Desmoplastic fibroma of bone often has thickened internal trabeculations and arises within the ceter of medullary canal. Purchase bone and soft tissue pathology 1st edition. Desmoplastic fibroma nonossifying fibroma ossifying fibroma. Pathology and genetics of tumors and soft tissue and bone. Desmoplastic fibroma of the bone is an extremely rare bone tumor that resembles a desmoid tumor of the soft tissues. It usually affects craniofacial bones, mandible most frequently, long bones metaphyseal femur, tibia, humerus.
They are considered to be a bony counterpart of soft tissue desmoid tumors and are histologically identical. Bone tumours are dealt with in the bone tumours article. An intraosseous osteolytic lesion in the metaphysis of the right lower fe. Collagenous fibroma desmoplastic fibroma of the palate. A gigantic desmoplastic fibroma resembling a malignant. D esmoplastic fibroma of bone is a benign, locally aggressive tumor with varied recurrence rates found in the literature. Its a very rare tumor with slow growth and produces collagen. It includes the latest diagnostic information in this challenging subspecialty, while its unique image collection serves as an exceptional educational aid. Cumulatively, these series are far outnumbered by single case reports. Nonossifying fibroma and solitary congenital fibromatosis of bone can be confused with desmoplastic fibroma. Numerous authors have subsequently attempted to clarify the histological criteria and, in 20, the. Presents a variable production of curvilinear immature bone trabeculae regularly disposed inside a slightly. The mr imaging characteristics in our case are compared to those previously reported.
A 5yearold chinese boy had dull pain in his right knee for about one year. Computed tomography of the chest shows an expansile, lytic bone lesion extending through the cortex. Desmoplastic fibroma of the rib archives of pathology. A colourful illustrated quick guide for bone tumour diagnosis, written in a practical way. Bone was designed for practicing pathologists who need access to uptodate, comprehensive, and concise bone pathology knowledge in one convenient place.
Herein, a case of massive and unusual df, with simultaneous involvement of ilium and ischium, is described. Desmoplastic fibroma of bone is a very rare benign tumor, which may be locally aggressive. 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. Desmoplastic fibroma of bone is a rare benign tumor consisting of thin, wavy fibroblasts set in an abundant matrix of collagen fibers. 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 of the rib albert einstein college. Desmoplastic fibroma of bone is a rare locally aggressive, but nonmetastatic tumor. Bone pathology is one of the most subspecialized and challenging areas of pathology. The most common site is the mandible, followed by the femur and pelvis. Jaffe first described it as a benign tumor characterized by the formation of abundant collagen fibers by the tumor cells. Diagnosis of tumors and pseudotumoral lesions of bones and joints. Atlas of soft tissue and bone pathology 9781620700372demos. The book contains a strong blend of archival material and new cases, and incorporates the latest knowledge about the grading and staging of these tumors.
Because of its rarity, desmoplastic fibroma of bone. Desmoplastic fibroma df, a benign locally aggressive lesion of the bone is recognized as an. Allograft bone in the treatment of desmoplastic fibroma. Desmoplastic fibroma of bone inwards 1991 cancer wiley. Save time identifying and diagnosing pathology specimens with high yield bone and soft tissue pathology, edited by drs. Hufnagel tj, artiles c, piepmeier j, kier l, kim jh. Desmoplastic fibroma of bone is a rare benign primary bone tumor that histological ly resembles the extraabdominal desmoid tumor of soft tissues. Aug 12, 2014 the desmoplastic fibroma df is a rare, fibroblastic lesion of bone that histologically resembles the desmoid tumor of soft tissue. In this case report, we present a desmoplastic fibroma in an unusual location, the clavicle.
Desmoplastic fibroma of parietal bone simulating eosinophilic granuloma. Desmoplastic fibroma of bone may be extremely difficult to distinguish. Desmoplastic fibroma df is a benign but locally aggressive neoplasm of the bones 1, 2 and it is very rare in the mandible like some other intraoral tumours 3. A 29yearold man suffered minor pain in his right hip for 2 years. Specifically localized, desmoplastic fibroma begins in the bone it is not a cancer that begins elsewhere in the body and metastasizes to the bone.
