{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,8]],"date-time":"2026-09-08T08:55:31Z","timestamp":1788857731654,"version":"build-2803163510"},"reference-count":30,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2022,7,9]],"date-time":"2022-07-09T00:00:00Z","timestamp":1657324800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Journal of Surgical Oncology"],"published-print":{"date-parts":[[2022,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Merkel cell carcinoma (MCC) is a rare, aggressive cutaneous malignancy that usually occurs in the head\/neck or extremities. However, there are reports of MCC developing in the lymph nodes or parotid gland without evidence of a primary cutaneous lesion.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We reviewed 415 patients with biopsy\u2010proven MCC. Patients with MCC of unknown primary (<jats:italic>n<\/jats:italic>\u2009=\u200937, 9%, MCCUP) made up the study cohort. The primary endpoints of the study were rate of recurrence, disease\u2010free survival, and overall survival.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Patients with MCCUP presented with tumors in lymph nodes (<jats:italic>n<\/jats:italic>\u2009=\u200934) or parotid gland (<jats:italic>n<\/jats:italic>\u2009=\u20093). Nodal disease was most commonly detected in the inguinal\/external iliac (<jats:italic>n<\/jats:italic>\u2009=\u200915) or axillary (<jats:italic>n<\/jats:italic>\u2009=\u200914) regions. The mean age at diagnosis was 70 years and 24% were female. Patients presented with distant metastases in 24.3% of cases. Patients with stage IIIA disease treated with regional lymph node dissection (RLND) had a lower risk of disease recurrence (hazard ratio 0.26, <jats:italic>p<\/jats:italic>\u2009=\u20090.046). Recurrence\u2010free survival was 59.3% at 5 years. Disease\u2010specific survival was 63.3% at 5 years.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Patients with MCCUP have a high risk of recurrence and mortality. The optimal treatment for MCCUP has yet to be elucidated, although therapeutic RLND appears beneficial for these patients.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jso.27010","type":"journal-article","created":{"date-parts":[[2022,7,9]],"date-time":"2022-07-09T15:49:43Z","timestamp":1657381783000},"page":"1080-1086","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Merkel cell carcinoma of unknown primary: Clinical presentation and outcomes"],"prefix":"10.1002","volume":"126","author":[{"given":"Samuel E.","family":"Broida","sequence":"first","affiliation":[{"name":"Department of Orthopaedic Surgery Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiao T.","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Orthopaedic Surgery Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian 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