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PubMed1995

Cutaneous sclerotic fibroma. Immunohistochemical evidence of a fibroblastic neoplasm with ongoing type I collagen synthesis.

The sclerotic fibroma (SF) is a tumor of the skin that may occur sporadically or in the context of Cowden's syndrome. The authors studied four examples of this tumor in an effort to understand better the nature of the fibrous matrix in SF and its significance. A novel antibody was utilized that is directed against the immunoreactive amino-terminal precuror domain of human type I procollagen (AP). This peptide is usually identified only at sites of active or recent collagen synthesis and deposition. All examples of SF showed strong cellular and stromal staining for AP. The matrix also demonstrated variable staining for type IV collagen and laminin. The overall immunophenotype of this lesion suggests that it may be a specialized fibroblastic ("dermal dendrocytic?") tumor. Despite its hypocellularity and hyalinized nature, SF is thought to exhibit ongoing matrical production that supports a neoplastic character for this lesion.

Adult↗
PubMed1999

Differential expression of CD34 and Ki-M1p in pleomorphic fibroma and dermatofibroma with monster cells.

Pleomorphic fibroma (PF) and dermatofibroma with monster cells (DFMC) are characterized by the presence of numerous cells with large atypical nuclei. Despite cytologic similarities, the two entities are likely to be unrelated, but their histogenesis is poorly understood. In this study, we examined six cases of PF and eleven cases of DFMC by immunohistochemistry using antibodies against vimentin, alpha-smooth muscle actin, S-100 protein, CD34, factor XIIIa, and the pan-monocytic marker Ki-M1p. Strong vimentin expression was seen in all tumors, whereas none of them expressed S-100 protein. PF consistently exhibited CD34 staining but appeared to be depleted of Ki-M1p positive cells compared with the surrounding normal skin. Conversely, all cases of DFMC contained numerous Ki-M1p positive cells including atypical multinucleate cells, but virtually no CD34 reactivity was observed. A weak staining for alpha-smooth muscle actin was occasionally seen in a subset of the cells of both entities. Our results indicate that PF and DFMC are histogenetically distinct entities that may arise from two different types of dermal dendritic cells defined by their reactivity for CD34 and Ki-M1p, respectively. Immunohistochemistry using these two antibodies permits an easy and reliable discrimination between PF and DFMC.

Actins↗
PubMed2004

Sporadic sclerotic fibroma of the oral soft tissues.

Sclerotic fibroma (SF) is an uncommon, benign fibrous neoplasm that may present either as a sporadic, small, solitary cutaneous mass, in otherwise healthy individuals, or as solitary or multiple, discrete skin nodules in patients with Cowden syndrome. Oral SF has been reported in patients with Cowden syndrome; however we now report the first documented series of sporadic SF originating within the oral mucosa. We describe 5 cases of SF arising in 3 women and 2 men with an age range of 43 to 66 years. The buccal mucosa was the site of involvement in 4 patients and the lower lip in 1 patient. Microscopically, each of the tumors was characterized by an unencapsulated, well-circumscribed, hypocellular submucosal nodule that was sharply demarcated from the surrounding tissues. The neoplasms were primarily composed of thick collagen bundles that were occasionally arranged in a storiform pattern. Prominent clefts separated many of the collagen bundles. In all cases, spindle and stellate-shaped cells containing fusiform or stellate-shaped nuclei and inconspicuous nucleoli were found scattered throughout the lesion. Occasional stellate-shaped, multinucleated cells were also seen. Many of the cells also exhibited long dendritic cytoplasmic processes. The tumor cells strongly expressed CD34 and vimentin, and occasionally factor XIIIa, but were negative for markers of myofibroblastic, neural or melanocytic differentiation. These findings confirm that oral SF represents a unique entity and should be differentiated from more commonly occurring benign fibrous lesions of the oral soft tissues.

Adult↗
PubMed1979

Desmoplastic fibroma of bone. An ultrastructural study.

The ultrastructure of three cases of desmoplastic fibroma of bone is presented. The lesion is principally characterized by myofibroblasts admixed with lesser numbers of fibroblasts and primitive mesenchymal cells. Thus, the cellular composition is similar to that described in a variety of nonneoplastic proliferative processes of soft tissue. It is postulated that the myofibroblastic proliferation develops in response to unknown factors acting on marrow fibroblasts or primitive mesenchymal cells.

Adolescent↗
PubMed1988

Three types of spindle cell tumors of the pleura. Fibroma, sarcoma, and sarcomatoid mesothelioma.

