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Cutaneous Scapular Lesion in an Elderly Woman

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

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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Article Information

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 (lamberti.giu88@gmail.com).

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.

References
1.
Fitzgerald  TL, Dennis  S, Kachare  SD, Vohra  NA, Wong  JH, Zervos  EE.  Dramatic increase in the incidence and mortality from Merkel cell carcinoma in the United States.  Am Surg. 2015;81(8):802-806.PubMedGoogle Scholar
2.
Heath  M, Jaimes  N, Lemos  B,  et al.  Clinical characteristics of Merkel cell carcinoma at diagnosis in 195 patients: the AEIOU features.  J Am Acad Dermatol. 2008;58(3):375-381. doi:10.1016/j.jaad.2007.11.020PubMedGoogle ScholarCrossref
3.
Coggshall  K, Tello  TL, North  JP, Yu  SS.  Merkel cell carcinoma: an update and review: pathogenesis, diagnosis, and staging.  J Am Acad Dermatol. 2018;78(3):433-442. doi:10.1016/j.jaad.2017.12.001PubMedGoogle ScholarCrossref
4.
Schmults  CD, Blitzblau  R, Aasi  SZ,  et al. National Comprehensive Cancer Network guidelines Merkel cell carcinoma version 2.2019. https://www.nccn.org/professionals/physician_gls/pdf/mcc.pdf. Accessed June 11, 2019.
5.
Fields  RC, Busam  KJ, Chou  JF,  et al.  Five hundred patients with Merkel cell carcinoma evaluated at a single institution.  Ann Surg. 2011;254(3):465-473. doi:10.1097/SLA.0b013e31822c5fc1PubMedGoogle ScholarCrossref
6.
Martel-Jantin  C, Filippone  C, Cassar  O,  et al.  Genetic variability and integration of Merkel cell polyomavirus in Merkel cell carcinoma.  Virology. 2012;426(2):134-142. doi:10.1016/j.virol.2012.01.018PubMedGoogle ScholarCrossref
7.
Lyngaa  R, Pedersen  NW, Schrama  D,  et al.  T-cell responses to oncogenic Merkel cell polyomavirus proteins distinguish patients with Merkel cell carcinoma from healthy donors.  Clin Cancer Res. 2014;20(7):1768-1778. doi:10.1158/1078-0432.CCR-13-2697PubMedGoogle ScholarCrossref
8.
Kaufman  HL, Russell  J, Hamid  O,  et al.  Avelumab in patients with chemotherapy-refractory metastatic Merkel cell carcinoma: a multicentre, single-group, open-label, phase 2 trial.  Lancet Oncol. 2016;17(10):1374-1385. doi:10.1016/S1470-2045(16)30364-3PubMedGoogle ScholarCrossref
9.
D’Angelo  SP, Russell  J, Lebbé  C,  et al.  Efficacy and safety of first-line avelumab treatment in patients with stage IV metastatic Merkel cell carcinoma: a preplanned interim analysis of a clinical trial.  JAMA Oncol. 2018;4(9):e180077. doi:10.1001/jamaoncol.2018.0077PubMedGoogle ScholarCrossref
10.
Nghiem  PT, Bhatia  S, Lipson  EJ,  et al.  PD-1 blockade with pembrolizumab in advanced Merkel-cell carcinoma.  N Engl J Med. 2016;374(26):2542-2552. doi:10.1056/NEJMoa1603702PubMedGoogle ScholarCrossref
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