Pathology and genetics of tumours of soft tissue and bone. Desmoplastic fibroma histologically and biologically mimics the soft tissue desmoid tumor. The desmoplastic fibroma df is a rare, fibroblastic lesion of bone that. Desmoplastic fibroma of the frontal bone lath r, ranjan a. In this case report, we define the clinicopathological and radiographic features of. Desmoplastic fibroma of bone is a rare primary tumor of bone. Sabo, dpm, facfas2 the foot and ankle online journal 8 2. Approximately, 180 cases have been reported in the english medical literature, most of which were case reports. Desmoplastic fibroma df is a rare, benign but locally aggressive, intraosseous lesion with a high tendency of local recurrence. Osteochondroma 45% osteoid osteoma 10% enchondroma 10% haemangioma 10% nonossifying fibroma 4% osteoblastoma 2% chondroblastoma 2% chrondromyxoid fibroma 2% clinical feature. Desmoplastic fibroblastoma in maxillary alveolar bone. Wittig, i am writing at a loss to find adequate words to thank you for all that you have done for me. Allograft bone in the treatment of desmoplastic fibroma. The who defines a desmoplastic fibroma as a rare, benign bone tumour composed of spindle cells with minimal atypia and abundant collagen production.
Desmoplastic fibroma of the mandible in a young adult. Bone and soft tissue pathology bone peripheral neuropathy. Pdf collagenous fibroma desmoplastic fibroblastoma of. Two cases of ossifying fibroma of long bones are presented. Bone and soft tissue pathology free download as powerpoint presentation. 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. Histopathological diagnosis of this tumour is also sometimes not easy. Although classified as benign, it frequently demonstrates aggressive behavior, often causing tooth mobility, extensive bone destruction, and has a moderate to high recurrence rate. Desmoplastic fibromas histologically are identical to soft tissue desmoid tumors. Desmoplastic fibroma is a very rare bone tumor with a reported incidence of 0. If cortical destruction and a soft tissue mass is noted, desmoplastic fibroma of bone may resemble more sinister pathology, such as fibrosarcoma, intraosseous osteosarcoma and metastases. Nov 24, 2009 desmoplastic fibroma df is a rare, benign but locally aggressive, intraosseous lesion with a high tendency of local recurrence.
Ossifying fibromas are benign bone lesions that should be differentiated from nonossifying fibromas and fibrous dysplasia. Folpe is the author of over 170 medical publications, principally in the areas of soft tissue pathology and diagnostic immunohistochemistry, the coauthor of the 6th edition of enzinger and weiss soft tissue tumors and the most recent armed forces institute of pathology fascicle on soft tissue tumors, the coauthor of bone and soft tissue pathology. Desmoplastic fibroma of bone is now considered to be the intraosseous counterpart of the soft tissue desmoid or fibromatosis 2. Desmoplastic fibroma of bone 288 fibrosarcoma of bone 289 fibrohistiocytic tumours 291 benign fibrous histiocytoma of bone 292 malignant fibrous histiocytoma of bone 294 14 ewing sarcoma primitive neuroectodermal tumour 297 ewing tumour pnet 298 15 haematopoietic tumours 301 plasma cell myeloma 302 malignant lymphoma 306 16 giant cell. Since his original description, a number of case reports have been publish ed, as many as 150. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. Fibrocartilaginous mesenchymoma of bone is fcmb is an extremely rare tumor first described in 1984. We report a rare case of desmoplastic fibroma df of the midshaft femur presenting as a pathological fracture. 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.
Desmoplastic fibroma of the rib archives of pathology. Desmoplastic fibromas of bone have been described as a rare, nonmetastasizing, locally aggressive, benign lesions that histologically resemble a desmoid tumour of the soft tissue. The treatment modalities for this tumour are nonuniform and often controversial. Jun 30, 2015 d esmoplastic fibroma of bone is a benign, locally aggressive tumor with varied recurrence rates found in the literature. Oral fibroma, typically referred to as simply fibroma, is a very common benign lesion in oral pathology it is also known as focal fibrous hyperplasia, peripheral fibroma, peripheral ossifying fibroma, fibroid epulis old term, and fibroepithelial polyp. Hence, desmoplastic fibroma of bone is also referred to as desmoid tumor of bone 2. Whitesides and ackerman 2 documented three cases in 1960, and sheer and kuhlman 3 reported an additional case in 1963. Extremely rare benign bone tumor arising in young adults. The incidence of desmoplastic fibroma is reported to be 0. Clinically, the tumor tends to present as a slowgrowing intrabony mass most often located in the region of the. It is a fibrous tumour of the bone, histologically appearing similar to soft tissue desmoid tumour. A case of desmoplastic fibroma of bone with ctnnb1 point. Jaffe first described it as a benign tumor characterized by the formation of abundant collagen fibers by the tumor cells. This rare benign bone tumor was treated with an acute en bloc excision and femoral shortening over an intramedullary nail.
