Fibrocartilaginous Dysplasia of the Proximal Part of the Femur with a Pathological Fracture in a Child

Henderson, Eric; Monforte, Hector; Neustadt, Jeffrey B.

doi:10.2106/jbjs.cc.m.00254

Cited by 3 publications

(6 citation statements)

References 25 publications

(59 reference statements)

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“…The polyostotic variant usually is associated with McCune-Albright syndrome. 8,9 Our case was monostotic and did not have syndromic association.…”

Section: Discussionmentioning

confidence: 59%

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Fibrocartilagenous dysplasia in distal femur: A rare histopathological finding

Patkar¹,

Neelakantan²,

Mishra³

2021

ACHR

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Fibrocartilaginous dysplasia (FCD) or massive cartilaginous differentiation in fibrous dysplasia are interchangeably used terms. It is a rare variant of fibrous dysplasia (FD) which is benign, lytic, and expansile bone lesion and causes progressive deformity in the bones and may lead to pathological fracture. Radiologically, FCD may confused with cartilaginous benign and malignant bone tumours. FCD usually shows calcification in imaging. Surgical curettage or corrective osteotomy and histopathological examination of these lesions is necessary to differentiate it from other cartilaginous tumours. Here we report case of fibrocartilaginous dysplasia of distal femur in a 4-year-old male child.

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“…The polyostotic variant usually is associated with McCune-Albright syndrome. 8,9 Our case was monostotic and did not have syndromic association.…”

Section: Discussionmentioning

confidence: 59%

“…3 A missense mutation in the guanine nucleotide binding protein/α subunit 1 gene on chromosome 20 causes FCD. 8,9 It is a genetic noninherited condition. The polyostotic variant usually is associated with McCune-Albright syndrome.…”

Section: Discussionmentioning

confidence: 99%

Fibrocartilagenous dysplasia in distal femur: A rare histopathological finding

Patkar¹,

Neelakantan²,

Mishra³

2021

ACHR

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“…Another method in differential diagnosis is to show GNAS1 gene mutation localized on chromosome 20. It was reported that the GNAS1 mutation is found in 50-70% of FD cases, playing a role in pathogenesis of FD [2], which supports the diagnosis of FCD [12]. In fibroosseous lesions of the head and neck region, although there are few reported cases, GNAS1 mutations in FCM have not been demonstrated [18,19].…”

Section: Discussionmentioning

confidence: 95%

“…Eighteen cases were monostotic, 8 were polyostotic, with the most common location of the lesions in the proximal femur (80%). The most frequent symptoms are pain, swelling, deformity, pathological fracture, and limping, all of which have been reported [3][4][5][6][7][8][9][10][11][12][13][14][15]. In cases with clinical and follow-up data, symptom duration ranged from 8 weeks to 18 years (mean 62.2 months), with no recurrence or malignant transformation observed in mean follow-up of 21.71 (2-60) months after treatment.…”

Section: Discussionmentioning

confidence: 99%

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Fibrocartilaginous dysplasia (fibrous dysplasia and massive cartilaginous differentiation): Case report and literature review

Yılmaz¹,

Canaz²,

Yılmaz³

et al. 2022

J Surg Med

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Fibrocartilaginous dysplasia (fibrous dysplasia and massive cartilaginous differentiation or fibrochondroplasia, FCD) is a rare variant of fibrous dysplasia and a term used for cases of fibrous dysplasia with prominent cartilage tissue. A limited number of FCD cases have been reported in the literature, which can be seen in both clinical forms. A 16-year-old male patient, who had been followed for ten years with a diagnosis of polyostotic fibrous dysplasia in the left hip and cranium, presented with pain in the left leg after a fall. A subtrochanteric pathological fracture in the left femur was detected on exam, the lesion area was curetted, and osteotomy and fixation were applied. Microscopic assessment revealed a fibro-osseous lesion of benign spindle cell fibrous connective tissue with woven bone trabeculae, without osteoblastic rim or large areas of benign cartilage nodules. The final diagnosis was fibrocartilaginous dysplasia. In our literature review, 26 cases of FCD were reported so far. Age distribution of patients ranged from 4 to 53 years (mean 15.9) and the male / female ratio was 15/11 = 1.36. Eighteen cases were monostotic, and 8 were polyostotic. In cases with noted clinical and follow-up data, symptom duration ranged from 8 weeks to 18 years (mean 62.2 months), with no recurrence or malignant transformation in a mean follow-up of 21.71 (2-60) months post-treatment. In the cartilage component, there was increased cellularity, some nuclear atypia, binucleation, and myxoid degeneration. This situation simulates benign and malignant entities such as enchondroma, fibrocartilaginous mesenchymoma, well-differentiated intramedullary osteosarcoma, and chondrosarcoma with a differential diagnosis. FCD is a benign and very rare lesion with a prominent chondroid component, but may cause difficulty with differential diagnosis. Awareness of the histopathological and radiological features of FCD cases, their age range, and involvement areas provides an approach to distinguish them from lesions that may be confusing in a differential diagnosis.

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Fibrocartilaginous Dysplasia – A Report of Five Cases with Review of Literature

Lakshmanan¹,

Parameswaran²

2022

JOCR

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Introduction:Fibrocartilaginous dysplasia (FCD) is a variant of fibrous dysplasia (FD) with extensive cartilaginous differentiation. This has been reported in both monostotic and polyostotic types of FD, the proximal femur being the most common site involved. Case Report:We report five cases of fibrocartilaginous dysplasia with varying degrees of cartilaginous differentiation. The age of the patients ranged from 7 to 30 years, and there was a female predominance (M:F ratio of 1:4). The proximal femur was the site of involvement in all the cases. Imaging showed well demarcated radiolucent lesions with stippled calcifications. Histologically, cartilaginous areas were noted juxtaposed to typical areas of fibrous dysplasia. Four of the patients were treated with curettage and one with a marginal resection. None of the five cases had recurrences at the past follow-up. Conclusion:FCD is a rare variant of fibrous dysplasia which needs to diagnosed and treated early, as there is a high risk of pathological fracture. Keywords:Fibrous dysplasia, fibrocartilaginous, cartilaginous tumors, enchondroma, chondrosarcoma.

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Fibrocartilaginous Dysplasia of the Proximal Part of the Femur with a Pathological Fracture in a Child

Cited by 3 publications

References 25 publications

Fibrocartilagenous dysplasia in distal femur: A rare histopathological finding

Fibrocartilagenous dysplasia in distal femur: A rare histopathological finding

Fibrocartilaginous dysplasia (fibrous dysplasia and massive cartilaginous differentiation): Case report and literature review

Fibrocartilaginous Dysplasia – A Report of Five Cases with Review of Literature

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