Vulpio C, Destito C, Carlucci I, Mercuri M, Ruffini M V, Wiel Marin A
Istituto di Clinica Chirurgica, Università Cattolica del Sacro Cuore, Policlinico A. Gemelli, Roma.
Minerva Chir. 1996 Dec;51(12):1129-33.
Extraskeletal osteosarcomas, localized in the retroperitoneal space, are very rare neoplasms (only 200 cases have been described); the case indicated is the second one observed in Literature. The extraskeletal osteosarcomas are very malignant mesenchymal neoplasms reproducing osseous and cartilaginous tissue disconnected from the skeletal system. From a histological point of view, the extraskeletal osteosarcomas are constituted of osseous and cartilaginous tissue with osteo-fibro-chondroblastic cells: the different representation of three types of cells define the variety of extraskeletal osteosarcomas (osteoblastic, chondroblastic, fibroblastic). Histological and ultrastructural study failed to find differences between skeletal and extraskeletal osteosarcomas. In the clinical history of some patients traumas or irradiation has been referred; although extraskeletal osteosarcomas etiology is frequently unknown. Diagnosis is generally delayed because symptoms are often absent of uncertain. Prognosis of these neoplasms is always very poor, and specially in relation to the dimension and to the possibility of its radical removal. All patients died until 2-3 years after surgery.
骨外骨肉瘤，位于腹膜后间隙，是非常罕见的肿瘤（仅报道过200例）；所提及的病例是文献中观察到的第二例。骨外骨肉瘤是非常恶性的间叶性肿瘤，可产生与骨骼系统无关的骨和软骨组织。从组织学角度来看，骨外骨肉瘤由骨和软骨组织以及骨-纤维-软骨母细胞组成：三种类型细胞的不同表现形式界定了骨外骨肉瘤的种类（成骨细胞型、软骨母细胞型、纤维母细胞型）。组织学和超微结构研究未能发现骨内和骨外骨肉瘤之间存在差异。在一些患者的临床病史中，曾提及有创伤或放疗史；尽管骨外骨肉瘤的病因通常不明。诊断通常会延迟，因为症状往往不明显或不确定。这些肿瘤的预后总是非常差，特别是考虑到其大小以及能否彻底切除。所有患者在术后2至3年内死亡。
