Lymphadenopathic form of PAX 3-7/FKHR fusion gene and ALK negative, solid type of alveolar rhabdomyosarcoma in an infant: A rare entity mimicking lymphoma with a review of literature.
Keywords:
Lymphadenopathic form, Solid alveolar rhabdomyosarcoma, ALK-fusion gene negativeAbstract
Alveolar rhabdomyosarcoma is a high-grade neoplasm, which forms about 30% of rhabdomyosarcomas. A rare solid variant has been described. Cervical and axillary lymph node enlargement due to metastatic rhabdomyosarcoma, without an obvious primary tumor, is a rare finding. We report a case of multiple cervical and axillary lymphadenopathies in a 6 month old infant, clinically suspected as lymphoma. On histopathology examination diagnosed to be a case of solid variant of alveolar rhabdomyosarcoma, which was confirmed by immunohistochemical and genetic studies.Tumour was negative for anaplastic lymphoma kinase (ALK) gene aberrations and for PAX 3-7/FKHR fusion gene studies on RT-PCR, a rare subset of alveolar rhabdomyosarcoma. The pediatric and adolescent cases of this rare tumor reported in English language literature are reviewed.
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Copyright (c) 2016 Chetan Sudhakar Chaudhari, Ganesh Ramdas Kshirsagar, Prashant Vijay Kumavat, Nitin M Gadgil
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