Desmoplastic fibroma of the rib archives of pathology. Pdf desmoplastic fibroma df is a rare bone tumor, which is known to involve mostly the gnathic bones. Pdf desmoplastic fibroma of bone is a rare benign primary bone tumor that histologically. Presentation in the rib is extremely rare with only six cases reported previously.
Moreover, a report of an interesting case is included localized in the mandibular corpus. This showed a cystic enlargement at the left angle of the mandible with no cortical disruption or soft tissue involvement. Although classified benign, this intraosseous lesion exhibits local aggressiveness and has a high potential for recurrence. If cortical destruction and a soft tissue mass is noted, desmoplastic fibroma of bone may resemble more sinister pathology, such as fibrosarcoma, intraosseous. The mr imaging characteristics in our case are compared to those previously reported. Desmoplastic fibromas are extremely rare bone tumors that do not metastasize but may be locally aggressive. 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 df is a rare bone tumor, which is known to involve mostly the gnathic bones. Desmoplastic fibroma of bone is a rare myofibroblastic tumor, locally aggressive, benign lesion that histologically resembles a desmoid tumor of the softtissue.
Desmoplastic fibroma of bone is a very rare benign tumor, which may be locally aggressive. A rare and benign chondrogenic lesion characterized by variable amounts of chondroid, fibromatoid and myxoid elements. This is a case report on an unusual presentation of desmoplastic fibroma of bone. Df earned its appellation as a result of its morphological resemblance to desmoidtype. Nielsen gp, oconnell jx, dickersin gr, rosenberg ae. Desmoplastic fibroma may also be misdiagnosed as a nonossifying fibroma.
Df earned its appellation as a result of its morphological resemblance to desmoidtype fibromatosis. It characterized also by aggressive intramedullary proliferation of myofibroblasts. Desmoplastic fibroma of bone is a rare locally aggressive, but nonmetastatic tumor. Desmoplastic fibroma was first recognized as a distinct kind of tumor in 1958 by henry l. Case report the aim of this report is to present a rare case of a child with a desmoplastic fibroma df at the mandibular angle. Imaging findings of desmoplastic fibroma rarely involving. Pathology and genetics of tumours of soft tissue and bone. 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. Scanning power view reveals a tumour comprised of multiple nodules situated within the dermis figure 1. Imaging findings of desmoplastic fibroma rarely involving the.
Desmoplastic fibroma of the rib with cystic change. Radiologically, the tumor is usually characterized by an expansile lytic bone lesion. 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 benign but locally aggressive tumor arising usually from the mandible, pelvis and long bones with a potential for recurrence. 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.
The majority of dfs occur in the long bones, mandible and ilium. Classified as one of the tumours of the follicular epithelium, trichoepithelioma is thought to arise from the hair germ. Jul 31, 20 dermatopathology reference describes oral fibroma histopathology including histologic features and provides links to additional medical references. Desmoplastic fibroma of the mandible a rare benign tumor. Desmoplastic fibroma of bone is an intraosseous, locally aggressive neoplasm, constituted by. Among the ameloblastomas, the desmoplastic variation is rare. This article reports a new case of this unusual neoplasm in a 66 yearold woman in which the main complaint was an asymptomatic swelling located in the right body of mandible. The incidence of desmoplastic fibroma is reported to be 0. Unlike most other benign lesions, a desmoplastic fibroma tends to be very aggressive at the local site.
Although classified as benign, it frequently demonstrates aggressive behavior, often causing tooth mobility, extensive bone destruction, and has a moderate to high recurrence rate. Hence, desmoplastic fibroma of bone is also referred to as desmoid tumor of bone 2. Aug 12, 2014 the desmoplastic fibroma df is a rare, fibroblastic lesion of bone that histologically resembles the desmoid tumor of soft tissue. Desmoplastic fibroma is a rare primary tumor of bone that histologically and biologically mimics the extraabdominal desmoid tumor of soft tissue. On histopathologic examination, this tumor shows gradual progression with well differentiated cells that produce collagen. Desmoplastic fibroma is a benign but aggressive tumor composed of fibroblasts and myoblasts in heavily collagenized stroma. Mar 27, 2020 a desmoplastic fibroma is a type of benign bone tumor. The desmoplastic ameloblastoma da is characterized by specific clinical, imaging, and histological features. Most commonly affected site in the head and neck region is mandible. By contrast, desmoplastic fibroma has an expansile destructive pattern. Specific fibromas include nonossifying fibroma, found in the large long bones. Microcystic adnexal carcinoma of the upper lip misdiagnosed benign desmoplastic trichoepithelioma. The desmoplastic fibroma df is a rare, fibroblastic lesion of bone that. 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.
