A ctscan and mriscan were performed and revealed a bone cyst with sclerotic borders without destruction of the cortical bone figs. We report a case of desmoplastic fibroma of the mandible with a 5 year followup without any signs of recurrence and discuss the utilization of resorbable hardware in the surgical. Desmo plastic fibroma of the extremities was first reported by jaffel in 1958. The second represents a softtissue desmoid invading bone secondarily. Desmoplastic fibroma in the spine is extremely rare, and only a few cases have been reported. Desmoplastic fibroma of bone can imitate the pattern of a lowgrade intraosseous osteosarcoma, but df lacks osteoid matrix production, as previously discussed. Desmoplastic fibroma histologically and biologically mimics the soft tissue desmoid tumor. Collagenous fibroma desmoplastic fibroma of the palate. Desmoplastic fibromas are discovered most often in the first three decades of life, in the mandible the femur and pelvis are also favored sites. Pdf desmoplastic fibroma df is a rare, benign but locally aggressive, intra osseous lesion with a. Wed like to understand how you use our websites in order to improve them. Desmoplastic fibroma of bone is a very rare benign tumor, which may be locally aggressive. The present study reports a case of desmoplastic fibroma of the bone with the longest published follow. Desmoplastic fibroma of bone is a rare primary tumor of bone.
Clinically, it is treated using surgery as the preferred choice. Desmoplastic fibroma of the bone is now considered the intraosseous counterpart of the common softtissue desmoid or fibromatoses. Because of its rarity, desmoplastic fibroma of bone. Five examples of this distinctive lesion of bone were described by jaffe 1 in 1958. Desmoplastic fibroblastoma is commonly seen in middleage and older adults 4070 years.
A desmoplastic fibroma is a type of benign bone tumor. Original article clinical application of mpr nerve display. Fibrosarcoma, however, will have cellular atypia, occasional mitotic figures, and a herringbone arrangement of cells. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. In contrast to the welldocumented radiological appearance, the literature on mr imaging features of this tumor is scarce. Moreover, a report of an interesting case is included localized in the mandibular corpus. Although there is a considerable overlap in the mr imaging features with other. Whitesides and ackerman 2 documented three cases in 1960, and sheer and kuhlman 3 reported an additional case in 1963. Desmoplastic fibroma the open orthopaedics journal, 20, volume 7 41 pain was attributable to his physically demanding job. Desmoplastic fibroblastoma also known as collagenous fibroma is a benign relatively paucicellular, soft tissue tumor characterized by spindle to stellateshaped fibroblasts and myofibroblasts embedded in a prominent collagenous background.
Jun 30, 2015 desmoplastic fibroma of bone, although considered benign, can be a debilitating condition leading to loss of limb 17. Biopsy confirmed a chondromyxoid fibroma which was removed surgically. Desmoplastic fibroma was first recognized as a distinct kind of tumor in 1958 by henry l. Herford, dds, md, paul reder, md,t and yadranko ducic, md, desmoplastic fibroma is a locally aggressive benign fibrous tumor of bone that has been reported in both the maxillofacial skeleton and the long bones. 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. D esmoplastic fibroma of bone is a benign, locally aggressive tumor with varied recurrence rates found in the literature. Desmoplastic fibroma article about desmoplastic fibroma by. Desmoplastic fibroma df, a benign locally aggressive lesion of the bone is recognized as an intraosseous counterpart of soft tissue fibromatosis and is usually. Desmoplastic fibroma of the frontal bone lath r, ranjan a. Cryoablation, image processing, computerassisted, desmoplastic fibroma introduction desmoplastic fibroma is a rare tumor between benign and malignant, and surrounds adjacent vital structures such as nerves and blood vessels with invasive growth pattern. Benign intraosseous fibroma desmoplastic fibroma associated with paget disease of the iliac bone skeletal radiol 1988.
A rare case of desmoplastic fibroma in the maxillary corpus is being presented with its pathology, clinical diagnostic methods, treatment and prognosis. Pathologic elements of this tumor include fibroblasts, collagen fibers, and elongated nuclei. Desmoplastic fibroma article about desmoplastic fibroma. 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. Its a very rare tumor with slow growth and produces collagen. A rare type of primary bone tumor that is characteristically composed of welldifferentiated cells that produce collagen. The mr imaging characteristics in our case are compared to those previously reported.
Desmoplastic fibroma, skull introduction desmoplastic fibroma is a rare benign but locally aggressive. Desmoplastic fibromas histologically are identical to soft tissue desmoid tumors, with abundant collagenous stroma and little cellularity or pleomorphism. Although surgical resection of df arising in the spine is commonly regarded as a recommended treatment, it is difficult to achieve satisfactory results. In contrast to the welldocumented radiological appearance, the literature on mr imaging features of this. Left foramen ovale arrow is enlarged compared with normal right foramen ovale arrowhead, suggesting perineural spread of tumor. Desmoplastic fibroma an overview sciencedirect topics. In 2002 df was defined by the who as a rare benign bone tumor composed of spindleshaped cells with minimal cytological atypia and abundant collagen production 2.
Desmoplastic fibroma of the jaws is most commonly discovered in the second and third decades with the mean age of 15. Desmoplastic fibroma definition of desmoplastic fibroma by. This soft tissue tumor typically infiltrates into the surrounding fascia or muscle and has a predilection for recurrence after surgical removal. Case reports of desmoplastic fibroma of the bone mention the presence of low to intermediate signal intensity on t2weighted mri sequences and vary in the degree of emphasis on this observation. Figure 4 photomicrograph showing monomorphic, wavy, spindled cells with. Desmoplastic fibroma of bone inwards 1991 cancer wiley. Desmoplastic fibroma of the maxilla peoples journal of scientific. Only 14 examples of desmoplastic fibroma are recorded in mayo clinic files 1. Desmoplastic fibroma is a benign but aggressive tumor composed of fibroblasts and myoblasts in heavily collagenized stroma.
