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[Large ossifying fibroma of the jaws and hyperparathyroidism in a chronic hemodialyzed patient].

OBJECTIVE: Describe the exceptional and fortuitous character of the association of ossifying fibroma and hyperparathyroidism in a chronic hemodialyzed patient. CASE REPORT: A twenty-year-old man who had undergone dialysis for ten years as the result of an indeterminate nephropathy was admitted for a functional disability in the standing position and a serious dysmorphic syndrome with swelling of the jaws that hindered proper closure of the mouth. RESULTS: Medical imagery revealed a polyostotic attack pleading in favor of renal osteodystrophy or a fibrous dysplasia. The surgical reduction of the tumor of the jaws shown, from the histological viewpoint, a large aggressive ossifying fibroma of the jaws. CONCLUSION: The association of ossifying fibroma and hyperparathyroidism in a chronic hemodialyzed patient is exceptional and fortuitous. The difficult treatment points out the need for early rigorous prevention of hyperparathyroidism in a chronic hemodialyzed patient.

Adult↗

Pleural fibromas: a clinical review and report of six patients.

Localized pleural fibromas are a definite clinical entity. Their origin is much debated. Though most arise from the mesothelial cell, occasionally some arise from the pleural fibroblast. The former retain the potential to become malignant. Distinguishing between the two origins can aid prognosis after treatment. The characteristic cell is a spindle-shaped fibroblast. Slits or clefts lined by flattened cells are often present in the tumor. Clinically, pleural fibromas are usually asymptomatic, space-occupying lesions. Chest symptoms are nonspecific. Extrathoracic symptoms, especially arthritis, are not uncommon and occur only in the benign variety. Excision is the treatment of choice, but long-term follow-up is essential, for recurrence is not unknown and is often heralded by the region of arthritic symptoms. Recurrences may be benign or malignant, the latter having a poor prognosis, with most patients dying within 2 years. Experience with benign pleural fibromas seen in 6 patients over a 25-year period is presented.

Diagnosis, Differential↗

The clinical spectrum of cardiac fibroma with diagnostic and surgical considerations: noninvasive imaging enhances management.

A cardiac fibroma was successfully resected from the interventricular septum of a 25-year-old woman. The clinical data were correlated with a review of the data on 144 other patients, thereby providing a clinical profile and management strategy for this type of tumor. Initial manifestations of a fibroma were determined to be congestive heart failure (21%), tachyarrhythmias (13%), and chest pain (3.5%). A majority of patients were asymptomatic (36%) with abnormal physical findings or an abnormal chest roentgenogram. Finding the tumor at autopsy incidentally or on sudden death (23%) indicated the lethal potential. A few (3.5%) of the reports on patients with cardiac fibroma were without clinical data. Noninvasive imaging by echocardiography, computed tomography, and nuclear magnetic resonance improved the diagnosis. Surgical treatment was successful in 53 of the 84 patients for whom it was attempted.

Adult↗

Epibulbar subconjunctival fibroma. A tumor possibly arising from Tenon's capsule.

A fibroma, a benign neoplasm arising from the periorbita or tendon sheaths, is described in a 74-year-old woman as an indolent, lobular tumor situated in the episcleral and subconjunctival connective tissues with superior limbal fixation. Because most fibromas arise from tendon sheaths or fascial planes, we speculate that our lesion arose from Tenon's capsule. The rarity of periocular fibroma is documented by a review of the modern ophthalmic literature. Histopathologically, the tumor was composed of a paucicellular population of fibroblasts, sometimes displaying hyperchromatic nuclei and widely separated by abundant broad bundles of collagen. Mast cells were infrequently dispersed in the lesion. A wide local excision was performed, and no recurrence has developed during an 18-month follow-up period.

Aged↗

Translocation (1;4)(p31;q34) in nonossifying fibroma.

