Merkel Cell Carcinoma of the Buccal Mucosa and Lower Lip

Mohammed N. Islam, Hardeep K. Chehal, Molly Housley Smith, Sarah Islam, Indraneel Bhattacharyya

Research output: Contribution to journalArticle

Abstract

Merkel cell carcinoma (MCC) is an uncommon relatively aggressive neuroendocrine dermal neoplasm first described in 1972 as a tumor of the sun exposed skin. Although most MCC affect the skin of the head and neck, rare primarily oral mucosal cases have been documented. Merkel cells are nondendritic neuroendocrine cells that are found not only in the skin but also the oral mucosa and give rise to MCC. Neuroendocrine cells may be found as aggregates in organs or as diffuse or isolated cells within organs and their epithelial lining. They contain peptide hormones and biogenic amines and occur in two forms: dendritic, which are not associated with nerve fibers and non-dendritic, which are associated with nerve fibers. Merkel cells as well as MCC express simple epithelium-type Cytokeratins (8, 18, 19, 20), neurosecretory substances; chromogranin A, synaptophysin, neuron-specific enolase (NSE), adhesion molecules, and villin (intermediate filament). Though weakly, they also express neural markers such as S-100 protein. Cytokeratin 20, and Cluster of differentiation 56, are the two key diagnostic markers for Merkel cells and MCC. Etiology includes UV radiation, the recently described Merkel cell polyomavirus, and long term systemic immunosuppression. The cutaneous and mucosal variants of MCC are considered aggressive tumors with a high risk for local recurrence and metastasis and should be considered in the differential diagnosis of head and neck mucosal lesions. We present two cases of primary Merkel cell carcinoma, one on the buccal mucosa and the other on the lower lip, and discuss the salient histologic, immunohistochemical and clinical features.

Original languageEnglish (US)
Pages (from-to)1-7
Number of pages7
JournalHead and Neck Pathology
DOIs
StateAccepted/In press - Oct 4 2017

Fingerprint

Merkel Cell Carcinoma
Mouth Mucosa
Lip
Merkel Cells
Neuroendocrine Cells
Skin
Nerve Fibers
Merkel cell polyomavirus
Neck
Head
Keratin-20
Keratin-8
Keratin-18
Chromogranin A
Synaptophysin
Biogenic Amines
Intermediate Filaments
S100 Proteins
Phosphopyruvate Hydratase
Peptide Hormones

All Science Journal Classification (ASJC) codes

  • Pathology and Forensic Medicine
  • Otorhinolaryngology
  • Oncology

Cite this

Merkel Cell Carcinoma of the Buccal Mucosa and Lower Lip. / Islam, Mohammed N.; Chehal, Hardeep K.; Smith, Molly Housley; Islam, Sarah; Bhattacharyya, Indraneel.

In: Head and Neck Pathology, 04.10.2017, p. 1-7.

Research output: Contribution to journalArticle

Islam, Mohammed N. ; Chehal, Hardeep K. ; Smith, Molly Housley ; Islam, Sarah ; Bhattacharyya, Indraneel. / Merkel Cell Carcinoma of the Buccal Mucosa and Lower Lip. In: Head and Neck Pathology. 2017 ; pp. 1-7.
@article{ba23dae3133e41cda49a3a23c9b3f0ff,
title = "Merkel Cell Carcinoma of the Buccal Mucosa and Lower Lip",
abstract = "Merkel cell carcinoma (MCC) is an uncommon relatively aggressive neuroendocrine dermal neoplasm first described in 1972 as a tumor of the sun exposed skin. Although most MCC affect the skin of the head and neck, rare primarily oral mucosal cases have been documented. Merkel cells are nondendritic neuroendocrine cells that are found not only in the skin but also the oral mucosa and give rise to MCC. Neuroendocrine cells may be found as aggregates in organs or as diffuse or isolated cells within organs and their epithelial lining. They contain peptide hormones and biogenic amines and occur in two forms: dendritic, which are not associated with nerve fibers and non-dendritic, which are associated with nerve fibers. Merkel cells as well as MCC express simple epithelium-type Cytokeratins (8, 18, 19, 20), neurosecretory substances; chromogranin A, synaptophysin, neuron-specific enolase (NSE), adhesion molecules, and villin (intermediate filament). Though weakly, they also express neural markers such as S-100 protein. Cytokeratin 20, and Cluster of differentiation 56, are the two key diagnostic markers for Merkel cells and MCC. Etiology includes UV radiation, the recently described Merkel cell polyomavirus, and long term systemic immunosuppression. The cutaneous and mucosal variants of MCC are considered aggressive tumors with a high risk for local recurrence and metastasis and should be considered in the differential diagnosis of head and neck mucosal lesions. We present two cases of primary Merkel cell carcinoma, one on the buccal mucosa and the other on the lower lip, and discuss the salient histologic, immunohistochemical and clinical features.",
author = "Islam, {Mohammed N.} and Chehal, {Hardeep K.} and Smith, {Molly Housley} and Sarah Islam and Indraneel Bhattacharyya",
year = "2017",
month = "10",
day = "4",
doi = "10.1007/s12105-017-0859-1",
language = "English (US)",
pages = "1--7",
journal = "Head and Neck Pathology",
issn = "1936-055X",
publisher = "Humana Press",

