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多灶性骨肉瘤:多原发性肿瘤还是转移瘤?1例罕见病例报告及文献复习

Multifocal Osteosarcoma: Multiple Primaries or Metastases? A Report of Rare Case and Review of Literature.

作者信息

Agrawal Mohit, Patil Akkamahadevi, James Tina, Kumar Nuthan, Premalata C S

机构信息

Department of Pathology, Kidwai Memorial Institute of Oncology, Dr. M. H. Marigowda Road, Bengaluru, Karnataka. India.

Department of Pediatric Oncology, Kidwai Memorial Institute of Oncology, Dr. M. H. Marigowda Road, Bengaluru, Karnataka. India.

出版信息

J Orthop Case Rep. 2020 Nov;10(8):97-100. doi: 10.13107/jocr.2020.v10.i08.1878.

Abstract

INTRODUCTION

Multifocal osteosarcoma (MFOS) is characterized by multicentricity of osseous osteosarcomas, either synchronous or metachronous, without visceral involvement. They account for about 1.5% of all osteosarcomas. Most synchronous MFOS has one dominant lesion with one to four and very rarely five or more secondary lesions. The distal femur followed by the proximal tibia is the most common site of dominant lesions. Its prognosis remains extremely poor even with combined chemotherapy and surgery.

CASE REPORT

We describe a rare case of MFOS in a 10-year-old boy who presented with a short history of severe aching pain in the right lower limb following a trivial fall. Initial workup and relevant investigations revealed a synchronous multicentric osteosarcoma with extensive involvement of appendicular and axial skeletal system. The dominant lesion was at the lower end of the right femur with multiple secondary lesions in the right tibia, left femur, bilateral humeri, pelvis, cervical and dorsolumbar spine, ribs, and sternum. The patient received one cycle of doxorubicin and cisplatin-based chemotherapy but unfortunately succumbed to progressive disease, a month after initiation of chemotherapy.

CONCLUSION

MFOS is a very rare presentation of osseous osteosarcoma. The non-specific clinical manifestation, despite the presence of generalized skeletal involvement, presents a diagnostic difficulty for both the clinician and the radiologist. Only biopsy and histopathological examination can confirm the diagnosis of this highly malignant disease and help in proper management.

摘要

引言

多灶性骨肉瘤(MFOS)的特征是骨骨肉瘤多中心发生,可为同时性或异时性,不累及内脏。它们约占所有骨肉瘤的1.5%。大多数同时性MFOS有一个主要病灶,伴有1至4个继发性病灶,很少有5个或更多继发性病灶。最常见的主要病灶部位是股骨远端,其次是胫骨近端。即使采用联合化疗和手术,其预后仍然极差。

病例报告

我们描述了一例罕见的10岁男孩MFOS病例,该男孩在轻微跌倒后出现右下肢严重疼痛的病史较短。初步检查和相关调查显示为同时性多中心骨肉瘤,累及四肢和轴向骨骼系统。主要病灶位于右股骨下端,右胫骨、左股骨、双侧肱骨、骨盆、颈椎和胸腰椎、肋骨及胸骨有多个继发性病灶。患者接受了一个周期的以阿霉素和顺铂为基础的化疗,但不幸的是,在化疗开始后一个月因疾病进展而死亡。

结论

MFOS是骨骨肉瘤非常罕见的表现形式。尽管存在全身骨骼受累,但非特异性临床表现给临床医生和放射科医生的诊断带来了困难。只有活检和组织病理学检查才能确诊这种高度恶性疾病,并有助于进行适当的治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4765/7933644/917bdef08bf9/JOCR-10-97-g001.jpg

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