Merkel Cell Carcinoma of the Maxillary Sinus: An Unusual Presentation of a Common Tumor
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CASE REPORTS
Merkel Cell Carcinoma of the Maxillary Sinus: An Unusual Presentation of a Common Tumor Jesse D. Sheldon1 · Abberly A. Lott Limbach1 Received: 16 June 2020 / Revised: 27 August 2020 / Accepted: 29 August 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Merkel cell carcinoma is most commonly seen in the skin of sun exposed areas, particularly the head and neck and is associated with Merkel cell polyomavirus. Merkel cell carcinoma at an extracutaneous mucosal site of the head and neck is rare. We report a case of a 74-year-old women who presented with an enlarging thyroid mass found to be neuroendocrine carcinoma consistent with Merkel cell carcinoma (positive for synaptophysin, chromogranin, CK20). Subsequent work up revealed a maxillary sinus mass with extension into the nasal cavity. Biopsy was diagnostic for Merkel cell carcinoma (positive for synaptophysin, chromogranin, CK20 and Merkel cell polyomavirus). There are only case reports and small case series of Merkel cell carcinoma arising in the mucosal sites of the head and neck most commonly in the oral cavity, rarely the sinonasal mucosa. Merkel cell carcinoma metastasizing to the thyroid has only been reported in three other case reports, all from skin primaries. In addition to our case, we review the literature of extracutaneous sinonasal Merkel cell carcinoma and metastases to the thyroid. Keywords Merkel cell carcinoma · Nasal cavity · Maxillary sinus · Head and neck carcinoma · Thyroid gland · Merkel cell polyomavirus
Introduction Merkel cell carcinoma (MCC) is a rare skin cancer that was first described by Toker as “trabecular carcinoma” [1]. Merkel cells are nondendritic neuroendocrine cells and MCC has been found to contain electron-dense neurosecretory granules as well as neurofilaments in the cytoplasm of tumor cells placing it in the family of neuroendocrine tumors [2–4]. Risk factors for MCC include UV exposure, Merkel cell polyomavirus, and long-term systemic immunosuppression [5]. In the head and neck, MCC frequently occurs on sunexposed skin of elderly patients [6, 7]. Often, by the time of diagnosis, the tumor has already metastasized with a predilection for regional lymph nodes, although occasionally distant metastases occur [8]. Rarely, MCC in the skin can “regress”, with metastases to regional lymph nodes the only evidence of disease [8]. * Abberly A. Lott Limbach [email protected] 1
Department of Pathology, The Ohio State University Wexner Medical Center, E410 Doan Hall, 410 W 10th Ave, Columbus, OH 43210, USA
While Merkel cell carcinoma is generally thought of as a skin tumor, it has been reported to arise in non-skin sites as well; case reports and case series describe MCC arising in the oral cavity and lip [9], nasal cavity [7, 10–12], nasopharynx [12, 13], and ethmoid sinus [14]. Even in the head and neck, MCC metastasizes primarily to regional lymph nodes. In later stages of the disease visceral metastasis has been reported, including metastases to liver, bone, lung/p
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