Article

Minute synovial sarcomas of the hands and feet: a clinicopathologic study of 21 tumors less than 1 cm.

Sikl's Department of Pathology, Faculty Hospital, Pilsen, Czech Republic, and the Department of Soft Tissue Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.
American Journal of Surgical Pathology (Impact Factor: 5.15). 06/2006; 30(6):721-6.
Source: PubMed

ABSTRACT

Synovial sarcoma, one of the most common types of soft tissue sarcomas, usually presents in the proximal or middle portions of the extremities, often as a large mass with an aggressive clinical behavior. Gland-forming biphasic and spindle cell fibrous monophasic tumors are the most common subtypes. In this study, we evaluated 21 minute synovial sarcomas, <1 cm in diameter, from the hands and feet. These tumors occurred in 14 females and 7 males with a median age of 29 years (range, 8-60 years). Clinically, all tumors were thought to be benign processes such as a ganglion cyst or glomus tumor, and on microscopic examination, they were also often initially misinterpreted as benign lesions such as nerve sheath or (myo) fibroblastic tumors. Histologically, 7 tumors were biphasic and 14 were monophasic spindle cell variants. Microscopic calcifications were present in 8 cases and were prominent in 3 tumors. All monophasic tumors tested had elements positive for EMA, and all but one had reactivity for a keratin cocktail. S-100 protein-positive neuroma-like neural proliferations were commonly present in the monophasic but not in biphasic tumors. SYT-SSX fusion transcripts were demonstrated in 5 cases studied by polymerase chain reaction assay. All tumors were enucleated, followed by local reexcision of the site, and often combined with postoperative radiation. Three patients had amputation of the involved digit or metatarsal. Four patients had local recurrences, 2 of which were successfully treated; 2 of these patients were lost to follow-up. Despite some variation in treatment, all 12 patients with complete follow-up were alive and well, 2 to 32.2 years after surgery (median, 14.7 years), including 2 patients who received neither amputation nor postoperative radiation. Minute synovial sarcomas of hands and feet are clinically favorable tumors if completely excised; there is some evidence to suggest that they may be managed more conservatively than larger tumors. These tumors should be recognized as part of the spectrum of synovial sarcomas.

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    • "Typical SS are from 3 to 10 cm in diameter [7], although lesions can be small, especially when they involve the hands or head and neck [27]. " Minute " SSs are less than 1 cm in diameter and often arise in the hands and feet and are clinically often thought to be benign processes such as ganglion cysts or glomus tumors [28]. No association of pathologic subtypes with specific imaging findings is noted [25]. "
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    ABSTRACT: Synovial sarcoma (SS) is a malignant mesenchymal neoplasm with variable epithelial differentiation, with a propensity to occur in young adults and which can arise at almost any site. It is generally viewed and treated as a high-grade sarcoma. As one of the first sarcomas to be defined by the presence of a specific chromosomal translocation leading to the production of the SS18-SSX fusion oncogene, it is perhaps the archetypal “translocation-associated sarcoma,” and its translocation remains unique to this tumor type. Synovial sarcoma has a variety of morphologic patterns, but its chief forms are the classic biphasic pattern, of glandular or solid epithelial structures with monomorphic spindle cells and the monophasic pattern, of fascicles of spindle cells with only immunohistochemical or ultrastructural evidence of epithelial differentiation. However, there is significant morphologic heterogeneity and overlap with a variety of other neoplasms, which can cause diagnostic challenge, particularly as the immunoprofile is varied, SS18-SSX is not detected in 100% of SSs, and they may occur at unusual sites. Correct diagnosis is clinically important, due to the relative chemosensitivity of SS in relation to other sarcomas, for prognostication and because of the potential for treatment with specific targeted therapies in the near future. We review SS, with emphasis on the diagnostic spectrum, recent immunohistochemical and genetic findings, and the differential diagnosis.
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    • "Synovial sarcoma is a relatively rare malignancy that typically occurs in adolescents and young adults between the ages of 15 and 50 years of age and most commonly affects the extremities in the vicinity of large joints such as the knee or the thigh (Cadman et al., 1965; Cordon-Cardo, 1997; Nicholson et al., 1998; Cappello and Barnes, 2001; Kumar et al., 2005c) and the hands or feet (Michal et al., 2006). Because synovial sarcoma can be difficult to distinguish from reactive mesothelial proliferation and sarcomatoid mesothelioma by use of histology and immunohistochemical markers alone (Shiraki et al., 1989; Moran et al., 1992; Nicholson et al., 1998; Miettinen et al., 2001; Carbone et al., 2002; Gladish et al., 2002; Vohra et al., 2004; Taylor et al., 2005; Michal et al., 2006; Rdzanek et al., 2006), it represents another alternative carcinoma to consider when evaluating suspected sarcomatoid mesothelioma cases. "
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    ABSTRACT: The diagnosis of mesothelioma is not always straightforward, despite known immunohistochemical markers and other diagnostic techniques. One reason for the difficulty is that extrapleural tumors resembling mesothelioma may have several possible etiologies, especially in cases with no meaningful history of amphibole asbestos exposure. When the diagnosis of mesothelioma is based on histologic features alone, primary mesotheliomas may resemble various primary or metastatic cancers that have directly invaded the serosal membranes. Some of these metastatic malignancies, particularly carcinomas and sarcomas of the pleura, pericardium and peritoneum, may undergo desmoplastic reaction in the pleura, thereby mimicking mesothelioma, rather than the primary tumor. Encasement of the lung by direct spread or metastasis, termed pseudomesotheliomatous spread, occurs with several other primary cancer types, including certain late-stage tumors from genetic cancer syndromes exhibiting chromosomal instability. Although immunohistochemical staining patterns differentiate most carcinomas, lymphomas, and mestastatic sarcomas from mesotheliomas, specific genetic markers in tumor or somatic tissues have been recently identified that may also distinguish these tumor types from asbestos-related mesothelioma. A registry for genetic screening of mesothelioma cases would help lead to improvements in diagnostic criteria, prognostic accuracy and treatment efficacy, as well as improved estimates of primary mesothelioma incidence and of background rates of cancers unrelated to asbestos that might be otherwise mistaken for mesothelioma. This information would also help better define the dose-response relationships for mesothelioma and asbestos exposure, as well as other risk factors for mesothelioma and other mesenchymal or advanced metastatic tumors that may be indistinguishable by histology and staining characteristics.
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    • "Small sarcomas of various types are well known for having a relatively good circumscription. This is true for example in minute synovial sarcomas of the hands and feet [30] and small superficial epithelioid sarcomas as well as small alveolar soft part sarcomas in the tongue and endocervix. We found no light microscopical or immunohistochemical evidence of melanocytic, endothelial, smooth muscle, rhabdomyoblastic, myofibroblastic or mesothelial differentiation. "
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