Facial Plast Surg 2020; 36(03): 249-254
DOI: 10.1055/s-0039-3401805
Original Research
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Merkel Cell Carcinoma of the Head and Neck Compared to Other Anatomical Sites in a Real-World Setting: Importance of Surgical Therapy for Facial Tumors

Michael C. Kirchberger*
1   Department of Dermatology, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Erlangen, Germany
,
Markus V. Heppt*
2   Department of Dermatology and Allergy, Munich University Hospital (LMU), Munich, Germany
,
Gerold Schuler
1   Department of Dermatology, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Erlangen, Germany
,
Carola Berking
2   Department of Dermatology and Allergy, Munich University Hospital (LMU), Munich, Germany
,
Lucie Heinzerling
1   Department of Dermatology, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Erlangen, Germany
› Author Affiliations
Further Information

Publication History

Publication Date:
30 December 2019 (online)

Abstract

Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine skin tumor with a high propensity for nodal involvement, local recurrence, and distant metastases. Up to 50% of MCC arises on head and neck (HN), which may impede oncological treatment due to insufficiently wide excisions and a lower rate of sentinel lymph node detection due to more complicated lymph drainage. Several studies have compared the clinical outcome of HN-MCC with those of non-head and neck (NHN) MCC yielding inconsistent results. This single-center, retrospective analysis compared the clinical outcome of 26 HN-MCC patients with 30 NHN-MCC patients. Overall survival (OS) and disease-free survival (DFS) were calculated with the Kaplan–Meier method assuming proportional hazards. The mean resection margins were 1.6 and 2.0 cm for the HN and NHN cohort, respectively. Local relapses were more frequently observed in patients with HN-MCC (19 vs. 10%). Patients with HN-MCC had a median OS of 4.3 years compared with 7.5 years in patients with NHN-MCC (p = 0.277). The median OS by tumor stage was 11, 3, 2, and 3 years in stage I, II, III, and IV disease, respectively (p = 0.009). The median DFS in HN-MCC was 10 years and not reached in the cohort with NHN-MCC patients (p = 0.939). Our data suggest a trend toward poorer outcomes of HN-MCC compared with NHN-MCC. Patients with MCC on the head and neck carry a higher risk for local relapse, requiring resolute surgical treatment also in facial localizations at early stages.

* Both authors contributed equally to the manuscript.


