Merkel cell carcinoma (MCC), firstly described by Toker as “trabec- ular carcinoma of the skin” [1], is a neuroendocrine cancer that for a long time was believed to arise from the skin, where Merkel cells are found in the basal layer of the epidermis [2], but it probably arises from stem cells [3]. It typically affects older whites, and it is characterized by an aggressive behaviour with a high rate of local recurrences, regional lymph nodes, and distant metastases [4]. The primary involvement of major salivary glands in small cell car- cinoma (SmCC) is very rare; Merkel-cell-like SmCC and pulmonary-like SmCC are the two variants of SMCC, and according to the work of Nagao and colleagues, Mekel-cell-like SmCC can be differentiated from pul- monary variants basing on cytokeratin 20 immunoreactivity [5,6]. Recently, several authors have suggested the similarity of Merkel-cell- like SmCC to MCC. To the best of our knowledge, only 12 cases of pri- mary Merkel-cell-like SmCC of major salivary glands have been re- ported, and only 1 case of primary Merkel-cell-like SmCC of submandibular gland. In this paper, we present the most representative case series of pri- mary Merkel-cell-like SmCC of major salivary glands, including the second case ever described of Merkel-cell-like SmCC of submandibular glands.

Small cell neuroendocrine carcinoma “Merkel-like” of major salivary glands: Presentation of a multicenter case series of this exceptional histological entity

Scarpa A.;Salzano F. A.;
2023-01-01

Abstract

Merkel cell carcinoma (MCC), firstly described by Toker as “trabec- ular carcinoma of the skin” [1], is a neuroendocrine cancer that for a long time was believed to arise from the skin, where Merkel cells are found in the basal layer of the epidermis [2], but it probably arises from stem cells [3]. It typically affects older whites, and it is characterized by an aggressive behaviour with a high rate of local recurrences, regional lymph nodes, and distant metastases [4]. The primary involvement of major salivary glands in small cell car- cinoma (SmCC) is very rare; Merkel-cell-like SmCC and pulmonary-like SmCC are the two variants of SMCC, and according to the work of Nagao and colleagues, Mekel-cell-like SmCC can be differentiated from pul- monary variants basing on cytokeratin 20 immunoreactivity [5,6]. Recently, several authors have suggested the similarity of Merkel-cell- like SmCC to MCC. To the best of our knowledge, only 12 cases of pri- mary Merkel-cell-like SmCC of major salivary glands have been re- ported, and only 1 case of primary Merkel-cell-like SmCC of submandibular gland. In this paper, we present the most representative case series of pri- mary Merkel-cell-like SmCC of major salivary glands, including the second case ever described of Merkel-cell-like SmCC of submandibular glands.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11386/4817251
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