Adult Prostate Sarcoma: The Memorial Sloan Kettering Experience

Abstract

Objectives To present our institutional experience with adult prostate sarcoma over 30 years. Methods We reviewed 38 cases of adult prostate sarcoma diagnosed and treated at our institution between 1982 and 2012. Univariate Cox proportional hazards regression was used to determine if there was an association between specific disease characteristics (tumor size, histology, AJCC stage, and metastasis at diagnosis) and cancer-specific survival (CSS). Results A total of 38 patients were included, with a median age of 50 (range 17–73). Most men presented with lower urinary tract symptoms (45%), hematuria (24%), or acute urinary retention (21%). Diagnosis was established with prostate needle biopsy (68%) or transurethral resection of the prostate (18%). The predominant histologic subtypes were leiomyosarcoma (13 cases, 34%) and rhabdomyosarcoma (12 cases, 32%). Rhabdomyosarcoma was associated with poorer CSS (hazard ratio = 3.00; 95% CI 1.13, 7.92; p = 0.027) compared to leiomyosarcoma. We did not observe a significant relationship between tumor size and CSS. Overall, median CSS was 2.9 years (95% CI 1.5, 5.4), with 7.7 years for clinically localized disease (95% CI 2.5, not reached) and 1.5 years for metastatic disease (95% CI 1.1, 2.7). Conclusions Adult prostate sarcoma has a poor prognosis, especially in cases of metastatic disease at the time of diagnosis. Surgery remains the standard of care, but it provides limited benefit to those with metastatic disease or as a consolidation therapy after partial response to systemic therapy.

Adult prostatic sarcoma: A contemporary multicenter Rare Cancer Network study

“…Because of their different natural history, sarcomas arising from different parts of the urogenital system should be managed accordingly. In a population of 38 PS patients, Musser et al confirmed that the presence of metastatic disease is a poor prognostic factor for CSS (HR: 5.91, 95%CI: 2.31‐15.11, P = 0.0002). These authors also showed that rhabdomyosarcoma patients have the poorest CSS compared to leiomyosarcomas patients (HR: 3.00, range: 1.13‐7.92, P = 0.027).…”

Synchronous Malignant Peripheral Nerve Sheath Tumor and Adenocarcinoma of the Prostate: Case Report and Literature Review

“…Sarcomas of the prostate account for 0.7% of all malignant prostatic tumors [4]. Forty percent of MPNSTs are sporadic and the incidence in the general population is 0.001% [1, 3].…”

Primary Intraprostatic Synovial Sarcoma

“…The remaining sarcomas described include prostatic stromal sarcoma, synovial sarcoma, malignant peripheral nerve sheath tumor, osteosarcoma, Ewing sarcoma/primitive neuroectodermal tumor, angiosarcoma, undifferentiated sarcoma, and mixed sarcomas. 13,[19][20][21] CURRENT TREATMENT The rare incidence of intraprostatic synovial sarcoma presents a challenge to the establishment of best practices. Each case presented in the literature thus far has been treated with a combination of surgical resection, chemotherapy, and/or radiation therapy.…”