Vulvar Merkel Cell Carcinoma: A Rare Entity

ABSTRACT Merkel cell carcinoma is a primary small cell carcinoma of the skin that resembles oat cell carcinomas of the lung. This tumor has a predilection for head and neck region but may rarely occur as isolated vulvar tumor. It has aggressive malignant behavior and should not be missed while evaluating vulvar masses. We report a case of 45-year-old Mrs I, who presented with recurrent vulvar swelling and was diagnosed as stage III Merkel cell carcinoma of the vulva. Synonyms Primary neuroendocrine carcinoma, Trabecular carcinoma, Primary small cell carcinoma, Cutaneous apudoma.

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Immunostaining for Thyroid Transcription Factor 1 and Cytokeratin 20 Aids the Distinction of Small Cell Carcinoma From Merkel Cell Carcinoma, But Not Pulmonary From Extrapulmonary Small Cell Carcinomas

Archives of Pathology & Laboratory Medicine ◽

10.5858/2001-125-0228-ifttfa ◽

2001 ◽

Vol 125 (2) ◽

pp. 228-231 ◽

Cited By ~ 22

Author(s):

W. Cheuk ◽

M. Y. Kwan ◽

Saul Suster ◽

John K. C. Chan

Keyword(s):

Transcription Factor ◽

Cell Carcinoma ◽

Small Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Small Cell ◽

Cytokeratin 20 ◽

Merkel Cell ◽

Thyroid Transcription Factor 1 ◽

Thyroid Transcription Factor ◽

Transcription Factor 1

Abstract Objective.—To study the expression of thyroid transcription factor 1 (TTF-1) and cytokeratin 20 (CK20) in pulmonary small cell carcinomas, extrapulmonary small cell carcinomas, and Merkel cell carcinomas, and thereby determine whether these markers are helpful in distinguishing these 3 groups of small cell neuroendocrine carcinomas. Materials and Methods.—Immunostaining for TTF-1 and CK20 was performed in 102 cases of small cell carcinoma (pulmonary, 52; extrapulmonary, 50) and 23 cases of Merkel cell carcinoma. The results for the 3 groups were compared. Results.—Thyroid transcription factor 1 was expressed in 82.7% of pulmonary small cell carcinomas, 42.0% of extrapulmonary small cell carcinomas (range, 33.3–53.3% for the various sites), and 0% of Merkel cell carcinomas. Cytokeratin 20 staining was consistently negative in pulmonary small cell carcinomas, and positive in 4.0% of extrapulmonary small cell carcinomas and 100% of Merkel cell carcinomas. Conclusions.—Immunostaining for TTF-1, especially when combined with immunostaining for CK20, can aid in the distinction between Merkel cell carcinoma and small cell carcinoma (both pulmonary and extrapulmonary). However, in individual cases, these markers cannot be used to distinguish between pulmonary and extrapulmonary small cell carcinomas due to the extensive overlap in immunophenotypes.

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Immunohistochemical examination of 25 cases of Merkel cell carcinoma: A comparison with small cell carcinoma of the lung and oesophagus, and a review of the literature

Apmis ◽

10.1111/j.1699-0463.1990.tb04995.x ◽

1990 ◽

Vol 98 (7-12) ◽

pp. 741-752 ◽

Cited By ~ 29

Author(s):

LEIF Johansson ◽

JAN Tennvall ◽

MÅNS ÅKerman

Keyword(s):

Cell Carcinoma ◽

Small Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Small Cell ◽

Merkel Cell ◽

Review Of The Literature ◽

Immunohistochemical Examination

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Electrochemotherapy as a new therapeutic strategy in advanced Merkel cell carcinoma of head and neck region

Radiology and Oncology ◽

10.2478/raon-2013-0059 ◽

2013 ◽

Vol 47 (4) ◽

pp. 366-369 ◽

Cited By ~ 15

Author(s):

Daniele Scelsi ◽

Niccolò Mevio ◽

Giulia Bertino ◽

Antonio Occhini ◽

Valeria Brazzelli ◽

...

Keyword(s):

Cell Carcinoma ◽

Head And Neck ◽

Merkel Cell Carcinoma ◽

Residual Life ◽

Objective Response ◽

Locally Advanced ◽

Basal Layer ◽

Neck Region ◽

Merkel Cell ◽

Head And Neck Region

Abstract Background. Merkel Cell Carcinoma (MCC) is a rare and aggressive tumour, arising from a cutaneous mechanoceptor cell located in the basal layer of epidermis, with poor prognosis. The treatment of choice for the initial stage of the disease is surgery and/or radiotherapy. The treatment of recurrent or advanced disease is still controversial. Case report. We report a case of 84 years old woman with a recurrent MCC of the chin treated with electrochemotherapy (ECT). During the period of 20 months, four sessions of ECT were employed, which resulted in an objective response of the tumour and good quality of residual life. Conclusions. Our case shows the effectiveness of ECT in the treatment of locally advanced MCC of the head and neck region in a patient not suitable for standard therapeutic options.

