Epidermotropic pagetoid spread and squamous cell carcinoma in situ in the overlying epidermis of Merkel cell carcinoma

Yamamoto, Toshiyuki

doi:10.7241/ourd.20142.54

Cited by 1 publication

(1 citation statement)

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“…Of them, an 82-year-old female was diagnosed with MCC overlapping BD by histopathology but refused further evaluation and operative treatment (Jeong et al 2018 ). Seven patients underwent direct extensive local excision treatment, followed by histopathological detection of the tissue post-surgery (Tono et al 2015 ; Swain et al 2022 ; Choe et al 2014 ; Miraflor et al 2016 ; Ishida et al 2013 ; Kiyohara et al 2019 ; Yamamoto 2014 ; McGowan et al 2016 ; Casari et al 2018 ). A 77-year-old woman was found to have lymph node and liver metastases after surgical treatment, followed by radiation and chemotherapy (Choe et al 2014 ).…”

Section: Resultsmentioning

confidence: 99%

Merkel cell carcinoma overlapping Bowen’s disease: two cases report and literature review

Chen,

Song,

Huang

et al. 2024

J Cancer Res Clin Oncol

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Purpose Merkel cell carcinoma (MCC) is a rare neuroendocrine tumor of the skin, which mainly occurs in the sun exposed sites of white patients over 65 years, with a higher recurrence and metastasis rate. Clinically, MCC overlapping Bowen’s disease (BD) is a very rare subtype of MCC. Few cases in the literature have been described and the management is not well defined. We summarize and update the epidemiology, clinical and histopathological features, metastasis characteristics, local recurrence rate and management of it by presenting two cases of MCC overlapping BD and reviewing the literature over the last 11 years. Design We consulted databases from PubMed, ResearchGate and Google Scholar by MeSh “Merkel cell carcinoma” and “Bowen’s disease”, “Bowen disease” or “squamous cell carcinoma in situ”, from January 2013 to December 2023 and reviewed the literatures. We reported two additional cases. Results Total 13 cases of MCC overlapping BD were retrospectively analyzed, in whom mainly in elderly women over 70 years, the skin lesions were primarily located on the faces, followed by the extremities and trunk. Most of them were asymptomatic, firm, dark red nodules arising on rapidly growing red or dark brown patches, or presenting as isolated nodules. Dermoscopy evaluation was rarely performed in the pre-operative diagnostic setting. All cases were confirmed by histopathology and immunohistochemistry. The most definitive treatment was extended local excision, but local recurrences were common. Of the 13 cases, 4 cases experienced local or distant metastasis. One suffered from an in-transit recurrence of MCC on the ipsilateral leg after local excision and lymph node dissection, whose metastasis completely subsided after avelumab treatment and without recurrence or metastasis during 6 months of follow-up. Conclusions MCC overlapping BD is a very rare skin tumor mainly predisposed on the faces, with high misdiagnosis rate and recurrence rate. Advanced disease at diagnosis is a poor prognostic factor, suggesting that earlier detection may improve outcome. The acronym, AEIOUN, has been proposed to aid in clinical identification. Our reports and the literature review can provide a better awareness and management of it.

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