Cases reported "Fibroma, Desmoplastic"

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1/19. Collagenous fibroma (desmoplastic fibroblastoma): genetic link with fibroma of tendon sheath?

    We observed clonal chromosome abnormalities in two fibrous soft tissue tumors diagnosed as collagenous fibroma (desmoplastic fibroblastoma). The involvement of the same band of the long arm of chromosome 11, 11q12, was observed in both tumors. The presence of hitherto unreported similar chromosomal abnormalities in this tumor supports the neoplastic nature of this lesion. In addition, a possible relationship with fibroma of tendon sheath, which also shows rearrangement of 11q12, is suggested. 11q12 might be a common genetic denominator of benign fibroblastic lesions, such as collagenous fibroma and fibroma of tendon sheath.
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2/19. Collagenous fibroma (desmoplastic fibroblastoma).

    Collagenous fibroma (desmoplastic fibroblastoma) is a recently described tumor that may arise in the subcutaneous tissue or skeletal muscle. We report a case of collagenous fibroma, occurring on the forehead of a 67-year-old man. An awareness of this entity is necessary to avoid confusion with other soft tissue neoplasms, especially extraabdominal fibromatosis.
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keywords = neoplasm, soft
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3/19. Selected case from the Arkadi M. Rywlin International pathology Slide Seminar: desmoplastic fibroblastoma (collagenous fibroma).

    Desmoplastic fibroblastoma (collagenous fibroma) is a rare and recently described benign soft tissue tumor. It is more common in men, median age is 50 years, the history is usually long, and the usual size is around 3 cm. Most lesions are subcutaneous but approximately 25% involve skeletal muscle. Histologically, they are sparsely cellular with stellate and spindle-shaped fibroblasts-myofibroblasts that are separated by a collagenous or myxocollagenous matrix. Mitoses are minimal or absent. Tumor cells are focally positive for muscle-specific and alpha smooth muscle actins, and rarely, for keratins. The S-100 is usually negative but a very few cases have been positive. It can be confused with one of the fibromatoses, myxomas of various kinds and neural tumors. It neither recurs nor metastasizes.
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4/19. Collagenous fibroma (desmoplastic fibroblastoma) of the palate: a case report.

    Collagenous fibroma is an uncommon benign soft tissue lesion that has a wide anatomic distribution. We describe a case of a collagenous fibroma that appeared in the left soft and hard palate of a 37-year-old woman as a 5.0-cm solitary, firm nodule. Microscopically, it was composed of stellate or spindle-shaped cells embedded in hypovascular fibrous stroma. Entrapment of fat was focally identified at the edges. Mitotic figures and tumor necrosis were absent. Tumor cells were immunopositive for vimentin, and a few cells were positive for alpha-smooth muscle actin. Tumor extracellular matrix was immunopositive for type I and type III collagen, as well as for fibronectin. These findings satisfied the diagnostic criteria for collagenous fibroma (desmoplastic fibroblastoma). This case, to our knowledge, represents the first report of this tumor in the mouth. The differential diagnosis of fibrous lesions of the mouth is discussed.
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5/19. Cross-sectional imaging patterns of desmoplastic fibroma.

    The aim of this study was to work out the cross-sectional imaging characteristics of desmoplastic fibroma (DF). In 3 patients with histologically proven DF, the imaging characteristics obtained with cross-sectional techniques were reviewed retrospectively. Radiographs and CT scans were available in all patients, and plain and contrast-enhanced MR examinations in 2 patients. Compared with conventional radiographs, CT allowed more accurate assessment of the extent of bone destruction including cortical breakthrough and articular invasion. Intramedullary tumor growth and soft tissue extension was best detected with MRI. Apart from heterogeneity on MR images, DF displayed nonspecific low signal intensity on unenhanced T1-weighted images and an intermediate to high signal intensity including areas of low intensity on T2-weighted images. Desmoplastic fibroma showed a distinct, inhomogeneous gadolinium enhancement. Although cross-sectional imaging features of DF are nonspecific, some MR characteristics, such as inhomogeneous contrast enhancement and the presence of low-intensity regions on T2-weighted images, are helpful in determining the differential diagnosis. Cross-sectional imaging of DF is useful for local staging of the tumor because it provides valuable information about the extent of bone destruction as well as medullary and extraosseous spread.
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6/19. Desmoplastic fibroma of the rib.

