Original ContributionMerkel cell carcinoma with eccrine differentiation: a clinicopathologic study of 7 cases☆
Introduction
Primary cutaneous endocrine carcinoma is characterized by a small cell population with a neuroendocrine phenotype. Toker [1] first described this tumor as trabecular carcinoma and suggested its sudoriferous origin. However, after the demonstration of dense-core neurosecretory granules and neuroendocrine markers in neoplastic cells, the term Merkel cell carcinoma was used and became popular [2], [3], [4], [5]. The neoplastic cells were thought to arise from the specialized Merkel cells of the epidermis, hair sheath, and sweat ducts [6], [7]. During the last 2 decades, however, several nonendocrine cell types as well as sarcomatous elements have also been recognized in some of the tumors [8], [9], [10], [11], [12], [13], [14], suggesting that Merkel cell carcinoma probably arises from a stem cell that is able to differentiate along different cell lines.
The purpose of this investigation is to report 7 examples of Merkel cell carcinoma that showed eccrine differentiation and determine whether or not this feature is present in metastatic deposits and alters the prognosis of the tumors.
Section snippets
Material and methods
Seven cases of Merkel cell carcinoma with eccrine differentiation were retrieved from the consultation files of one of the authors (J-AS). Hematoxylin-eosin–stained sections and immunostains for cytokeratin (CK) 7, CK20, carcinoembryonic antigen (CEA), chromogranin, synaptophysin, and neuron-specific enolase were available for review in all cases. Positive and negative control immunostains were also available and examined. Clinical information and follow-up were obtained from the surgical
Results
The patients, 5 men and 2 women, ranged in age from 63 to 81 years, with a mean age of 73 years. There was no history of immunosuppression in any of the patients. Six tumors were located on the head and neck: ear, 3; cheek, 1; forehead, 1; and eyebrow, 1. One tumor was located on the sole of the left foot. All tumors were surgically excised. One patient received postoperative radiotherapy. A modified radical neck dissection was performed in 2 patients. Three patients developed local recurrence,
Discussion
Merkel cell carcinoma is an aggressive neoplasm that commonly arises in the sun-exposed skin of elderly and often immunocompromised patients [5], [15], [16], [17], [18], [19]. It has also been reported in the vulva [20], on the oral mucosa [21], and in lymph nodes in patients with no primary cutaneous lesion [15]. Approximately 40% of the tumors recur locally, and 75% of these tumors eventually metastasize in lymph nodes or in distant organs. Several investigators have recently detected Merkel
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Cited by (24)
Divergent differentiation of Merkel cell carcinoma between primary and metastatic lesions
2021, Annales de Dermatologie et de VenereologieUpdate on Merkel Cell Carcinoma: Epidemiology, Etiopathogenesis, Clinical Features, Diagnosis, and Staging
2017, Actas Dermo-SifiliograficasMelanotic and non-melanotic malignancies of the face and external ear - A review of current treatment concepts and future options
2014, Cancer Treatment ReviewsCitation Excerpt :Sarkomatoid differentiated tumor cells have also been identified in MCC. Thus, the origin of MCC is most likely a pluripotential stem cell [131,132]. In recent times an ubiquitously found Polyomavirus, the MCC-Polyomavirus (MCPyV) was identified as a factor that plays a key role in the pathogenesis of MCC tumor development [123,133,134].
Merkel cell carcinosarcoma: Merkel cell carcinoma with embryonal rhabdomyosarcoma-like component
2012, Annals of Diagnostic PathologyCitation Excerpt :Merkel cell carcinoma also appears to be a highly plastic tumor, with a potential to differentiate into cell types distinct from neuroendocrine cells, as has been comprehensively reviewed by Plaza and Suster [4]. Sweat duct, melanocytic, neuroblastic, and ganglion cell differentiations have been reported; and there are case reports describing MCC in combination with squamous cell carcinoma or sarcomatous elements, including leiomyosarcoma, fibrosarcoma, and rhabdomyosarcoma [5-13]. We describe a highly unusual case of MCC that shows biphasic pattern comprising classic MCC round cell and spindle cell components that resembles embryonal rhabdomyosarcoma and discuss the diagnostic issues and the histogenetic dilemma.
Ductal differentiation: A rare phenomenon in Merkel cell carcinoma
2023, Journal of Cutaneous Pathology
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This work was supported by La Fundación La Cruz Azul.