Osteosarcoma of the skull resembling desmoplastic fibroma. Numerous authors have subsequently attempted to clarify the histological criteria and, in 20, the who. This website is created to introduce trainees in pathology to the basic concepts in diagnosis of bone tumors. It occurs typically in adolescents and young adults. Desmoplastic fibroma of the rib archives of pathology. Desmoplastic fibroma df is a rare, locally aggressive benign bone tumour with a reported incidence of 0. Allograft bone in the treatment of desmoplastic fibroma. Desmoplastic fibroma df is a rare fibroblastic lesion of bone that histologically resembles the desmoid tumor of soft tissue. Fibrosarcoma, however, will have cellular atypia, occasional mitotic figures, and a herringbone arrangement of cells. Desmoplastic fibroma of bone is a rare locally aggressive, but nonmetastatic tumor.
Desmoplastic fibroma is a benign but locally aggressive tumor arising usually from the mandible, pelvis and long bones with a potential for recurrence. It usually affects craniofacial bones, mandible most frequently, long bones metaphyseal femur, tibia, humerus. Axial ct reconstruction of lower extremity, shows a lytic lesion with posterior cortical destruction during ct guided biopsy. Desmoplastic fibroma of bone 288 fibrosarcoma of bone 289 fibrohistiocytic tumours 291 benign fibrous histiocytoma of bone 292. This website is created to introduce trainees in pathology to. In contrast to the welldocumented radiological appearance, the literature on mr imaging features of this tumor is scarce. The radiographs from 107 previously published cases of df and seven cases from the authors institution were analyzed to better understand and define its radiographic characteristics. Desmoplastic fibroma is a rare primary bone tumour first described by jaffe in 1958. Hisao shigematsu, keizou naka, seiji suzuki, nobuyuki utsumi. Animated gif showing a photomicrograph of lamellar bone. Jun 08, 2015 findings, and surgical management of this pathology.
Recognition of desmoplastic fibrom a is important because on radiology and histology, the lesion may be mistaken. The diagnosis of desmoplastic fibroma, possibly the first in a hong kong chinese, was based on the clinical, radiological and histological findings. In oral mucosa only two cases have been described in the literature. Although it does not tend to metastatize, it has a high local recurrence and. It s a nonmetastasizing but locally aggressive tumour. Df represents one of the most uncommon bone diseases as it has an incidence of only 0. Nov 24, 2009 desmoplastic fibroma df is a rare, benign but locally aggressive, intraosseous lesion with a high tendency of local recurrence.
Desmoplastic fibroma of the mandible a rare benign tumor. We report here on a pathologically proven case of osteosarcoma that presented as a painless mass in the frontal bone of a 7yearold boy. It was first described by jaffe in 1958 and there are around 270 reported cases in literature accounting for less than 0. Collagenous fibroma desmoplastic fibroma of the palate. Although there is a considerable overlap in the mr imaging features with other bone tumors, an. Recurrences will depend on the quality of the treatment. Desmoplastic fibroma of bone is a rare myofibroblastic tumor. Desmoplastic fibroma of the bone wiley online library.
Desmoplastic fibromas histologically are identical to soft tissue desmoid tumors. The incidence of desmoplastic fibroma is reported to be 0. The mr imaging characteristics in our case are compared to those previously reported. Desmoplastic fibroma df is a rare benign intraosseous tumor with provincially aggressive nature. Desmoplastic fibroma of bone is a very rare benign tumor, which may be locally aggressive. Approximately, 180 cases have been reported in the english medical literature, most of which were case reports. The purpose of this study was to evaluate the imaging features of desmoplastic fibroma of the bone, with an emphasis on mri signal characteristics. Who classification of tumours of soft tissue and bone. Desmoplastic fibroma of proximal femur surgical reconstruction. Mar 16, 2020 desmoplastic fibroblastoma collagenous fibroma is a benign fibrous tumor that presents as a slowgrowing, painless mass. About us introductory course case studies unknoun slides. Two cases of ossifying fibroma of long bones are presented. It was initially described by jaffe in 1958 who highlighted the histological resemblance to aggressive fibromatosis desmoid tumour.
Desmoplastic fibroma is a rare primary bone tumor which morphologically resembles its soft tissue counterpart the desmoidtype fibromatosis commonly seen in the abdominal wall. We describe the case of a 42yearold white woman whose complaint was a painless, slowgrowing mass under the prosthesis. Collagenous fibroma desmoplastic fibroblastoma is a rare benign soft tissue tumor with a fibroblastic origin. Krishnan unni fredrik mertens larcpress lyon, 2002. A rare case of desmoplastic fibroma in the maxillary corpus is being presented with its pathology, clinical diagnostic methods, treatment and prognosis. Desmoplastic fibroma of the bone is now considered the intraosseous counterpart of the common softtissue desmoid or fibromatoses. Positive diagnosis is based on surg ical pathology. Most patients are adults and it is more common in males m. They have been described involving a wide range of anatomic sites but most commonly arise on the upper extremity. The first would be considered a desmoplastic fibroma belonging with the group in table i by the pathologic criteria of some authorities 4, 16, but a periosteal desmoid by others 12. To our knowledge, no series has analyzed the mri characteristics of desmoplastic fibroma of the bone. Desmoplastic fibroma is a benign intraosseous neoplasm that is recognized as the intraosseous counterpart of. Desmoplastic fibroma of the bone is an extremely rare bone tumor that resembles a desmoid tumor of the soft tissues. Desmoplastic fibroma histologically and biologically mimics the soft tissue desmoid tumor.
