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An 81-year-old woman presented with a 30 cm-wide, easily bleeding, violaceous, cutaneous lesion of the left scapular region with a superinfected necrotic central portion surrounded by satellite telangiectatic papules (Figure, A). The patient first noticed a rapidly expanding, itchy, painful nodule, which measured 3 cm in diameter, 5 months prior to presentation (Figure, B).
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C. Merkel cell carcinoma
Skin punch biopsy results demonstrated dermal and hypodermal infiltrates from malignant cells with neuroendocrine features (Figure, C). Immunostaining was positive for synaptophysin, pan-cytokeratin, and cytokeratin 20 (with the characteristic paranuclear dotlike pattern) and negative for cytokeratin 7, CD45, CD20, S100, and SOX-10, which is consistent with Merkel cell carcinoma (MCC). Staging with fluorine-18 fluorodeoxyglucose positron emission tomography revealed cutaneous and subcutaneous radiotracer uptake in the left scapular region (maximum standardized uptake value [SUVmax], 13.7); contralateral subcutaneous nodules; and multiple bulky, bilateral laterocervical and axillary lymph nodes (SUVmax, 15).
Merkel cell carcinoma is a rare, aggressive neuroendocrine neoplasm of the skin with a rising incidence.1 Clinical presentation may vary broadly, and diagnosis can be challenging, even for experienced dermatologists.2 Usually, MCC presents as an erythematous-to-violaceous, easily bleeding, painless or mildly painful, firm nodule occurring on areas of sun-exposed skin in elderly white men. In up to 10% of individuals, there is concurrent immune suppression. Rapid growth with or without ulceration is common. As in this case, telangiectatic papules and itching (on lower extremities) are reported, as well as papules, plaques, cystic or pedunculated lesions, and subcutaneous masses.3 Definitive diagnosis is confirmed by complete examination of skin and lymph nodes and a biopsy, which is triggered by a high level of clinical suspicion.4 Diagnosis is usually made when the median lesion diameter is 1.8 cm2.
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Corresponding Author: Giuseppe Lamberti, MD, Department of Experimental, Diagnostic, and Specialty Medicine, Policlinico di Sant’Orsola University Hospital, Università di Bologna, Via Zamboni, 33, 40126 Bologna, Italy (firstname.lastname@example.org).
Published Online: July 18, 2019. doi:10.1001/jamaoncol.2019.1754
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient for granting permission to publish this information.
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