Fibroma Cardíaco: seguimento a longo prazo por ressonância magnética cardíaca / Cardiac Fibroma: long-term follow-up by cardiac magnetic resonance

As neoplasias cardíacas primárias são raras, e o diagnóstico correto é essencial para planejar o tratamento mais adequado. O objetivo deste estudo foi demonstrar o papel da ressonância magnética cardíaca na avaliação, no diagnóstico e no acompanhamento de fibroma cardíaco. Paciente do sexo feminino, 21 anos, com massa miocárdica ao ecocardiograma. Realizou ressonância magnética com diagnóstico de fibroma cardíaco. Foi acompanhada durante 6 anos com estabilidade do quadro. Fibromas cardíacos correspondem à segunda neoplasia mais comum em crianças e jovens. À ressonância magnética, caracterizam-se por realce tardio intenso e homogêneo.(AU)

Chondromyxoid Fibroma of the Mastoid: A Rare Entity with Comprehensive Literature Review.

Chondromyxoid fibroma (CMF) is the least commonly occurring bone tumor of cartilaginous origin. It is usually situated in the metaphysis of long bones of the lower limbs. Localization of the tumor in the skull is extremely rare. The definitive diagnosis is challenging and depends on radiological and histological examinations. To the best of our knowledge, only 14 cases of CMF involving the temporal bone have been reported to date, 7 of which were within the mastoid. The most common clinical symptom is headache; however, these symptoms vary greatly according to site, size, and extension of the lesion. Surgical removal is the treatment of choice. A literature review of the diagnostic challenges, histological difficulties in differential diagnosis, imaging, clinical features, and recommended modalities of treatment have been discussed in the present case.Chondromyxoid fibroma (CMF) is the least commonly occurring bone tumor of cartilaginous origin. It is usually situated in the metaphysis of long bones of the lower limbs. Localization of the tumor in the skull is extremely rare. The definitive diagnosis is challenging and depends on radiological and histological examinations. To the best of our knowledge, only 14 cases of CMF involving the temporal bone have been reported to date, 7 of which were within the mastoid. The most common clinical symptom is headache; however, these symptoms vary greatly according to site, size, and extension of the lesion. Surgical removal is the treatment of choice. A literature review of the diagnostic challenges, histological difficulties in differential diagnosis, imaging, clinical features, and recommended modalities of treatment have been discussed in the present case.

Fibroma-like PEComa: A Tuberous Sclerosis Complex-related Lesion.

Perivascular epithelioid cell tumor (PEComa), mesenchymal tumors morphologically characterized by epithelioid cells, coexpress melanocytic and muscle markers. Herein, we describe a heretofore-undescribed tuberous sclerosis complex (TSC)-related neoplasm, morphologically resembling a soft tissue fibroma-like lesion, but showing an immunophenotype resembling PEComa. We identified 3 soft tissue fibroma-like lesions in individuals with TSC. We also evaluated 6 TSC-related periungual fibroma as well as a range of non-TSC fibroma-like lesions (n=19). Immunohistochemistry for HMB-45, desmin, smooth muscle actin, TFE3, and S100 was performed on the TSC-related fibromas. Periungual fibromas and non-TSC fibroma-like lesions were also stained for HMB-45. All 3 TSC patients were female, ranging in age from 4 to 51 years (mean, 26.7 y). Two tumors were located in extremities and 1 on the chest wall. The tumors showed elongated to stellate spindle-shape cells, prominent collagenous background, and lacked mitotic activity and cytologic atypia. Immunohistochemically, all 3 tumors were positive for HMB-45; smooth muscle actin or desmin was positive in both tumors tested. TFE3 was negative. All patients were alive with no evidence of disease with median follow-up of 55 months (range, 6 to 131 mo). Non-TSC fibroma-like lesions and oral and periungual fibromas were negative for HMB-45. Fibroma-like PEComa, a newly recognized soft tissue tumor with a strong association with TSC, mimics soft tissue fibroma but shows reactivity with melanocytic markers.

Fibroma odontogénico central: reporte de dos casos / Central odontogenic fibroma: report of two cases

El fibroma odontogénico central es una lesión poco común dentro de los tumores odontogénicos. La variante central incrementa esta rara incidencia, representando solamente 1.5 por ciento de éstos. En el presente artículo se reportan dos casos de fibroma odontogénico central en la región maxilar, así como el tratamiento empleado para la resección del mismo y la reconstrucción de la región, mediante técnica de injerto óseo, malla de titanio y osteosíntesis...

Elastofibroma dorsi: a case report with an immunohistochemical and ultrastructural studies.

