Biphenotypic sinonasal sarcoma with diffuse infiltration and intracranial extension: a case report

Area of Science:

• Oncology
• Pathology
• Surgical Pathology

Background:

• Biphennsic sinonasal sarcoma (BSNS) is an emerging tumor characterized by spindle cells with neural and myogenic features.
• A recurring PAX3 gene rearrangement is a hallmark of BSNS.
• BSNS is recognized as a distinct clinicopathological entity within sinonasal sarcomas.

Purpose of the Study:

• To present a case of biphennsic sinonasal sarcoma (BSNS) with diffuse infiltration.
• To analyze the clinicopathological characteristics of this BSNS case.
• To discuss the differential diagnosis and prognostic factors of BSNS.

Main Methods:

• Case presentation of a patient diagnosed with BSNS.
• Histopathological examination of tumor tissue.
• Review of clinical data and relevant literature.

Main Results:

• The presented case exhibited diffuse infiltration, a less common presentation of BSNS.
• BSNS demonstrates low-grade spindle cell morphology with biphennsic differentiation.
• Differential diagnoses considered include schwannoma, malignant peripheral nerve sheath tumor, synovial sarcoma, and spindle cell rhabdomyosarcoma.

Conclusions:

• BSNS is a low-grade spindle cell sarcoma requiring careful differential diagnosis.
• While BSNS commonly recurs and rarely metastasizes, extensive invasion can lead to a poor prognosis.
• Accurate diagnosis and understanding of BSNS behavior are crucial for patient management.