Alveolar rhabdomyosarcoma in children with histomorphological review

S. K. Nema, Arjun Singh, Honey Bhasker, Pawan Bhambani, H. S. Sharma, Kumud Julka

Abstract


Rhabdomyosarcomas (RMS) are aggressive malignant neoplasm of mesenchymal origin, related to skeletal muscle lineage. These are the most common soft tissue tumors in children. The diagnosis is made by microscopic analysis and ancillary techniques like immunohistochemistry, electron microscopy, cytogenetics and molecular biology. We encountered a case of a 03 years old child who presented with a tender, reddish, soft swelling over cheek for three weeks. The FNAC was reported as a small round cell tumor, Probably Primitive Neuroectodermal Tumor (PNET). The biopsy of tumor revealed a small round cell tumor with an alveolar pattern. Tumor giant cells were absent and mitotic figures were infrequent. Hence, differentials of alveolar rhabdomyosarcoma and PNET were rendered. Immunohistochemistry (IHC) demonstrated desmin positivity. Thus, a final diagnosis of alveolar rhabdomyosarcoma was offered.


Keywords


Alveolar rhabdomyosarcoma (ARMS), Primitive neuroectodermal tumor (PNET), Immunohistochemistry (IHC), Fine needle aspiration cytology (FNAC)

Full Text:

PDF

References


Dagher R, Helman L. Rhabdomyosarcoma: an overview. Oncol. 1999;4:34-44.

McDowell H. Update on childhood rhabdomyosarcoma: a review. Arch Dis Child. 2003;88:354-7.

Huang Y, Tseng C, Yang L, et al. Rhabdomyosarcoma of the adult nasopharynx: a case report. J Intern Med Taiwan. 2005;16: 146-50.

Vankalakunti M, Das A, Rao N. Postauricular congenital alveolar rhabdomyosarcoma: a case report of an unusual entity. Diagn Pathol. 2006;1:37.

Parham D, Ellison D. Rhabdomyosarcomas in adults and children: an update. Arch Pathol Lab Med. 2006;130:1454-65.

Wu T, Huang J, Wang H et al. Long-term survivors of adult rhabdomyosarcoma of maxillary sinus following multimodal therapy: case reports and literature reviews. Chang Gung Med J. 2010;33:466-71.

Gordón-Núñez MA, Piva MR, Dos Anjos ED, Freitas RA. Orofacial rhabdomyosarcoma: report of a case and review of the literature. Med Oral Patol Oral Cir Bucal. 2008;13(12):E765-9.

Barr FG. Soft tissue tumors: Alveolar rhabdomyosarcoma. Atlas Genet Cytogenet Oncol Haematol. 2009;13(12):981-5.

Wexler L, Hand Ladanyi M. Diagnosing alveolar rhabdomyosarcoma: morphology must be coupled with fusion confirmation. J Clin Oncol. 2010;28:2126-8.

Astekar M, Metgud R, Sharma P, Ramesh G. Oral alveolar rhabdomyosarcoma: a case report with immunohistochemical analysis. Clin Pract. 2012;2:e17.

Bharath C, Rakesh. Extraosseous Ewing’s/PNET: a case report by FNAC. J Evolution Med Dent Sci. 2013;2(2):156-8.

Guiter GE, Gamoboni MM, Zakowski MF. The cytology of extra skeletal Ewing’s sarcoma. Cancer Cytopathol. 1999;87:141-8.

Pohar MZ. Difficulties in diagnosing small round cell tumors of childhood from fine needle aspiration cytology samples. Cytopathol. 2008;19(2):67-79.

Franchi A, PasquInelli G, Cenacchi G et al. Immunohistochemical and ultrastructural investigation of neural differentiation in Ewing sarcoma/PNET of bone and soft tissues. Ultrastructural Pathol. 2001;25(3):219-25.