Desmoplastic fibroma of bone fibrosarcoma of bone 18 fibrohistiocytic tumours nonossifying fibroma and benign fibrous histiocytoma of bone 19 ewing sarcoma 20 haematopoietic neoplasms plasma cell myeloma solitary plasmacytoma of bone primary nonhodgkin lymphoma of bone 21 osteoclastic giant cellrich tumours giant cell lesion of the small bones. Oral and maxillofacial radiology desmoplastic fibroma of. The tumor constitutes book provides essential, internationally applicable information in the area of orthopedic pathology with emphasis on practical diagnostic aspects, including many illustrations. There are 2 types synovial or nonsynovial synovial joints also known as diarthroses. In this case report, we present a desmoplastic fibroma in an unusual location, the clavicle.
Its distinction from fibrous dysplasia and its association with adamantinoma of long bone. In 1958, jaffe first described desmoplastic fibroma of the bone as a distinct entity when he documented five cases of a previously unclassified osseous fibrous tumor that histologically was similar to abdominal desmoid tumor. Histopathology revealed that the tumor was composed of uniform spindle cells with elongated and wavy nuclei arranged in short fascicles with the stroma being. About one year later the only abnormality was a small rounded radiological lucency, static for 4 months. Space between ends of bones formed by endochondral ossification. Desmoplastic fibroma of the bone, the intraosseous counterpart of softtissue fibromatoses, also is known as desmoid tumor of bone. Hence, desmoplastic fibroma of bone is also referred to as desmoid tumor of bone 2. Surgical treatment ranged from primary amputation to intralesional curettage. Tumours often spread to bone and occasionally arise in bone.
The role of chemotherapy in the treatment of bone and softtissue sarcomas pdf version. They are considered to be a bony counterpart of soft tissue desmoid tumors and are histologically identical. Significant t2 shortening of a nonsclerotic fibroosseous lesion should place desmoplastic fibroma high among the diagnostic considerations. Desmoplastic fibroma of bone is a rare primary tumor of bone.
Download pdf 1712k download meta ris compatible with endnote, reference manager, procite, refworks. Desmoplastic fibroma df is a rare, benign but locally aggressive, intraosseous lesion with a high tendency of local recurrence. Moreover, a report of an interesting case is included localized in the mandibular corpus. Desmoplastic fibroma df, a benign locally aggressive lesion of the bone is recognized as an. Desmoplastic fibroma is a rare benign primary bone tumor, histologically identical to the extraabdominal desmoid tumor of soft tissues. Desmoplastic fibroma of bone often has thickened internal trabeculations and arises within the ceter of medullary canal. Desmoplastic fibroma, skull introduction desmoplastic fibroma is a rare benign but locally aggressive.
Desmoplastic fibroma df of bone is a rare, nonmetastasizing but locally aggressive tumor that has been discussed infrequently in the radiology literature. The second represents a softtissue desmoid invading bone secondarily. Desmoplastic fibroma is a very rare bone tumor with a reported incidence of 0. Because of its rarity, desmoplastic fibroma of bone. Desmoplastic fibroma is a very rare bone tumor which histopathologically and biologically very much similar to extraabdominal desmoid tumor of the soft tissue. Pathology and genetics of tumours of soft tissue and bone edited by christopher d. It is characterized by a locally aggressive spindle cell tumour that resembles the pattern of fibromatosis in soft tissues. Desmoplastic fibroma of bone may be extremely difficult to distinguish from lowgrade fibrosarcoma of bone. Pdf we identified thirteen patients with desmoplastic fibroma of bone. They are covered by hyaline cartilage, strengthened by dense fibrous capsule continuous with periosteum of bones and an inner synovial membrane, and reinforced by ligaments and muscles. Lesions of fibrous dysplasia that show little woven bone may also be confused with desmoplastic fibroma.
The incidence of desmoplastic fibroma of bone in all patients with benign bone tumours in our population is 0. Pathology and genetics of tumors and soft tissue and bone. We report a case of desmoplastic fibroma of the frontal bone in a young male. Benign bone tumor, desmoplastic fibroma, mandible, recurrence. At least 20 publication records of articles and or books related to the field of experimental and. The incidence in m in the present study 18 cases from the files of the netherlands committee on bone tumors are reported, with an emphasis on the radiographic features. In 1958, jaffe 7 first differentiated this bony lesion from other fibromas of bone, and termed it desmoplastic fibroma based on the presence of fibroblasts and abundant collagenous tissue 21. This unusual form of osteosarcoma had features of desmoplastic fibroma in a large portion of the tumor. En bloc resection is the treatment of choice in view of.
Pathology and genetics of tumours of soft tissue and bone. The tumor constitutes of bone tumors, and mandibular involvement is common with a reported frequency of approximately 40% of the various bony sites. In medicine, a desmoplastic fibroma is a benign, but locally aggressive, fibrous and rare tumor of the bone, affecting children and young adults, potentially resulting in cortical bone destruction. Desmoplastic fibroma an overview sciencedirect topics. Welcome to our bone tumor pathology site bone tumors represent a unique group of pathologic conditions for which little time is assigned during the training period in most pathology residency programs. The who defines a desmoplastic fibroma as a rare, benign bone tumour composed of spindle cells with minimal atypia and abundant collagen production. Desmoplastic fibromas are extremely rare bone tumors that do not metastasize but may be locally aggressive. On the basis of material obtained from the pathology files at two institutions, we describe and discuss the imaging features of desmoplastic fibroma of the bone, with an emphasis on radiologic and mri characteristics.1332 282 1420 1221 387 957 782 261 914 734 687 289 1237 64 709 1329 1242 1382 537 813 989 570 1127 308 947 180 1016 274 1327 1519 1234 983 1056 1044 917 321 1414 852 426