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.

  • Chetan Sudhakar Chaudhari Assistant Professor,Dept of Pathology LTMMC & LTMGH,Sion,Mumbai
  • Ganesh Ramdas Kshirsagar Assistant Professor,Dept of Pathology LTMMC & LTMGH,Sion,Mumbai
  • Prashant Vijay Kumavat Assistant Professor,Dept of Pathology LTMMC & LTMGH,Sion,Mumbai
  • Nitin M Gadgil Professor,Dept of Pathology LTMMC & LTMGH,Sion,Mumbai
Keywords: Lymphadenopathic form, Solid alveolar rhabdomyosarcoma, ALK-fusion gene negative


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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