Document Type : Original Article(s)
Authors
- Omar Hamdy 1
- Rehab T Eldesouky 2
- Ahmed S Elsaid 3
- Elissia F Gerges 3
- Eyad M Elsakti 3
- Kareem K Belal 3
- Mohamed Elbadrawy 1
1 Surgical Oncology Department, Oncology Center, Mansoura University, Mansoura, Egypt
2 Pathology Department, Faculty of Medicine, Mansoura University, Mansoura, Egypt
3 Intern, Mansoura University Hospitals, Mansoura, Egypt
Abstract
Background: Merkel cell carcinoma (MCC) is a rare, aggressive cutaneous neuroendocrine tumor, which is often misdiagnosed, resulting in most cases presenting with early metastasis. The present study aimed to highlight the incidence, clinical and diagnostic features, pathological characteristics, treatment, and outcomes of MCC.
Method: This is a retrospective single-center cohort study that included MCC patients who referred to our center between January 2008 and December 2024. Continuous variables were expressed as median values with interquartile ranges or mean values with standard deviation. Categorical variables were presented as frequencies and percentages. Statistical significance was set at P < 0.05.
Results: A total number of nine patients were included (three males, six females). The mean age at diagnosis was 66.3 ± 6.2 years. The lower limb was the most common tumor location (eight patients), and the mean tumor diameter was 54 ± 28 mm. Of these, six patients (66.7%) had clinically positive lymph nodes, and three (33.3%) had distant metastasis at presentation. Only one patient did not undergo surgery for the primary tumor. Also, four patients (44.4%) underwent regional lymphadenectomy. Adjuvant radiotherapy and chemotherapy were administered to 3 (33.3%) and 2 (22.2%) patients, respectively. During follow-up, one patient developed local recurrence, and two patients developed distant metastasis. Eventually, three patients died (33.3%), while six patients (66.7%) were lost to follow-up. The mean disease-free survival value was 4.6 ± 4.1 months, while the mean overall survival was 9.6 ± 9.1 months.
Conclusion: MCC exhibited distinct characteristics in our locality, particularly in tumor location and size. Early diagnosis and radical surgical intervention may improve outcomes.
Highlights
Omar Hamdy (google scholar)
Keywords
Main Subjects
Please cite this article as: Hamdy O, Eldesoky RT, Elsaid AS, Gerges EF, Elsakti EM, Belal KK, et al. Merkel Cell Carcinoma of the Skin: An Exceptionally Rare Entity with Unique Locality-Related Characteristics. Middle East J Cancer. 2026: in press. doi: 10.30476/mejc.2026.110574.2402.
- Singh N, McClure EM, Akaike T, Park SY, Huynh ET, Goff PH, et al. The evolving treatment landscape of Merkel cell carcinoma. Curr Treat Options Oncol. 2023;24(9):1231-58. doi: 10.1007/s11864-023-01118-8. PMID: 37403007; PMCID: PMC11260505.
- Lewis DJ, Sobanko JF, Etzkorn JR, Shin TM, Giordano CN, McMurray SL, et al. Merkel cell carcinoma. Dermatol Clin. 2023;41(1):101-15. doi: 10.1016/j.det.2022.07.015. PMID: 36410971.
- Badiu IM, Korecka K, Orzan AO, Spadafora M, Longo C, Forsea AM, et al. A review of non-invasive skin imaging in Merkel cell carcinoma: Diagnostic utility and clinical implications. Cancers (Basel). 2024;16(21):3586. doi: 10.3390/cancers16213586. PMID: 39518026; PMCID: PMC11545219.
- Gauci ML, Aristei C, Becker JC, Blom A, Bataille V, Dreno B, et al. Diagnosis and treatment of Merkel cell carcinoma: European consensus-based interdisciplinary guideline - Update 2022. Eur J Cancer. 2022;171:203-31. doi: 10.1016/j.ejca.2022.03.043. PMID: 35732101.
- Becker JC, Stang A, DeCaprio JA, Cerroni L, Lebbé C, Veness M, et al. Merkel cell carcinoma. Nat Rev Dis Primers. 2017;3:17077. doi: 10.1038/nrdp.2017.77. PMID: 29072302; PMCID: PMC6054450.
- Patel P, Hussain K. Merkel cell carcinoma. Clin Exp Dermatol. 2021;46(5):814-9. doi: 10.1111/ced.14530. PMID: 33252781.
- Iyer JG, Storer BE B, Phillips JL, Bichakjian CK, et al. Relationships among primary tumor size, number of involved nodes, and survival for 8044 cases of Merkel cell carcinoma. J Am Acad Dermatol. 2014;70(4):637-43. doi: 10.1016/j.jaad.2013.11.031. PMID: 24521828; PMCID: PMC3959572.
- Siqueira SOM, Campos-do-Carmo G, Dos Santos ALS, Martins C, de Melo AC. Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease. An Bras Dermatol. 2023;98(3):277-86. doi: 10.1016/j.abd.2022.09.003. PMID: 36870886; PMCID: PMC10173065.
- Schmults CD, Blitzblau R, Aasi SZ, Alam M, Amini A, Bibee K, et al. NCCN Guidelines® Insights: Merkel Cell Carcinoma, Version 1.2024. J Natl Compr Canc Netw. 2024;22(1D):e240002. doi: 10.6004/jnccn.2024.0002. PMID: 38244274.
