Fibrocartilaginous mesenchymoma of bone (FCMB) is an extremely rare
tumor first described in 1984.[1][2] About 26 cases have been reported in literature, with patient ages spanning from 9 to 25 years, though a case in a male infant aged 1 year and 7 months has been reported.[3][4] Quick growth and bulky size are remarkable features of this tumor.
Diagnosis
The most common locations are the shaft and epyphises of long bones (
fibula and
humerus) but the
spine,
metatarsal bones, and
ilium have been involved as well. Radiologic examination evidences
osteolytic areas with a
lobulated framework comprising
radiolucent and
radiodense foci admixed to speckled
calcification. Cortical destruction is a common finding with no soft tissue expansion in many cases.
Histopathology of the lesion shows large areas of mature fibrous
stroma undergoing
hyaline cartilagemetaplasia resulting in conspicuous lobules or gradual transformation into chondroid foci. Both hyaline cartilage and chondroid in turn undergo calcification and endochondral
cancellous bone formation mimicking epiphyseal plate-like cartilage.[citation needed]
A full account of imaging findings on
radiography,
bone scan, CT and magnetic resonance has been provided by Sumner et al.[8]
Treatment
Surgery is curative despite possible local relapses. Wide resection of the tumor and resection
arthrodesis with an intramedullary nail,
vertebrectomy and
femoral headallograft replacement of the
vertebral body, resection of the iliac wing and
hip joint disarticulation have been among the performed procedures.[2][5][6]
The close resemblance of FCMB to fibrocartilaginous dysplasia has suggested to some scholars that they might be closely related entities, although the latter features woven bone
trabeculae without
osteoblastic rimming, which is a quite distinctive aspect. Instead, the occurrence of
epiphyseal plate-like cartilage is peculiar of the former.[citation needed]
^
abCozzutto C, Cornaglia-Ferraris P (March 1991). "Fibrocartilaginous mesenchymoma of bone". Pathology, Research and Practice. 187 (2–3): 279–83.
doi:
10.1016/S0344-0338(11)80784-3.
PMID2068011.
^Gambarotti M, Righi A, Vanel D, Cocchi S, Benini S, Elli FM, et al. (July 2017). "Fibrocartilaginous mesenchymoma of bone: a single-institution experience with molecular investigations and a review of the literature". Histopathology. 71 (1): 134–142.
doi:
10.1111/his.13201.
hdl:11577/3223054.
PMID28239886.
S2CID4787327.
^
abGedikoglu G, Aksoy MC, Ruacan S (August 2001). "Fibrocartilaginous mesenchymoma of the distal femur: case report and literature review". Pathology International. 51 (8): 638–42.
doi:
10.1046/j.1440-1827.2001.01247.x.
PMID11564220.
S2CID30961094.
^
Cherradi N, Jelthi A, Alhamany Z, Miri A, Forest M (1999). "[Fibrocartilaginous mesenchymoma of bone. A case report]". Clinical and Experimental Pathology. 47 (5): 249–55.
PMID10598375.
Lin J, Shulman SC, Steelman CK, Oskouei SV, Reith JD, Simoneaux SF, et al. (November 2011). "Fibrocartilaginous mesenchymoma, a unique osseous lesion: case report with review of the literature". Skeletal Radiology. 40 (11): 1495–9.
doi:
10.1007/s00256-011-1189-0.
PMID21560005.
S2CID2621119.
Martínez-Lage JF, Alarcón F, Hernández-Barceló JE, Almagro MJ, Alfaro R, Galera-Miñarro A (March 2010). "Fibrocartilaginous mesenchymoma of the spine in a child: a case report". Child's Nervous System. 26 (3): 385–9.
doi:
10.1007/s00381-009-1042-0.
PMID20183926.
S2CID9246389.
^Sumner TE, Ward WG, Kilpatrick SE, Opatowsky MJ (May 2000). "Fibrocartilaginous mesenchymoma of bone: case report and review of the literature". Pediatric Radiology. 30 (5): 315–7.
doi:
10.1007/s002470050747.
PMID10836593.
S2CID29836066.
References
Bhaduri A, Deshpande RB (December 1995). "Fibrocartilagenous mesenchymoma versus fibrocartilagenous dysplasia-: are these a single entity?". The American Journal of Surgical Pathology. 19 (12): 1447–8.
doi:
10.1097/00000478-199512000-00015.
PMID7503369.
S2CID44361999.
Ishida T, Dorfman HD (September 1993). "Massive chondroid differentiation in fibrous dysplasia of bone (fibrocartilaginous dysplasia)". The American Journal of Surgical Pathology. 17 (9): 924–30.
doi:
10.1097/00000478-199309000-00009.
PMID8352377.