Nonossifying fibroma is a benign, lytic lesion of fibrous origin most often observed in the metaphyseal region of the long bones in children and adolescents. It is frequently asymptomatic and is often characterized by a history of spontaneous resolution. Herein, we report a clinicohistopathologically typical case of nonossifying fibroma arising in the tibia of an 18-year-old skeletally mature female. Conventional cytogenetic analysis revealed a reciprocal translocation involving bands 1p31 and 4q34 [t(1;4)(p31;q34)]. To the best of our knowledge, this is only the second reported case of a clonally aberrant nonossifying fibroma.

Adolescent↗

[Desmoplastic fibroma of the rib: two case reports].

Desmoplastic fibroma is a very rare primary tumor of bone, closely related to aggressive fibromatosis of soft tissue. Although considered a benign lesion, it can be very aggressive locally and has a high rate of local recurrence after incomplete surgical excision. Radiologically, the lesion shows no distinctive features, often simulating osteosarcoma, fibrous dysplasia, or fibrosarcoma. Rib involvement by desmoplastic fibroma is extremely rare. We present two cases of desmoplastic fibroma involving this unusual location with lytic costal lesion and chest wall extension. Histological examination after surgical resection revealed that the tumor consisted of spindle cells with small, elongated nuclei in a background of numerous collagen fibers and infiltrating lamellar bone. There was no evidence of nuclear atypia, mitotic activity, or necrosis. We also discuss histological differential diagnosis as well as clinical features and the radiological and pathologic findings of this rare disease. Recognition of this entity is important to ensure proper surgical treatment.

Biomarkers, Tumor↗

Benign fibromatous tumor (fibroma) of the kidney: a case report.

Benign fibromatous tumor (fibroma) of the urogenital tract is a distinctive pathologic entity occurring in the testis, paratesticular structures and renal peripelvis. We report a well-circumscribed tumor replacing the cortex and the medulla of more than half of the upper kidney, radiographically thought to be renal cell carcinoma. Histologically, the tumor was characterized by a variable cellularity and was composed of bland spindle-shaped cells loosely dispersed in a fibromyxoid to densely fibrous stroma in which calcifications and chronic inflammation were not observed. Immunocytochemistry showed that cells were positive for vimentin and, only focally, stained positive for desmin and alpha-smooth muscle actin. Differential diagnosis included a wide spectrum of benign and malignant spindle cell tumors. The clinicopathologic features were consistent with benign fibromatous tumor (fibroma) of the kidney. To our knowledge, this is the first case of benign fibromatous tumor (fibroma) of the kidney reported in the English literature.

Aged↗

Periungual fibroma.

Two cases of periungual fibroma of the hand are described. Lesions caused by periungual fibroma place pressure on the nail matrix, which results in marked nail deformities. In both cases, the nail deformities were improved by excision of the fibroma.

Adult↗

Distinguishing features of focal cemento-osseous dysplasia and cemento-ossifying fibromas. II. A clinical and radiologic spectrum of 316 cases.

The distinguishing histopathologic features of focal cemento-osseous dysplasia (FCOD) (including lesions occurring in both anterior and posterior jaws) and cemento-ossifying fibroma (COF) (ossifying fibroma and cementifying fibroma) were demonstrated in our earlier work. The aim of the current study was to further refine their clinical and radiographic features. We have assessed 18 clinical and radiographic parameters by univariate comparisons (chi-squared and Student t tests), and a multivariate assessment (logistic regression) in 241 cases of FCOD and 75 of COF. These cases were diagnosed from a combination of clinical, radiographic, and histopathologic information. FCOD was seen predominantly in black women, with a peak incidence in the fourth and fifth decades, whereas COF showed no female predilection except in the fourth decade (p < 0.005). COF occurred in patients an average of 10 years younger than patients with FCOD (p < 0.0001). Most patients with FCOD were asymptomatic (62%); the average lesion size was 1.8 cm. More than half of patients with COF displayed jaw expansion and a considerably larger size lesion (mean 3.8 cm, p < 0.001). The mandible was the most frequent site for both FCOD (86%) and COF (70%). Radiographically, a well-defined border was observed in 53% of cases of FCOD and 85% of cases of COF (p < 0.01). Cases of FCOD mostly demonstrated an irregularly mixed radio-opacity (69%), whereas 53% of COFs presented as a radiolucency (p < 0.005). In FCOD, there was a close association with tooth apices (70.6%, p < 0.0001) or with previous extraction sites (21%, p < 0.05); however, the majority of COF cases (86%) showed no relationship with either. Combining the radiographic feature of a periapical location with the pathology of multiple curetted fragments and "ginger root" bony trabeculae, allowed 90% sensitivity and 89% specificity in a logistic regression model to predict the lesion to be an FCOD. These findings provide guidelines not only to distinguish these two entities clinically, but also aid in reaching an accurate diagnosis histopathologically.