- Lugowska I, Becker JC, Ascierto PA, Veness M, Blom A, Lebbe C, et al. Electronic address: clinicalguidelines@esmo.org. Merkel-cell carcinoma: ESMO-EURACAN Clinical Practice Guideline for diagnosis, treatment and follow-up. ESMO Open. 2024;9(5):102977. doi: 10.1016/j.esmoop.2024.102977. PMID: 38796285; PMCID: PMC11145756.
- Brickley SA, Franco A, Somers K. Skin cancer: Back to basics-merkel cell carcinoma. J Dermatol Nurses Assoc. 2021;13:102-5.
- Bichakjian CK, Olencki T, Aasi SZ, Alam M, Andersen JS, Blitzblau R, et al. Merkel cell carcinoma, version 1.2018, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw. 2018;16(6):742-74. doi: 10.6004/jnccn.2018.0055. PMID: 29891526; PMCID: PMC9440150.
- Rutkowski P, Owczarek W, Nejc D, Jeziorski A, Wysocki WM, Słowińska M, et al. Expert recommendation on diagnostic-therapeutic management in skin carcinomas. Oncol Clin Pract. 2022;18:69-91.
- Lebbe C, Becker JC, Grob JJ, Malvehy J, Del Marmol V, Pehamberger H, et al. Diagnosis and treatment of Merkel cell carcinoma. European consensus-based interdisciplinary guideline. Eur J Cancer. 2015;51(16):2396-403. doi: 10.1016/j.ejca.2015.06.131. PMID: 26257075.
- Fojnica A, Ljuca K, Akhtar S, Gatalica Z, Vranic S. An updated review of the biomarkers of response to immune checkpoint inhibitors in Merkel cell carcinoma: Merkel cell carcinoma and immunotherapy. Cancers (Basel). 2023;15(20):5084. doi: 10.3390/cancers15205084. PMID: 37894451; PMCID: PMC10605355.
- Nghiem PT, Bhatia S, Lipson EJ, Kudchadkar RR, Miller NJ, Annamalai L, et al. PD-1 blockade with pembrolizumab in advanced Merkel-cell carcinoma. N Engl J Med. 2016;374(26):2542-52. doi: 10.1056/NEJMoa1603702. PMID: 27093365; PMCID: PMC4927341.
- Lipson EJ, Vincent JG, Loyo M, Kagohara LT, Luber BS, Wang H, et al. PD-L1 expression in the Merkel cell carcinoma microenvironment: association with inflammation, Merkel cell polyomavirus and overall survival. Cancer Immunol Res. 2013;1(1):54-63. doi: 10.1158/2326-6066.CIR-13-0034. PMID: 24416729; PMCID: PMC3885978.
- Zwijnenburg EM, Lubeek SFK, Werner JEM, Amir AL, Weijs WLJ, Takes RP, et al. Merkel cell carcinoma: New trends. Cancers (Basel). 2021;13(7):1614. doi: 10.3390/cancers13071614. PMID: 33807446; PMCID: PMC8036880.
- Pedersen EA, Verhaegen ME, Joseph MK, Harms KL, Harms PW. Merkel cell carcinoma: updates in tumor biology, emerging therapies, and preclinical models. Front Oncol. 2024;14:1413793. doi: 10.3389/fonc.2024.1413793. PMID: 39136002; PMCID: PMC11317257.
- Harms KL, Healy MA, Nghiem P, Sober AJ, Johnson TM, Bichakjian CK, et al. Analysis of prognostic factors from 9387 Merkel cell carcinoma cases forms the basis for the new 8th edition AJCC staging system. Ann Surg Oncol. 2016;23(11):3564-71. doi: 10.1245/s10434-016-5266-4. PMID: 27198511; PMCID: PMC8881989.
- Farley CR, Perez MC, Soelling SJ, Delman KA, Harit A, Wuthrick EJ, et al. Merkel cell carcinoma outcomes: Does AJCC8 underestimate survival? Ann Surg Oncol. 2020;27(6):1978-85. doi: 10.1245/s10434-019-08187-w. Erratum in: Ann Surg Oncol. 2020;27(Suppl 3):983. doi: 10.1245/s10434-020-08871-2. PMID: 32103415.
- Zaggana E, Konstantinou MP, Krasagakis GH, de Bree E, Kalpakis K, Mavroudis D, et al. Merkel cell carcinoma-Update on diagnosis, management and future perspectives. Cancers (Basel). 2022;15(1):103. doi: 10.3390/cancers15010103. PMID: 36612102; PMCID: PMC9817518.
- Mohsen ST, Price EL, Chan AW, Hanna TP, Limacher JJ, Nessim C, Set al. Incidence, mortality and survival of Merkel cell carcinoma: a systematic review of population-based studies. Br J Dermatol. 2024;190(6):811-24. doi: 10.1093/bjd/ljad404. PMID: 37874770.
- Moshiri AS, Doumani R, Yelistratova L, Blom A, Lachance K, Shinohara MM, et al. Polyomavirus-negative Merkel cell carcinoma: A more aggressive subtype based on analysis of 282 cases using multimodal tumor virus detection. J Invest Dermatol. 2017;137(4):819-27. doi: 10.1016/j.jid.2016.10.028. PMID: 27815175; PMCID: PMC5565758.
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