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

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 discuss clinical effects of surgical treatment for desmoplastic fibroma of bone.

METHODSBetween June 2000 and June 2010, 15 cases of desmoplastic fibroma were treated by surgical operation including 4 males and 7 females with an average age of 39 years old (ranged from 18 to 64 years old). The site of tumor was proximal femur in 4 cases, distal femur in 3, distal tibia in 2, proximal humerus in 1, distal humerus in 1, scapula in 1, pelvic in 2, manubrium of sternum in 1. The simple intralesional curettage was performed in 1 case. The other 14 cases were divided into two groups, 7 cases had an aggressive curettage with inactivation and the last 7 cases had a wide resection. Recurrence condition were observed after operation. The function was valuated in two groups after the operation according to Enneking's standard.

RESULTSThe mean duration of follow-up was 56 months (ranged, 18 to 132 months). Two cases recurred, but no metastasis. The patient with simple intralesional curettage recurreed, 1 of the 7 patients with a wide resection recurred. The recurrence rate was 13.3% (2/15). There was no recurrence in the group with an aggressive curettage with inactivation. According to Enneking's standard, Enneking scoring was 21.6 +/- 3.8 in the group with a wide resection and 28.3 +/- 1.3 in another group, The results were excellent in 2 cases and good in 5 in the group with a wide resection, excellent in 7 in the other group.

CONCLUSIONThe aggressive curettage with inactivation has better functional recovery than the wide resection,and it should be chosen when the lesion is small or located in an area where reconstruction is difficult.

Adolescent ; Adult ; Bone Neoplasms ; pathology ; surgery ; Female ; Fibroma, Desmoplastic ; pathology ; surgery ; Humans ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; epidemiology ; Young Adult

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 fibroma, which develops predominantly in long bones and the mandible, is a rare and benign but locally aggressive tumor. Desmoplastic fibroma of the cranium is extremely rare. We report a case of desmoplastic fibroma of the frontal bone in a young man. Because of its locally aggressive behavior, complete surgical excision with a safety margin is essential.
Fibroma, Desmoplastic ; Frontal Bone ; Mandible ; Skull

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

Fibroma, Desmoplastic ; pathology ; Humans ; Male ; Middle Aged ; Nose Neoplasms ; pathology

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

Skeletal desmoplastic fibroma is an intraosseous neoplasm that is recognized as a very scare benign tumor. It has a propensity for locally aggressive behavior and local recurrence. The aim of this article is to report a case of skeletal desmoplastic fibroma in right mandible of a 4-year-old boy. The patient was found to have a large skeletal desmoplastic fibroma in right mandible, which was resected by surgical intervention. The defect was successfully restored with a titanium plate. In the report, the etiopathogenisis, pathological, radiographic features, clinical diagnosis, therapy and prognosis of skeletal desmoplastic fibroma were diccussed.
Fibroma, Desmoplastic ; Humans ; Male ; Mandible ; Titanium

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