Adolescent↗

Central odontogenic fibroma of mandible: a case report and review of the literature.

Central odontogenic fibroma is an extremely rare benign tumor, accounting for less than 0.1% of all odontogenic tumors. The most recent literature review revealed that only 69 cases have so far been reported in the English literature, and only 1 of these cases showed radiologic appearance of a dentigerous cyst and this involved impacted maxillary incisors. A case of central odontogenic fibroma occurring in the mandible of a 30-year old female with radiological appearance of a dentigerous cyst involving an impacted lower first molar, presented here, appears to be the first reported case of central odontogenic fibroma of mandible with radiological appearance of dentigerous cyst in the English-language literature.

Adult↗

[Juvenile aponeurotic fibroma. A case report with a review of the literature].

Juvenile aponeurotic fibroma is a rare benign tumour which occurs mainly in young patients under 20 years of age and especially during childhood. Clinical presentation is a unique, hard and painless tumour of the palm or sole. The treatment commonly accepted for this locally recurrent tumour is complete excision with function preservation. In this paper, we report on a case of juvenile aponeurotic fibroma of the thenar area with a thickening of the first metacarpal bone shaft. Bone involvement in juvenile aponeurotic fibroma is a very rare condition. We only found one report of a comparable case in literature.

Adolescent↗

Superficial collagenous fibroma: immunohistochemical, ultrastructural, and flow cytometric study of three cases, including one pemphigus vulgaris patient with a dermal mass.

Collagenous fibroma (desmoplastic fibroblastoma) is an extremely rare benign soft tissue tumor of fibroblastic origin. The majority of reported cases have been located in the deep subcutis, fascia, aponeurosis, or skeletal muscle of the extremities, limb girdles, or head and neck regions. There has been no mention of underlying diseases in patients who developed this tumor. We here report an additional three cases of superficial collagenous fibroma, one of which was a dermal lesion occurring in the abdomen of a 26-year-old male patient with a 5-year history of pemphigus vulgaris prior to development of the tumor. To the best of our knowledge, an association between collagenous fibroma and pemphigus vulgaris has not previously been reported. The remaining two tumors were located in the superficial subcutaneous tissue of the infrascapular area and right foot, respectively. There was no tumor recurrence or metastasis during follow up of 18, 25, and 47 months, respectively. All three tumors were well-circumscribed and unencapsulated without infiltrating borders. Histologically, the common denominator of all three cases was paucicellular proliferation of spindle or stellate fibroblasts enmeshed within an extensively collagenous background. Immunohistochemically, there was diffuse strong staining for vimentin and intense focal reaction for smooth muscle actin in two tumors tested. Electron microscopy revealed features consistent with a fibroblastic or myofibroblastic lineage. Flow cytometry in two cases demonstrated a diploid DNA content with low S-phase fractions, which correlated with minimal MIB-1 nuclear labeling (less than 1%) and benign behavior of this entity.

Actins↗

Hybrid central giant cell granuloma and central odontogenic fibroma-like lesions of the jaws.

Ten lesions from eight cases are presented of a rare intra-osseous jaw lesion with the combined histological features of giant cell granuloma and central odontogenic fibroma. Lesions arose over a wide age range and presented as monolocular or multilocular radiolucencies with cortical expansion and, in one case, perforation. Two lesions recurred after curettage, one being eradicated by a second curettage and one by conservative excision. Histologically, zones of typical giant cell granuloma lay in a fibrous stroma containing islands, strands and clusters of epithelial cells. Islands often contained duct-like spaces or hyaline basement membrane globules. Trabeculae of osteoid were present in five lesions. Recurrent lesions showed features identical to the initial lesion, including recurrence of the prominent epithelial component. These features cannot be conclusively ascribed to a variant of either giant cell granuloma, central odontogenic fibroma or aneurysmal bone cyst, but the clinical features are slightly more suggestive of giant cell granuloma. Attention is drawn to the characteristic and potentially confusing histological appearances. The presence of giant cell granuloma-like areas in central odontogenic fibroma-like lesions is associated with an increased risk of recurrence following curettage.

