Primary Rhabdomyosarcoma of the Breast in a 13-Year-Old Girl: Report of a Case
Department of Breast and Endocrine Surgery, Jikei University School of Medicine, 3-25-8 Nishi-shinbashi, Tokyo 105-8461, Japan.Surgery Today (Impact Factor: 1.53). 02/2007; 37(1):38-42. DOI: 10.1007/s00595-006-3326-2
We report a case of primary alveolar rhabdomyosarcoma of the breast in a 13-year-old Japanese girl. The patient initially presented with a 13 x 8-cm mass in her left breast, which was diagnosed as alveolar rhabdomyosarcoma after an excisional biopsy. Genetic expression of the tumor revealed t(2;13)(q35-37;q14). She underwent modified radical mastectomy (Bt + Ax) and nine lymph nodes were found to be involved. Systemic examinations showed multiple bone and lung metastases 2 weeks after her operation. Despite chemotherapy with doxorubicin, ifosfamide, and actinomycin D, which resulted in remission for 6 months, she died of the disease 8 months after surgery.
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ABSTRACT: We recently established a cell line derived from pleural effusion from a 13-year-old girl with primary alveolar rhabdomyosarcoma (RMS with a chromosomal translocation t[2;13]) in the breast tissue. The cell line was designated as HUMEMS. Cases of primary alveolar RMS swelling in the breast are extremely rare (about 0.2% of all RMSs). Therefore, the HUMEMS cell line is an important material for studying therapeutics for malignant tumors in children. The HUMEMS cell line we isolated consisted of two morphological subtypes. One type (SSN cells) is small in size and has a single nucleus. Another (LMN cells) is large in size and has two or more nuclei. Both SSN cells and LMN cells were immunohistochemically positive for desmin and slightly positive for myoglobin. Our data suggested LMN cells are well-differentiated SSN cells. Moreover, in some of the LMN cells, rapid cell contractions (1-5 times/10 sec) were observed. We investigated the anticancer drug susceptibility of the HUMEMS cell line with an oxygen electrode apparatus (Daikin, DOX-10, JPN) and effect of all-trans-retinoic acid (atRA) to the cell line. The atRA-treatment inhibited proliferation of the HUMEMS cells.
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ABSTRACT: To determine incidence trends and outcomes for pediatric patients with malignant breast disease. The Surveillance, Epidemiology, and End Results registry was examined for all females 19 years of age and younger diagnosed with a malignant breast tumor between 1973 and 2004. A total of 75 patients with malignant breast tumors were identified. Overall, 14.5% of patients had in situ tumors, and 85.5% had invasive disease. Tumors were classified as being either carcinomas (n = 41, 54.7%) or sarcomas (n = 34, 45.3%). The majority of sarcomatous lesions were phyllodes tumors (n = 29, 85.5%), whereas most carcinomas were of a ductal etiology (n = 19, 46.3%). The age-adjusted incidence of all malignant pediatric breast tumors in 2003 was 0.08 cases per 100,000 people (0.03 carcinoma and 0.06 sarcoma cases per 100,000 people). In the carcinoma group, regionally advanced disease was present in 11 patients (26.8%), whereas only 3 patients (7.3%) presented with metastatic disease. All patients with sarcomatous tumors presented with localized disease. Adjuvant radiation therapy was administered in only 9.8% of carcinomas and 8.8% of sarcomas, and 85.4% of carcinoma patients and 97.1% of sarcoma patients underwent surgical resection for their primary disease. Subgroup analysis revealed 5- and 10-year survival rates of 89.6% for patients with sarcomatous tumors and 63.1% and 54.3% for carcinomas. Malignant pediatric breast malignancies remain relatively rare. The two most common histologies of breast neoplasms in children are malignant carcinomas followed by sarcomas. Although uncommon, malignant disease must be considered in the differential diagnosis of the pediatric patient with a breast mass.
Article: Unusual Tumors of the Breast[Show abstract] [Hide abstract]
ABSTRACT: The emergency digital mammogram, MRI, and accurate large core diagnosis have emphasized the need for interdisciplinary care and close communication of the radiologist, pathologist, medical oncologist, and surgeon. Ultimately, this will help us prevent overtreatment and needless side effects. Such interdisciplinary care and communication and a working knowledge of unusual types of tumors and their presentation are the gold standard necessary to achieve optimal care for our breast cancer patients.