    Desmoplastic fibroma is a very rare primary tumor of bone, closely related to aggressive fibromatosis of soft tissue. 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. Recognition of this entity is important to ensure proper surgical treatment. According to the published data, the tumor is most common in the long tubular bones (56%), the mandible (26%), and the pelvis (14%). Rib involvement by desmoplastic fibroma is extremely rare, and to our knowledge, only 3 cases have been reported in the literature to date. We present the case of a desmoplastic fibroma in the rib of a 19-year-old man, adding a fourth case to the previously reported cases involving this unusual location. The clinical history and the radiological and pathologic findings are presented.
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7/19. Desmoplastic fibroma of the cranium: case report and review of the literature.

    OBJECTIVE AND IMPORTANCE: Desmoplastic fibroma is a benign bone tumor that can be locally aggressive. It usually occurs in the long bones and mandible. We report on a patient with a desmoplastic fibroma arising in the temporal bone and review previously published cases of desmoplastic fibroma originating within the cranium. CLINICAL PRESENTATION: A 43-year-old woman presented with a 12-year history of progressively worsening head asymmetry. magnetic resonance imaging and computed tomography demonstrated a mass originating from the bone and involving the adjacent soft tissues. INTERVENTION: A temporal parietal craniectomy was performed with excision of a large tumor involving the bone. An acrylic cranioplasty was used to replace the bone. Pathological examination of the lesion identified desmoplastic fibroma of the cranium. After surgery, the patient's cranial asymmetry was corrected. CONCLUSION: Desmoplastic fibroma of the cranium is rare. Surgical resection is the treatment of choice.
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8/19. Collagenous fibroma (desmoplastic fibroblastoma).

    A Collagenous fibroma (Desmoplastic Fibroblastoma) is a rare, benign, slowly growing, fibroblastic, soft tissue lesion. Here, the case of a 28-year-old woman, who presented with a 1-year history of a slowly growing painless mass in the right anterior aspect of her neck, is described. This type of tumor was first described by Evans in 1995, and named as a Desmoplastic fibroblastoma but was renamed, by Nielsen in 1996 as a Collagenous fibroma. This type of tumor is frequently reported in men with a mean age at occurance of 50 years. Clinically, a Collagenous fibroma presents as a firm, well-circumscribed subcutaneous, or intramuscular, painless mass of long duration. They are mostly located in the neck and extremities. The tumors range in size from 1 to 20 cm and predominantly occurs within the subcutaneous tissue, but fascial and skeletal muscle involvement is common. The treatment of a Collagenous fibroma is a total surgical excision. No tumor recurrence has been reported the literature during the follow-up period and no tumor recurrence was observed in our case at the 1-year follow-up.
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9/19. Desmoplastic fibroma-like tumor of maxillofacial region associated with tuberous sclerosis.

    Desmoplastic fibroma is a rare primary tumor of bone that histologically and biologically mimics the extra-abdominal desmoid tumor of soft tissue. It usually presents in patients during the first three decades of life and often involves the mandible or long bones of the skeleton. Its clinical behavior is characterized by a locally aggressive, infiltrating, and destructing course, often with invasion of surrounding tissues but without metastasis. We present herein the clinicopathological features of a desmoplastic fibroma-like tumor involving the left maxillofacial region in a 14-year-old Hispanic boy with tuberous sclerosis.
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10/19. Collagenous fibroma (desmoplastic fibroblastoma): a new case originating in the palate.

    A 49-year-old woman presented with a painless slowly growing but huge polypoid palatal mass. On magnetic resonance imaging (MRI), areas of low signal intensity were consistent with a fibrous tumour. T(1) and T(2) weighted MRI after Gd-DTPA administration highlighted a well-circumscribed, purely soft tissue process. Histological analysis of the operative specimen detected collagenous fibroma (desmoplastic fibroblastoma). The patient remains disease-free 4 years after excision.
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