Literature DB >> 9275327

Spontaneous malignant transformation of fibrous dysplasia.

D Xu1, H Luan, A Zhan, W Feng, X Sun, F Meng.   

Abstract

OBJECTIVE: To evaluate the clinical and radiological findings in diagnosing spontaneous malignant transformation of fibrous dysplasia.
METHODS: Fifteen cases of sarcomatous transformation proved by operation and pathological examinations were found in a group of 356 patients with fibrous dysplasia, and their radiological manifestations were retrospectively studied. The 15 cases included 8 osteosarcomas, 5 fibrosarcomas and 2 chondrosarcomas. All the 15 patients were known to have long-standing fibrous dysplasia, but no radiation therapy was ever received. Eleven patients had polyostotic fibrous dysplasia and 4 had monostotic type.
RESULTS: Malignant transformation most frequently occurs in the cystic expansive lesion of the long tubular bone. Pains, swelling and late appearance of a bony mass are the main clinical manifestations. The early radiological features of sarcomatous transformation in fibrous dysplasia are moth-eaten or cystic areas of osteolysis located in the involved bone. The cortical destruction and gradual formation of a soft tissue mass that contains tumor-bone are highly suspicious of osteosarcomatous transformation, while ring-like and spotty calcification in the tumor matrix is indicative of chondrosarcoma. Fibrosarcoma usually shows simple osteolytic destruction.
CONCLUSIONS: According to the clinical radiological findings, patients of sarcomatous transformation can be detected in the early stage. These radiological findings may be used as a clue for differentiating various kinds of sarcomatous transformation.

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Year:  1996        PMID: 9275327

Source DB:  PubMed          Journal:  Chin Med J (Engl)        ISSN: 0366-6999            Impact factor:   2.628


  1 in total

1.  Pseudomyogenic hemangioendothelioma secondary to fibrous dysplasia of the left lower extremity in a 14-year-old female: a case report.

Authors:  Conglin Ye; Xiaolong Yu; Jin Zeng; Hucheng Liu; Min Dai
Journal:  World J Surg Oncol       Date:  2016-07-28       Impact factor: 2.754

  1 in total

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