PDF icon Download PDF
J Neurol Surg B Skull Base 2019; 80(01): 051-058
DOI: 10.1055/s-0038-1667146
Original Article
Georg Thieme Verlag KG Stuttgart · New York

Biphenotypic Sinonasal Sarcoma—Case Report and Review of Clinicopathological Features and Diagnostic Modalities

Chandala Chitguppi
1   Division of Rhinology and Endoscopic Skull Base Surgery, Department of Otolaryngology - Head and Neck Surgery, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, United States
,
Ian Koszewski
1   Division of Rhinology and Endoscopic Skull Base Surgery, Department of Otolaryngology - Head and Neck Surgery, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, United States
,
Kaitlin Collura
1   Division of Rhinology and Endoscopic Skull Base Surgery, Department of Otolaryngology - Head and Neck Surgery, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, United States
,
Mark Curtis
1   Division of Rhinology and Endoscopic Skull Base Surgery, Department of Otolaryngology - Head and Neck Surgery, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, United States
,
Gurston Nyquist
1   Division of Rhinology and Endoscopic Skull Base Surgery, Department of Otolaryngology - Head and Neck Surgery, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, United States
,
Mindy Rabinowitz
1   Division of Rhinology and Endoscopic Skull Base Surgery, Department of Otolaryngology - Head and Neck Surgery, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, United States
,
Marc Rosen
1   Division of Rhinology and Endoscopic Skull Base Surgery, Department of Otolaryngology - Head and Neck Surgery, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, United States
› Author Affiliations
Further Information

Publication History

08 February 2018

06 June 2018

Publication Date:
16 July 2018 (online)

Also available atMedOne
   Permissions and Reprints
Preview

Abstract

Background Biphenotypic sinonasal sarcoma is a recently described malignancy showing dual differentiation with both myogenic and neural elements. Due to its histologic similarities to other sinonasal malignancies, it is a diagnostic challenge.

Objective The main purpose of this article is to report a case of biphenotypic sinonasal sarcoma and to consolidate data and provide a comprehensive review regarding pathological differences between biphenotypic sarcoma and other sinonasal malignancies and diagnostic modalities used for biphenotypic sarcoma.

Material and Methods A systematic review of all cases of biphenotypic sinonasal sarcoma was performed using electronic databases (PubMed and Medline). Data collected included age, gender, symptoms, sub-site of origin, immunophenotyping, metastasis, recurrence, treatment, duration of follow-up, and survival outcomes.

Results Ninety-five cases of biphenotypic sarcoma were found with mean age at diagnosis of 52.36 years (range, 24–87 years). Female to male ratio was 2.27:1. Extra-sinonasal extension was present in 28%. Immunophenotyping revealed that S-100 and SMA (smooth muscle actin) were consistently positive, while SOX-10 was consistently negative. PAX3-MAML3 fusion [t (2; 4) (q35; q31.1)] was the most common genetic rearrangement. Surgical excision with or without adjuvant radiotherapy was the most frequent treatment modality used. Recurrence was observed in 32% of cases with follow-up. None of the cases reported metastasis. Three patients had died at the time of publication that included one case with intracranial extension.

Conclusion Biphenotypic sarcoma is distinct sinonasal malignancy with unique clinicopathological features. Testing involving a battery of myogenic and neural immunomarkers is essential for diagnostic confirmation and is a clinically useful endeavor when clinical suspicion is high.

Keywords

sarcoma - sinus - nasal - biphenotypic - spindle cell

Comment to this article:

Addendum: Biphenotypic Sinonasal Sarcoma—Case Report and Review of Clinicopathological Features and Diagnostic ModalitiesJ Neurol Surg B Skull Base 2019; 80(01): e1-e1
DOI: 10.1055/s-0038-1677011