The clinicopathologic features of 17 fibrous tumors of the pleura are presented. Eight were benign localized fibrous tumors; all of these were negative when stained with antibodies to keratin. Eight were diffuse malignant tumors that demonstrated intense immunohistochemical staining of the spindle cells with antibodies to keratin. One case presented as a histologically malignant spindle cell tumor that was initially localized but recurred subpleurally three times in 12 years. This spindle cell tumor was consistently negative when stained for keratin. We conclude that there are three types of fibrous tumors of the pleura: (a) a localized, histologically benign, keratin-negative spindle cell tumor that might be termed "fibroma," (b) a diffuse, histologically malignant, keratin-positive neoplasm that might appropriately be termed a "sarcomatoid mesothelioma," and (c) a histologically malignant, keratin-negative, spindle cell tumor with the potential to spread under the pleura that might appropriately be termed a "sarcoma." Immunohistochemistry proved to be useful in distinguishing the sarcomatoid mesothelioma from the sarcoma.

Adult↗
PubMed1991

Pleomorphic fibroma of tendon sheath.

We report an example of fibroma of tendon sheath with pleomorphic bizarre cells without mitoses. The tumor appears to be morphologically identical to a similar tumor in the skin and represents yet another example of a benign soft-tissue neoplasm with disturbing morphological features.

Fibroma↗
PubMed1994

Fibroepithelial tumor of the breast with digital fibroma-like inclusions in the stromal component. Case report with immunocytochemical and ultrastructural analysis.

A case of fibroepithelial tumor of the breast in a 34-year-old woman, showing digital fibroma-like inclusions in the stromal component is presented. Morphological, immunocytochemical, and ultrastructural findings indicate that the peculiar eosinophilic intracytoplasmic inclusions observed may represent tightly packed actin filaments. Although they are more frequently detected within digital fibrous proliferations of infancy, their rare occurrence at either unusual site or in adulthood has been reported. Similar inclusions have also been rarely observed in intranodal myofibroblastoma and in benign leiomyomas. These findings suggest that these inclusions may be the result of some metabolic or organisational abnormality shared by neoplastic actin-rich cells. Myofibroblastic and smooth muscle lesions are probably the commonest to show this defect.

Actins↗
PubMed2004

Distinctive prepubertal vulval fibroma: a hitherto unrecognized mesenchymal tumor of prepubertal girls: analysis of 11 cases.

Eleven cases of a distinctive previously unrecognized mesenchymal tumor that arises in the vulvar region in the prepubertal years are described. The tumors presented in the vulva (8 cases arising from labia majora) of prepubertal girls (range, 4-12 years; median, 8 years). The preoperative diagnoses were labial mass or swelling, not otherwise specified in 3 cases, hemangioma in 2 cases, lipoma in 2 cases, and lymphangioma and Bartholin cyst/lymphedema in 1 case each. The tumors were unilateral, ill defined, located in the submucosa or subcutaneous tissue, and ranged in size from 2.0 to 8.0 cm in maximum dimension. Microscopically, they were poorly marginated, hypocellular neoplasms composed of bland spindle-shaped cells in a variably collagenous to edematous or myxoid stroma, diffusely infiltrating between preexisting normal vascular, adipose, and neural tissues. No cytologic atypia was identified. Mitotic activity was minimal with no abnormal mitotic figures. The tumor cells were immunoreactive for CD34 (8 of 9 cases) but not for smooth muscle actin, desmin, and S-100 protein. Treatment was by local excision. From the follow-up data available for 9 patients, 3 patients showed locally recurrent tumor after 4, 6, and 13 months, respectively, of which the second case showed a second recurrence 18 months after the first excision. The term "prepubertal vulval fibroma" is suggested because it reflects the distinct features of this seemingly unique, previously uncharacterized, site- and age-specific mesenchymal tumor.

Child↗
PubMed1987

Desmoplastic fibroma of the mandible.

A desmoplastic fibroma of the mandible in an 11-year-old girl provided a difficult diagnosis because of its similarity, both clinically and histologically, to a low-grade fibrosarcoma following its rapid recurrence after primary local curettage. Immediate one-stage microvascular musculocutaneous reconstruction served to allow complete excision of the lesion and minimize the degree of psychological trauma. The differential diagnosis and the significance of cartilagenous elements present in the lesion are discussed.

Child↗
PubMed1987

Detection of benign cardiac fibroma on thallium-201 imaging in an adult.

