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骨平滑肌肉瘤:原发性还是继发性?一例病例报告及文献复习

Leiomyosarcoma in bone: primary or secondary? A case report and review of the literature.

作者信息

Fornasier V L, Paley D

出版信息

Skeletal Radiol. 1983;10(3):147-53. doi: 10.1007/BF00357768.

Abstract

Our case demonstrates how metastases may be present and symptomatic long before they produce sufficient osseous changes to render the lesions radiologically detectable. A case of leiomyosarcoma arising in the uterus and initially presenting as a femoral lesion is presented. It required an intensive review of the case history and of the previous pathology in order to identify the primary site in the uterus. In the literature nine cases of metastatic leiomyosarcoma presenting initially in bone are reviewed. Ten additional cases from the literature showed bone metastases as the first site of recurrence. When this is assessed together with some 14 cases of primary leiomyosarcomas reported arising in bone, one is impressed that this is an uncommon tumour in bone either as a primary or secondary. In all patients in whom a leiomyosarcoma is histologically proven in bone, all primary sites should be specifically and categorically excluded before the lesion is accepted as an osseous primary. This should include special stains for myofibrils and electron microscopy if at all possible, in order to differentiate such tumours from other sarcomas such as fibrosarcoma. This applies especially to patients with uterine lesions that have been previously treated, even if they have been regarded as benign initially. All previous pathology and in particular hysterectomy specimens should be carefully reviewed. The need to specifically identify a leiomyosarcoma as a primary lies in the different modalities of treatment used: aggressive ablative surgery for primary lesions as opposed to radiation and chemotherapy for metastatic disease.

摘要

我们的病例表明,转移瘤在产生足以使病变在放射学上可检测到的骨质改变之前,可能早就存在并出现症状。本文介绍了一例起源于子宫、最初表现为股骨病变的平滑肌肉瘤病例。为了确定子宫内的原发部位,需要对病史和既往病理进行深入回顾。本文回顾了文献中9例最初表现为骨转移的平滑肌肉瘤病例。另外从文献中选取了10例以骨转移为首次复发部位的病例。将这些病例与报道的约14例原发于骨的平滑肌肉瘤病例一起评估时,人们会印象深刻地发现,无论是作为原发性还是继发性肿瘤,平滑肌肉瘤在骨中都不常见。在所有经组织学证实骨内存在平滑肌肉瘤的患者中,在将病变认定为骨原发性病变之前,都应特别明确地排除所有原发部位。如果可能的话,这应包括进行肌原纤维特殊染色和电子显微镜检查,以便将此类肿瘤与其他肉瘤如纤维肉瘤区分开来。这尤其适用于先前接受过治疗的子宫病变患者,即使这些病变最初被认为是良性的。所有既往病理,尤其是子宫切除标本都应仔细复查。明确将平滑肌肉瘤认定为原发性肿瘤的必要性在于所采用的不同治疗方式:原发性病变采用积极的切除手术,而转移性疾病则采用放疗和化疗。

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