Extraskeletal neoplasm resembling Ewing's sarcoma

Abstract

This article reviews the pathologic features and the behavior of 39 small, round, or oval cell sarcomas occurring in the soft tissues and considered histologically indistinguishable from Ewing's sarcoma of bone. The tumors affected chiefly young adults (median age 20 years) and most commonly involved the soft tissues of the lower extremity and the paravertebral region. Microscopically, they consisted of solidly packed small, round, or ovoid cells of great uniformity, arranged in sheets or lobules separated by strands of fibrous connective tissue. The nucleus of the tumor cells contained finely divided chromatin, a distinct nuclear membrane, and frequently a minute nucleolus. The scanty ill-defined cytoplasm contained varying amounts of glycogen. Sometimes the histologic picture was dominated by a "peritheliomatous" pattern, or by large areas of necrosis or hemorrhage. Followup data ranging from 1 month to 14 years were available in 35 of the 39 cases (93%). Of these, 13 were alive and 22 had died. In the majority of the fatal cases, the clinical course was rapid; metastatic lesions developed within a few months after the primary tumor was excised. The lungs and the skeleton were the two most common sites of metastasis. Cure may be achieved by wide local excision of the tumor at an early stage of the disease, combined with radiation therapy and chemotherapy.

Establishment and Characterization of a Clonal Human Extraskeletal Ewing's Sarcoma Cell Line, EES1

“…2), which is consistent with previously reported histological findings (Angervall and Enzinger 1975;Ahmad et al 1999). Rosette-like structures were infrequently seen.…”

“…Angervall and Enzinger (1975) first reported the pathological features and the behavior of EES, which are round, or oval cell sarcomas occurring in the soft tissues, and they also reported that this tumor wad histologically indistinguishable from Ewing's sarcoma of bone. The tumors affect mostly young adults (median age 20 years) and most commonly involve the soft tissues of the lower extremity and the paravertebral region.…”

Orthopaedic Case of the Month: A 14-Year-Old Boy With a Medial Thigh Soft Tissue Mass

“…On immunohistochemistry, S-100 positivity frequently is seen [13,34]. Extraosseous Ewing's sarcoma is a malignant small, round, blue-cell tumor that occurs mostly during or after adolescence, with a slight male predominance [2,31,44,52]. The extremities are the most common sites of involvement [41,48].…”

Superficial primitive Ewing's sarcoma: a clinicopathologic and molecular cytogenetic analysis of 14 cases

“…Since the first description of PNET/ES arising in superficial locations (ie, in the skin and/or subcutis) by Angervall and Enzinger in 1975, 13 32 cases diagnosed on at least histology and CD99 positivity have been reported, more often as single cases [1][2][3][4][5] with three series of 8, 5, and 13 cases. [6][7][8] A review of the reported cases (Table 7) shows that they occurred in 8 male and 24 female patients aged from 2 to 67 years (median, 18), with no site predilection.…”