A rare case of benign cardiac fibroma causing a focal defect on Tl-201 imaging in an adult patient is presented. The abnormalities on the Tl-201 scan are correlated with other studies performed on the same patient. The usefulness of the Tl-201 myocardial perfusion scan as an early, noninvasive test in cardiac workup is stressed.

Adult↗
PubMedDate not supplied

Ossifying fibroma of sphenoid bone with coexistent mucocele: CT and MRI.

A case of a 26-year-old woman with decreasing vision in her right eye, diplopia, headache, and galactorrhea is presented. Both CT and MR studies showed a large sphenoid fibroosseous lesion that had a ground glass appearance on CT and low-to-intermediate proton density and low T2-weighted and low T1-weighted signal intensities on MR. After contrast medium administration, this process diffusely enhanced on CT and MR. There was also an expansile mass in the sphenoid sinus that had intermediate proton density, high T2-weighted and low T1-weighted signal intensities compared with brain. This mass did not enhance but had an intensely enhancing, uniformly thin rim. The pathologic diagnosis was ossifying fibroma with a sphenoid sinus mucocele. There are only isolated reports in the literature of benign fibrosseous lesions causing mucoceles. This association is reviewed as are the findings in this case.

Adult↗
PubMed1991

Ossifying fibroma in the sylvian fissure: case report.

A rare case of an ossifying fibroma that developed in the sylvian fissure is reported here. During surgery, branches of the middle cerebral artery were encased in osseous tumor. The encased branches were divided and reanastomosed after the mass was removed. Postoperative angiograms demonstrated patency of the anastomosed arteries and a postoperative magnetic resonance imaging scan revealed no infarction distal to the arterial anastomoses. The authors recommend division and anastomosis of the encased arteries rather than partial removal of tumor from around the encased arteries, resulting in residual tumor being left behind.

Adult↗
PubMed1977

Desmoplastic fibroma of the hand: case report.

We present a patient who had a desmoplastic fibroma at the base of the metacarpal of his long finger. The operation to remove the lesion and to reconstruct the metacarpal is described. The history of the experiences with this lesion is reviewed.

Adult↗
PubMed1982

Resection of a giant ossifying fibroma through an intraoral approach in a 9-year-old child: immediate reconstruction and 6-year cephalometric follow-up.

An unusual opportunity was afforded to study the growth and development of the facial structures of a 9-year-old child who underwent major mandibular reconstruction. The longitudinal studies confirmed the present concepts of the factors responsible for mandibular growth as well as their repercussions on other facial structures. An intraoral subperiosteal resection of a major portion of the right hemimandible sparing the upper part of the mandibular ramus was required to eradicate a large ossifying fibroma. An iliac bone graft consisting of the outer table of cortical bone and cancellous bone was placed within the mucoperiosteal sac to repair the defect. It also was used to control the ramus remnant. The patient was followed for 6 years. The growth of the reconstructed mandible was in effect nearly symmetrical with the unaffected contralateral portion of the mandible, resulting in minimal facial asymmetry. The mucoperiosteum provided a vascular bed for the bone graft and the buccal sulcus was preserved, thus providing a retentive ridge and sulcus for a denture without the need to perform a skin or mucosal graft inlay procedure. The symmetrical growth of the mandible is attributed to the growth of the ramus by remodeling, resorption, deposition, and relocation and by the muscle-bone interface (the functional matrix).

Bone Transplantation↗
PubMed1984

Bilateral calcified ovarian fibromas.

A 12-year-old girl with abdominal pain and occasional dark vaginal discharge was found upon abdominal x-ray examination to have amorphous pelvic calcification. Calcification can occur in ovarian fibromas and should be considered as a cause of amorphous pelvic calcification in female patients. When bilateral, calcified, and nodular, the possibility of basal cell nevus syndrome is suggested.

Calcinosis↗
PubMed2001

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.

Fibroma, Desmoplastic↗
PubMed2004

Juvenile active ossifying fibroma with massive involvement of the mandible.

Fibro-osseous lesions of the maxillofacial complex are often difficult to diagnose from both a clinical and a histopathologic point of view. The parameters for the diagnosis of juvenile active ossifying fibroma are as follows: a patient under 15 years of age, localization of the tumor, the radiologic aspect, and the tendency to recur. Although many authors favor conservative surgery rather than radical en bloc resection, immediate recurrence characterized by a high aggressive growth rate and the absence of a distinct separation between the tumor and the adjacent bone requires ex- tensive surgery, with wide demolition of the involved bone.

Child↗