To our knowledge, no series has analyzed the mri characteristics of desmoplastic fibroma of the bone. Bone tumours which produce chondroid matrix, at least focally. Diagnosis is not easy and is often made by excluding other tumours. It usually affects craniofacial bones, mandible most frequently, long bones metaphyseal femur, tibia, humerus. Page 247250 seven cases have been reported in the oral cavity so far in the buccal mucosa, palate, parotid and gingiva. The second represents a softtissue desmoid invading bone secondarily. Desmoplastic fibroma df of bone is a rare, nonmetastasizing but locally aggressive tumor that has been discussed infrequently in the radiology literature. This website is created to introduce trainees in pathology to the basic concepts in diagnosis of bone tumors.
D esmoplastic fibroma of bone is a benign, locally aggressive tumor with varied recurrence rates found in the literature. Desmoplastic fibroma of bone is a rare benign primary bone tumor. The lesion was filled with autologous bone graft from the iliac crest. You were so generous to give up your time on a weekend to operate on my femur. Bone pathology is the second edition of the book, a compendium of skeletal. Computed tomography of the chest shows an expansile, lytic bone lesion extending through the cortex. We identified thirteen patients with desmoplastic fibroma of bone treated at our institute over a 30 year period.
Fewer than 20 cases have been reported, with patient ages spanning from 9 to 25 years, though a case in a male infant aged 1 year and 7 months has been reported. As the clinical presentation, imaging features and histopathology of df and dflike lgos show much overlap, the objective of this study was to investigate the value of cytogenetic analysis, molecular pathology. It is the benign primary fibroblastic bone tumor that may be locally aggressive. Desmoplastic fibroblastoma in maxillary bone turkish journal of pathology vol. Bone pathology is the second edition of the book, a compendium of skeletal pathology that published 10 years ago. Quick growth and bulky size are remarkable features of this tumor.
Read desmoplastic fibroma of bone with extensive cartilaginous metaplasia, annals of diagnostic pathology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. We report a case of desmoplastic fibroma of the frontal bone in a young male. Desmoplastic fibroma of the mandible dentomaxillofacial. Bone and soft tissue tumors fabrizio remotti md definition zsoft tissue pathology deals with tumors of the connective tissues. 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.
Specific fibromas include nonossifying fibroma, found in the large long bones. Once union of the acute shortening had been achieved, further surgery was undertaken to lengthen the femur with the use of intramedullary skeletal kinetic. Desmoplastic fibromas are extremely rare bone tumors that do not metastasize but may be locally aggressive. The mr imaging characteristics in our case are compared to those previously reported.
Desmoplastic fibroma of bone, when found in the foot, is an exceedingly rare occurrence 1. Printable fibroma of tendon sheath surgical pathology. Desmoplastic fibroma is a rare primary bone tumor which morphologically resembles its soft tissue counterpart the desmoidtype fibromatosis commonly seen in the abdominal wall. A rare case of desmoplastic fibroma in the maxillary corpus is being presented with its pathology, clinical diagnostic methods, treatment and prognosis. Osteofibrous dysplasia is considered as a separate pathological entity in view of its different presentation and treatment, although histopathologically similar to ossifying fibroma. Desmoplastic fibroma of bone dfb is a rare bone tumor with an incidence rate of less than 0. It was first described by jaffe in 1958 and there are around 270 reported cases in literature accounting for less than 0. Specimen radiograph showing a characteristic soap bubble appearance of desmoplastic. In contrast to the welldocumented radiological appearance, the literature on mr imaging features of this tumor is scarce. Desmoplastic fibroma of the femur with atypical image. Desmoplastic fibroma is an uncommon locally aggressive benign tumor of the bone. Desmoplastic fibroma is a rare benign tumour of bone. Significant t2 shortening of a nonsclerotic fibroosseous lesion should place desmoplastic fibroma high among the diagnostic considerations.
