Table of Contents
Definition / general | Terminology | Epidemiology | Sites | Clinical features | Prognostic factors | Case reports | Treatment | Clinical images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Differential diagnosis | Additional referencesCite this page: Mannan R, Yuan S. Neuroendocrine carcinoma, including small cell carcinoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/nasalsmallcell.html. Accessed April 2nd, 2023.
Definition / general
- High grade neuroendocrine tumor, morphologically similar to those occurring in pulmonary or other extrapulmonary sites
Terminology
- Also known as small cell carcinoma, neuroendocrine type, small cell neuroendocrine carcinoma
Epidemiology
- Rare tumor, with no gender or racial predilection
- Age range is from 26 - 77 years, with a mean age of 49 years
Sites
- Commonly arises in nasal cavity, often extends into maxillary or ethmoid sinuses
- Primary tumors of maxillary or ethmoid sinuses are less common
Clinical features
- Epistaxis and nasal obstruction are common symptoms, followed by facial pain, palpable facial mass and exophthalmos
- No known etiologic association
Prognostic factors
- Aggressive tumor with poor prognosis and frequent local recurrence and distant metastasis despite multimodal therapy
Case reports
- 22 year old woman with small cell neuroendocrine carcinoma of paranasal sinus (Natl J Maxillofac Surg 2013;4:111)
Treatment
- There are no specific management guidelines, due to rarity of the tumor
- Surgery, radiotherapy and chemotherapy alone or in combination have been used with variable results
Microscopic (histologic) description
- Sheets or ribbons of closely packed oval to spindle shaped cells with high nuclear cytoplasmic ratio, dense chromatin and inconspicuous nucleoli
- Nuclear molding, mitoses, necrosis, angiolymphatic and perineural invasion are common
- Rosettes may be seen
Microscopic (histologic) images
Positive stains
- Chromogranin, synaptophysin, neuron specific enolase
- Cytokeratin AE1 / AE3 (paranuclear, punctuate pattern), EMA
Negative stains
Differential diagnosis
Additional references