Rhabdomyosarcoma of the nasal cavity

Case contributed by A.Prof Frank Gaillard

Presentation

Presented with repeated epistaxis.

Patient Data

Age: 20 years
Gender: Male

CT sinuses

Modality: CT

In the posterior aspect of the right nasal passage a soft tissue mass is present, just posterior to the inferior turbinate. There is no destructive bony change, and perhaps minimal bony remodelling. 

MRI

Modality: MRI

MRI confirms the presence of a right nasal cavity mass, which demonstrates solid enhancement. There is no evidence of local extension or convincing perineural spread. 

The patient went on to have resection of the mass. 

Histology 

The sections show respiratory mucosa extensively infiltrated by sheets of loosely cohesive and in places discohesive large cells with pleomorphic large nuclei, abundant mitoses, abundant apoptotic cells and focal areas of necrosis. Prominent vessels are seen within the lesion but no vessel invasion or perineural invasion is seen. The overlying respiratory epithelium is ulcerated in places but no atypia of the epithelium is seen.

The tumour cells are strongly positive for smooth muscle actin, desmin, myogenin and CD56 and negative for CD20, CD79a, CD3, S100, melan A, AE1/AE3, Chromogranin and synaptophysin.

FINAL DIAGNOSIS: Rhabdomyosarcoma (the lesion is favoured to be a solid variant of alveolar rhabdomyosarcoma).

Case Discussion

The alveolar subtype of rhabdomyosarcoma is less common than than the embryonal subtype, typically occurs in this age group (10-20 years of age) and more commonly involves the deep compartments of the extremities 1-2

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Case Information

rID: 17456
Case created: 16th Apr 2012
Last edited: 6th Nov 2015
System: Head & Neck
Inclusion in quiz mode: Included

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