Desmoplastic fibroma of bone is an extremely rare tumor that was first described by Jaffe in 1958. It histologically resembles the desmoid tumor of soft tissue. It is known as locally aggressive tumor but we experienced definitely benign and resembling simple bone cyst radiographically. We report a case of desmoplastic fibroma of bone and it should be included in the differential diagnosis list of any lytic bone lesion. The radiograph, MR imaging features, radiological and pathological differential diagnosis of the case are described, and literatures are reviewed.
Bone Cysts ; Diagnosis, Differential ; Female ; Fibroma, Desmoplastic ; Fibromatosis, Aggressive ; Humans

Central odontogenic fibroma is a rare odontogenic neoplasm that originates from odontogenic ectomesenchyme. Here, a case of central odontogenic fibroma in a 17-year-old male is reported. Since the present case showed a multilocular radiolucency with partially ill-defined border between the right mandibular condyle and the distal root of the right mandibular third molar, differential diagnosis involved a wide range of pathosis from benign lesions like ameoloblastic fibroma and odontogenic myxoma to more aggressive lesions such as desmoplastic fibroma, juvenile aggressive fibromatosis, or fibrosarcoma.
Adolescent ; Diagnosis, Differential ; Fibroma ; Fibroma, Desmoplastic ; Fibromatosis, Aggressive ; Fibrosarcoma ; Humans ; Male ; Mandibular Condyle ; Molar, Third ; Myxoma ; Odontogenic Tumors

Authors reviewed 21 cases of histologically confirmed rib tumor except metastasis and myeloma from Nov. 1973 to Dec. 1982 at Seoul National University Hospital. The resuls are as follows: 1. Major clinical findings are painand mass. 2. Pathologic diagnosis of rib tumors are 5 cases of fibrous dysplasia, 3 chondroma, 2 osteochondroma, 2desmoplastic fibroma, 3 chondroma, 2 osteochondroma, 2 desmoplastic fibroma, 5 osteosarcoma, 1 aneurysmal bonecyst, 1 eosinophilic granuloma, 1 cavernous hemangioma, 1 chondrosarcoma. 3. Radiologic findings are osteolyticchange and cortical expansion without marginal sclerosis in fibrous dysplasia, central location, well-definedosteolytic change with marginal sclerosis and no cortical destruction in desmoplastic fibroma, corticaldestruction, soft tissue mass and pleural effusion in malignant tumors.
Aneurysm ; Chondroma ; Chondrosarcoma ; Diagnosis ; Eosinophilic Granuloma ; Fibroma ; Fibroma, Desmoplastic ; Hemangioma, Cavernous ; Neoplasm Metastasis ; Osteochondroma ; Osteosarcoma ; Pleural Effusion ; Ribs ; Sclerosis ; Seoul

Desmoplastic fibroma is a rare benign primary bone tumor that is histologically similar to the soft tissue desmoid tumor. It most often involves the mandible, large long bone or iliac bone. Desmoplastic fibroma in a toe has been extremely rarely reported. Authors report a rare case of desmoplastic fibroma of bone occurring in the distal phalanx of a foot, with descriptions of the radiographic and MRI findings, correlation of the radiologic and pathologic findings, and discussion on the differential diagnosis of the tumor.
Adolescent ; Bone Neoplasms/*diagnosis ; Diagnosis, Differential ; Female ; Fibroma, Desmoplastic/*diagnosis ; Humans ; Magnetic Resonance Imaging ; Male ; Toes/*pathology

Desmoplastic fibromas are rare intraosseous bone tumors. They are benign but locally aggressive and frequently found in the long bones and mandible. We report radiographic and histopathologic finding of a case desmoplastic fibroma involving right temporal skull bone. A 53-year-old woman presented at our hospital complaining of continuous right side headaches for a year. Simple skull X-ray film showed 3 x 2.5 cm lytic lesion with mild sclerotic margin on right temporal area. A large craniectomy 1cm lateral to margin was fashioned. The resected mass showed encapsulated mass colored white gray. Histologic diagnosis was compatible with that of a the desmoplastic fibroma. There was no evidence of recurrence during the 15months of follow-up period.
Diagnosis ; Female ; Fibroma, Desmoplastic* ; Follow-Up Studies ; Headache ; Humans ; Mandible ; Middle Aged ; Recurrence ; Skull* ; Temporal Bone ; X-Ray Film

PURPOSE: We analyzed the diagnosis and the treatment outcomes of patients with central low grade osteosarcoma. MATERIALS AND METHODS: We retrospectively reviewed 16 patients with central low grade osteosarcoma were treated at out institution between 1994 and 2011. RESULTS: There were 4 men and 12 women with mean age of 26 years. Eleven patients were correctly diagnosed but 5 patients were misdiagnosed as osteoid osteoma, non ossifying fibroma, aneurysmal bone cyst, desmoplastic fibroma. 15 patients finally received wide margin en bloc excision and one of them treated under neoadjuvant chemotherapy. Final survival status was continuous disease free in 14 and 1 patient died of renal cell cancer. Remaining 1 with multifocal lesions is alive with disease for 7 years only treated radiation therapy on residual tumors. Nine (56%) of 16 tumors showed extra-osseous extension of tumor (56%) and 1 of them showed extra-compartmental tumors. CONCLUSION: The diagnosis of central low grade osteosarcoma is challenging, however, considering of the clinical suspicion, the typical findings of radiologic and pathologic features, proper diagnosis is needed. This tumor should be treated with wide excision, even after an intralesional excision, to avoid local recurrence or transformation to higher histologic grade.
Aneurysm ; Bone Cysts ; Carcinoma, Renal Cell ; Diagnosis* ; Drug Therapy ; Female ; Fibroma, Desmoplastic ; Fibroma, Ossifying ; Humans ; Male ; Neoplasm, Residual ; Osteoma, Osteoid ; Osteosarcoma* ; Prognosis* ; Recurrence ; Retrospective Studies

