Lay Summary: Merkel Cell Carcinoma is a rare skin cancer; treatment options are discussed.
Merkel cell carcinoma (MCC) is an aggressive yet uncommon neoplasm that often arises on the head and neck. Because of the rarity of the tumor, however, diagnosis and treatment have previously been based more on anecdotal data than on scientific data. Because of the high degree of local recurrence and early lymph node and distant metastases in patients with NEC, patients should be treated aggressively at the time of initial diagnoses. Although no widely adopted classification system exists, treatment guidelines have been based on three clinical stages of disease: local disease without lymph node or systemic involvement (stage I), regional lymph node development without systemic disease (stage II), and systemic metastases (stage III). Most treatment guidelines include wide excision of the primary tumor, alone or in combination with adjuvant radiation therapy, therapeutic regional lymph node dissection, or elective regional lymph node dissection. This patient has loco-regional disease which is usually treated with wide resection and adjuvant radiation. Some recommend adjuvant chemo and there is little information on which to base firm recommendations.
Clinical stage III disease usually presents in the bone, abdomen, skin, mediastinum, lung, liver, or basin. The usual time span from diagnosis of stage III disease to death is 8 months. Chemotherapy is the treatment most often employed within this setting. However, as in the case of all other treatment modalities used against this tumor, the rarity of the condition precludes the availability of statistically significant comparisons. No firmly established chemotherapy for MCC exists. Because of the neuroendocrine features of this tumor, it has been treated with etoposide and cisplatin as well as with cisplatin and 5-fluorouracil. More recently, there are anecdotal reports of responses to paclitaxel. Unfortunately, the rarity of this tumor has prevented cooperative efforts to establish a firm basis for a recommended therapy.
NCCN mentions adriamycin, cytoxan AND vincristine or cisplatin or carboplatin/etoposide for metastatic disease but for first line only. Recently David et al rep0rted a case of response to Votrient and others who treat this disease anecdotally also reported success. However, there remain no prospective trials of this treatment.
http://www.nccn.org/professionals/physician_gls/PDF/mcc.pdf, p.12, 2012
Davids MS, Charlton A, Ng SS, Chong ML, Laubscher K, Dar M, Hodge J, Soong R, Goh BCResponse to a novel multitargeted tyrosine kinase inhibitor pazopanib in metastatic Merkel cell carcinoma.J Clin Oncol. 2009 Sep 10;27(26):e97-100 .
Management of merkel cell carcinoma: what we know.
Arch Dermatol, June 1, 2006; 142(6): 771 – 774.
M. J. Veness
Merkel Cell Carcinoma: Improved Outcome With the Addition of Adjuvant Therapy
J. Clin. Oncol., October 1, 2005; 23(28): 7235 – 7236.