Small cell neuroendocrine carcinoma of the nasal cavity and paranasal sinuses Journal Article


Authors: Perez-Ordonez, B.; Caruana, S. M.; Huvos, A. G.; Shah, J. P.
Article Title: Small cell neuroendocrine carcinoma of the nasal cavity and paranasal sinuses
Abstract: Small cell neuroendocrine carcinomas (SNECs) of the sinonasal tract are extremely uncommon tumors. We reviewed the clinicopathologic features of six cases of this neoplasm. There was no sex preponderance with three females and three males and a mean age at presentation of 51 years (range, 38 to 68). Two patients had disease limited to the nasal cavity, and in four the tumor involved the nasal cavity and maxillary or ethmoid sinuses. Involvement of the orbit was present in two patients. Surgery was the primary treatment. After a mean follow-up of 37 months, one patient died of local disease and liver metastases, four were alive with recurrent or metastatic disease, and one died of unrelated causes. The tumors were composed of sheets, nests, and trabeculae with extensive areas of necrosis and hemorrhage. The individual cells were small to intermediate in size and had scanty cytoplasm. The nuclei were oval or round and hyperchromatic with absent or inconspicuous nucleoli. Nuclear molding and crush artefact were present in five cases. All tumors had a high mitotic rate with frequent abnormal mitotic figures. All cases stained for Cam 5.2, neuron-specific enolase, and chromogranin. Five cases were positive for AE1:AE3, and four for synaptophysin. No case stained for S-100 protein, or neurofilaments. O-13 stained one case. No case contained EBV- RNA. SNECs of the nasal cavity and paranasal sinuses are aggressive tumors with pathological features similar to those of anaplastic small cell carcinomas of the lung. They exhibit morphological and immunophenotypic features different from olfactory neuroblastoma and should be distinguished from this tumor.
Keywords: adult; human tissue; aged; middle aged; cancer surgery; major clinical study; histopathology; follow up; follow-up studies; differential diagnosis; tumor markers, biological; neuroendocrine tumor; immunoenzyme techniques; immunophenotyping; neoplasm invasiveness; chromogranin; paranasal sinus neoplasms; esthesioneuroblastoma; nose neoplasms; paranasal sinuses; orbital neoplasms; clinical examination; neuroendocrine carcinoma; small cell carcinoma; carcinoma, small cell; neuron specific enolase; synaptophysin; nasal cavity; mitotic index; paranasal sinus carcinoma; nose cavity cancer; olfactory neuroblastoma; orbit cancer; liver metabolism; humans; human; male; female; article
Journal Title: Human Pathology
Volume: 29
Issue: 8
ISSN: 0046-8177
Publisher: Elsevier Inc.  
Date Published: 1998-08-01
Start Page: 826
End Page: 832
Language: English
DOI: 10.1016/s0046-8177(98)90452-x
PUBMED: 9712424
PROVIDER: scopus
DOI/URL:
Notes: Article -- Export Date: 12 December 2016 -- Source: Scopus
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  1. Jatin P Shah
    722 Shah
  2. Andrew G Huvos
    289 Huvos