Mariconda M, Tacchetti C, Misiti C M, Lotti G, Ammendolia A, Millo R, Milano C
Clinique Orthopédique, Faculté de Médecine de Catanzaro, Université de Reggio Calabria, Italie.
Rev Chir Orthop Reparatrice Appar Mot. 1995;81(4):338-43.
Bone leiomyosarcoma is a rare tumor, whether it may be primary or secondary. The authors report on the case of a woman, aged 67, admitted in January 1992 complaining of pain in the left hip and the upper end of the femur.
In 1985 the patient underwent surgical excision of a soft tissue tumor in the right thigh, histologically diagnosed as a benign fibrous tumor. This lesion recurred locally four times and repeated excisions were performed throughout the years, always with a histological diagnosis of a benign lesion. On admission to hospital, the physical examination as well as laboratory data and plain roentgenograms were unremarkable. Both tomography and MRI showed a lesion in the upper end of the left femur. An isotopic bone scan showed marked increased uptake in the left hip extending to the femoral diaphysis. An open biopsy was performed for histology, immunohistochemistry and electron microscopy. A diagnosis of metastatic leiomyosarcoma was made. The retrospective histological examination of specimens of the soft tissue tumor excised in 1985 showed the same immunohistochemical features of the contralateral leiomyosarcoma. On this basis, one stage resection of the left hip and the upper end of the femur was performed and a Kotz modular prosthesis was inserted. Postoperative healing was achieved without any complications and the function of the operated limb was satisfactory. Three months after the operation pulmonary lesions were noted on chest radiographs and CT scan. The patient died two years after the first admission for widespread metastasis.
In the reported case, the bony metastasis appeared to be the presenting finding of the soft tissue tumor of the contralateral thigh. This presentation is rare in previously published series. The misdiagnosis of the primary tumor had caused local recurrences, and an increased malignity occurred. According to the literature, a soft tissue leiomyosarcoma can be easily confused with other spindle cell lesions. Therefore an accurate histological and ultrastructural diagnosis is necessary for adequate surgical treatment.
骨平滑肌肉瘤是一种罕见的肿瘤，无论是原发性还是继发性。作者报告了一例67岁女性病例，该患者于1992年1月入院，主诉左髋部和股骨上端疼痛。
1985年，患者接受了右大腿软组织肿瘤的手术切除，组织学诊断为良性纤维瘤。该病变局部复发4次，多年来进行了多次切除，组织学诊断均为良性病变。入院时，体格检查、实验室检查数据及X线平片均无异常。断层扫描和磁共振成像均显示左股骨上端有病变。同位素骨扫描显示左髋部摄取明显增加，延伸至股骨干。进行了开放活检以进行组织学、免疫组织化学和电子显微镜检查。诊断为转移性平滑肌肉瘤。对1985年切除的软组织肿瘤标本进行回顾性组织学检查，显示与对侧平滑肌肉瘤具有相同的免疫组织化学特征。在此基础上，对左髋部和股骨上端进行了一期切除，并植入了Kotz模块化假体。术后愈合良好，无任何并发症，手术肢体功能满意。术后3个月，胸部X线片和CT扫描发现肺部病变。患者首次入院两年后因广泛转移死亡。
在本报告病例中，骨转移似乎是对侧大腿软组织肿瘤的首发表现。这种表现在以往发表的系列病例中很少见。原发性肿瘤的误诊导致了局部复发，并增加了恶性程度。根据文献，软组织平滑肌肉瘤很容易与其他梭形细胞病变混淆。因此，准确的组织学和超微结构诊断对于充分的手术治疗是必要的。
