Fibrocartilaginous Mesenchymoma Research Articles

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

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.

Fibrocartilaginous mesenchymoma with an unusual location in the rib.

Fibrocartilaginous mesenchymoma is a rare bone tumor, with fewer than 35 cases reported in the literature since 1984. This tumor usually occurs in the long bones of children and adolescents. In the current case, the tumor affected a rib. A 17-year-old boy presented with a mass in the right fifth rib. Radiologic findings revealed an osteolytic mass with cortical destruction and calcification; en bloc resection was performed. The tumor showed three distinct histologic features: bland spindle cell proliferation, benign cartilage nodules, and epiphyseal plate-like enchondral ossification. The pathologic diagnosis was fibrocartilaginous mesenchymoma. The patient remains free of disease 1 year after the surgery. Pathological diagnosis of fibrocartilaginous mesenchymoma can be challenging, especially when the tumor occurs in an unusual site. When any fibro-osseous lesion with a cartilaginous component is encountered, the possibility of fibrocartilaginous mesenchymoma should be considered because of its locally aggressive behavior.

Fibrocartilaginous mesenchymoma of bone: a single-institution experience with molecular investigations and a review of the literature.

Fibrocartilaginous mesenchymoma is a rare intraosseous lesion, with a total of 26 cases described in the literature. This study describes the clinical, radiological and histological features of eight new cases of fibrocartilaginous mesenchymoma collected at a single institution between 1982 and 2016. The presence of GNAS and IDH1/2 mutations and MDM2 amplification was explored to evaluate possible links between fibrocartilaginous mesenchymoma, fibrous dysplasia, de-differentiated chondrosarcoma and low-grade osteosarcoma. Eight new cases of fibrocartilaginous mesenchymoma of bone identified in our archives, dating from 1982 to 2016, were reviewed. The diagnosis was not performed on the initial biopsy in any of these cases, due mainly to the absence of obvious cartilaginous differentiation. On imaging, the tumour contained cartilaginous calcifications and showed a very strong uptake of contrast medium after injection. Histologically, the tumour was characterized by spindle cell proliferation mimicking a low-grade spindle cell sarcoma, associated with epiphyseal growth-plate-like nodules of cartilage and bone production. Molecularly, no GNAS and IDH1/2 mutations or MDM2 amplification were found in the cases analysed. Only one case recurred 1 year following intralesional resection. None died of disease. This very rare bone tumour has a typical radiological and histological pattern and a favourable survival outcome after treatment. Local recurrences can be prevented with complete surgery. Fibrocartilaginous mesenchymoma does not seem to be related genetically to fibrous dysplasia, low-grade osteosarcoma and de-differentiated chondrosarcoma.

Fibrocartilaginous mesenchymoma arising in the pubic bone: A case report

Fibrocartilaginous mesenchymoma (FCM) of the bone is a rare tumor, with only 21 reported cases since 1984. It usually occurs in the long bones of children and adolescents, but in this case, the tumor arose in the pubic bone. The pathological diagnosis of FCM can be challenging, and the treatment should be a wide resection because of its locally aggressive behavior. Histologically, our tumor was composed of a lobular proliferation of spindle cells juxtaposed to the cartilaginous tissue, lacking cytological atypia. Enchondral ossification was observed at the periphery of the cartilaginous nodules, and hypertrophic chondrocytes was recognized, reminiscent of an epiphyseal plate. Intralesional resection followed by phenol and ethanol cauterization was performed in place of the usual wide resection. We report a case of FCM arising in a rare anatomical site, the pubic bone, successfully treated by intralesional resection. One year after the surgery, the patient is free of disease.

