable mass in the left axilla. Ultrasonography showed a 17-mm lymph node. FNAB was performed, and the pathology examination confirmed the presence of follicular dendritic sarcoma cells. The patient underwent total body CT scan, x-ray of the chest, and bone biopsy: there was no evidence of disease. In November 2004 the patient underwent complete left axillary dissection at the European Institute of Oncology. Among 12 lymph nodes of the first level, 9 lymph nodes of the second level and 7 lymph nodes of the third level, only 1 first-level node was positive for follicular dendritic cell sarcoma. The complete report of this patient was discussed during the interdisciplinary
Acknowledgments: We are extremely grateful to William Russell-Edu for the revision of the English text, and to Maria Grazia Villardita for making the manuscript formally suitable for publication. Correspondence to: Dr Giovanna Gatti, Division of Breast Surgery, European Institute of Oncology, Via Ripamonti 435, 20141 Milan, Italy. Tel +39-02-57489215; fax +39-02-57489210; e-mail
[email protected] Received May 25, 2005; accepted October 14, 2005.
AXILLARY METASTASES FROM BREAST SARCOMA
meeting held in our center (EIO) every week: we decided to perform regular follow-up with no further treatment. The patient is currently disease-free. Discussion
Breast sarcoma is a tumor that arises from mesenchymal tissue; it is part of a wide spectrum of connective tissue tumors, accounting for less than 1% of malignant breast lesions10-12. The incidence, pathology type and clinical course of breast sarcoma are not well established: this is due to the rarity of the tumor and the lack of definition in various reports. Malignant phyllodes tumors and lymphomas, and even carcinomas with pseudo-sarcomatous changes have been considered by some authors as breast sarcomas. Axillary lymph node involvement is very rare; this pattern is similar to that of other soft tissue sarcomas9. Some reports suggest that axillary node disease is quite rare in the clinically negative axilla13-17, and routine axillary dissection is therefore not recommended. Even though there are few reports on this disease, the most common indication is that lymph node involvement is usually associated with advanced-stage, disseminated disease9-18. Lymph node metastases seem to carry a prognosis poorer than positive surgical margins; tumor size is of less prognostic value. Breast sarcoma with a high mitotic rate has a poor prognosis19. From the therapeutic point of view, simple mastectomy with clear resection margins gives good results13. Complete axillary dissection is important in case there is clinical involvement of the axillary nodes20. Adjuvant radiotherapy may be indicated when the primary tumor is bulky and/or when local clearance is not satisfactory21. Brachytherapy can be used as a high local dose technique, in order to attempt good local control of the disease. The role of adjuvant chemotherapy has not yet been defined22. Breast sarcoma does not have hormonal receptors: endocrine therapy is therefore ineffective4. Follicular dendritic cells are immune accessory or antigen presenting cells of the immune system within Bcell areas of lymph nodes. Follicular dendritic cell sarcoma is an unusual malignancy; the behavior of this tumor
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is more similar to that of a low grade soft tissue sarcoma than a malignant lymphoma, and is characterized by local recurrence and occasional distant metastases23. Microscopically, follicular dendritic cell tumors are composed of cells which are oval- to spindle-shaped with eosinophilic cytoplasm arranged in sheets, fascicles and whorls. The tumor cells typically express CD35, Ki-M4p, Ki-FDRC1p and vimentin, with occasional positivity for S-100 protein, muscle-specific actin, and epithelial membrane antigen23. Follicular dendritic cell tumors originate in germinal follicular lymph nodes and are considered as hematopoietic disorders24. In 70% of cases the tumor presents in the lymph nodes, in 30% there are other presentations such as the abdomen, neck and thorax. Breast presentation is extremely rare; only two cases have been reported in the world literature24,25. As already mentioned, the role of chemotherapy is not clear because of the small number of reported cases. There are reports on the use of chemotherapy in follicular dendritic cell tumors in the abdomen, thorax and neck: in such cases a protocol for aggressive lymphomas (CHOP for six courses followed by six courses of cisplatin and etoposide) seems effective26. There is one case report on axillary lymph node involvement (12 cm x 11 cm x 9 cm): the patient underwent surgical resection and chemotherapy with six courses of the EORTC lymphoma protocol followed by radical radiotherapy to the axillary region. This patient is alive and free of disease at 5.5 years26. Complete surgical resection is the treatment of choice whenever feasible. Adjuvant radiotherapy or chemotherapy is indicated in cases with adverse pathological features and in recurrent or incompletely resected lesions27. Axillary lymph node involvement by follicular cell sarcoma of the breast is rare, because the dissemination is usually hematogenous. The rate of axillary involvement is unknown: in the literature two cases of follicular dendritic cell sarcoma of the breast with no axillary involvement have been reported24,25. The patient we are reporting on is the first case of axillary involvement from follicular dendritic cell sarcoma of the breast.
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