Treatment Algorithm
The treatment of Merkel cell carcinoma (MCC) is based on data from institutional case series. Local disease treatment consists of surgical excision, radiation monotherapy, or postoperative adjuvant radiation. In cases where tumor diameter ≤2 cm, surgical
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Douglas Winstanley and Seaver Soon
Summary The approach to the treatment of Merkel cell carcinoma (MCC) presents a clinical conundrum. The lack of prospective evidence-based studies due to the rarity of the tumor results in a nebulous body of data that can be conflicting. More recently, mounting evidence has provided a framework in which the clinician can logically approach, and optimize, treatment for this very aggressive disease. The treatment of local disease consists of surgical excision to obtain clear margins. In cases where tumor diameter £2 cm, surgical margins of 1 cm are reasonable. For tumors >2 cm, 2 cm margins are recommended. Due to the high rate of sentinel node positivity, sentinel lymph node biopsy (SLNB) should be performed in nearly every case of MCC, unless comorbidities or potential postoperative complications provide compelling reason not to do so. In cases where excision prior to identification of the sentinel lymph node (SLN) may compromise lymphatic channels and identification of the SLN, adjuvant radiation therapy (RT) to the primary site, nodal beds, and in-transit lymphatics should
D. Winstanley (*) • S. Soon Division of Dermatology and Dermatologic Surgery, Scripps Clinic, 10666 North Torrey Pines Road, MS112A, La Jolla, CA 92037, USA e-mail: [email protected]; [email protected]
be considered. Patients with a positive SLN should undergo completion lymph node dissection (CLND) or radiation monotherapy to the regional basin. Regular surveillance should then be employed. Multidisciplinary tumor boards are helpful in planning treatment for patients with regional or systemic disease. Treatment of regional disease consists of lymphadenectomy with or without adjuvant radiation, radiation monotherapy, or possibly adjuvant chemotherapy. Overall survival for metastatic MCC is low. Treatment of distant disease primarily consists of palliative chemotherapy, with the addition of radiation treatment and surgery where indicated.
Introduction Developing an approach to the management of MCC is complicated by a paucity of rigorous, prospective trials, due largely to the rarity of this tumor. Evidence from institutional, retrospective studies, however, has recently provided a conceptual framework to inform treatment decisions related to local, regional, and distant disease. Treatment options for local disease include surgery—specifically, wide excision or Mohs micrographic surgery—or radiation, either as monotherapy or in the postoperative setting. Local disease management should almost always include assessment of the regional nodal basin by SLNB. Treatment of regional and distant disease should be considered within the context of a
M. Alam et al. (eds.), Merkel Cell Carcinoma, DOI 10.1007/978-1-4614-6608-6_11, © Springer Science+Business Media New York 2013
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multidisciplinary tumor board. Treatment options for regional disease include lymphadenectomy, with or without adjuvant radiotherapy, or radiation monotherapy. Options for
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