}

TY - JOUR

T1 - Merkel Cell Carcinoma of the Buccal Mucosa and Lower Lip

AU - Islam, Mohammed N.

AU - Chehal, Hardeep K.

AU - Smith, Molly Housley

AU - Islam, Sarah

AU - Bhattacharyya, Indraneel

PY - 2017/10/4

Y1 - 2017/10/4

N2 - Merkel cell carcinoma (MCC) is an uncommon relatively aggressive neuroendocrine dermal neoplasm first described in 1972 as a tumor of the sun exposed skin. Although most MCC affect the skin of the head and neck, rare primarily oral mucosal cases have been documented. Merkel cells are nondendritic neuroendocrine cells that are found not only in the skin but also the oral mucosa and give rise to MCC. Neuroendocrine cells may be found as aggregates in organs or as diffuse or isolated cells within organs and their epithelial lining. They contain peptide hormones and biogenic amines and occur in two forms: dendritic, which are not associated with nerve fibers and non-dendritic, which are associated with nerve fibers. Merkel cells as well as MCC express simple epithelium-type Cytokeratins (8, 18, 19, 20), neurosecretory substances; chromogranin A, synaptophysin, neuron-specific enolase (NSE), adhesion molecules, and villin (intermediate filament). Though weakly, they also express neural markers such as S-100 protein. Cytokeratin 20, and Cluster of differentiation 56, are the two key diagnostic markers for Merkel cells and MCC. Etiology includes UV radiation, the recently described Merkel cell polyomavirus, and long term systemic immunosuppression. The cutaneous and mucosal variants of MCC are considered aggressive tumors with a high risk for local recurrence and metastasis and should be considered in the differential diagnosis of head and neck mucosal lesions. We present two cases of primary Merkel cell carcinoma, one on the buccal mucosa and the other on the lower lip, and discuss the salient histologic, immunohistochemical and clinical features.

AB - Merkel cell carcinoma (MCC) is an uncommon relatively aggressive neuroendocrine dermal neoplasm first described in 1972 as a tumor of the sun exposed skin. Although most MCC affect the skin of the head and neck, rare primarily oral mucosal cases have been documented. Merkel cells are nondendritic neuroendocrine cells that are found not only in the skin but also the oral mucosa and give rise to MCC. Neuroendocrine cells may be found as aggregates in organs or as diffuse or isolated cells within organs and their epithelial lining. They contain peptide hormones and biogenic amines and occur in two forms: dendritic, which are not associated with nerve fibers and non-dendritic, which are associated with nerve fibers. Merkel cells as well as MCC express simple epithelium-type Cytokeratins (8, 18, 19, 20), neurosecretory substances; chromogranin A, synaptophysin, neuron-specific enolase (NSE), adhesion molecules, and villin (intermediate filament). Though weakly, they also express neural markers such as S-100 protein. Cytokeratin 20, and Cluster of differentiation 56, are the two key diagnostic markers for Merkel cells and MCC. Etiology includes UV radiation, the recently described Merkel cell polyomavirus, and long term systemic immunosuppression. The cutaneous and mucosal variants of MCC are considered aggressive tumors with a high risk for local recurrence and metastasis and should be considered in the differential diagnosis of head and neck mucosal lesions. We present two cases of primary Merkel cell carcinoma, one on the buccal mucosa and the other on the lower lip, and discuss the salient histologic, immunohistochemical and clinical features.

UR - http://www.scopus.com/inward/record.url?scp=85030545239&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85030545239&partnerID=8YFLogxK

U2 - 10.1007/s12105-017-0859-1

DO - 10.1007/s12105-017-0859-1

M3 - Article

SP - 1

EP - 7

JO - Head and Neck Pathology

JF - Head and Neck Pathology

SN - 1936-055X

ER -