Epithelioid sarcoma: one institution's experience with a rare sarcoma

Abstract

Background Epithelioid sarcomas (ES) are extremely rare soft tissue sarcomas. As such, their clinical behavior and response to treatment are poorly described in the literature. Methods We queried the centralized cancer registry and pathology archives at the Johns Hopkins Medical Institution and identified 22 patients with a diagnosis of ES. We excluded two patients because of inadequate data. A pathologist reviewed patient charts and reexamined available histological slides. This study was performed with institutional review board approval. Results The median age at diagnosis was 27.8 y; most patients (75%) were male. Regional lymph node metastases were present in 10% of patients at presentation. The majority of tumors (57.9%) recurred and 35% recurred more than once, although the number of recurrences did not affect survival (P = 0.48). Patients did not experience a decrease in time to recurrence with increasing number of resections. The median time between resection and recurrence was 1.23 y and the maximum was 18.8 y. Median overall survival was 56.2 mo and 5-y survival was 92%. Conclusions Our study reveals that ES is an extremely rare tumor with a protracted and recurrent course, but overall survival may be more favorable than in the past. Patients benefit from aggressive and repeated resection. Epithelioid sarcoma is unique because it metastasizes to regional nodal basins. Extended surveillance is indicated, because recurrences can appear after decades of quiescence.

Soft Tissue Sarcomas

“…The majority of these tumors (more than 90 %) carry giant chromosomes composed of translocated portions of chromosomes 17 and 22. This tumor is sensitive to imatinib, mainly the tumors with the t (17,22) translocation. Imatinib has been approved by the FDA as a first line of treatment for advanced disease [2,3,11,12].…”

“…Regional lymph node metastasis is also fairly common and it tends to propagate along fascial planes, tendon sheaths, and nerve sheaths making it very difficult to treat. Patients benefit from repeated resections and extended surveillance is indicated with recurrences appearing decades after quiescence [2,3,17].…”

Phase 2 study of dasatinib in patients with alveolar soft part sarcoma, chondrosarcoma, chordoma, epithelioid sarcoma, or solitary fibrous tumor

“…For example, the rate of metastasis in patients with alveolar soft part sarcoma (ASPS) is greater than 50%, but the median survival is more than 3 years after the diagnosis of metastases . Solitary fibrous tumor (SFT) and epithelioid sarcoma (ES) are other soft tissue sarcomas with an indolent growth rate but a high propensity for recurrence and metastasis . Chordoma and low‐ to intermediate‐grade chondrosarcoma (CS) are types of sarcoma arising in the bone that also exhibit a low mitotic rate but have a risk of local recurrence and metastasis.…”

Acral manifestations of soft tissue tumors

“…Extended surveillance is indicated, because recurrences can appear after decades of quiescence. 55,[57][58][59][60] Acral myxoinflammatory fibroblastic sarcoma…”

“…Due to the young patient age at presentation, a benign diagnosis is often presumed initially, resulting in substantial diagnostic delays of several month. 60 Epithelioid sarcoma is adeptly named in two ways: 1) it mimics carcinoma in its pattern of metastatic spread to lymph nodes, as alluded to above, and 2) it mimics carcinoma microscopically and immunohistochemically. Microscopic assessment finds variably cohesive, polygonal cells with voluminous cytoplasm, sometimes growing in waves around a zone of necrosis ( Figure 18).…”