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 negative
Abstract
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.References
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2. Rosenblum MK, Bibao JM, Ang LC. Neuromuscular system: Central nervous system. In: Rosai J, editor. Rosai and Ackerman's Surgical Pathology. 9 th ed., Vol. 2. India: Thomson Press, Mosby, Elsevier 2004: 2301-6.
3. Parham DM. Pathologic classification of rhabdomyosarcomas and correlations with molecular studies. Mod Pathol 2001; 14:506-14.
4. Reinecke P, Gerharz CD, Thiele KP, et al: Alveolar rhabdomyosarcoma presenting as acute leukemia. Verh Dtsch Ges Pathol 1998;82:336-339.
5. Duran Padilla MA, Rodriguez Martinez HA, Chavez Mercado L, et al: Atypical biological behavior of alveolar rhabdomyosarcoma in five patients. Rev Invest Clin1999;51:17-22.
6. Mekni A, Bouraoui S, Boussen H, et al: Lymphadenopathic form of alveolar rhabdomyosarcoma: A case report. Tunis Med 2004;82:241-244.
7. Chen L, Shah HO, Lin JH: Alveolar rhabdomyosarcoma with concurrent metastases to bone marrow and lymph nodes simulating acute hematologic malignancy. J Pediatr Hematol Oncol 2004;26:696-697.
8 Ganesan P, Thulkar S, Rajan A, Bakhshi S. Solid variant of alveolar rhabdomyosarcoma mimicking non-Hodgkin lymphoma: Case report and review of literature. J Pediatr Hematol Oncol 2008;30:772-4
9. Parham DM, Qualman SJ, Teot L, Barr FG, Morotti R, Sorensen PH, Triche TJ; Soft Tissue Sarcoma Committee of the Children's Oncology Group. Correlation between histology and PAX/FKHR fusion status in alveolar rhabdomyosarcoma: a report from the Children's Oncology Group. Am J Surg Pathol. 2007 Jun;31(6):895-901
10 . Egas-Bejar D, Huh WW. Rhabdomyosarcoma in adolescent and young adult patients: current perspectives. Adolesc Health Med Ther. 2014; 5: 115–125
Published
2016-11-05
Section
Case Report
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