Synovial sarcoma of bone: Sarcoma typically of soft tissues presenting as a primary bone tumor

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Abstract

Synovial sarcoma typically presents as periarticular soft tissue mass in adolescent and young adult patients. Very rarely, soft tissue sarcomas may arise primarily within bone posing a significant diagnostic challenge as primary osseous malignancies such as osteosarcoma and metastatic disease are much more common. While tissue sampling with immunohistochemical and genetic testing are required for definitive diagnosis, radiologists and orthopedic oncologists should consider alternate etiologies when typical imaging features of more common bone tumors are not identified. As an example, we present a 33-year-old male referred with a pathologic hip fracture proven to represent primary synovial sarcoma of bone.

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Malignant soft-tissue tumors in a large referral population: distribution of diagnoses by age, sex, and location.

Mark Kransdorf (1994)

The purpose of this study was to determine the relative prevalence, age at presentation, sex distribution, and skeletal distribution of malignant soft-tissue tumors and to ascertain the relative frequency of these tumors in specific anatomic locations and age groups among a population of patients in a large pathologic consultation service. The computer diagnoses of 39,179 lesions occurring in 38,484 patients seen by soft-tissue pathologists at the Armed Forces Institute of Pathology during the 10-year period from January 1, 1980, to December 31, 1989, were retrospectively reviewed. All lesions were placed in one of 121 major categories in accordance with the classification system used by the World Health Organization and coded to one of 32 anatomic locations, such as hand, wrist, forearm, and so forth. Age and sex also were recorded. For purposes of analysis, all lesions were placed in one of 10 categories: hand and wrist, upper extremity, proximal limb girdle (axilla and shoulder), foot and ankle, lower extremity, hip and buttocks region, head and neck, trunk, retroperitoneum, and other lesions. The study group included 31,047 mesenchymal lesions, of which 12,370 were malignant. More than 80% of malignant tumors were classified into eight diagnostic categories: malignant fibrous histiocytoma (24%), liposarcoma (14%), leiomyosarcoma (8%), malignant schwannoma (6%), dermatofibrosarcoma protuberans (6%), synovial sarcoma (5%), fibrosarcoma (5%), and sarcoma, not classified further (12%). Approximately 79% of all malignant tumors were classified into five diagnoses for each age and location. With the distal upper extremity (hand and wrist) as an example, 50% of malignant lesions in the 16-25-year-old group were classified as epithelioid sarcoma (29%), malignant fibrous histiocytoma (13%), and synovial sarcoma (8%). For the same location but for children 5 years old or younger, almost 50% of malignant tumors were classified as infantile fibrosarcoma. Despite the multitude of pathologic possibilities, most malignant soft-tissue tumors are classified into a small number of diagnoses. These may be further defined when the location of the lesion and the age of the patient are considered. Knowledge of tumor prevalence will assist radiologists in establishing a suitably ordered differential diagnosis when a soft-tissue tumor has a nonspecific radiologic appearance.

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From the archives of the AFIP: Imaging of synovial sarcoma with radiologic-pathologic correlation.

Mark D Murphey, Michael Gibson, Bryan Jennings … (2015)

Synovial sarcoma is the fourth most common type of soft-tissue sarcoma, accounting for 2.5%-10.5% of all primary soft-tissue malignancies worldwide. Synovial sarcoma most often affects the extremities (80%-95% of cases), particularly the knee in the popliteal fossa, of adolescents and young adults (15-40 years of age). Despite its name, the lesion does not commonly arise in an intraarticular location but usually occurs near joints. Histologic subtypes include monophasic, biphasic, and poorly differentiated; the cytogenetic aberration of the t(X;18) translocation is highly specific for synovial sarcoma. Although radiographic features of these tumors are not pathognomonic, findings of a soft-tissue mass, particularly if calcified (30%), near but not in a joint of a young patient, are very suggestive of the diagnosis. Cross-sectional imaging features are vital for staging tumor extent and planning surgical resection; they also frequently reveal suggestive appearances of multilobulation and marked heterogeneity (creating the "triple sign") with hemorrhage, fluid levels, and septa (creating the "bowl of grapes" sign). Two features associated with synovial sarcoma that may lead to an initial mistaken diagnosis of a benign indolent process are slow growth (average time to diagnosis, 2-4 years) and small size (< 5 cm at initial presentation); in addition, these lesions may demonstrate well-defined margins and homogeneous appearance on cross-sectional images. Synovial sarcoma is an intermediate- to high-grade lesion, and, despite initial aggressive wide surgical resection, local recurrence and metastatic disease are common and prognosis is guarded. Understanding and recognizing the spectrum of appearances of synovial sarcoma, which reflect the underlying pathologic characteristics, improve radiologic assessment and are important for optimal patient management. Copyright RSNA, 2006.