Betacatenin is usually positive in the cytoplasm of neoplastic cells. Although considered a benign lesion, it is locally destructive, can extend into the soft tissues, and has a high rate of local recurrences after incomplete surgical excision. A gigantic desmoplastic fibroma resembling a malignant neoplasm. Primary bone tumors chapter 12 pathology of bone and. It affects mainly adolescents and young adults, the mean age at presentation is 1525 years 3. Mar 16, 2020 desmoplastic fibroblastoma collagenous fibroma is a benign fibrous tumor that presents as a slowgrowing, painless mass.
Desmoplastic fibroblastoma surgical pathology criteria. Although there is a considerable overlap in the mr imaging features with other bone tumors, an. The usual sites of involvement are subcutaneous tissue or skeletal muscles of upper or lower extremities. Desmoplastic fibroma definition of desmoplastic fibroma by. The authors are expert educators in surgical and cytopathology and experienced diagnosticians in the complexities of soft tissue and bone pathology.
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 fibroma is a rare primary bone tumor which morphologically resembles its soft tissue counterpart the desmoidtype fibromatosis commonly seen in the abdominal wall. Desmoplastic fibroma of bone may be extremely difficult to distinguish from lowgrade fibrosarcoma of bone. This type of bone cancer has been found in patients ranging from 20 months to 60 years old, but it typically affects adolescents and young adults between the ages of 15 and 25 years old. Specimen radiograph showing a characteristic soap bubble appearance of desmoplastic. The book contains a strong blend of archival material and new cases, and incorporates the latest knowledge about the grading and.
Desmoplastic fibroma is an extremely rare tumor with less than 200 cases in the published literature. In 2002, the world health organization defined df as a rare, benign bone tumor composed of spindle cells with minimal cytologic atypia and abundant collagen production. Atlas of soft tissue and bone pathology 9781620700372. Bone is a scaffold it bears weight and occasionally gets infected. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment.
Abstract desmoplastic fibroma is a rare primary tumor of bone that histologically and biologically mimics the extra. Desmoplastic fibromas are extremely rare bone tumors that do not metastasize but. Otherwise known as collagenous fibroma, desmoplastic fibroblastoma is a benign tumour that presents as slowgrowing masses in the subcutis and mainly occur in middle age. Jun 08, 2015 findings, and surgical management of this pathology. The tumor constitutes of bone tumors, and mandibular involvement is common with a reported frequency of approximately 40% of the various bony sites. It is a slowly progressing tumor with welldifferentiated cells that produce collagen. At times it is difficult to distinguish desmoplastic fibroma from other fibrous lesions, especially lowgrade fibrosarcomas. 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.
Dfb can occur in any bone, but is predominant in the jaw and the femur bones. It was first described by jaffe in 1958 and there are around 270 reported cases in literature accounting for less than 0. Axial ct reconstruction of lower extremity, shows a lytic lesion with posterior cortical destruction during ct guided biopsy. Desmoplastic fibroma is a benign but aggressive tumor composed of fibroblasts and myoblasts in heavily collagenized stroma. Fibrosarcoma, however, will have cellular atypia, occasional mitotic figures, and a herringbone arrangement of cells. Pathology desmoplastic fibromas histologically are identical to soft tissue desmoid tumors, with abundant collagenous stroma and little cellularity or pleomorphism. This benign tumor is characterized by aggressive local infiltration. Desmoplastic fibromas are extremely rare bone tumors that do not metastasize but may be locally aggressive. Fletcher cdm, bridge ja, hogendoorn pcw, mertens f, eds. It represents one of the rarest bone diseases, with 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 is a very rare bone tumor with a reported incidence of 0. Desmoplastic fibroma df is a rare benign intraosseous tumor with provincially aggressive nature. 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 is a rare benign primary bone tumor, histologically identical to the extraabdominal desmoid tumor of soft tissues. Desmoplastic fibroma formerly described as endosteal fibroma andor desmoid tumor of bone is a rare, benign, locally aggressive tumor of bone. Desmoplastic fibroma malignant fibrous histiocytoma pleomorphic sarcoma fibrosarcoma of bone. 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. It was initially described by jaffe in 1958 who highlighted the histological resemblance to aggressive fibromatosis desmoid tumour. The incidence of desmoplastic fibroma of bone in all patients with benign bone tumours in our population is 0.