OBJECTIVETo investigate the clinical and pathological features of paratesticular desmoplastic small round cell tumor (DSRCT), and to improve the diagnosis and treatment of the disease.

METHODSOne case of paratesticular DSRCT was studied retrospectively and a considerable amount of related literature from Medline and Chinese journals reviewed. The patient was a 27-year-old man presenting with a painless testicular mass in the left hemiscrotum. On physical examination, a cystic mass was palpable while the testis was not in the left hemiscrotum.

RESULTSDuring the operation the paratesticular area was found full of multiple nodular tumor masses of various sizes ranging from 0.5 cm to 1.5 cm in diameter. Pathological examination showed the characteristic histological pattern of nests of small undifferentiated cells embedded in a dense fibrous stroma. The tumor presented an immunohistochemical feature of epithelial, mesenchymal as well as neural multidirectional differentiation. Following testicular tumor orchiectomy, chemotherapy was performed with DDP, VP16, ifosfamide and EPI. Three years follow-up found no tumor recurrence.

CONCLUSIONDesmoplastic small round cell tumor has a specific clinicopathologic stigmata, usually occurring in young males, for which surgical resection with chemotherapy is the treatment of choice. DSRCT located in the paratesticular region may have a better prognosis than its more frequently abdominal counterpart.

Adult ; Carcinoma, Small Cell ; diagnosis ; drug therapy ; surgery ; Combined Modality Therapy ; Fibroma, Desmoplastic ; diagnosis ; drug therapy ; surgery ; Humans ; Male ; Testicular Neoplasms ; diagnosis ; drug therapy ; surgery ; Treatment Outcome

Desmoplastic fibroma of bone is a rare locally aggressive, but non-metastatic tumor. In this case report, we present a desmoplastic fibroma in an unusual location, the clavicle. Desmoplastic fibroma involving the clavicle is extremely rare, with only 2 reported cases before 1985. We report the imaging findings of a desmoplastic fibroma of the clavicle with a review of the relevant literature.
Adult ; Bone Neoplasms/*diagnosis/pathology/radiography ; *Clavicle/pathology/radiography ; Fibroma, Desmoplastic/*diagnosis/pathology/radiography ; Humans ; Magnetic Resonance Imaging ; Male ; Tomography, X-Ray Computed

From Sept. 1987 to Mar. 1993, 14 cases of pelvic bone tumors took surgical treatment. Male to female ratio was 7:7, with an average age of 31.9 years. Pathological diagnosis were chondrosarcoma 7(1 ; secondary), metastatic thyroid cancer 2, and each one case of giant cell tumor, desmoplastic fibroma, metastatic synovial sarcoma, malignant fibrous histiocytoma(MFH), and enchondroma. According to the Enneking's classification for the type of surgical resection, we performed 4 cases of type II+III resection, 4 type IA, 2 type I+II, 2 type III, and each one of II and IIA+III. Types of reconstruction were hip fusion(3 cases), heat treated autogenous bone graft combined with total hip replacement arthroplasty(4), bone graft(2), cement spacer(1) or saddle prothesis(1). Simple resection was done for 3 cases. Wide surgical margin was achieved in 12 cases, marginal margin in 1 and intralesional magin in 1. Complications occurred in 3 cases(21.4%) : 1 graft failure ; 1 lymphedema ; and 1 delayed union. The average follow-up period was 27.4(8-60) months. During follow up, lung metastasis developed in MFH case but no local recurrence was noted in all. By the criteria of American Musculoskeletal Tumor Society(MSTS), the average score for functional activities was 21(70% of normal). The function of the extremity with reconstructed or undestroyed pelvic ring was better than that with disrupted pelvic ring(functional scores ; 24.6 vs 12.3 respectively). Due to the Korean life style using hypocaust, fused hip was not good for indoor activity. For the malignant pelvic bone tumor not involving femoral neurovascular bundle, limb-salvage using internal pelvectomy and reconstruction of pelvic ring can be indicated as a surgical procedure, instead of the hindquarter amputation.
Amputation ; Arthroplasty, Replacement, Hip ; Chondroma ; Chondrosarcoma ; Classification ; Diagnosis ; Extremities ; Female ; Fibroma, Desmoplastic ; Follow-Up Studies ; Giant Cell Tumors ; Hip ; Hot Temperature ; Humans ; Life Style ; Lung ; Lymphedema ; Male ; Neoplasm Metastasis ; Pelvic Bones ; Recurrence ; Sarcoma, Synovial ; Thyroid Neoplasms ; Transplants