Congenital mesenchymoma of tibia: Case report and review of literature

Fibrocartilaginous mesenchymoma is a rare bone tumor arising from long bones in children and adolescents. With only 21 cases reported in the literature, it is often not included in the differential diagnosis in patients with a bone tumor. Previously, the youngest case reported was in a 19-month-old boy, and a congenital variant had not been reported.We describe a case of congenital fibrocartilaginous mesenchymoma of the proximal tibia that presented shortly after birth. Biopsy of a suspected malignancy on imaging confirmed the diagnosis. The child was temporarily lost to follow up and presented again with genu varum and limb shortening but surprisingly, the tumor had completely resolved without surgical intervention.

Fibrocartilaginous mesenchymoma, a unique osseous lesion: case report with review of the literature

Fibrocartilaginous mesenchymoma is a rare osseous tumor that primarily arises in the long bones of children and adolescents. This lesion can grow quickly and reach a considerable size, despite its benign nature. It has proved challenging to diagnose and can be mistaken for a spectrum of benign and malignant bone tumors. The histological presentation of unique epiphyseal plate-like cartilage with destruction of the surrounding cortical bone and exhibition of dense fibrous stroma are important indicators for the diagnosis of fibrocartilaginous mesenchymoma. An 11-year-old boy presented with a left proximal humerus mass thought to be an aneurysmal bone cyst. The patient was lost to follow-up and came back 3 years later with massive growth of the lesion. Owing to the aggressive nature of the tumor, a left forequarter amputation was performed. Histological examination demonstrated numerous islands of cartilage with an exuberant spindle cell component characteristic of FCM. No distant metastases or local recurrences were identified at 2 years post-amputation. Because of the rapid growth of this lesion, it should be considered in the differential diagnosis of bone lesions in children and young adults.

Fibrocartilaginous mesenchymoma of the spine in a child: a case report

Originally described by Dahlin et al. in 1984, fibrocartilaginous mesenchymoma (FCM) constitutes a rare bone tumor of children and adolescents that mainly affects the long bones. A spinal location of this tumor is exceptional. Only two previous instances of vertebral FCM have been reported in the current literature, and both occurred in young adults. We report the case of a 9-year-old boy with backache caused by a neoplasm that involved the Th12 vertebra treated with tumor excision. Histopathological diagnosis was confirmatory of FCM. During a follow-up period of 2 years, there was no evidence of disease progression. Our patient, thus, represents the first case of FCM occurring in a child. Given the rarity of spinal FCM, there are no guidelines about its management. However, treatment of this neoplasm seems to be mainly surgical aiming at total removal of the lesion, if feasible, as FCM may recur locally.

Fibrocartilaginous Mesenchymoma of Bone. Whatʼs in a Name? Sometimes, a Lot

On: Fibrocartilagenous Mesenchymoma of Bone. Bulychova IV, Unni KK, Bertoni F, Beabout JW

Fibrocartilagenous Mesenchymoma of Bone

We studied 12 cases of a rare primary bone neoplasm--fibrocartilaginous mesenchymoma--including five from the original report on this condition. The seven male and five female patients were 9 to 25 years old. The metaphyses of the long bones were the most common site, with the fibula accounting for a third of all cases. Histologically, the lesion contained spindle cells, bone trabeculae, and islands of cartilage. At least some of the cartilage was in the form of plates that resembled epiphyseal plates. Intralesional excision led to a high rate of recurrence, but no metastasis or death was related to the tumor. Our results indicate that the entity described by Dahlin and co-authors is histologically distinct and that its behavior does not merit the term malignant.

Fibrocartilaginous Mesenchymoma of Bone

Two bone tumors in children were characterized by prevalent low-cellular fibrous stroma of mature appearance and lobules of hyaline cartilage or chondroid tissue. Endochondral ossification was an additional finding. Clinically the two cases presented with osteolytic lesions of considerable size and relapsing course thus enhancing the suspicion of malignancy. The pathological diagnosis was controversial but both patients are disease-free several years after diagnosis. Histopathologically, the process duplicated the consecutive steps of embryonal endochondral bone formation. In a review of the pertinent literature it was found that these two cases reflect to a large extent the bone lesion reported in 1984 by Dahlin et al. and designated fibrocartilaginous mesenchymoma of the bone.