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‘Telangiectatic’ Transformation in Soft Tissue Sarcomas. A Clinicopathology Analysis of an Aggressive Feature of High-grade Sarcomas

¹ Department of Orthopedic Oncology, Washington Cancer Institute, Washington Hospital Center, Washington, DC, USA
² Department of Pathology, Washington Cancer Institute, Washington Hospital Center, Washington, DC, USA
³ Department of Radiology, Washington Cancer Institute, Washington Hospital Center, Washington, DC, USA
⁴ Georgetown University, Washington, DC, USA
⁵ Pediatric and Surgery Branch, National Cancer Institute, Bethesda, USA

Correspondence: Address correspondence and reprint requests to: Amir Sternheim, MD; E-mail: amirsternheim{at}gmail.com

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.

Key Words: Soft tissue sarcoma • Tumor Necrosis • Hemorrhagic Sarcoma