Desmoplastic fibroma of the frontal bone lath r, ranjan a. Desmoplastic fibromas of bone have been described as a rare, nonmetastasizing, locally aggressive, benign lesions that histologically resemble a. Benign bone tumor, desmoplastic fibroma, mandible, recurrence. 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 an overview sciencedirect topics.
Desmoplastic fibroma df of bone is a rare, nonmetastasizing but locally aggressive tumor that has been discussed infrequently in the radiology literature. Desmoplastic fibroma is an extremely rare benign tumor that progresses slowly with welldifferentiated cells that produce collagen. Desmoplastic fibroma of the mandible a rare benign tumor. Surgical treatment ranged from primary amputation to intralesional curettage.
There is no metastasis but beside of their destructive growth they show a high frequent recurrence after local resection 4. Pathology and genetics of tumours of soft tissue and bone, world health organization classification of tumours 2002. Moreover, a report of an interesting case is included localized in the mandibular corpus. Because of its relative rarity, desmoplastic fibroma of the bone has been described in only a few small series in the orthopedic, pathology, and radiology literature. Abstract desmoplastic fibroma df of bone is a rare, nonmetastasizing but locally aggressive tumor that has been discussed infrequently in the radiology literature. Desmoplastic fibroma of bone dfb is an extremely rare benign bone tumor, which accounts for approximately 0. The senior editors and authors of this book are well recognized and highly regarded bone and soft tissue pathologists and radiologists. Who classification of tumours of soft tissue and bone tumors. Desmoplastic fibroma of bone, when found in the foot, is an exceedingly rare occurrence 1. The second represents a softtissue desmoid invading bone secondarily. This tumor grows locally in bone in an aggressive manner and destroys the bone.
Its main incidence is in the second and third decade of life. Developed by renowned radiologists in each specialty, statdx provides comprehensive decision support you can rely on desmoplastic fibroma. Desmoplastic fibroma df is a rare, locally aggressive benign bone tumour with a reported incidence of 0. Typically, dfb is more common in the distal femoral metaphysis when it occurs in the femur. Lesions of fibrous dysplasia that show little woven bone may also be confused with desmoplastic fibroma. This richly illustrated and concise reference will be a practical and indispensible tool for general pathologists and pathologists in training, who are required to diagnose bone and soft tissue. F ibrous lesions fibrous dysplasia osteofibrous dysplasia desmoplastic fibroma nonossifying fibroma ossifying fibroma. The incidence of desmoplastic fibroma is reported to be 0. Authored and updated by experts in the field, diagnostic. Desmoplastic fibroma is a benign bone tumor that can be locally aggressive. Approximately, 180 cases have been reported in the english medical literature, most of which were case reports. Dahlins bone tumors presents a succinct, profusely illustrated summary of the largest single collection of welldiagnosed bone tumors anywhere in the world. Desmoplastic fibroma df is a rare benign neoplasm of the bone that currently accounts for 0.
Cells are spindle and arranged in a whorled fashion or in long fascicles, similar to what happens in soft tissue desmoplastic fibroma. It characterized also by aggressive intramedullary proliferation of myofibroblasts. Tumours often spread to bone and occasionally arise in bone. Enzinger and weisss soft tissue tumors, 5th edition, 2008. Desmoplastic fibroma df is a rare, locally invasive but benign bone tumor. They have been described involving a wide range of anatomic sites but most commonly arise on the upper extremity.
If cortical destruction and a soft tissue mass is noted, desmoplastic fibroma of bone may resemble more sinister pathology, such as fibrosarcoma. Df was most common in the mandible, pelvis, and femur. The classic text on the mayo clinics experience with bone tumors is now in its updated sixth edition. Desmoplastic fibroma of the femur with atypical image. Low grade intraosseous osteosarcoma, another tumour that can mimic desmoplastic fibroma, can also be excluded by identification of bone formation. Desmoplastic fibroma of bone is an intraosseous, locally aggressive neoplasm, constituted by spindle cells and abundant collagen production.
Massive rare desmoplastic fibroma of the ilium and ischium. Most patients are adults and it is more common in males m. The main cell types that are seen include fibroblasts, myofibroblasts, and undifferentiated mesenchymal cells 9. Desmoplastic fibromas histologically are identical to soft tissue. Animated gif showing a photomicrograph of lamellar bone.