|LETTER TO THE EDITOR
|Year : 2015 | Volume
| Issue : 3 | Page : 295-296
Primary mammary rhabdomyosarcoma in a nineteen year old female: A case report and review of literature
AA Kallianpur1, Praveen1, NK Shukla1, SVS Deo1, P Khanna1, P Durgapal2
1 Department of Surgical Oncology, All India Institute of Medical Science, New Delhi, India
2 Department of Pathology, All India Institute of Medical Science, New Delhi, India
|Date of Web Publication||18-Feb-2016|
A A Kallianpur
Department of Surgical Oncology, All India Institute of Medical Science, New Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Kallianpur A A, Praveen, Shukla N K, Deo S, Khanna P, Durgapal P. Primary mammary rhabdomyosarcoma in a nineteen year old female: A case report and review of literature. Indian J Cancer 2015;52:295-6
|How to cite this URL:|
Kallianpur A A, Praveen, Shukla N K, Deo S, Khanna P, Durgapal P. Primary mammary rhabdomyosarcoma in a nineteen year old female: A case report and review of literature. Indian J Cancer [serial online] 2015 [cited 2019 Jun 16];52:295-6. Available from: http://www.indianjcancer.com/text.asp?2015/52/3/295/176702
Among the soft tissue tumors in children and adolescence, rhabdomyosarcoma is one of the most common sarcomas, comprising around 5% of malignant neoplasia in this age group.,, Its location in the breast is rare and generally represents metastatic disease from a primary site in another part of the body., Primary rhabdomyosarcoma of the breast has rarely been reported in the literature. We report a 19 year old female with a primary rhabdomyosarcoma of the breast and review the management aspect of this uncommon malignancy.
A nineteen year old adolescent girl was admitted with a large progressive tumor in her right breast that had begun as a small nodule 12 months previously. She underwent incisional biopsy of the lump at a local hospital which showed invasive ductal carcinoma histopathologically. She received one cycle of Cyclophosphamide, adriamycin and 5-Flurouracil based neoadjuvant chemotherapy at that hospital. Since she had a rapidly progressive disease on neoadjuvant chemotherapy, she was referred to our institute. Repeat biopsy was done which revealed malignant phyllodes hence she was planned for surgery. On local examination she had a large fungating mobile mass of size 30 × 20 cm involving the right breast [Figure 1]. She had no palpable lymph nodes over her axilla or supraclavicular region. Computerized tomography of the chest showed mass at the right breast not involving the chest wall. Positron emission tomography of the whole body revealed disease only in the right breast. The patient then underwent a right mastectomy with a tumor free margin and the defect was closed with thoracoepigastric fasciocutaneous flap. Gross examination of the specimen showed a large lobulated, firm tumor weighing 5.1 kilogram [Figure 2]. Histologic examination revealed infiltrative neoplasia comprising small hyperchromatic cells with round, eccentric nuclei and sparse eosinophilic cytoplasm of rhabdoid appearance [Figure 3] and [Figure 4]. Immunohistochemical evaluation revealed positivity for desmin and myogenin, thereby confirming the diagnosis of rhabdomyosarcoma. With the final diagnosis of primary rhabdomyosarcoma she was planned for 50 Grays external beam radiotherapy and adjuvant chemotherapy. She received two cycles of Ifosphamide and Adriamycin based chemotherapy and is disease free since two months of surgery.
|Figure 1: Represents a large fungating mass involving the right breast with the overlying skin displaying a shiny appearance and translucent enough to reveal underlying breast veins|
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|Figure 2: Represents the mastectomy specimen with a large firm lobulated tumor replacing the whole breast|
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|Figure 3: Represents the pleomorpic population of sheets of cell with thin walled blood vessels suggestive of rhabdomyosarcoma (low power field)|
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|Figure 4: Represents the increased mitoses and pleomorphism as seen on the high power field|
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Breast rhabdomyosarcomas are usually metastatic with primary sites usually originating from the head, neck, genital or urinary tracts, retroperitoneal tract, extremities or trunk.,,, These rare metastases of rhabdomyosarcoma of the breast are seen principally in adolescent girls. A complete physical examination and diagnostic imaging of other parts of the body should be carried out to eliminate the possibility of primary neoplasia at other sites that have metastasized to the breast.,,, Primary rhabdomyosarcoma of the breast is very rare with only 17 cases have been reported in the English literature.,,,,,,
Primary breast rhabdomyosarcomas are aggressive, rapidly growing tumor. Majority are diagnosed at a locally advanced or metastatic stage. In this case, there was no metastasis or evidence of a primary tumor in other locations. The treatment of rhabdomyosarcoma, in this uncommon site, includes a wide excision and even radical mastectomy followed by chemotherapy using multiple agents. Vincristine, actinomycin D, cyclophosphamide has been the gold standard for combination chemotherapy in the treatment of rhabdomyosarcoma. Rhabdomyosarcomas have a poor prognosis irrespective of therapy with majority presenting with distant metastasis.,, The favorable prognosis has been reported with embryonal and botryoid histological variant of rhabdomyosarcoma, tumor size less than 5 cm, R0 (complete) resection and age less than 10 years.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]