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DEK expression in Merkel cell carcinoma and small cell carcinoma

Journal of Cutaneous Pathology ◽

10.1111/j.1600-0560.2012.01941.x ◽

2012 ◽

Vol 39 (8) ◽

pp. 753-757 ◽

Cited By ~ 5

Author(s):

Rajiv M. Patel ◽

Laura L. Walters ◽

Ferdinand Kappes ◽

Rohit Mehra ◽

Douglas R. Fullen ◽

...

Keyword(s):

Cell Carcinoma ◽

Small Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Small Cell ◽

Merkel Cell

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Neuroendocrine Carcinomas of the Upper Airways: A Small Case Series with Histopathological Considerations

Tumori Journal ◽

10.1177/030089160709300517 ◽

2007 ◽

Vol 93 (5) ◽

pp. 499-503 ◽

Cited By ~ 18

Author(s):

Marco Capelli ◽

Giulia Bertino ◽

Patrizia Morbini ◽

Chiara Villa ◽

Stefano Zorzi ◽

...

Keyword(s):

Cell Carcinoma ◽

Head And Neck ◽

Small Cell Carcinoma ◽

Radical Surgery ◽

Neck Region ◽

Small Cell ◽

Upper Airways ◽

Head And Neck Region ◽

Poorly Differentiated ◽

Neuroendocrine Carcinomas

Neuroendocrine carcinomas are rare tumors. In the head and neck region they are most common in the larynx, where they represent 0.5-1% of epithelial cancers. Diagnosis requires the recognition of the typical neuroendocrine architecture and morphology and the immunohistochemical confirmation of neuroendocrine differentiation. In the 1991 WHO classification laryngeal neuroendocrine carcinomas have been divided into carcinoids, atypical carcinoids, small cell carcinomas and paragangliomas. Atypical carcinoids in the head and neck region usually show an aggressive behavior analogous to poorly differentiated carcinomas, and are resistant to chemo- and radiotherapy. For this reason, it was recently proposed to change their designation to “moderately differentiated neuroendocrine carcinomas”. We present the clinical and histopathological features of 2 moderately differentiated neuroendocrine carcinomas of the larynx, one large cell poorly differentiated neuroendocrine carcinoma of the oropharynx, and one small cell carcinoma of the minor salivary glands of the tongue. The patient with small cell carcinoma was free from disease 26 months after radical surgery, while the other patients showed liver, lung and bone metastases 18, 26 and 24 months after the diagnosis despite radical surgery or concomitant intra-arterial chemotherapy and radiotherapy.

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Optimal Postoperative Treatment for Composite Laryngeal Small Cell Carcinoma

Case Reports in Otolaryngology ◽

10.1155/2013/806284 ◽

2013 ◽

Vol 2013 ◽

pp. 1-3

Author(s):

Koji Ebisumoto ◽

Akihiro Sakai ◽

Kenji Okami ◽

Ryousuke Sugimoto ◽

Kosuke Saito ◽

...

Keyword(s):

Adjuvant Chemotherapy ◽

Cell Carcinoma ◽

Small Cell Carcinoma ◽

Treatment Strategies ◽

Neck Region ◽

Small Cell ◽

Postoperative Treatment ◽

Pathological Examination ◽

Tumor Biopsy ◽

Head And Neck Region

Small cell carcinoma (SmCC) most commonly occurs in the lung and rarely arises from the head and neck region. Further, composite SmCC is extremely rare. Therefore, no postoperative treatment strategy has been established. We report a 59-year-old male patient referred to our outpatient clinic for further examination and treatment of a laryngeal tumor. Biopsy from the tumor revealed squamous cell carcinoma (SCC). The preoperative diagnosis was supraglottic SCC (T3N2bM0), and total laryngectomy and bilateral neck dissection were performed. Pathological examination revealed 2 individual cancer components: SmCC and SCC. Postoperative chemoradiotherapy (2 courses of cisplatin (CDDP) and etoposide (VP-16)) was indicated. Following the postoperative chemoradiotherapy, 2 courses of adjuvant chemotherapy were administered. The patient is currently alive with no evidence of disease at 36 months following the completion of therapy. Postoperative chemoradiotherapy and adjuvant chemotherapy are optimal treatment strategies for laryngeal composite SmCC.

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