An Extremely Rare Case of Advanced Metastatic Small Cell Neuroendocrine Carcinoma of Sinonasal Tract

Abstract

Small cell neuroendocrine carcinoma (SNEC) is a rare form of malignancy. It mainly presents as bronchogenic neoplasm, and the extrapulmonary form accounts for only 0.1% to 0.4% of all cancers. These extrapulmonary tumors have been described most frequently in the urinary bladder, prostate, esophagus, stomach, colon and rectum, gall bladder, head and neck, cervix, and skin. Primary SNEC of the sinonasal tract is extremely rare with only less than 100 cases reported in the literature. Because of extreme rarity and aggressiveness of the tumor, the management for this entity varies considerably mandating multimodality approach. In this paper, we report a patient presented with left-sided facial swelling, and the histopathologic examination confirmed primary SNEC of left sinonasal tract. The tumor involved multiple paranasal sinuses with invasion into the left orbit and left infratemporal fossa and metastasized to cervical lymph nodes and bone. The patient encountered devastating outcome in spite of optimal medical management and treatment with palliative chemotherapy highlighting the necessity for further research of primary SNEC of head and neck.

Small Cell Neuroendocrine Carcinoma Presenting as Recurrent Dacryocystitis: Case Report of a Rare Entity

“…5 There are reports of neuroendocrine carcinoma occurring as primary lesions of the orbit, ocular adnexa, and lacrimal gland, as well as the paranasal sinuses, with one previous reported entity of a primary lacrimal sac tumor. 5–16…”

Nasopharyngeal neuroendocrine neoplasms: Systematic review of the literature and case presentation

“…Most aerodigestive NENs occur in the lung, and EPNENs account for 9% of all cases 8 . Primary NENs of the nasopharyngeal cavity are rare (3% of sinonasal tumours and 0.1% of all cancers) 9,10 . Because of the rarity of occurrence, initial workup must include searching for a primary tumour at a more common site such as the lung.…”

Extrapulmonary small cell carcinoma. Literature review

“…МР синоназального тракта -крайне редкие опухоли (0,3% всех опухолей этой локализации). Используется мультимодальная терапия [51]. Отношение к ПОМ у разных исследователей неоднозначное.…”