Presentation
Presented with repeated epistaxis.
Patient Data
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 remodeling.
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 tumor 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 favored 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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