Carcinoma with Triphasic Differentiation Arising from Inverted Papilloma in Sinonasal Sinus: A Rare Case with Molecular Characterization

Small cell neuroendocrine carcinoma (SNEC) is a rare subset of tumors in the sinonasal sinus. Combined tumors are exceedingly rare. Here, we describe a 65-year-old male with a mixed tumor of SNEC and sarcomatoid carcinoma arising in an inverted papilloma, containing squamous cell carcinoma in situ (...

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Main Authors: So-Woon Kim, Kiyong Na
Format: Article
Language:English
Published: MDPI AG 2021-10-01
Series:Diagnostics
Subjects:
Online Access:https://www.mdpi.com/2075-4418/11/10/1827
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author So-Woon Kim
Kiyong Na
author_facet So-Woon Kim
Kiyong Na
author_sort So-Woon Kim
collection DOAJ
description Small cell neuroendocrine carcinoma (SNEC) is a rare subset of tumors in the sinonasal sinus. Combined tumors are exceedingly rare. Here, we describe a 65-year-old male with a mixed tumor of SNEC and sarcomatoid carcinoma arising in an inverted papilloma, containing squamous cell carcinoma in situ (SqCCis) in the sinonasal sinus. We evaluated the molecular characteristics of the two separate carcinoma components using next-generation sequencing. The patient presented with a nasal obstruction. Computed tomography showed a mass infiltrating the right ethmoid and maxillary sinuses. An excisional biopsy was performed. The tumor was found to have three morphologically distinct components. The first was SqCCis arising in an inverted papilloma, which was positive for cytokeratin and P40. The second consisted of nests of densely packed small round cells representing SNEC-positive neuroendocrine markers. The third was a solid sheet of anaplastic spindle cell proliferation, which was negative for the above markers. Oncogenic mutations such as <i>FBXW7, TP53</i>, and <i>EGFR</i> were detected in both SNEC and sarcomatoid carcinoma, and <i>MYCL</i> amplification was observed only in the SNEC component. This case highlights an extremely rare presentation of combined SNEC and sarcomatoid carcinoma arising from an inverted papilloma in the sinonasal sinus.
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spelling doaj.art-e6a51ef4e8a744c7ad742b61235299342023-11-22T17:57:22ZengMDPI AGDiagnostics2075-44182021-10-011110182710.3390/diagnostics11101827Carcinoma with Triphasic Differentiation Arising from Inverted Papilloma in Sinonasal Sinus: A Rare Case with Molecular CharacterizationSo-Woon Kim0Kiyong Na1Department of Pathology, Kyung Hee University Hospital, College of Medicine, Kyung Hee University, Seoul 02447, KoreaDepartment of Pathology, Kyung Hee University Hospital, College of Medicine, Kyung Hee University, Seoul 02447, KoreaSmall cell neuroendocrine carcinoma (SNEC) is a rare subset of tumors in the sinonasal sinus. Combined tumors are exceedingly rare. Here, we describe a 65-year-old male with a mixed tumor of SNEC and sarcomatoid carcinoma arising in an inverted papilloma, containing squamous cell carcinoma in situ (SqCCis) in the sinonasal sinus. We evaluated the molecular characteristics of the two separate carcinoma components using next-generation sequencing. The patient presented with a nasal obstruction. Computed tomography showed a mass infiltrating the right ethmoid and maxillary sinuses. An excisional biopsy was performed. The tumor was found to have three morphologically distinct components. The first was SqCCis arising in an inverted papilloma, which was positive for cytokeratin and P40. The second consisted of nests of densely packed small round cells representing SNEC-positive neuroendocrine markers. The third was a solid sheet of anaplastic spindle cell proliferation, which was negative for the above markers. Oncogenic mutations such as <i>FBXW7, TP53</i>, and <i>EGFR</i> were detected in both SNEC and sarcomatoid carcinoma, and <i>MYCL</i> amplification was observed only in the SNEC component. This case highlights an extremely rare presentation of combined SNEC and sarcomatoid carcinoma arising from an inverted papilloma in the sinonasal sinus.https://www.mdpi.com/2075-4418/11/10/1827small cell neuroendocrine carcinomasarcomatoid carcinomasquamous cell carcinomasinonasal sinuscomposite tumor
spellingShingle So-Woon Kim
Kiyong Na
Carcinoma with Triphasic Differentiation Arising from Inverted Papilloma in Sinonasal Sinus: A Rare Case with Molecular Characterization
Diagnostics
small cell neuroendocrine carcinoma
sarcomatoid carcinoma
squamous cell carcinoma
sinonasal sinus
composite tumor
title Carcinoma with Triphasic Differentiation Arising from Inverted Papilloma in Sinonasal Sinus: A Rare Case with Molecular Characterization
title_full Carcinoma with Triphasic Differentiation Arising from Inverted Papilloma in Sinonasal Sinus: A Rare Case with Molecular Characterization
title_fullStr Carcinoma with Triphasic Differentiation Arising from Inverted Papilloma in Sinonasal Sinus: A Rare Case with Molecular Characterization
title_full_unstemmed Carcinoma with Triphasic Differentiation Arising from Inverted Papilloma in Sinonasal Sinus: A Rare Case with Molecular Characterization
title_short Carcinoma with Triphasic Differentiation Arising from Inverted Papilloma in Sinonasal Sinus: A Rare Case with Molecular Characterization
title_sort carcinoma with triphasic differentiation arising from inverted papilloma in sinonasal sinus a rare case with molecular characterization
topic small cell neuroendocrine carcinoma
sarcomatoid carcinoma
squamous cell carcinoma
sinonasal sinus
composite tumor
url https://www.mdpi.com/2075-4418/11/10/1827
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AT kiyongna carcinomawithtriphasicdifferentiationarisingfrominvertedpapillomainsinonasalsinusararecasewithmolecularcharacterization