Esthesioneuroblastoma, Neuroendocrine Carcinoma, and Sinonasal Undifferentiated Carcinoma: Differentiation in Diagnosis and Treatment

Abstract

Introduction Malignant sinonasal tumors comprise less than 1% of all neoplasms. A wide variety of tumors occurring primarily in this site can present with an undifferentiated or poorly differentiated morphology. Among them are esthesioneuroblastomas, sinonasal undifferentiated carcinomas, and neuroendocrine carcinomas. Objectives We will discuss diagnostic strategies, recent advances in immunohistochemistry and molecular diagnosis, and treatment strategies. Data Synthesis These lesions are diagnostically challenging, and up to 30% of sinonasal malignancies referred to the University of Texas MD Anderson Cancer Center are given a different diagnosis on review of pathology. Correct classification is vital, as these tumors are significantly different in biological behavior and response to treatment. The past decade has witnessed advances in diagnosis and therapeutic modalities leading to improvements in survival. However, the optimal treatment for esthesioneuroblastoma, sinonasal undifferentiated carcinoma, and neuroendocrine carcinoma remain debated. We discuss advances in immunohistochemistry and molecular diagnosis, diagnostic strategies, and treatment selection. Conclusions There are significant differences in prognosis and treatment for esthesioneuroblastoma, neuroendocrine carcinoma, and sinonasal undifferentiated carcinoma. Recent advances have the potential to improve oncologic outcomes but further investigation in needed.

Small round blue cell tumors of the sinonasal tract: a differential diagnosis approach

“…4,45,[49][50][51][52][53] When more basaloid growth and rhabdoid features are present, the lack of SMARCB1 (INI-1) protein by immunohistochemistry may suggest a different tumor type. 54,55 Special studies.…”

“…There is a strong and diffuse expression of epithelial markers (AE1/AE3, CK7, OSCAR, CAM5.2, EMA; Figure 6), consistent p16 and CD117 reactions, and only focal, patchy nuclear reaction with p63, whereas CK5/6, p40, CEA, EBER, CD34, desmin, S100 protein, and calretinin are consistently negative. 53,[56][57][58][59][60][61] Focal, patchy, and/or weak reactions with neuroendocrine markers (NSE, synaptophysin, chromogranin, CD56) may be present, but there is no corresponding neuroendocrine morphology.…”

“…The overall prognosis is poor, but with aggressive multimodality therapy, including primary surgical resection, 5-year survival rates or 35-63% are achieved, but with a median overall survival of about 2 years. [46][47][48][49]53,60,[62][63][64][65][66] Local recurrence is common, but nodal metastases are relatively uncommon, although distant metastases are frequent.…”

“…Patients present in middle age (40-55 years), with men more commonly affected than women. 52,[79][80][81] A smoking 53 and high-risk HPV 82 association are rare. Symptoms are non-specific, with tumors involving the ethmoid sinus, followed by nasal cavity and maxillary sinus, with advanced local disease (pT3 or T4) and regional or distant metastases at presentation common.…”

“…Symptoms are non-specific, with tumors involving the ethmoid sinus, followed by nasal cavity and maxillary sinus, with advanced local disease (pT3 or T4) and regional or distant metastases at presentation common. 52,53,80,83,84 Histopathology. In general, the tumors are clinically large and destructive, frequently accompanied by tumor necrosis (Figure 8), showing bone destruction, perineural invasion, and lymphovascular invasion.…”