Elastofibroma is a rare, benign, fibrous tumor formed by the proliferation of characteristic elastic fibers that commonly occurs between the lower margin of the scapula and the ribcage. We undertook a histochemical, immunohistochemical, and ultrastructural study of an elastofibroma dorsi beneath the right scapula of a 77-year-old woman. Tumor cells comprised collagen fiber bundles, numerous elastic fibers, and spindle cells resembling fibroblasts. The elastic and collagen fibers in the tumor were stained positively with Elastic van Gieson and Masson trichrome staining, respectively. Immunostaining showed that the fibroblasts were strongly positive for CD34, positive for vimentin, and weakly positive for α-smooth muscle actin. Ultrastructural observations revealed elastin and microfibrils between numerous irregularly arranged collagen fiber bundles. Signs suggestive of elastin deposition were also evident in the tangled collagen fibers themselves. The fibroblasts contained a large amount of rough endoplasmic reticulum and were surrounded on the outside of cells by microfibrils and collagen fibers. Although fibroblasts may produce large quantities of elastin, microfibrils, and collagen, our findings suggested that the deposition of elastin on collagen fibers may be involved in the formation of abnormal elastic fibers.

Superficial acral fibromyxoma: immunohistochemical and ultrastructural analysis of a case, with literature review.

Superficial acral fibromyxoma (SAFM) is an uncommon tumor of the superficial soft tissues of acral sites. SAFM is a proliferation of fibroblastic cells, within a myxoid to collagenous stroma. The published cases mostly expressed immunoreactivity for CD34, CD99, EMA, and, less frequently, CD10. The authors report an additional case that did not express any of the previously reported markers, including CD34, and antigens of mesenchymal stromal lineage. Ultrastructural study confirmed the tumor cells were typical fibroblasts with cytoplasmic intermediate filaments and numerous cisternae of rough endoplasmic reticulum. The authors describe the first example of SAFM, ultrastructurally studied, with pure fibroblastic immunoprofile.

Active neovascularization and possible vascular-centric development of gastric and periscapular elastofibromas.

An elastofibroma is a benign and rare fibrous lesion that most commonly occurs in the periscapular region. A gastrointestinal elastofibroma is extremely rare. In the present study, six cases of elastofibromas including a case in the stomach were evaluated. The gastric case revealed widely distributed lesions in the submucosal layer with perivascular fibrotic lesions (PVFLs) and some PVFLs were distributed to the skip lesions of elastofibroma. These PVFLs were also observed in all five periscapular cases and invariably contained elastic fibers which showed various degree of maturation. CD34-positive stromal cells were observed not only in elastofibromas but also in PVFLs in each case. These findings suggested the possibility of the PVFLs were the primary lesions of elastofibroma and their vascular-centric development. The percentage of the CD105-positive vessels in elastofibroma group was significantly higher than in the control group. This result indicates active neovascularization in elastofibromas.

Elastofibroma: a histochemical, immunohistochemical, and ultrastructural study of two patients.

Elastofibroma is a rare neoplasm. In this article, we report our study of the pathogenesis of fibrosis in elastofibroma. Three tumors obtained from two patients were selected. One patient was a 57-year-old Japanese woman who had a bilateral tumor, and the other patient was a 83-year-old Japanese man. All tumors occurred in the infrascapular region. Macroscopically, the cut surface of all tumors showed a poorly defined and whitish mass with yellowish foci. Microscopically, the tumor consisted of collagen fiber bundles, abnormal elastic fibers, and spindle cells suggestive of fibroblasts. Elastica-van Gieson and Masson-trichrome stain identified abnormal elastic fibers and abundant collagen fibers, respectively, in elastofibroma. Immunohistochemically, fibroblasts were positive for CD34 but negative for alpha-smooth muscle actin and h-caldesmon. Additionally, the cytoplasm of many fibroblasts was positive for TGF-beta in all tumors. Ultrastructurally, some fibroblasts with abundant organelles in one tumor were observed in the adjacent area to amorphous elastic mass and bundles of collagen fibers. However, no myofibroblasts were ultrastructurally identified in the tumor. Finally, our study supplies further evidence that elastofibroma may show the proliferation of CD34-positive fibroblasts and contain no myofibroblasts, and that fibroblasts may produce both abnormal elastic fibers and collagen fibers through the secretion of TGF-beta.

Postoperative fibromatosis-type fibromas in the Bhd gene mutant (Nihon) rat.

Fibromatosis-type fibromas were found to develop at abdominal surgical sites in 4 heterozygous Nihon rats, a model for the human Birt-Hogg-Dubé syndrome. In all 4 rats, solitary and firm nodules were located within the lateral abdominal musculature involving the full thickness of the abdominal wall at the sites of laparotomy. Histologically, the nodules consisted of well-differentiated fibroblastic spindle-shaped cells. These cells were surrounded by large amounts of collagen fibers, and appeared to infiltrate within the abdominal musculature. A portion of the spindle-shaped cells showed features of myofibroblasts. These characteristics are consistent with desmoid tumors in human. Although the etiology of desmoid tumors in human remains unclear, they are known to occur in association with hormonal factors, surgical trauma, and familial adenomatous polyposis. In animals, they have been reported in dogs, cats, horses, and genetically modified mouse models for human familial adenomatous polyposis. The development of the tumors in the Nihon rats was apparently associated with surgical incisions. Genetic factor should be involved in the occurrence of the tumor, since it was found only in the Nihon rats among many rats. Our present data suggest that Bhd gene mutation is not likely to be a candidate.

