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Year : 2017  |  Volume : 10  |  Issue : 5  |  Page : 502-503  

Polyostotic fibrocartilaginous dysplasia


1 Department of Orthopaedics, Armed Forces Medical College, Pune, Maharashtra, India
2 Department of Pathology, Armed Forces Medical College, Pune, Maharashtra, India

Date of Web Publication14-Nov-2017

Correspondence Address:
Manoj Gopal Madakshira
Department of Pathology, Armed Forces Medical College, Pune - 411 040, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/MJDRDYPU.MJDRDYPU_301_16

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How to cite this article:
Manibanakar NB, Madakshira MG. Polyostotic fibrocartilaginous dysplasia. Med J DY Patil Univ 2017;10:502-3

How to cite this URL:
Manibanakar NB, Madakshira MG. Polyostotic fibrocartilaginous dysplasia. Med J DY Patil Univ [serial online] 2017 [cited 2019 Aug 25];10:502-3. Available from: http://www.mjdrdypu.org/text.asp?2017/10/5/502/218190



Sir,

Fibrous dysplasia is known to have microscopic islands of cartilage along with the typical “Chinese-letter” pattern of bony trabeculae. These trabecular are devoid of rimming by osteoblasts with the intervening spindle cell rich stroma having a storiform or whorled disposition. The presence of exuberant cartilaginous differentiation in place of the spindle cell rich stroma is however extremely rare and is called as fibrocartilaginous dysplasia (FCD).[1] We discuss a peculiar case who presented with a deformed left arm.

A 25-year-old male presented to the orthopedic outpatient department of a tertiary care hospital with gradually increasing hard painful swellings over the left humerus, thumb, and index finger since 15 years [Figure 1]a. However, there was no restriction in the range of movements at the adjacent joints. Radiology revealed well-defined, expansile, ground glass osteolytic lesions occupying the upper two-thirds of the left humerus, distal, and middle phalanx of index finger and distal phalanx of thumb [Figure 1]b and [Figure 1]c. Provisional diagnosis considered included fibrous dysplasia, anuersymal bone cyst, and enchondromatosis. Multiple trucut biopsies were taken from the lesions. Histopathology revealed tissue cores composed of well-demarcated lobules of abundant cartilage surrounded by curvilinear fibro-osseous tissue. The cartilaginous tissue was moderately cellular with the chondrocytes displaying bland chromatin pattern [Figure 1]d. Based on radiology and histopathological features, a diagnosis of polyostotic FCD was offered. In view of the preserved restriction of movements and extensive involvement of the humerous which mandated major reconstruction of the bony defect, the patient made an informed decision to opt for conservative management.
Figure 1: ( a) Deformed left upper limb with conspicuous swelling in the proximal part of the arm. (b) X-ray showing an expansile osteolytic lesion in the proximal part of the left humerus. (c) X-ray of the left hand showing similar lesions in the distal and middle phalanx of index finger and distal phalanx of thumb. (d) H and E stained section (×400) showing curvilinear bony tissue enclosing abundant chondroid material

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FCD is considered as a variant of fibrous dysplasia.[2] This entity is characterized by the presence of extensive cartilaginous differentiation.[1],[2] The possible origins of the cartilage are thought to be from cartilage rests near the growth plate of long bones.[3] The etiopathogenesis is similar to that of fibrous dysplasia, in which there is an arrest in the bone maturation.[1] This results in abnormal disposition of the bone matrix and formation of these dysplastic bony lesions. The genetic basis is said to be due to mutation in the α-subunit of G-protein which results in an increased cyclic adenosine monophosphate levels (cAMP). This increase in second messenger (cAMP) leads to induction of target genes such as c-jun, interleukin (IL)-6, IL-11, and c-fos.[4] Both the sexes are equally affected with a wide range of age at presentation (4–26 years).[5] Long bones of the lower limb are frequently affected with the presenting symptoms being pain, deformity, and pathological fracture.[1],[2] These lesions usually are monostotic, but polyostotic forms are also known, as seen in our case.[1],[5] Radiology shows a well-demarcated expansile ground glass radiolucent medullary lesion.[3] However, histopathology is needed to establish the diagnosis and differentiate it from the entities such as enchondroma and the more sinister chondrosarcoma. Treatment remains curettage, correction of deformity, and repair of fractures. Reconstruction may be required for extensive lesions.[1]

The importance in identifying FCD is in recognizing the benign nature of the cartilaginous differentiation of fibrous dysplasia and avoiding its misinterpretation as a chondroid neoplasm.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Bokhari A, Benevenia J, Heller DS, Hameed MR. A 10-year-old boy with down syndrome and right hip and lower back pain after a falling episode. Arch Pathol Lab Med 2005;129:1185-6.  Back to cited text no. 1
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2.
Vargas-Gonzalez R, Sanchez-Sosa S. Fibrocartilaginous dysplasia (fibrous dysplasia with extensive cartilaginous differentiation). Pathol Oncol Res 2006;12:111-4.  Back to cited text no. 2
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3.
Drolshagen LF, Reynolds WA, Marcus NW. Fibrocartilaginous dysplasia of bone. Radiology 1985;156:32.  Back to cited text no. 3
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4.
Marie PJ. Cellular and molecular basis of fibrous dysplasia. Histol Histopathol 2001;16:981-8.  Back to cited text no. 4
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5.
Ishida T, Dorfman HD. Massive chondroid differentiation in fibrous dysplasia of bone (fibrocartilaginous dysplasia). Am J Surg Pathol 1993;17:924-30.  Back to cited text no. 5
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