Atypical teratoid rhabdoid tumor of the central nervous system: Case series from a regional Tertiary Care Cancer Centre in South India.

BACKGROUND: Atypical teratoid rhabdoid tumors (AT/RT) constitute a rare group of pediatric brain tumors. AIM: To study the clinical, histopathological, and immunohistochemical (IHC) profile, management and outcome of children with AT/RT of the central nervous system who presented between the years 2007 and 2015 in a regional tertiary care center in South India. MATERIALS AND METHODS: This was a retrospective study. Demographic and clinical data were obtained from the clinical case files. Archived slides and tissue blocks were retrieved. All cases had hematoxylin and eosin stained sections. IHC was available in all the cases. RESULTS: There were eight cases with the mean age of presentation being 4 years (range: 4 months to 15 years) and with slight male predominance (male:female = 1.66:1). Most of the presenting complaints were due to raised intra-cranial tension. The median duration of symptoms was 0.75 months. About 62.5% of the tumors were infratentorial in location. The tumors were heterogeneous showing variable expression of cytokeratin, epithelial membrane antigen, glial fibrillary acid protein, and synaptophysin. Loss of integrase interactor-1 expression was demonstrated in seven cases in which it was done. Multimodal treatment comprising surgical resection, radiotherapy and chemotherapy was tailored based on location of tumor, resectability and patient's age. The median overall survival was 2.5 months (range: 1.5-30 months). CONCLUSION: Awareness of this tumor is important as it portends a poor outcome in most patients, in spite of multi-modal treatment. Several new molecules which aim to prolong survival and improve quality of life are being developed to combat this enigmatic tumor.

Darier-Roussy Sarcoidosis Mimicking Metastatic Breast Cancer.

Subcutaneous sarcoidosis (also known as 'Darier-Roussy sarcoid') is a cutaneous condition characterized by numerous deep-seated nodules on the trunk and extremities. Coexistence of sarcoidosis and breast cancer are reported in the literature, but there will always be a chance of misdiagnosis. It is very crucial to obtain a tissue diagnosis of suspicious metastatic lesions. We report a case of breast cancer presenting with a subcutaneous sarcoid lesion masquerading as a metastatic lesion. A 50-year-old female patient, who had had cancer of the left breast, was on hormone therapy 2 years after treatment with surgery, radiotherapy and chemotherapy. The patient presented with a sudden onset of a forehead subcutaneous swelling mimicking metastasis which on excision biopsy was proved to be sarcoidosis. In India, sarcoidosis is reported rarely. We have to keep in mind that there is a chance of the metastatic lesions being of sarcoidosis origin or another granulomatous disease. To avoid misdiagnosis, it is better to obtain a tissue diagnosis.