Merkel cell carcinoma of the upper extremity
Abstract
Merkel cell carcinoma is a rare, aggressive skin cancer which is usually characterized by lymph node involvement, distant metastases, and disease relapse. It is commonly seen in elder people and it affects the head, neck, and extremities. It can be difficult to differentiate macroscopically Merkel cell carcinoma from other small cells neoplasms. Herein is the case of a 66-year-old male patient, admitted in our department for a local recurrence of Merkel cell carcinoma on the posterolateral side of the proximal third right forearm. The surgical treatment included wide local excision with 2 cm safety margins, sentinel lymph node biopsy and grafting the residual defect using split-thickness skin. Postoperative no local or general complications were encountered. The final histological examination revealed Merkel cell carcinoma with all the excisional margins free of tumor and two positive sentinel lymph nodes. The patient has performed postoperative radiotherapy with monthly clinical examination. Six months after the last surgery and after undergoing radiotherapy, there were no local or distant signs of recurrence. Due to high rates of recurrence, close surveillance is demanded.
##plugins.themes.bootstrap3.article.details##
Merkel cell carcinoma, sentinel lymph node biopsy, local recurrence, radiotherapy
2. Kim JA, Choi AH. Effect of radiation therapy on survival in patients with resected Merkel cell carcinoma: a propensity score surveillance, epidemiology, and end results database analysis. JAMA Dermatol 2013; 149(7):831-838.
3. Toker C. Trabecular carcinoma of the skin. Arch Dermatol 1972; 105(1):107-110.
4. Engels EA, Frisch M, Goedert JJ, Biggar RJ,Miller RW. Merkel cell carcinoma and HIV infection. Lancet 2002; 359:497‐498.
5. Feng H, Shuda M, Chang Y, Moore PS. Clonal integration of a polyomavirus in human Merkel cell carcinoma. Science 2008; 319:1096‐1100.
6. Penn I, First MR. Merkel's cell carcinoma in organ recipients: report of 41 cases. Transplantation 1999; 68:1717‐1721.
7. 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; 58:375‐381.
8. Mojica P, Smith D, Ellenhorn JD. Adjuvant radiation therapy is associated with improved survival in Merkel cell carcinoma of the skin. J Clin Oncol 2007; 25:1043-1047.
9. Coggshall K, Tello TL, North JP, Yu SS. Merkel cell carcinoma: An update and review: Pathogenesis, diagnosis, and staging. J Am Acad Dermatol 2018; 78:433-442.
10. Agelli M, Clegg LX. Epidemiology of primary Merkel cell carcinoma in the United States [abstract]. J Am Acad Dermatol 2003; 49(5):832-841.
11. Hodgson NC. Merkel cell carcinoma: changing incidence trends. J Surg Oncol 2005; 89(1):1-4.
12. Albores-Saavedra J, Batich K, Chable-Montero F, Sagy N, Schwartz AM, Henson DE. Merkel cell carcinoma demographics, morphology, and survival based on 3870 cases: a population based study. J Cutan Pathol 2010; 37:20-27.
13. Bichakjian CK, Lowe L, Lao CD, et al. Merkel cell carcinoma: critical review with guidelines for multidisciplinary management. Cancer 2007; 110:1-12.
14. Lemos BD, Storer BE, Iyer JG, et al. Pathologic nodal evaluation improves prognostic accuracy in Merkel cell carcinoma: analysis of 5823 cases as the basis of the first consensus staging system. J Am Acad Dermatol 2010; 63(5):751-761.
15. Akhtar S, Oza KK, Wright J. Merkel cell carcinoma: report of 10 cases and review of the literature. J Am Acad Dermatol 2000; 43:755-767.
16. Gillenwater AM, Hessel AC, Morrison WH, et al. Merkel cell carcinoma of the head and neck: effect of surgical excision and radiation on recurrence and survival. Arch Otolaryngol Head Neck Surg 2001; 127:149-154.
17. Medina-Franco H, Urist MM, Fiveash J, Heslin MJ, Bland KI, Beenken SW. Multimodality treatment of Merkel cell carcinoma: case series and literature review of 1024 cases. Ann Surg Oncol 2001; 8:204-208.
18. Bobos M, Hytiroglou P, Kostopoulos I, Karkavelas G, Papadimitriou CS. Immunohistochemical distinction between merkel cell carcinoma and small cell carcinoma of the lung. Am J Dermatopathol 2006; 28:99-104.
19. Hanly AJ, Elgart GW, Jorda M, Smith J, Nadji M. Analysis of thyroid transcription factor-1 and cytokeratin 20 separates Merkel cell carcinoma from small cell carcinoma of lung. J Cutan Pathol 2000; 27:118–120.
