Extraskeletal telangiectatic osteosarcoma

Abstract

The first three well‐documented cases of pure, extraskeletal telangiectatic osteosarcoma of the soft tissues are presented in this article. The distinctive gross features were a predominance of large, blood‐filled spaces, which, on histologic examination, were large blood‐filled cavities in association with cyst‐like walls containing anaplastic spindle cells and definite osteoid production in two of three cases. One of the cases is of additional clinical interest because the teen‐aged daughter of the woman patient had died 1 year previously of a high‐grade intramedullary osteosarcoma of bone.

Mesenteric extraskeletal osteosarcoma with telangiectatic features: a case report

“…In an article on telangiectatic osteosarcoma of bone, osteoid was not found in five of 25 cases (25%) [8]. The first detailed report on three cases of telangiectatic EOS also noted the absence of osteoid in one case [1]. Before those reports on the telangiectatic subtype, the diagnosis of osteosarcoma was strictly defined as a mesenchymal tumor characterized by the direct production of osteoid or bone by malignant cells.…”

“…Extraskeletal osteosarcoma (EOS) is a rare malignant mesenchymal tumor characterized by the production of neoplastic osseous tissues, without attachment to bone or the periosteum [1]. It occurs predominantly in the lower extremity, upper extremity, and retroperitoneum, and rarely in the visceral organs [2].…”

“…It occurs predominantly in the lower extremity, upper extremity, and retroperitoneum, and rarely in the visceral organs [2]. Of the different histologic variants of EOS, the telangiectatic variant has only a small number of subtypes, and its pattern is more often mentioned as a minor component of other variants [1-3]. To date, only two cases of extraskeletal osteosarcoma in the mesentery have been reported [4,5].…”

Fine-needle aspiration biopsy of skeletal versus extraskeletal osteosarcoma

“…22 Extraskeletal telangiectatic OS represents one of the rarest subtypes. 22,23 Morphologically, the tumors are hemorrhagic and comprised of aneurysmal bone cyst-like septa containing frankly anaplastic tumor cells and infrequent deposits of osteoid. One of our cases of extraskeletal OS represented the telangiectatic subtype.…”

Telangiectatic Osteosarcoma

“…The most common location at presentation is the metaphyses of long bones, with the following location distribution pattern 11 : distal femur (41.6%), proximal tibia (16.9%), proximal humerus (9.2%), proximal femur (7.7%), mid-femur (6.2%), mid-humerus (4.6%), mid-tibia (3.1%), pelvis (3.1%), fibula (1.5%), skull bones (1.5%), and ribs (1.5%). Occasional cases of extraosseous TO are described in the literature, 12 with TO arising in the soft tissues of the popliteal fossa, thigh, and forearm.…”