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Sinonasal undifferentiated carcinoma with orbital invasion: report of three cases
1Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia, USA.
Purpose:
To report three patients with sinonasal undifferentiated carcinoma (SNUC) that invaded the orbit.
Methods:
Retrospective small case series. The clinical, radiographic, and pathologic features of three patients with SNUC were reviewed.
Results:
Three patients with SNUC that invaded the orbit were evaluated. A biopsy was performed on the tumors, which were composed of small, hyperchromatic cells with numerous mitoses and areas of necrosis. Immunohistochemical staining was positive for cytokeratins AE1.3, epithelial membrane antigen, and neuron-specific enolase in all three tumors. Electron microscopic examination showed absence of neurosecretory granules and presence of basement membrane production. Two patients were treated with surgical resection and postoperative chemotherapy and/or radiation. One patient was treated with preoperative radiation and chemotherapy.
Conclusions:
Sinonasal undifferentiated carcinoma is a high-grade tumor that arises in the nasal and paranasal sinuses and may invade the orbit. SNUC should be distinguished from other small, round, blue cell tumors, in particular, esthesioneuroblastoma.
Insights
Sinonasal undifferentiated carcinoma (SNUC) can invade the orbit. This high-grade tumor requires careful distinction from other small round blue cell tumors like esthesioneuroblastoma.
Area of Science:
- Otolaryngology
- Oncology
- Ophthalmology
Background:
- Sinonasal undifferentiated carcinoma (SNUC) is an aggressive malignancy originating in the nasal cavity and paranasal sinuses.
- Orbital invasion by SNUC presents a diagnostic and therapeutic challenge due to its rarity and potential for visual compromise.
Observation:
- This case series details three patients with SNUC exhibiting orbital invasion.
- Pathological analysis revealed small, hyperchromatic cells with high mitotic activity and necrosis.
- Immunohistochemistry confirmed positivity for cytokeratins AE1.3, epithelial membrane antigen, and neuron-specific enolase.
Findings:
- Electron microscopy showed an absence of neurosecretory granules and the presence of basement membrane production.
- Treatment modalities included surgical resection, chemotherapy, and radiation therapy, with varied patient responses.
- Distinguishing SNUC from esthesioneuroblastoma is critical for appropriate management.
Implications:
- Early recognition and accurate diagnosis of orbital SNUC are crucial for effective treatment planning.
- Understanding the distinct pathological features of SNUC aids in differentiating it from other orbital tumors.
- Multidisciplinary approaches involving oncology, otolaryngology, and ophthalmology are essential for managing this rare condition.
