Anzeige
Erschienen in:
01.04.2021 | Other Articles
S-100 Immuno-Positive Low Grade Myofibroblastic Sarcoma of Nasal Cavity: A Rare Case Presentation and Review of Literature
Erschienen in: Indian Journal of Otolaryngology and Head & Neck Surgery | Sonderheft 2/2022
Einloggen, um Zugang zu erhaltenAbstract
Introduction
Myofibroblastic neoplasms comprise a spectrum of benign/malignant neoplasms. Only low-grade malignant forms have been reproducibly characterized as a diagnostic entity in the WHO classification. Low grade myofibroblastic sarcoma (LGMFS) confined to the nasal cavity is extremely rare.
Objective
To review previously reported cases of nasal cavity LGMFS and provide a better insight regarding its clinical and immunohistochemical features.
Data synthesis
A review was performed involving two databases (PubMed and Google Scholar). Four cases of nasal cavity LGMFS were included. The lesion showed no gender or nasal-side predilection. All cases underwent wide excision. None showed distant metastasis while half recurred locally. Histologically, mitotic rate ranged from 1 to 3/10 high-power-field (HPF) and none exhibited spontaneous necrosis. Immuno-expression of calponin, smooth muscle actin (SMA) and vimentin were seen in either all four or three-fourth of cases. Diffuse S-100 expression was a unique finding in present case and not reported previously, that caused a diagnostic dilemma with schwannomas.
Conclusion
LGMFS of nasal cavity is extremely rare. A wide resection is the primary treatment of choice. Adjuvant therapies (chemotherapy or radiotherapy) are of uncertain significance. Distant metastasis is rather unusual. Calponin, SMA and vimentin are highly sensitive immuno-markers. Diffuse S-100 expression is a possible finding. Mitotic rate < 6/10 HPF and absence of spontaneous necrosis are characteristic indolent features differentiating from high grade lesions. Trifecta of clinical and morphological features plus immunohistological phenotype, are sufficient for a definitive diagnosis. Electron microscopy is the most definitive confirmation test, however, should be reserved only for equivocal/atypical immunostaining pattern.