Adult Onset Sinonasal Rhabdomyosarcoma - A Rare Case Report With Cytohistological Features
- Author:
Neelam Sood
;
Nisha Sehrawat
- Publication Type:Case Reports
- Keywords:
Rhabdomyosarcoma;
cambium zone;
parameningeal;
small round cell tumour
- From:The Malaysian Journal of Pathology
2016;38(2):169-173
- CountryMalaysia
- Language:English
-
Abstract:
Rhabdomyosarcoma (RMS) is a fast growing, malignant tumour arising from immature mesenchymal
cells, committed to skeletal muscle differentiation. It is more often seen in the paediatric population
and constitutes less than 1% of all malignancies and less than 3% of all soft tissue tumours. RMS
of the paranasal sinuses constitutes 10-15 % of adult head and neck RMS, ethmoidal and maxillary
sinuses being the most common. We report a 56-year-oldman presenting with left nasal obstruction,
epistaxis on and off and left cheek swelling. Nasal endoscopy revealed a reddish friable mass, bleeding
on touch, in the left nasal cavity. CECT scan showed a heterogeneous growth in the left maxillary
sinus eroding the medial orbital wall and lateral nasal wall. FNAC of the left cheek swelling yielded
highly cellular smears showing predominantly singly scattered round to ovoid neoplastic cells with
scanty cytoplasm and indistinct nucleoli. Few of the cells had eccentric nuclei with moderate
amount of eosinophilic cytoplasm. Attempted pseudorossette formation was seen. An impression
of round cell tumour was given. A diagnosis of an adult onset sinonasal rhabdomyosarcoma was
made on histopathological examination of the nasal biopsy, supported by immunohistochemistry
(IHC) showing strong myogenin positivity, focal positivity for PAX8 and negativity for CK, LCA,
S-100 and CD99. Parameningeal RMS is rare in adults especially the elderly. However, it needs to
be considered whenever a poorly-differentiated neoplasm is seen in this age and IHC is a useful aid.
- Full text:P020161005052850429808.pdf
