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Histopathology Jul 2017Fibrocartilaginous mesenchymoma is a rare intraosseous lesion, with a total of 26 cases described in the literature. This study describes the clinical, radiological and... (Review)
Review
AIMS
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.
METHODS AND RESULTS
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.
CONCLUSIONS
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.
Topics: Adolescent; Adult; Bone Neoplasms; Child; Child, Preschool; Female; Humans; Infant; Male; Mesenchymoma; Young Adult
PubMed: 28239886
DOI: 10.1111/his.13201 -
ANZ Journal of Surgery Oct 2023
Topics: Humans; Mesenchymoma; Adrenal Glands; Adrenal Gland Neoplasms
PubMed: 37249155
DOI: 10.1111/ans.18514 -
Skeletal Radiology Apr 2023Fibrocartilaginous mesenchymoma (FM) is a rare bone tumor mimicking other fibrocartilaginous lesions on imaging and histologically. Hence, it is difficult to diagnose...
Fibrocartilaginous mesenchymoma (FM) is a rare bone tumor mimicking other fibrocartilaginous lesions on imaging and histologically. Hence, it is difficult to diagnose this entity especially on small biopsies. In this article, we report a case of FM mimicking desmoplastic fibroma on biopsy. A 36-year-old male presented with pain in the left hip. Imaging showed a large expansile lytic lesion involving the acetabulum and pubis. The differential diagnosis was suggestive of giant cell tumor, aneurysmal bone cyst, intraosseous desmoplastic fibroma, and chondrosarcoma. Biopsy revealed a low-grade spindle cell lesion with no evidence of osteoid or chondroid matrix. The lack of cartilaginous nodules in the biopsy prompted a preoperative diagnosis of desmoplastic fibroma. The excised mass showed bland spindle cell proliferation, benign cartilage nodules, and epiphyseal plate-like enchondral ossification suggestive of fibrocartilaginous mesenchymoma. Negative immunostaining for SATB2, CDK4, and MDM2 ruled out low-grade central osteosarcoma. Though GNAS mutations were not performed in this case, rimming of the bony trabeculae at the periphery of the epiphyseal growth plate-like cartilaginous nodule ruled out fibrous dysplasia. The absence of cartilaginous component misleads the diagnosis preoperatively in small biopsies.
Topics: Male; Humans; Adult; Mesenchymoma; Fibroma, Desmoplastic; Bone Neoplasms; Bone and Bones; Pelvis
PubMed: 36042034
DOI: 10.1007/s00256-022-04167-6 -
Zhonghua Bing Li Xue Za Zhi = Chinese... Jan 2021
Topics: Bone Neoplasms; Humans; Mesenchymoma
PubMed: 33396990
DOI: 10.3760/cma.j.cn112151-20200522-00403 -
Cancer Feb 1996Malignant mesenchymomas are rare soft tissue tumors that contain two or more distinct histologic subtypes of sarcoma within the same tumor (exclusive of a...
BACKGROUND
Malignant mesenchymomas are rare soft tissue tumors that contain two or more distinct histologic subtypes of sarcoma within the same tumor (exclusive of a fibrosarcomatous or hemangiopericytomatous component). They are generally considered high grade neoplasms and are associated with a poor prognosis, although experience with these tumors is limited.
METHODS
We report 8 patients seen at our center over the last 22 years and describe the clinical course of a patient with a malignant mesenchymoma arising in the retroperitoneum whose experience typifies the aggressive behavior of this tumor.
RESULTS
All eight patients had large, high grade tumors located in the retroperitoneum or thigh. Six of the 8 died of disease and 2 were alive with disease at a median of 30 months from diagnosis.
CONCLUSIONS
Malignant mesenchymoma represents a particularly aggressive form of soft tissue sarcoma. Our experience with this disease highlights the need for more effective treatment strategies for these patients.
Topics: Adult; Aged; Female; Humans; Male; Mesenchymoma; Middle Aged; Retroperitoneal Neoplasms; Soft Tissue Neoplasms; Thigh
PubMed: 8630953
DOI: 10.1002/(SICI)1097-0142(19960201)77:3<467::AID-CNCR7>3.0.CO;2-H -
Journal of Pediatric Surgery Dec 2012Fibrocartilaginous 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... (Review)
Review
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.
Topics: Biopsy, Needle; Bone Neoplasms; Female; Follow-Up Studies; Humans; Immunohistochemistry; Infant; Magnetic Resonance Imaging; Mesenchymoma; Risk Assessment; Tibia; Tomography, X-Ray Computed; Treatment Refusal
PubMed: 23217910
DOI: 10.1016/j.jpedsurg.2012.08.039 -
The Annals of Thoracic Surgery Aug 1991Benign chest wall mesenchymoma in children is an extremely rare disease. Only 20 patients have been reported in the world literature. We report a chest wall mesenchymoma... (Review)
Review
Benign chest wall mesenchymoma in children is an extremely rare disease. Only 20 patients have been reported in the world literature. We report a chest wall mesenchymoma in a 2-year-old boy who was admitted to the hospital after a routine chest roentgenogram showed a mass in the right upper chest wall. The patient was asymptomatic. Clinical examination was negative, but chest roentgenograms and computed tomography showed a mass in the right upper chest wall involving the third rib. A 2 x 2 x 1.5-cm tumor was excised totally with partial resection of the third rib. The histology of the lesion corresponded to a mesenchymoma (hamartoma) of the chest wall. Our patient has been followed up for 8 years without recurrence.