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. Although fibroma of tendon sheath is typically sclerotic and nodular fasciitis is typically not, there is sufficient variation in patterns to cause overlap. Ossifying fibroma of is a benign, nonodontogenic tumor of the jaw, a type of. Desmoplastic fibroblastoma collagenous fibroma of the. Moreover, a report of an interesting case is included localized in the mandibular corpus. Desmoplastic fibroma is a rare benign bone tumour which originates from connective tissue with characteristics of local aggression and a high recurrence rate. Axial ct reconstruction of lower extremity, shows a lytic lesion with posterior cortical destruction during ct guided biopsy. We studied two cases of rare fibrous bone tumors, namely desmoplastic fibroma df and lowgrade central osteosarcoma lgcos resembling desmoplastic fibroma dflike lgcos. Ossifying odontogenic fibroma e589 case report a 43yearold woman with noncontributory medical, social, and cultural records was referred to the oral and. Desmoplastic fibroma of bone is a recently described, rarely reported lesion. They are considered to be a bony counterpart of soft tissue desmoid tumors and are histologically identical. 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. Desmoplastic fibroma of bone with extensive cartilaginous metaplasia. Desmoplastic fibroma df of the bone, also know as desmoid tumour, is a rare nonmetastasizing but locally aggressive tumour.
Desmoplastic fibroma df, a benign locally aggressive lesion of the bone is. Most such lesions on the hands are labeled fibroma of tendon sheath. Desmoplastic fibroma formerly described as endosteal fibroma andor desmoid tumor of bone is a rare, benign, locally aggressive tumor of bone. Desmoplastic fibroma of bone with extensive cartilaginous. Desmoplastic fibroma of bone is a rare bone tumour, accounting for only 0. High power microscopic image showing dense connective tissue with collagen bundles and relatively few blood vessels and fibroblasts irritation fibroma polypoid fibrous hyperplasia. Desmoplastic fibroma of bone is a rare locally aggressive, but nonmetastatic tumor. A gigantic desmoplastic fibroma resembling a malignant. Desmoplastic fibroma of the bone, the intraosseous counterpart of softtissue fibroma toses, also is known as desmoid tumor of bone. In this case report, we present a desmoplastic fibroma in an unusual location, the clavicle. If cortical destruction and a soft tissue mass is noted, desmoplastic fibroma of bone may resemble more sinister pathology, such as fibrosarcoma. Thus, the incidence of desmoplastic fibroma of bone is approximately 1440 of osteosarcoma i. Because of its rarity, desmoplastic fibroma of bone.
Five examples of this distinctive lesion of bone were described by jaffe 1 in 1958. Its distinction from fibrous dysplasia and its association with adamantinoma of long bone. The purpose of this study was to evaluate the imaging features of desmoplastic fibroma of the bone, with an emphasis on mri signal characteristics. Desmoplastic fibroma of bone is a very rare benign tumor, which may be locally aggressive. Desmoplastic fibroma, skull introduction desmoplastic fibroma is a rare benign but locally aggressive. Desmoplastic fibroma is a benign bone tumor that can be locally aggressive. Radiologically and histologically, it can mimic a variety of tumors. In this case report, we present imaging findings of a histopathologically proven desmoplastic fibroma involving the left mandibular region in a 15yearold chinese patient. Scribd is the worlds largest social reading and publishing site. To date, small numbers of case reports and case series have been published regarding desmoplastic fibroma. Benign bone tumor prevalence of benign bone tumors.
Neoplastic mimics in soft tissue and bone pathology pathology of. Desmoplastic fibroma is a benign but locally aggressive tumor arising usually from the mandible, pelvis and long bones with a potential for recurrence. In 1958, jaffe first described desmoplastic fibroma of bone as a distinct entity 1. This article presents a report on three cases of desmoplastic fibroma in the proximal humerus, distal femur, and neck femur region.
Aug 14, 2014 if cortical destruction and a soft tissue mass is noted, desmoplastic fibroma of bone may resemble more sinister pathology, such as fibrosarcoma, intraosseous osteosarcoma and metastases. Immature bone trabeculae with entrapped osteocytes and lined by a dense. Recognition of desmoplastic fibrom a is important because on radiology and histology, the lesion may be mistaken. Similar to the prior edition, this book complements standard pathology texts and blends new but relatively established information on the molecular biology of the bone. A wellcircumscribed domeshaped soft tissue enlargement of the left buccal mucosa. Benign tumours are usually climically asymptomatic, found incidentally on xray investigation. Abstract desmoplastic fibroma is a rare primary tumor of bone that histologically and biologically mimics the extra. Choi department of pathology and department of orthopaedic surgery, university of hong kong, queen mary hospital, hong kong summary a 5yearold chinese boy had dull pain in his right knee for about one year. Biologic behaviour range from benign lesions to highly malignant neoplasms. Desmoplastic fibroma of bone dfb is an extremely rare benign bone tumor, which accounts for approximately 0.
Desmoplastic fibroblastoma of the oral cavity e89 journal section. Patients usually present with a longstanding history of pain and swelling. The removed tumor was sent for further histological examination to the department of pathology. 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.