INTRODUCTION Merkel cell carcinoma (MCC) is a cutaneous neuroendocrine neoplasm with propensity for lymphatic spread. The rarity of MCC has limited analysis of factors associated with a positive sentinel lymph node biopsy (SLNB) and survival. METHODS Review of a prospective MCC database was performed. Factors associated with +SLNB were analyzed. Univariate and multivariate analyses of factors associated with recurrence and survival were performed using the cumulative incidence (CI) function, treating death from other causes (DOC) as a competing risk. RESULTS From 1996–2010, 153 patients with localized MCC underwent SLNB, of whom 45 (29%) were positive. Factors associated with +SLNB were primary tumor size (25% ≤2 cm v. 45% >2 cm; p=0.02) and presence of lymphovascular invasion (LVI; 55% +LVI v. 4% −LVI; p<0.01). +SLNB patients were more likely to receive radiation or chemotherapy (60% vs. 7%, p<0.01). With median follow-up of 41 months, there were 16 nodal/distant recurrences (10%), 11 deaths from MCC (7%), and 27 DOC’s (18%). The 2-year CI’s of recurrence or death from MCC were 12% and 6%, respectively. There was no difference in recurrence or death from MCC between +SLNB and −SLNB patients. The 2-year CI’s of recurrence or death from MCC for +LVI patients were 30% and 15%, respectively. No −LVI patient recurred or died of MCC. CONCLUSION SLNB identifies occult nodal metastases in 29% of patients with localized MCC. Predictors of +SLNB are tumor size and presence of LVI. Patients with +SLNB are more likely to receive further treatment; however, SLN status is not associated with recurrence or survival. In contrast, LVI is strongly associated with both recurrence and survival.
Radiation-associated sarcomas are uncommon, constituting less than 5% of all sarcomas, and generally associated with a poor prognosis. We reviewed the medical records of 565 patients with sarcoma and a second malignancy seen at our institution between 1943 and 1989; 160 of these patients (28%) were considered to have a radiation-associated sarcoma. The most common diagnosis for which radiation had been given was breast cancer (26%), followed by lymphoma (25%) and carcinoma of the cervix (14%). The most common histologic types of radiation-associated sarcoma were osteogenic (21%), malignant fibrous histiocytoma (16%), and angiosarcoma/lymphangiosarcoma (15%). Most of the tumors were high grade (87%). Three variables had prognostic significance in multivariate analysis: the presence of metastatic disease, the completeness of operative resection in patients with localized disease, and the size of the primary tumor in patients who underwent complete resection of the sarcoma. Survival was independent of histologic subtype or site of disease.
OS is a poor measure of the influence of MCC on life expectancy. The presence of lymphovascular invasion and clinically, but not microscopically, positive lymph nodes were associated with increased DSD. These factors should be incorporated into MCC staging and treatment recommendations.
From July 1982 to December 1989, 43 of 1,583 adults (2.7%) with soft tissue sarcoma admitted to the Memorial Sloan-Kettering Cancer Center (MSKCC) had tumors arising from the urinary tract and male genital tract (urological sarcoma). The most common site of origin of the tumor was paratesticular (14 patients), followed by the prostate/seminal vesicle (12), bladder (10) and kidney (7). The most common histological type was leiomyosarcoma (19 patients), followed by rhabdomyosarcoma (14), liposarcoma (5) and 5 other histological sarcoma categories (angiosarcoma, malignant fibrous histiocytoma, mesenchymoma and 2 undifferentiated sarcomas). Most of the tumors were high grade (86%) and more than half (56%) were greater than 5 cm. in diameter. A total of 9 patients (21%) presented with metastatic disease, 8 of whom had rhabdomyosarcoma. Complete resection with negative microscopic margins was possible in 58% of the patients. Actuarial relapse-free survival for all patients at 3 and 5 years was 55% and 40%, respectively. There were no significant differences in survival based on patient age, sex or histological tumor type. Favorable prognostic variables by univariate analysis included tumor diameter less than 5 cm., low histological grade, paratesticular or bladder tumor site and complete surgical resection. Application of the MSKCC sarcoma staging system, which is based on grade, size, depth and presence or absence of metastasis, was useful to predict survival. In our experience patients with stage 3 (high grade, greater than 5 cm., 15 patients) or stage 4 (metastatic disease, 9 patients) had a combined 3-year relapse-free survival rate of only 26% and they should be considered candidates for adjuvant treatment protocols.
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