In 1958, jaffe delineated desmoplastic fibroma as a rare primary bone tumor usually found in young adults 7. The incidence of desmoplastic fibroma is reported to be 0. We report a case of desmoplastic fibroma of the frontal bone in a young male. Full text available fibroma is a benign tumor of fibrous connective tissue. Nichols 10 first called attention to the occurrence of desmoid tumors in extraabdominal sites 9. Desmoplastic fibroma is a rare benign primary bone tumor that is histologically similar to the soft tissue desmoid tumor. Desmoplastic fibroma df is a rare benign neoplasm of the bone that currently accounts for 0. Although it does not tend to metastatize, it has a high local recurrence and. The tissue is described as being poorly cellular with nuclei that are ovoid or elongated. It most often involves the mandible, large long bone or iliac bone. A desmoplastic fibroma often behaves in an aggressive manner and macroscopically has a firm consistency with welldefined advancing surfaces that may extend into surrounding soft tissue. Paucicellular, bland spindled stellate and reactive appearing fibroblasts and myofibroblasts separated by.
Desmoplastic fibroma df is a benign, yet locally aggressive, tumor of the connective tissue. However, ct and mri offer the best imaging of the lesion. Although classified as benign, it frequently demonstrates aggressive behavior, often causing tooth mobility, extensive bone destruction, and has a moderate to high recurrence rate. 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. Until jaffe differentiated it from other bone fibromas and established. There was a male predominance, and 74% of the patients were in. Aug 12, 2014 the desmoplastic fibroma df is a rare, fibroblastic lesion of bone that histologically resembles the desmoid tumor of soft tissue.
The desmoplastic fibroma df is a rare, fibroblastic lesion of bone that histologically resembles the desmoid tumor of soft tissue. Although classified as benign, it frequently demonstrates aggressive behavior, often causing tooth mobility, extensive bone destruction, and has a. Desmoplastic fibroma of the bone is an extremely rare primary benign tumor. Desmoplastic fibroma is a benign but locally aggressive tumor arising usually from the mandible, pelvis and long bones with a potential for recurrence. Desmoplastic fibroma in a toe has been extremely rarely reported.
Sabo, dpm, facfas2 the foot and ankle online journal 8 2. They have been described involving a wide range of anatomic sites but most commonly arise on the upper extremity. Desmoplastic fibroma is a primary bone tumour which occurs exceedingly rarely in the cranial bones. Desmoplastic fibroblastoma df, also known as collagenous fibroma is a distinct yet uncommon benign fibrous soft tissue tumor that typically occurs in the subcutaneous tissue or skeletal muscle in adults. Jan 14, 2008 desmoplastic fibroma is a primary bone tumour which occurs exceedingly rarely in the cranial bones. Desmoplastic fibroma of bone, when found in the foot, is an exceedingly rare occurrence 1. The operation was performed with the patient under general anaesthesia. Aug 24, 2018 desmoplastic fibroblastoma is commonly seen in middleage and older adults 4070 years. It is usually seen radiographically as a welldemarcated, radiolucent lesion. This tumor grows locally in bone in an aggressive manner and destroys the bone. The main cell types that are seen include fibroblasts, myofibroblasts, and undifferentiated mesenchymal cells 9. It usually affects craniofacial bones, mandible most frequently, long bones metaphyseal femur, tibia, humerus.
Bohm p, krober s, greschniok a, laniado m, kaiserling f. Desmoplastic fibroblastoma collagenous fibroma discussion. Desmoplastic fibroma is a benign intraosseous neoplasm that is recognized as the intraosseous counterpart of soft tissue fibromatosis in both gnathic and extragnathic sites. Pathology and genetics of tumors and soft tissue and bone. Desmoplastic fibroma of bone may be extremely difficult to distinguish from lowgrade fibrosarcoma of bone. Because of its intraosseus location the working diagnosis of desmoplastic fibroma was made.
It characterized also by aggressive intramedullary proliferation of myofibroblasts. With some extraabdominal desmoids, invasion of contiguous bone has been described 2, 8, 15. Printable desmoplastic fibroblastoma surgical pathology. Jaffe first described it as a benign tumor characterized by the formation of abundant collagen fibers by the tumor cells. Desmoplastic fibroma is generally classified as a benign tumor and has not been shown to metastasize. Authors report a rare case of desmoplastic fibroma of bone occurring in the distal phalanx. Although there is a considerable overlap in the mr imaging features with other bone tumors, an.
Desmoplastic fibroma is an aggressive fibrous lesion that may rarely be encountered in the jaw fig. Pdf desmoplastic fibroma of bone is a rare benign primary bone tumor that histologically resembles the extraabdominal desmoid tumor of soft tissues find. A ctscan and mriscan were performed and revealed a bone cyst with sclerotic borders without destruction of the cortical 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. Lesions of fibrous dysplasia that show little woven bone may also be confused with desmoplastic fibroma. Hence, desmoplastic fibroma of bone is also referred to as desmoid tumor of bone 2. Accordingly the indication for operative excision via curettage and grafting was established. Frontal there is a welldefined, highly expansile, purely lytic lesion within the proximal ulna which extends to the distal third of the diaphysis. Desmoplastic fibroma definition of desmoplastic fibroma. A rare case of desmoplastic fibroma in the maxillary corpus is being presented with its pathology.
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