Topics: Bone Neoplasms; Child, Preschool; Humans; Male; Mesenchymoma; Ribs
PubMed: 1863152
DOI: 10.1016/0003-4975(91)91355-y -
The British Journal of Oral &... Aug 2004The purpose of this paper is to present a case report of a benign mesenchymoma involving the lip. A brief review of relevant literature is presented. (Review)
Review
The purpose of this paper is to present a case report of a benign mesenchymoma involving the lip. A brief review of relevant literature is presented.
Topics: Humans; Lip Neoplasms; Male; Mesenchymoma; Middle Aged
PubMed: 15225956
DOI: 10.1016/j.bjoms.2004.03.002 -
Tumori 2010Malignant mesenchymoma of the thyroid is extremely rare. We report such a tumor involving the bilateral lobes of the thyroid which showed simultaneous... (Review)
Review
Malignant mesenchymoma of the thyroid is extremely rare. We report such a tumor involving the bilateral lobes of the thyroid which showed simultaneous chondrosarcomatous, osteosarcomatous, fibrosarcomatous and rhabdomyosarcomatous differentiation. The patient was a 52-year-old woman admitted with a history of facial swelling, neck thickness and swallowing discomfort of one month's duration. Sonographic examination indicated a thyroid mass involving the bilateral lobes. Macroscopically, the tumors of both lobes were well demarcated, solid, greyish-white, and multinodular on the cut surface. Some nodules were translucent in appearance and hard in texture. Microscopically, the tumor was composed of small primitive mesenchymal cells with osteoid formation resembling the small cell variant of osteosarcoma interspersed with multiple cartilaginous nodules that indicated chondrosarcomatous differentiation. Some tumor cells showed prominent rhabdomyoblastic differentiation with eosinophilic cytoplasm and eccentric nuclei. Fibrosarcomatous areas were also observed. Immunohistochemically, the small primitive mesenchymal cells were positive for vimentin and CD99 and negative for CD56, Syn, CgA, CK, TG, TTF-1, calcitonin, and S-100. The tumor cells in the rhabdomyosarcomatous area were MyoD1 and muscle-specific actin positive. Molecular analysis for BRAFand RAS gene alterations showed no point mutation. The tumor recurred four months after surgery and tumor thrombi were suspected in the bilateral internal carotid arteries on ultrasonography. Primary malignant mesenchymoma of the thyroid is a high-grade malignant tumor with a poor prognosis. Its differerential diagnosis includes anaplastic carcinoma and other rare sarcomas with chondroid, osteoid, and other mesenchymal metaplasia.
Topics: Female; Humans; Immunohistochemistry; Mesenchymoma; Middle Aged; Thyroid Neoplasms
PubMed: 20572598
DOI: 10.1177/030089161009600227 -
Zhonghua Bing Li Xue Za Zhi = Chinese... Jan 2023To investigate the clinical, radiological, histological and molecular features and the differential diagnosis of fibrocartilaginous mesenchymoma (FM). Four cases of FM...
To investigate the clinical, radiological, histological and molecular features and the differential diagnosis of fibrocartilaginous mesenchymoma (FM). Four cases of FM diagnosed in the Department of Pathology, the Sixth People's Hospital Affiliated to Shanghai Jiaotong University School of Medicine from 2020 to 2022 were analyzed. Related literature was also reviewed. Case 1 was a 10-year-old girl with bone destruction in the sacrum and L5 articular processes revealed by CT scan. Case 2 was a 7-year-old girl with an aggressive lesion in her right distal ulna. Case 3 was an 11-year-old boy with a lesion in the metaphysis of his left proximal tibia. Case 4 was an 11-year-old boy with bone destruction in the distal portion of a radius. Microscopically, the four tumors all consisted of numerous spindle cells, hyaline cartilage nodules, and bone trabeculae. The hypocellular to moderately cellular spindle cell component contained elongated cells with slightly hyperchromatic, mildly atypical nuclei arranged in bundles or intersecting fascicles. Benign-appearing cartilaginous nodules of various sizes and shapes were scattered throughout the tumors. There were areas mimicking epiphyseal growth-plate characterized by chondrocytes arranged in parallel columns and areas of enchondral ossification. The stroma was rich in mucus in case 1. Mutation of GNAS and IDH1/IDH2 and amplification of MDM2 gene were not found in any of the three tested cases. FM is very rare and tends to affect young patients. It most frequently occurs in the metaphysis of long tubular bones, followed by the iliac-pubic bones and vertebrae. FM is characterized by a mixed population of spindle cells, hyaline cartilage nodules and trabeculae of bone, without specific immunophenotypes and molecular alternations. As a borderline, locally aggressive neoplasm, surgical removal with a wide margin is generally the treatment of choice for FM.
Topics: Humans; Male; Female; Child; Mesenchymoma; China; Osteogenesis; Cartilage; Tomography, X-Ray Computed
PubMed: 36617902
DOI: 10.3760/cma.j.cn112151-20221026-00886