RESUMO
The review covers the current recommendations for Merkel cell carcinoma (MCC), with detailed discussion of many controversies. The 2010 AJCC staging system is more in-line with other skin malignancies although more complicated to use. The changes in staging system over time make comparison of studies difficult. A wide excision with margins of 2.5-3 cm is generally recommended. Even for primary = 1 cm, there is a significant risk of nodal and distant metastases and hence sentinel node biopsy should be done if possible; otherwise adjuvant radiotherapy to the primary and nodal region should be given. Difficulties of setting up trials owing to the rarity of the disease and the mean age of the patient population result in infrequent reports of adjuvant or concurrent chemotherapy in the literature. The benefit, if any, is not great from published studies so far. However, there may be a subgroup of patients with high-risk features, e.g. node-positive and excellent performance status, for whom adjuvant or concurrent chemotherapy may be considered. Since local recurrence and metastases generally occur within 2 years of the initial diagnosis, patients should be followed more frequently in the first 2 years. However delayed recurrence can still occur in a small proportion of patients and long-term follow-up by a specialist is recommended provided that the general condition of the patient allows it. In summary, physician judgment in individual cases of MCC is advisable, to balance the risk of recurrence versus the complications of treatment.
RESUMO
A case series of Merkel cell carcinoma (MCC) is reported here and illustrates some of the current controversies in clinical management of this disease. From 1988-2007, 145 cases (74 men and 71 women) were analyzed. These were combined with other cases in the literature, hence the total number of patients was 433. Nodal metastases occurred clinically at presentation in 9/105 (9%) patients with primary tumor size <1 cm. The rate of nodal metastases is too high to obviate sentinel node biopsies even for these small tumors. For the 87 patients with intermediate tumor size (>1 - <2 cm), nodal metastases occurred clinically in 11 of 87 patients (13%) at presentation and 23 of 87 patients (26%) during follow-up. Distant metastases occurred in 20 of 87 patients (23%) only at follow-up. The risks of nodal and distant failures for tumors of intermediate sizes were sufficient to be classified as high-risk for clinical study purposes.
Assuntos
Carcinoma de Célula de Merkel/terapia , Neoplasias Cutâneas/terapia , Carcinoma de Célula de Merkel/mortalidade , Carcinoma de Célula de Merkel/patologia , Feminino , Humanos , Metástase Linfática , Masculino , Prognóstico , Biópsia de Linfonodo Sentinela , Neoplasias Cutâneas/mortalidade , Neoplasias Cutâneas/patologiaRESUMO
The results of the multimodality management of 145 cases of cutaneous Merkel cell carcinoma (MCC) are reported herein. Patient information was obtained from medical records of four Canadian institutions and one French institution. These data included ages, pathological stages, disease sites, histological and treatment details. Cause-specific survival (CSS), overall survival (OS) and disease-free survival (DFS) rates were analyzed by the log-rank and Kaplan-Meier methods. From 1988 to 2007, 145 cases were analyzed. There were 74 men and 71 women. Median age was 78 years (range 47-95.2). Median follow-up was 21.5 months (range 0.5-169.1). During the follow-up period, 37 local, 37 regional nodal and 15 distant recurrences were determined to be either solitary or multifocal sites of treatment failure. Relapsing patients were treated with multimodal treatments provided that their general state of health did not preclude this approach. Either initially and/or at relapse, 30 patients were treated with either multiagent (mostly etoposide with cisplatin or carboplatin) or single-agent chemotherapy, including irinotecan, taxotere and topotecan. In conclusion, recurrence should be treated with multimodalities. Our experience of long-term survivors after treatment of recurrence is encouraging.