Malignant fibrous histiocytoma secondary to fibrous dysplasia

Ohmori, Kazuo; Matsui, Hisao; Koike, Masahiko; Yudoh, Kazuo; Yasuda, Tomomi; Terahata, Shintarou

doi:10.1007/s002640050104

Cited by 10 publications

(3 citation statements)

References 5 publications

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“…Apart from case reports [e.g. [15][16][17], the above mentioned series 12,14 and a more recent publication by Hoshi 18 little is known about the malignant transformation of FD. A risk factor seems to be a history of prior radiation.…”

Section: Discussionmentioning

confidence: 99%

A Multifocal Malignant Fibrous Histiocytoma of the Bone Evolving From Fibrous Dysplasia

Bruck¹

2012

UHOD

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Fibrous Dysplasia (FD) is a focal bone lesion occurring sporadically and in hereditary syndromes caused by activating mutations of the G protein alpha subunit. It rarely undergoes malignant transformation. Malignant Fibrous Histiocytoma (MFH) is a sarcoma of soft tissues and may be found as primary sarcoma of the bone or multifocally. Until now only a few cases of malignant transformation of FD into MFH of the bone have been described. We report a case of a 38 year old female with polyostotic FD. Years after the first diagnosis of FD, pain with sudden onset and diminution of force in the left lower limb finally led to the diagnosis of a multifocal MFH of the bone. No response to different chemotherapy schemes was found and the patient deceased shortly after. This is the first described case of a polyostotic FD with malignant transformation into a multifocal MFH of bone. Due to the rarity of this entity and the lack of response to chemotherapy no treatment recommendations can be concluded. Keywords: Multifocal malignant fibrous histiocytoma of the bone, Fibrous dysplasia, Polyostotic ÖZET Fibröz Displaziden Geliflen Kemi¤in Multifokal Malign Fibröz HistiyositomuFibröz displazi (FD), G protein alfa altbiriminin aktivasyonu sonucu ortaya ç›kan sporadik görülen, genetik geçiflli fokal kemik lezyonudur. Malign transformasyonu çok nadirdir. Malign fibröz histiyositoma (MFH), multifokal veya primer kemikte de görülebilen yumuflak doku sarkomudur. FD'nin malign transformasyonla MFH'a dönüflmesiyle ilgili literatürde çok az say›da vaka bildirilmifltir. Bu raporumuzda polyostotik FD olan 38 yafl›nda kad›n hasta sunulmufltur. ‹lk tan›dan y›llar sonra a¤r›, ve alt ekstremitede kuvvet kayb›yla ortaya ç›kan tablo multifocal MFH tan›s›n›n habercisi olmufltur. Kemoterapiye cevaps›z olmas› nedeniyle tan›dan k›sa süre sonra hasta kaybedilmifltir. Bu vaka raporumuz, polyostotik FD'nin multifokal MFH'a malign transfromasyonunu sunmaktad›r. Bu durumun nadirli¤i ve kemoterapi yan›ts›zl›¤› nedeniyleriyle tedavi önerisi sunamamaktay›z.

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Section: Discussionmentioning

confidence: 99%

A Multifocal Malignant Fibrous Histiocytoma of the Bone Evolving From Fibrous Dysplasia

Bruck¹

2012

UHOD

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“…4 Most reported cases of malignant degeneration of FD have occurred after radiation therapy. 23,25,26 Osteosarcoma is the most common histologic type of malignant transformation, followed by fibrosarcoma, chondrosarcoma, and malignant fibrohistiocytoma. 23,27,28 These malignancies are most commonly found in the maxilla and mandible, with rare involvement of the calvarium.…”

Section: Osteomamentioning

confidence: 99%

“…23,27,28 These malignancies are most commonly found in the maxilla and mandible, with rare involvement of the calvarium. 23,25,26 Diagnosis can be accomplished via radiographic appearance, although some investigators recommend biopsy of FD lesions. 5,6 On CT scan, the FD lesions are characterized by hazy borders and a fairly homogeneous "ground glass" appearance representing the disorganized spicules of bone that characterize FD"s histology.…”

Section: Osteomamentioning

confidence: 99%

Fibro-osseous lesions of the paranasal sinuses and the skull base

Alturaiki

Almomen

Azzeh

et al. 2018

Int J Otorhinolaryngol Head Neck Surg

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Background: Osteomas, ossifying fibromas, and fibrous dysplasia comprise a set of benign neoplasms known as fibro-osseous lesions (FO), which can arise in the paranasal sinuses. The vast majority of benign FO lesions (BFOL) are incidental findings on radiographs. They tend to be slow-growing tumors, and are infrequently symptomatic. The management strategy can vary significantly for theses lesions. This can range from serial, observations to aggressive surgical resection, based on symptomatology, size, and location of the lesion.Methods: Retrospective analysis of all the data of different symptomatic FO of the paranasal sinuses and the skull base was performed at King Fahad specialist hospital, Dammam, KSA (2006 to 2017). Results: A total of 21 patients were identified; 10 (46.7%) patients were diagnosed with osteoma, 8 (38.1%) with ﬁbrous dysplasia, 2 (9.5%) with ossifying fibroma and one (4.8%) with juvenile active ossifying (JAOF), 17 were adults and 4 were children, the range of f/u (3-60) months. 19 patients were managed by the endonasal endoscopic approach. They continue to be followed with no evidence of recurrence. Two cases required the revision combined endoscopic open approach to ensure adequate removal of the disease (residual and recurrence).Conclusions: Diagnostic dilemma of BFOL can be overcome with a combination of clinical, radiological and pathological criteria. Correct diagnosis of fibro-osseous tumors is crucial for adequate therapy as their treatment, prognosis, clinical aggressiveness and long term complications of individual entities vary significantly. Endoscopic computer-assisted surgery is the treatment of choice. Endoscopic resection remains a technical challenge.

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Malignant Fibrous Histiocytoma of the Liver: A Neoplasm of the Undifferentiated High-Grade Pleomorphic Sarcoma Group

Zimmermann¹

2016

Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

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Malignant fibrous histiocytoma secondary to fibrous dysplasia

Cited by 10 publications

References 5 publications

A Multifocal Malignant Fibrous Histiocytoma of the Bone Evolving From Fibrous Dysplasia

A Multifocal Malignant Fibrous Histiocytoma of the Bone Evolving From Fibrous Dysplasia

Fibro-osseous lesions of the paranasal sinuses and the skull base

Malignant Fibrous Histiocytoma of the Liver: A Neoplasm of the Undifferentiated High-Grade Pleomorphic Sarcoma Group

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