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Cellular and Histopathologic Classification

continued...

Vascular tumors

Angiosarcoma: A review of 20 years of experience in the Italian and German Soft Tissue Sarcoma Cooperative Group identified 12 children with angiosarcoma.[70] Only one objective response to chemotherapy was observed, and the overall behavior of this tumor was identical to angiosarcoma in adults. A subsequent retrospective study performed by the Polish and German Cooperative Paediatric Soft Tissue Sarcoma Study Groups identified four chemotherapy responses in ten children treated (total 14 children with angiosarcoma).[71] Another review of 15 patients demonstrated a 33% survival rate.[72] A review of 222 patients (median age, 62 years; range, age 15-90 years) showed an overall disease-specific survival (DSS) rate of 38% at 5 years. Five-year DSS was 44% in 138 patients with localized, resected tumors but only 16% in 43 patients with metastases at diagnosis.[73] Anti-angiogenesis therapy may prove useful in the treatment of this group of neoplasms.[74]

Hemangioendothelioma: Hemangioendotheliomas are tumors found in infants that arise within the liver or elsewhere and usually remain benign.[75] The tumors are sometimes associated with consumptive coagulopathy, also known as the Kasabach-Merritt syndrome (or phenomenon).[76,77,78] In older children and adults, hemangioendotheliomas may occur elsewhere in the body and can metastasize to lungs, lymph nodes, bones, and within the pleural or peritoneal cavities. The preferred pathologic designation for these lesions in older persons is epithelioid hemangioendothelioma, which connotes the possibility of distant spread. These latter lesions are considered of intermediate malignant potential, between benign hemangioma and angiosarcoma.[79,80]

Biopsy Technique for Soft Tissue Sarcoma

When a suspicious lesion is identified it is crucial that a complete workup followed by adequate biopsy be performed. Generally, it is better to image the lesion prior to any interventions. A core-needle biopsy or limited open biopsy that obtains an adequate amount of tissue for histopathology, immunohistochemistry, and molecular genetics is mandatory, given the diagnostic importance of translocations. Needle biopsy techniques must obtain an adequate tissue sample and usually require obtaining multiple cores of tissue. Image guidance using ultrasound, computed tomography scan, or magnetic resonance imaging may be necessary to ensure a representative biopsy.[81] Incisional biopsies are acceptable but should not compromise subsequent wide local excision, and extensive dissection around the lesion must be avoided. Transverse extremity incisions should be avoided to reduce skin loss, as should extensive surgical procedures prior to definitive diagnosis.

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