Adolescent↗

Nuchal fibroma associated with scleredema, diabetes mellitus and organic solvent exposure.

A case of scleredema diabeticorum of Buschke associated with nuchal fibroma and organic solvent exposure is reported. The patient presented with a neck mass causing discomfort and restriction of movement. Histological examination showed this to be a nuchal fibroma. Additionally, there was widespread induration of the skin of his trunk which was asymptomatic. A biopsy showed features of scleredema. This is the first reported association of these two conditions, both of which show increased and thickened collagen bundles without significant fibroblast proliferation. They differ by the occurrence of mucin in scleredema, although this is not always demonstrable, particularly in late lesions. The possibility that nuchal fibroma is an end stage, localized form of scleredema is canvassed. The patient's medical history included insulin-dependent diabetes mellitus with complications of retinal vessel thrombosis and peripheral neuropathy. The patient also had significant past exposure to a wide variety of chemicals, including organic solvents.

Diabetes Mellitus, Type 1↗

Testicular (gonadal stromal) fibroma: case report and review of the literature.

A 25-year-old man presented with complaint of a painless enlargement in his left testis. The solid, encapsulated, circumscribed and grayish-white testicular mass displayed the characteristics of testicular fibroma histologically. It was composed of acellular collagenized plaques and hypercellular areas of fibroblastic spindle cells. Immunohistochemically, the neoplastic cells were positive for vimentin and smooth muscle actin, but not for cytokeratin, S-100 protein, desmin, CD99/MIC2 (a protein expressed by Sertoli cells and granulosa cells) and CD34. Only 18 cases of testicular (gonadal stromal) fibroma composed exclusively of spindle cells have been reported to date. An additional case of fibroma, which lacks definite neoplastic sex cord elements, and its differential diagnosis from other mesenchymal lesions of testis are discussed here, together with other cases in the literature.

Actins↗

Use of CO2 laser in the treatment of periungual fibromas associated with tuberous sclerosis.

BACKGROUND: Tuberous sclerosis complex is an autosomal dominant disorder with variable clinical expression. In severe forms it may involve almost any organ system. Periungual fibromas are a common cutaneous manifestation and a frequent source of cosmetic and functional concern. Current treatment involves surgical excision of the lesions. OBJECTIVE: To evaluate laser vaporization of periungual fibromas and to compare results to the standard surgical treatment. METHODS: A patient with tuberous sclerosis and recurrent periungual fibromas was treated with a continuous wave CO2 laser in sequential focused and defocused modes at 10-15 W. RESULTS: Laser treatment required approximately 10 seconds per lesion and produced no intraoperative bleeding. The wounds healed well with good cosmetic results. CONCLUSION: Though similar to conventional surgery in terms of cosmetic satisfaction, CO2 laser presents a viable alternative to surgery through significantly reduced treatment time and amount of bleeding.

Carbon Dioxide↗

[Desmoplastic fibroma of the fibula. A difficult clinical, radiological and histological diagnosis].

Desmoplastic fibroma of bone is a rare benign tumor that is made up of wavy fibroblasts and abundant collagenous tissue. The case of a 18-year-old patient is presented with a two months history of weightbearing pain in the left knee. Neither native x-ray, CT nor MRI could detect the kind of tumor. The histological findings lead finally to the diagnosis of a desmoplastic fibroma. Wide resection prevented recurrence of the tumor for 8 1/2 years until now. Considering the semi-malignant character of the desmoplastic fibroma and the recurrence rate marginal or wide resection for the primary treatment is recommended. The superior imaging quality of MRI facilitates preoperative planning.

Adolescent↗