Merkel cell carcinoma of unknown primary: Clinical presentation and outcomes.
J Surg Oncol
; 126(6): 1080-1086, 2022 Nov.
Article
in En
| MEDLINE
| ID: mdl-35809230
ABSTRACT
BACKGROUND:
Merkel cell carcinoma (MCC) is a rare, aggressive cutaneous malignancy that usually occurs in the head/neck or extremities. However, there are reports of MCC developing in the lymph nodes or parotid gland without evidence of a primary cutaneous lesion.METHODS:
We reviewed 415 patients with biopsy-proven MCC. Patients with MCC of unknown primary (n = 37, 9%, MCCUP) made up the study cohort. The primary endpoints of the study were rate of recurrence, disease-free survival, and overall survival.RESULTS:
Patients with MCCUP presented with tumors in lymph nodes (n = 34) or parotid gland (n = 3). Nodal disease was most commonly detected in the inguinal/external iliac (n = 15) or axillary (n = 14) regions. The mean age at diagnosis was 70 years and 24% were female. Patients presented with distant metastases in 24.3% of cases. Patients with stage IIIA disease treated with regional lymph node dissection (RLND) had a lower risk of disease recurrence (hazard ratio 0.26, p = 0.046). Recurrence-free survival was 59.3% at 5 years. Disease-specific survival was 63.3% at 5 years.CONCLUSION:
Patients with MCCUP have a high risk of recurrence and mortality. The optimal treatment for MCCUP has yet to be elucidated, although therapeutic RLND appears beneficial for these patients.Key words
Full text:
1
Database:
MEDLINE
Main subject:
Skin Neoplasms
/
Neoplasms, Unknown Primary
/
Carcinoma, Merkel Cell
Type of study:
Observational_studies
/
Prognostic_studies
Limits:
Female
/
Humans
/
Male
Language:
En
Year:
2022
Type:
Article