Medicine:Fibrocartilaginous mesenchymoma of bone
| Fibrocartilaginous mesenchymoma of bone |
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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 cartilage metaplasia 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]
Differential diagnosis is concerned with fibrocartilaginous dysplasia of bone, desmoplastic fibroma, low-grade fibrosarcoma, chondromyxoid fibroma and low-grade chondrosarcoma.[5][6][7]
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 head allograft 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]
Notes
- ↑ "Fibrocartilaginous mesenchymoma with low-grade malignancy". Skeletal Radiology 12 (4): 263–9. 1984. doi:10.1007/bf00349507. PMID 6505732.
- ↑ 2.0 2.1 "Fibrocartilaginous mesenchymoma of bone". Pathology, Research and Practice 187 (2–3): 279–83. March 1991. doi:10.1016/S0344-0338(11)80784-3. PMID 2068011.
- ↑ "Fibrocartilaginous mesenchymoma of bone: a single-institution experience with molecular investigations and a review of the literature". Histopathology 71 (1): 134–142. July 2017. doi:10.1111/his.13201. PMID 28239886.
- ↑ "Fibrocartilagenous mesenchymoma of bone: the youngest reported case in a patient aged 1 year and 7 months". Journal of Clinical Pathology 58 (7): 782–3. July 2005. PMID 15976352.
- ↑ 5.0 5.1 "Fibrocartilaginous mesenchymoma of the distal femur: case report and literature review". Pathology International 51 (8): 638–42. August 2001. doi:10.1046/j.1440-1827.2001.01247.x. PMID 11564220.
- ↑ 6.0 6.1 "Fibrocartilaginous mesenchymoma of the fifth lumbar vertebra treated by vertebrectomy". Spine 19 (17): 1992–7. September 1994. doi:10.1097/00007632-199409000-00022. PMID 7997935.
- ↑
"[Fibrocartilaginous mesenchymoma of bone. A case report]". Clinical and Experimental Pathology 47 (5): 249–55. 1999. PMID 10598375.
- "Fibrocartilaginous mesenchymoma, a unique osseous lesion: case report with review of the literature". Skeletal Radiology 40 (11): 1495–9. November 2011. doi:10.1007/s00256-011-1189-0. PMID 21560005.
- "Fibrocartilaginous mesenchymoma of the spine in a child: a case report". Child's Nervous System 26 (3): 385–9. March 2010. doi:10.1007/s00381-009-1042-0. PMID 20183926.
- Sangüesa, M. J.; Laguarta, J. García; Valverde, C. (2007). "Mesenquimoma fibrocartilaginoso de hueso. Caso clínico" (in es). Revista Española de Cirugía Osteoarticular 42 (229): 30–33. http://www.cirugia-osteoarticular.org/adaptingsystem/intercambio/revistas/articulos/80_30.pdf.
- ↑ "Fibrocartilaginous mesenchymoma of bone: case report and review of the literature". Pediatric Radiology 30 (5): 315–7. May 2000. doi:10.1007/s002470050747. PMID 10836593.
References
- "Fibrocartilagenous mesenchymoma versus fibrocartilagenous dysplasia-: are these a single entity?". The American Journal of Surgical Pathology 19 (12): 1447–8. December 1995. doi:10.1097/00000478-199512000-00015. PMID 7503369.
- "Massive chondroid differentiation in fibrous dysplasia of bone (fibrocartilaginous dysplasia)". The American Journal of Surgical Pathology 17 (9): 924–30. September 1993. doi:10.1097/00000478-199309000-00009. PMID 8352377.
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