FET(EWSR1)-TFCP2 Rhabdomyosarcoma: An Additional Example of this Aggressive Variant with Predilection for the Gnathic Bones

An example of a mandibular rhabdomyosarcoma in a 15-year-old male is described featuring EWSR1-TFCP2 fusion with homolateral lymph node metastasis and apparent metastasis to the thoracic vertebra T7. This type of rhabdomyosarcoma has preference for the craniofacial skeleton. Histologically, the tumo...

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Bibliographic Details
Published in:Head & neck pathology (Totowa, N.J.) N.J.), 2021-03, Vol.15 (1), p.374-380
Main Authors: Koutlas, Ioannis G., Olson, Damon R., Rawwas, Jawhar
Format: Article
Language:English
Subjects:
Case Reports
Dentistry
Medicine
Medicine & Public Health
Oral and Maxillofacial Surgery
Otorhinolaryngology
Pathology
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Summary:An example of a mandibular rhabdomyosarcoma in a 15-year-old male is described featuring EWSR1-TFCP2 fusion with homolateral lymph node metastasis and apparent metastasis to the thoracic vertebra T7. This type of rhabdomyosarcoma has preference for the craniofacial skeleton. Histologically, the tumor was composed of spindle and epithelioid cells characterized by nuclear pleomorphism, cytologic atypia and brisk mitotic activity. Immunohistochemically, it featured diffuse positive nuclear staining MYOD1, only focal staining for myogenin and patchy cytoplasmic staining for desmin. Tumor cells were positive for keratins and nuclear staining for SATB2 was also observed. Interestingly, tumor cells were diffusely positive for calponin. Currently, the patient is under chemotherapy treatment.
ISSN:1936-0568
1936-055X
1936-0568
DOI:10.1007/s12105-020-01189-1