20. Scott MP, Helm KF. Cytokeratin 20: a marker for diagnosing Merkel cell carcinoma. Am J Dermatopathol 1999; 21:16-20.
21. Harms PW, Collie AM, Hovelson DH, et al. Next generation sequencing of cytokeratin 20-negative Merkel cell carcinoma reveals ultraviolet signature mutations and recurrent TP53 and RB1 inactivation. Mod Pathol 2016; 29(3):240–248.
22. Gruber SB, Wilson LD. Merkel cell carcinoma. Cutaneous Oncology: pathophysiology, diagnosis, and management. Science; 1998:710-721.
23. Schneider S, Thurnher D, Erovic BM. Merkel cell carcinoma: interdisciplinary management of a rare disease. J Skin Cancer 2013; 2013:189342.
24. Tai P. A practical update of surgical management of Merkel cell carcinoma of the skin. ISRN Surg 2013; 2013:850797.
25. Tai PT, Yu E, Tonita J, Gilchrist J. Merkel cell carcinoma of the skin. J Cutan Med Surg 2000; 4:186-195.
26. Howle JR, Hughes TM, Gebski V, Veness MJ. Merkel cell carcinoma: an Australian perspective and the importance of addressing the regional lymph nodes in clinically node-negative patients. J Am Acad Dermatol 2012; 67:33-40.
27. Fields RC, Busam KJ, Chou JF, et al. Recurrence and survival in patients undergoing sentinel lymph node biopsy for Merkel cell carcinoma: analysis of 153 patients from a single institution. Ann Surg Oncol 2011; 18:2529-2537.
28. Fang LC, Lemos B, Douglas J, Iyer J, Nghiem P. Radiation monotherapy as regional treatment for lymph node-positive Merkel cell carcinoma. Cancer 2010; 116:1783-1790.
29. Gupta SG, Wang LC, Peñas PF, Gellenthin M, Lee SJ, Nghiem P. Sentinel lymph node biopsy for evaluation and treatment of patients with Merkel cell carcinoma: the Dana-Farber experience and meta-analysis of the literature. Arch Dermatol 2006; 142(6):685-690.
30. Mehrany K, Otley CC, Weenig RH, Kim Phillips P, Roenigk RK, Nguyen TH. A meta-analysis of the prognostic significance of sentinel lymph node status in Merkel cell carcinoma. Dermatol Surg 2002; 28:113-117.
31. Smith VA, Camp ER, Lentsch EJ. Merkel cell carcinoma: identification of prognostic factors unique to tumors located in the head and neck based on analysis of SEER data. Laryngoscope 2012; 122:1283-1290.
32. Bichakjian CK, Olencki T, Aasi S, et al. NCCN clinical practice guidelines in oncology (NCCN guidelines) Merkel cell carcinoma, version 1 2018. J Natl Compr Canc Netw 2018; 16(6):742-774.
33. Asgari MM, Sokil MM, Warton EM, Iyer J, Paulson KG, Nghiem P. Effect of host, tumor, diagnostic, and treatment variables on outcomes in a large cohort with Merkel cell carcinoma. JAMA Dermatol 2014; 150:716-723.
34. Amin MB, Edge SB, Greene FL, et al. AJCC Cancer Staging Manual, 8th Edition. New York: Springer, 2017.
Archive of Clinical Cases is protected by copyright and may be used in accordance with copyright and other applicable laws. Content available at www.clinicalcases.eu and our digital applications is intended for personal noncommercial use.
Authors who submit a manuscript for publication in Archive of Clinical Cases agree to the following terms: a. Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal. b. Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal. c. Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) only after the final version of the manuscript was accepted and published, as it can lead to productive exchanges, as well as earlier and greater citation of published work (See The Effect of Open Access). d. It is compulsory that before submission authors ensure that their work was not published in any other medical journals or pending acceptance for publication and that "Archives of Clinical Cases" is the only beneficiary at that moment if their work/case will be accepted by us.
Guidelines for linking to www.clinicalcases.eu a. The main purpose of the site linking to the Archive of Clinical Casess site should be educational. b. Links should be made to the Archive of Clinical Casess home page (www.clinicalcases.eu) or to the articles abstract. c. It is forbidden to use the Archive of Clinical Casess cover by outside organizations unless permission has been granted in advance, notifying our Secretary. d. Material owned by the Archive of Clinical Cases (including the name, logo, cover, and text) may not be used in any manner that may induce the idea or suggest that the Archive of Clinical Cases is in some way recommending a specific company, product or service. e. You must not use or allow others to access or use, all or any part of our Site or the contents and/or applications on it for commercial purposes without our permission. To seek permission to do anything prohibited by or not contained in these TERMS, or which requires our prior consent or agreement, you can contact us.