Printable desmoplastic fibroblastoma surgical pathology. Up until now, less than 150 patients have been reported in the literature. Among visceral metastatic sites, cutaneous melanoma cm metastasises initially to the liver in 1420% of cases. Printable fibroma of tendon sheath surgical pathology. Liver metastases in cm patients are associated with both poor prognosis and poor resp. They are considered to be a bony counterpart of soft tissue desmoid tumors and are histologically identical. A case of desmoplastic trichoepithelioma with ossification. Lesions are well demarcated with a sclerotic rim and based on the cortex. We report a case of desmoplastic ameloblastoma in a 45yearold female with a painless swelling in the left anterior. An unusual case of a desmoplastic fibroma of the temporal bone is presented. Desmoplastic fibromalike tumor of maxillofacial region. Histopathologic examination of the decalcified specimen revealed. With symptoms persisting the patient underwent exploration of the area under general anaesthesia and histopathology which had previously been negative now revealed desmoplastic fibroma.
Desmoplastic fibroma histologically and biologically mimics the soft tissue desmoid tumor. Abstract desmoplastic fibroma is a very rare primary tumor of bone, closely. Desmoplastic fibroblastoma collagenous fibroma of the tongue. Desmoplastic fibroma of the vidian canal in a child.
The most common site of involvement is mandible reported incidence 22% of all desmoplastic fibroma cases followed by metaphysis of long bones. Dermoscopy of desmoplastic trichoepithelioma reveals other criteria to distinguish it from basal cell carcinoma. It usually affects craniofacial bones, mandible most frequently, long bones metaphyseal femur, tibia, humerus. Mar 18, 2011 desmoplastic ameloblastoma is a rare variant of ameloblastoma. Desmoplastic fibroma an overview sciencedirect topics. Pathology of desmoplastic fibroblastoma collagenous fibroma. Desmoplasia definition of desmoplasia by medical dictionary. 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.
Desmoplastic fibroma involving the clavicle is extremely rare, with only 2 reported cases before 1985. Desmoplastic fibroma of the frontal bone rahul lath, alok ranjan, b. Desmoplastic fibroblastoma collagenous fibroma of the. Its a very rare tumor with slow growth and produces collagen. Replacement and desmoplastic histopathological growth. Desmoplastic fibroma is an extremely rare tumor of bone with a reported incidence of 0. Small horn cysts, abortive hair follicles and calcification are frequently seen figure.
They have been described involving a wide range of anatomic sites but most commonly arise on the upper extremity. In this case report, we present a desmoplastic fibroma in an unusual location, the clavicle. We report a case of desmoplastic fibroma of the frontal bone in a young male. However, the radiographic features of nonossifying fibroma are characteristic. It usually presents in patients during the first three decades of life and often involves the mandible or long bones of the skeleton. In contrast to the welldocumented radiological appearance, the literature on mr imaging features of this tumor is scarce. Most such lesions on the hands are labeled fibroma of tendon sheath. Histopathology images of desmoplastic small round cell tumor. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. It primarily occurs in the abdominal cavity associated with mesentery and peritoneum and presents as large abdominal mass with peritoneal effusion. Desmoplastic fibroma of the frontal bone lath r, ranjan a.
Desmoplastic fibroma df is a rare, locally aggressive bone tumour, first described by jaffe in 1958. This tumor grows locally in bone in an aggressive manner and destroys the bone. This rare condition occurs predominantly in the mandible and in the long bones and is seldom seen in the calvarium. Pdf desmoplastic fibroma df is an exceedingly rare benign primary tumor of bone, first described in 1958. Desmoplastic fibroma of the temporal bone the journal of. Although there is a considerable overlap in the mr imaging features with other bone tumors, an. Desmoplastic fibroma df is a benign, rare fibroblastic intraosseous neoplasm histologically resembling a desmoid soft tissue tumor. The here presented retrospective analysis investigated the clinicoradiographic features of an overall of 115 dacases, having been reported in literature from 1984 to 2008. Request pdf desmoplastic fibroma of mandible in a child. Ratnam, swarnalata gowrishankar departments of neurosurgery and pathology, apollo hospitals, hyderabad 500 033, andhra pradesh, india desmoplastic fibroma is a benign but locally aggressive tumor arising usually from the mandible, pelvis and long. In this study we present a rare case of peripheral desmoplastic ameloblastoma and discuss its clinical features, histopathology, and inmunoshistochemical profile. Desmoplastic fibroma of bone is an uncommon myofibroblastic tumor characterized by local. In this setting, the clinical presentation is usually represented by a bulging tumor of the face.
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