TY - JOUR

T1 - Management Recommendations for Merkel Cell Carcinoma

T2 - A Danish Perspective

AU - Naseri, Simon

AU - Steiniche, Torben

AU - Ladekarl, Morten

AU - Bønnelykke-Behrndtz, Marie Louise

AU - Hölmich, Lisbet R.

AU - Langer, Seppo W.

AU - Venzo, Alessandro

AU - Tabaksblat, Elizaveta

AU - Klausen, Siri

AU - Larsen, Mathilde Skaarup

AU - Junker, Niels

AU - Chakera, Annette H.

PY - 2020/3

Y1 - 2020/3

N2 - Merkel cell carcinoma (MCC) is a rare malignant neuroendocrine carcinoma of the skin with a poor prognosis and an apparent increase in incidence. Due to its rarity, evidence-based guidelines are limited, and there is a lack of awareness among clinicians. This review constitutes the consensus management recommendations developed by the Danish MCC expert group and is based on a systematic literature search. Patients with localized disease are recommended surgical excision and adjuvant radiotherapy to the primary site; however, this may be omitted in patients with MCC with low risk features. Patients with regional lymph node involvement are recommended complete lymph node removal and adjuvant radiotherapy in case of extracapsular disease. Metastatic disease was traditionally treated with chemotherapy, however, recent clinical trials with immune therapy have been promising. Immune checkpoint inhibitors targeting the programmed cell death protein 1(PD-1)/programmed death-ligand 1(PD-L1) axis should therefore be strongly considered as first-line treatment for fit patients. A 5-year follow-up period is recommended involving clinical exam every 3 months for 2 years and every 6 months for the following 3 years and PET-CT one to two times a year or if clinically indicated. These national recommendations are intended to offer uniform patient treatment and hopefully improve prognosis.

AB - Merkel cell carcinoma (MCC) is a rare malignant neuroendocrine carcinoma of the skin with a poor prognosis and an apparent increase in incidence. Due to its rarity, evidence-based guidelines are limited, and there is a lack of awareness among clinicians. This review constitutes the consensus management recommendations developed by the Danish MCC expert group and is based on a systematic literature search. Patients with localized disease are recommended surgical excision and adjuvant radiotherapy to the primary site; however, this may be omitted in patients with MCC with low risk features. Patients with regional lymph node involvement are recommended complete lymph node removal and adjuvant radiotherapy in case of extracapsular disease. Metastatic disease was traditionally treated with chemotherapy, however, recent clinical trials with immune therapy have been promising. Immune checkpoint inhibitors targeting the programmed cell death protein 1(PD-1)/programmed death-ligand 1(PD-L1) axis should therefore be strongly considered as first-line treatment for fit patients. A 5-year follow-up period is recommended involving clinical exam every 3 months for 2 years and every 6 months for the following 3 years and PET-CT one to two times a year or if clinically indicated. These national recommendations are intended to offer uniform patient treatment and hopefully improve prognosis.

KW - Diagnosis

KW - Guideline

KW - Merkel cell carcinoma

KW - Review

KW - Treatment

U2 - 10.3390/cancers12030554

DO - 10.3390/cancers12030554

M3 - Review

C2 - 32121063

AN - SCOPUS:85081033884

VL - 12

JO - Cancers

JF - Cancers

SN - 2072-6694

IS - 3

M1 - 554

ER -