Katabi M, Anract P, Forest M, Tomeno B
Service d'Orthopédie B, Hôpital Cochin, Paris.
Rev Chir Orthop Reparatrice Appar Mot. 1996;82(3):208-15.
The authors reviewed eight patients treated for Low Grade Intramedullary Osteosarcoma (LGIO). They analyzed the radiographical and histological features and evaluated the prognosis of this disease.
Eight cases of LGIO were studied. The mean follow-up period was 3.9 years. LGIO is defined as a Broders classification grade I osteosarcoma. The study involved 3 males and 5 females. Mean age at the time of diagnosis was 28.
Clinical records, radiographs and C.T. Scan were available and reviewed in all cases. Five patients had had MRI. In addition to the conventional histological study, ultrastructural studies were conducted in three of the cases. On their last examination, all patients received a clinical and X-ray assessment.
The tumor was localized around the knee in six cases on the humerus and on the proximal femur in one case. The tumor was situated in the metaphysis and was most often lytic. A malignant tumor was suspected at the outset for only two of the patients. The scanner and the MRI revealed a cortical discontinuity and extraosseous involvement in five cases. Seven patients had a wide resection, which was intra-tumoral once. The histological examination of two of those resections revealed limited areas of anaplasia; complementary chemotherapy was then conducted. We did not observe any local recurrence in this group. We observed, however, an iliac metastasis, for which another wide resection was performed. This last patient remained free of disease three years later. One patient had radiotherapy and chemotherapy; he died from pulmonary metastasis after his LGIO turned into high grade osteosarcoma.
LGIO are rare, they represent 1.8 per cent of all Osteosarcomas in the Cochin file. The mean age of patients contracting LGIO is higher than that of conventional osteosarcoma patients. Observation of the radiographs and histological examination could lead to think the tumor is benign, and mistake it with a fibrous displasia. The C.T. Scan and the MRI have the advantage of showing soft tissue invasion and help the histological examination. The wide resection is the only treatment able to cure the patient. Chemotherapy would be useful for LGIO cases containing limited arcas of anaplasia.
LGIO is a slowly-evolving tumor. It remains localized for a long period of time. The histological diagnosis is difficult and requires a pathologist familiar with bone tumors. Its treatment requires surgery. LGIO must be distinguished from conventional osteosarcomas because of its better prognosis.
作者回顾了8例接受低级别髓内骨肉瘤（LGIO）治疗的患者。他们分析了影像学和组织学特征，并评估了该疾病的预后。
研究了8例LGIO病例。平均随访期为3.9年。LGIO被定义为布罗德斯分类I级骨肉瘤。该研究涉及3名男性和5名女性。诊断时的平均年龄为28岁。
所有病例均有临床记录、X线片和CT扫描，并进行了回顾。5例患者进行了MRI检查。除了常规组织学研究外，对其中3例进行了超微结构研究。在最后一次检查时，所有患者均接受了临床和X线评估。
6例肿瘤位于膝关节周围，1例位于肱骨，1例位于股骨近端。肿瘤位于干骺端，最常见为溶骨性。最初仅2例患者被怀疑为恶性肿瘤。CT扫描和MRI显示5例有皮质连续性中断和骨外侵犯。7例患者进行了广泛切除，其中1例为瘤内切除。其中2例切除标本的组织学检查显示有局限性间变区域，随后进行了辅助化疗。该组未观察到局部复发。然而，观察到1例髂骨转移，为此又进行了一次广泛切除。最后这名患者3年后无疾病复发。1例患者接受了放疗和化疗；他的LGIO转变为高级别骨肉瘤后死于肺转移。
LGIO罕见，在科钦档案中占所有骨肉瘤的1.8%。患LGIO的患者平均年龄高于传统骨肉瘤患者。观察X线片和组织学检查可能会认为肿瘤是良性的，并将其与纤维发育异常混淆。CT扫描和MRI的优势在于能显示软组织侵犯并有助于组织学检查。广泛切除是唯一能治愈患者的治疗方法。化疗对含有局限性间变区域的LGIO病例可能有用。
LGIO是一种进展缓慢的肿瘤。它在很长一段时间内局限于局部。组织学诊断困难，需要熟悉骨肿瘤的病理学家。其治疗需要手术。由于LGIO预后较好，必须将其与传统骨肉瘤区分开来。
