'Telangiectatic' transformation in soft tissue sarcomas. a clinicopathology analysis of an aggressive feature of high-grade sarcomas.

BACKGROUND: 'Telangiectatic' change, which contains a large fluid hemorrhagic component, occurs in a variety of high-grade soft tissue sarcomas. METHODS: In a retrospective database review, we identified 20 consecutive patients (3%) with 'telangiectatic' change in soft tissue sarcomas. RESULTS: Tumors were located in the thigh (55%), shoulder (15%), calf (15%), upper arm (10%), and buttock in one patient. All 20 tumors were high grade. Histological diagnoses were MFH (40%), leiomyosarcoma (15%), synovial sarcoma (10%), and one each of seven other sarcomas (35%). Tumor size was often large-more than 10 cm (35%), between 5 and 10 cm (60%), and less than 5 cm in one case. A history of contusion to the tumor site followed by swelling was recorded in 30% of patients and 80% presented with a painful mass. On MRI imaging, 60% of tumors appeared to contain more than 50% blood, 50% had a hemosiderin-laden rim, and 55% had well-defined tumor nodules within the wall of the hematoma. Limb-sparing surgery was carried out in 90% of patients, the other 10% underwent primary amputation. The 5-year, event-free survival rate was 30%. Of the patients, 15% presented initially with metastatic disease; in 53%, it developed within 2 years of diagnosis. The overall local recurrence rate was 30%. CONCLUSIONS: Telangiectatic transformation in soft tissue sarcomas is a rare feature of aggressive high-grade soft tissue sarcomas and is unique in its clinical presentation, MRI characteristics, pathological pattern, and a tendency for a worse-off prognosis.

Paratesticular leiomyosarcoma in an elderly man.

Soft tissue tumors, both benign and malignant, of the testes are relatively uncommon. Primary leiomyosarcoma of the testis is rare, and its clinical behavior is difficult to predict. We report a case of paratesticular leiomyosarcoma in a 71-year-old man. Routine histologic examination and immunohistochemical staining were performed. The tumor was composed of interlacing fascicles of smooth muscle cells with marked nuclear pleomorphism and three to four mitoses per high power field, with few atypical mitoses. On the basis of the microscopic examination and immunohistochemical studies, the tumor was identified as a paratesticular leiomyosarcoma. The published studies on this entity are reviewed.

Diagnosis of gastrointestinal stromal tumors: A consensus approach.

As a result of major recent advances in understanding the biology of gastrointestinal stromal tumors (GISTs), specifically recognition of the central role of activating KIT mutations and associated KIT protein expression in these lesions, and the development of novel and effective therapy for GISTs using the receptor tyrosine kinase inhibitor STI-571, these tumors have become the focus of considerable attention by pathologists, clinicians, and patients. Stromal/mesenchymal tumors of the gastrointestinal tract have long been a source of confusion and controversy with regard to classification, line(s) of differentiation, and prognostication. Characterization of the KIT pathway and its phenotypic implications has helped to resolve some but not all of these issues. Given the now critical role of accurate and reproducible pathologic diagnosis in ensuring appropriate treatment for patients with GIST, the National Institutes of Health convened a GIST workshop in April 2001 with the goal of developing a consensus approach to diagnosis and morphologic prognostication. Key elements of the consensus, as described herein, are the defining role of KIT immunopositivity in diagnosis and a proposed scheme for estimating metastatic risk in these lesions, based on tumor size and mitotic count, recognizing that it is probably unwise to use the definitive term "benign" for any GIST, at least at the present time.

Diagnosis of gastrointestinal stromal tumors: a consensus approach.

As a result of major recent advances in understanding the biology of gastrointestinal stromal tumors (GIST), specifically recognition of the central role of activating KIT mutations and associated KIT protein expression in these lesions, and the development of novel and effective therapy for GISTs using the receptor tyrosine kinase inhibitor STI-571, these tumors have become the focus of considerable attention among pathologists, clinicians, and patients. Stromal/mesenchymal tumors of the gastrointestinal tract have long been a source of confusion and controversy with regard to classification, line(s) of differentiation, and prognostication. Characterization of the KIT pathway and its phenotypic implications has helped to resolve some but not all of these issues. Given the now critical role of accurate and reproducible pathologic diagnosis in ensuring appropriate treatment for patients with GIST, the National Institutes of Health (NIH) convened a GIST workshop in April 2001 with the goal of developing a consensus approach to diagnosis and morphologic prognostication. Key elements of the consensus, as described herein, are the defining role of KIT immunopositivity in diagnosis and a proposed scheme for estimating metastatic risk in these lesions, based on tumor size and mitotic count, recognizing that it is probably unwise to use the definitive term benign for any GIST, at least at the present time.

Diagnosis of primary bone tumors with image-guided percutaneous biopsy: experience with 110 tumors.

PURPOSE: To determine the diagnostic accuracy of image-guided percutaneous biopsy in 110 primary bone tumors of varying internal compositions. MATERIALS AND METHODS: One hundred ten consecutive patients with primary bone tumors underwent biopsy with computed tomography (CT) or fluoroscopy. Ninety-one patients underwent surgical follow-up and 19 received medical treatment and underwent subsequent imaging studies. Final analysis of bone biopsy results included tumor type, malignancy, final tumor grade, biopsy complications, and effect on eventual treatment outcome. RESULTS: Seventy-seven tumors were malignant and 33 were benign. Most common tumors at biopsy were osteosarcoma (n = 20), lymphoma (n = 18), chondrosarcoma (n = 16), and giant cell tumor (n = 16). Correct final diagnosis was attained in 97 (88%) patients. Sixty-three lesions were solid nonsclerotic; 26, sclerotic; and 21, lytic with cystic centers containing internal areas of fluid, hemorrhage, or necrosis. In six of 21 lesions with a predominant cystic internal composition, problems occurred in determining a final diagnosis. In 13 patients, definite correct diagnosis was not obtained with initial percutaneous bone biopsy. Of these patients, benign bone tumors were better defined with surgical specimens in seven, a diagnosis of malignancy was changed to that of another malignancy in four, and the diagnosis was changed from benign to malignant in two. Nine patients underwent open surgical biopsy. Seven of the difficult cases were of cystic tumors with hemorrhagic fluid levels visible at CT or magnetic resonance imaging. The only complication was a small hematoma. CONCLUSION: Percutaneous biopsy of primary bone tumors is safe and accurate for diagnosis and grade of specific tumor. In cases with nondiagnostic biopsy, open-procedure biopsy is likely to be associated with similar diagnostic difficulties.