 
  • References

  • 1 Coggshall K, Tello TL, North JP, Yu SS. Merkel cell carcinoma: an update and review part 1. Pathogenesis, diagnosis, and staging. J Am Acad Dermatol 2018; 78 (03) 433-442
  • 2 Tello TL, Coggshall K, Yom SS, Yu SS. Merkel cell carcinoma: an update and review. Part II: current and future therapy. J Am Acad Dermatol 2017; 78 (03) 445-454
  • 3 Becker JC, Stang A, DeCaprio JA. , et al. Merkel cell carcinoma. Nat Rev Dis Primers 2017; 3: 17077
  • 4 Becker JC, Kauczok CS, Ugurel S, Eib S, Bröcker EB, Houben R. Merkel cell carcinoma: molecular pathogenesis, clinical features and therapy. J Dtsch Dermatol Ges 2008; 6 (09) 709-719
  • 5 Agelli M, Clegg LX, Becker JC, Rollison DE. The etiology and epidemiology of Merkel cell carcinoma. Curr Probl Cancer 2010; 34 (01) 14-37
  • 6 Deinlein T, Richtig G, Schwab C. , et al. The use of dermatoscopy in diagnosis and therapy of nonmelanocytic skin cancer. J Dtsch Dermatol Ges 2016; 14 (02) 144-151
  • 7 Kuwamoto S. Recent advances in the biology of Merkel cell carcinoma. Hum Pathol 2011; 42 (08) 1063-1077
  • 8 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 (01) 20-27
  • 9 Robertson JP, Liang ES, Martin RCW. Epidemiology of Merkel cell carcinoma in New Zealand: a population-based study. Br J Dermatol 2015; 173 (03) 835-837
  • 10 Youlden DR, Soyer HP, Youl PH, Fritschi L, Baade PD. Incidence and survival for Merkel cell carcinoma in Queensland, Australia, 1993-2010. JAMA Dermatol 2014; 150 (08) 864-872
  • 11 Zaar O, Gillstedt M, Lindelöf B, Wennberg-Larkö A-M, Paoli J. Merkel cell carcinoma incidence is increasing in Sweden. J Eur Acad Dermatol Venereol 2016; 30 (10) 1708-1713
  • 12 Hussain SK, Sundquist J, Hemminki K. Incidence trends of squamous cell and rare skin cancers in the Swedish national cancer registry point to calendar year and age-dependent increases. J Invest Dermatol 2010; 130 (05) 1323-1328
  • 13 Reichgelt BA, Visser O. Epidemiology and survival of Merkel cell carcinoma in the Netherlands. A population-based study of 808 cases in 1993-2007. Eur J Cancer 2011; 47 (04) 579-585
  • 14 Goon PKC, Greenberg DC, Igali L, Levell NJ. Merkel Cell Carcinoma: rising incidence in the East of England. J Eur Acad Dermatol Venereol 2016; 30 (12) 2052-2055
  • 15 Girschik J, Thorn K, Beer TW, Heenan PJ, Fritschi L. Merkel cell carcinoma in Western Australia: a population-based study of incidence and survival. Br J Dermatol 2011; 165 (05) 1051-1057
  • 16 Engels EA, Frisch M, Goedert JJ, Biggar RJ, Miller RW. Merkel cell carcinoma and HIV infection. Lancet 2002; 359 (9305): 497-498
  • 17 Ulrich C, Arnold R, Frei U, Hetzer R, Neuhaus P, Stockfleth E. Skin changes following organ transplantation: an interdisciplinary challenge. Dtsch Arztebl Int 2014; 111 (11) 188-194
  • 18 Lebbe C, Becker JC, Grob J-J. , et al; European Dermatology Forum (EDF), the European Association of Dermato-Oncology (EADO) and the European Organization for Research and Treatment of Cancer (EORTC). Diagnosis and treatment of Merkel Cell Carcinoma. European consensus-based interdisciplinary guideline. Eur J Cancer 2015; 51 (16) 2396-2403
  • 19 Henness S, Vereecken P. Management of Merkel tumours: an evidence-based review. Curr Opin Oncol 2008; 20 (03) 280-286
  • 20 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
  • 21 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
  • 22 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 (06) 1283-1290
  • 23 Pellitteri PK, Takes RP, Lewis JS. , et al. Merkel cell carcinoma of the head and neck. Head Neck 2012; 34 (09) 1346-1354
  • 24 Morand GB, Madana J, Da Silva SD, Hier MP, Mlynarek AM, Black MJ. Merkel cell carcinoma of the head and neck: poorer prognosis than non-head and neck sites. J Laryngol Otol 2016; 130 (04) 393-397
  • 25 Kokoska ER, Kokoska MS, Collins BT, Stapleton DR, Wade TP. Early aggressive treatment for Merkel cell carcinoma improves outcome. Am J Surg 1997; 174 (06) 688-693
  • 26 Allen PJ, Bowne WB, Jaques DP, Brennan MF, Busam K, Coit DG. Merkel cell carcinoma: prognosis and treatment of patients from a single institution. J Clin Oncol 2005; 23 (10) 2300-2309
  • 27 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 (05) 751-761
  • 28 Bichakjian CK, Olencki T, Aasi SZ. , et al. Merkel cell carcinoma, Version 1.2018, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw 2018; 16 (06) 742-774
  • 29 Boyer JD, Zitelli JA, Brodland DG, D'Angelo G. Local control of primary Merkel cell carcinoma: review of 45 cases treated with Mohs micrographic surgery with and without adjuvant radiation. J Am Acad Dermatol 2002; 47 (06) 885-892
  • 30 O'Connor WJ, Roenigk RK, Brodland DG. Merkel cell carcinoma. Comparison of Mohs micrographic surgery and wide excision in eighty-six patients. Dermatol Surg 1997; 23 (10) 929-933
  • 31 Hughes MP, Hardee ME, Cornelius LA, Hutchins LF, Becker JC, Gao L. Merkel cell carcinoma: epidemiology, target, and therapy. Curr Dermatol Rep 2014; 3 (01) 46-53
  • 32 Jackson PC, Wallis K, Allgar V, Lind MJ, Stanley PRW. Merkel cell carcinoma in East Yorkshire: a case series and literature review of current management. J Plast Reconstr Aesthet Surg 2015; 68 (05) 667-672
  • 33 Sundaresan P, Hruby G, Hamilton A. , et al. Definitive radiotherapy or chemoradiotherapy in the treatment of Merkel cell carcinoma. Clin Oncol (R Coll Radiol) 2012; 24 (09) e131-e136
  • 34 Chen MM, Roman SA, Sosa JA, Judson BL. The role of adjuvant therapy in the management of head and neck Merkel cell carcinoma: an analysis of 4815 patients. JAMA Otolaryngol Head Neck Surg 2015; 141 (02) 137-141
  • 35 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 (06) 685-690
  • 36 Servy A, Maubec E, Sugier PE. , et al. Merkel cell carcinoma: value of sentinel lymph-node status and adjuvant radiation therapy. Ann Oncol 2016; 27 (05) 914-919
  • 37 Mehrany K, Otley CC, Weenig RH, Phillips PK, Roenigk RK, Nguyen TH. A meta-analysis of the prognostic significance of sentinel lymph node status in Merkel cell carcinoma. Dermatol Surg 2002; 28 (02) 113-117
  • 38 Weber MM, Fottner C. Immune checkpoint inhibitors in the treatment of patients with neuroendocrine neoplasia. Oncol Res Treat 2018; 41 (05) 306-312
  • 39 Chin K, Chand VK, Nuyten DSA. Avelumab: clinical trial innovation and collaboration to advance anti-PD-L1 immunotherapy. Ann Oncol 2017; 28 (07) 1658-1666
  • 40 Kaufmann H, Russel J, Hamid O. , et al. Durable responses to avelumab (anti-PD-L1) in patients with Merkel cell carcinoma progressed after chemotherapy: 1-year efficacy update. Oral presentation at: American Association for Cancer Research Annual Meeting; June 2017; Chicago, IL
  • 41 Gallo M, Guarnotta V, De Cicco F, Rubino M, Faggiano A, Colao A. ; NIKE Group. Immune checkpoint blockade for Merkel cell carcinoma: actual findings and unanswered questions. J Cancer Res Clin Oncol 2019; 145 (02) 429-443