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    Merkel Cell Carcinoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - General Information About Merkel Cell Carcinoma

    Table 1. The Ten Most Common Sites for Merkel Cell Carcinoma (MCC), (SEER 1973-2006)a continued...

    Initial Staging Results

    The results of initial clinical staging of MCC vary widely in the literature, based on retrospective case series reported over decades. In 2009, 3,870 MCC cases were reported from the SEER Program registry. For invasive cancers, 48.6% were localized, 31.1% were regional, and 8.2% were distant.[15]

    MCC that presents in regional nodes without an identifiable primary lesion is found in a minority of patients, with the percent of these cases varying among the reported series. Tumors without an identifiable primary lesion have been attributed to either spontaneous regression of the primary or metastatic neuroendocrine carcinoma from a clinically occult site.[6,15,16,37,38]

    Clinical Progression

    In a review of patients from 18 case series, 279 of 926 patients (30.1%) developed local recurrence during follow-up, excluding those presenting with distant metastatic disease. These events have been typically attributed to inadequate surgical margins and/or a lack of adjuvant radiation therapy. In addition, 545 of 982 patients (55.5%) had lymph node metastases at diagnosis or during follow-up.[6]

    In the same review of 18 case series, the most common sites of distant metastases were distant lymph nodes (60.1%), distant skin (30.3%), lung (23.4%), central nervous system (18.4%), and bone (15.2%).[6] Many other sites of disease have also been reported, and the distribution of metastatic sites varies among case series.

    In one series of 237 patients presenting with local or regional disease, the median time-to-recurrence was 9 months (range, 2-70 months). Ninety-one percent of recurrences occurred within 2 years of diagnosis.[39]

    Potential Prognostic Factors

    The extent of disease at presentation appears to provide the most useful estimate of prognosis.[5]

    Diagnostic procedures, such as sentinel lymph node biopsy, may help distinguish between local and regional disease at presentation. One-third of patients who lack clinically palpable or radiologically visible nodes will have microscopically evident regional disease.[33] The likelihood is that nodal positivity may be substantially lower among patients with small tumors (e.g., ≤1.0 cm).[40]

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