An ultrastructural and immunohistochemical study of elastofibroma: CD 34, MEF-2, prominin 2 (CD133), and factor XIIIa-positive proliferating fibroblastic stromal cells connected by Cx43-type gap junctions.

Elastofibromas have been described as ill-defined tumors, composed of fibroblastic stromal cells and a dense collagenous stroma. A total of 5 elastofibromas from 4 Japanese patients were examined by ultrastructural and immunohistochemical methods. The proliferating fibroblastic stromal cells in the lesion showed Cx43-type gap junctions, isolated cilia, prominent nuclear fibrous laminae, and primitive cellular junctions with incomplete laminae. The active proliferating fibroblastic cells showed positive staining for vimentin, CD34, factor XIIIa, prominin 2 (CD133), and MEF 2. Conspicuous cell-to-matrix interactions were observed with abnormally unique elastins, collagens (type I, III, and IV), laminin, fibronectin, and amorphous extracellular matrix (GAGs; glycosaminoglycans). As for the origin of elastofibromas, the tumors in the present study were suggested to arise from subscapular or periosteal connective tissue, but further revealed some similarities to other tissues, such as human skin dermal tissue, as exemplified by the presence of an abundance of type I and III collagen, CD34/factor XIIIa-expressing stromal fibroblast-like cells, amorphous extracellular matrix, and a unique abnormal elastin. The elastofibromas might have arisen from stromal stem cell candidate populations of stromal fibroblastic cells (CD34(+), MEF2(+), prominin 2(CD133)(+), and factor XIIIa(+)).

[Chondromyxoid fibroma].

Chondromyxoid fibroma is a benign chondroid tissue tumor characterized by the lobular overgrowth of stellate or elongated cells in the myxoid or chondroid intracellular matrix. There may be giant multinucleate cells. They may be encountered in any skeletal bones, mainly at the age of 20-30 years. A positive reaction with S-100 protein, d smooth muscle actin, and CD 34 was immunohistochemically detected in chondromyxoid fibroma tissue. In chondromyxoid fibroma, the prognosis is favorable. Recurrence of the tumor is observed in 15% of the surgically treated cases.

Elastofibroma dorsi: histologic and echographic considerations.

BACKGROUND: Elastofibroma dorsi is a relatively rare soft-tissue tumor of the elderly with typical localization to the subscapular area. To date, few cases have been reported in the dermatology literature. The differential diagnosis includes frequently observed subcutaneous neoplasms such as lipoma, fibrolipoma or more aggressive tumors. The diagnosis is made with histologic examination. CASE REPORT: We present a typical case of elastofibroma dorsi, studied with ultrasound investigation and color-power Doppler, and discuss the correlation with the histologic picture. CONCLUSIONS: Considering the strict correspondence between the characteristic histologic findings of elastofibroma and the specific ultrasound pattern, we believe that ultrasound investigation with color and power Doppler allows, in typical cases, a definitive diagnosis of elastofibroma.

Application of a quick-freezing and deep-etching method to pathological diagnosis: a case of elastofibroma.

A case of elastofibroma in a middle-aged Japanese woman was examined by the quick-freezing and deep-etching (QF-DE) method, as well as by immunohistochemistry and conventional electron microscopy. The slowly growing tumor developed at the right scapular region and was composed of fibrous connective tissue with unique elastic materials called elastofibroma fibers. A normal elastic fiber consists of a central core and peripheral zone, in which the latter has small aggregates of 10 nm microfibrils. By the QF-DE method, globular structures consisting of numerous fibrils (5-20 nm in width) were observed between the collagen bundles. We could confirm that they were microfibril-rich peripheral zones of elastofibroma fibers by comparing the replica membrane and conventional electron microscopy. One of the characteristics of elastofibroma fibers is that they are assumed to contain numerous microfibrils. Immunohistochemically, spindle tumor cells showed positive immunoreaction for vimentin, whereas alpha-smooth muscle actin, desmin, S-100 protein and CD34 showed negative immunoreaction. By conventional electron microscopy, the tumor cell had thin cytoplasmic processes, pinocytotic vesicles and prominent rough endoplasmic reticulum. Abundant intracytoplasmic filaments were observed in some tumor cells. Thick lamina-like structures along with their inner nuclear membrane were often observed in the tumor cell nuclei. The whole image of the tumor cell was considered to be a periosteal-derived cell, which would produce numerous microfibrils in the peripheral zone of elastofibroma fibers. This study indicated that the QF-DE method could be applied to the pathological diagnosis and analysis of pathomechanism, even for surgical specimens obtained from a patient.

Fibroelastoma papilífero: experiência de uma instituição / Cardiac papillary fibroelastoma: experience of an institution

Os tumores cardíacos primários do coração são raros, com uma prevalência entre 0,0017 por cento e 0,19 por cento dos estudos de autópsia não selecionados. Cerca de 75 por cento são tumores benignos e quase a metade são mixomas. Os restantes se dividem entre rabdomiomas, lipomas e fibroelastomas. Os mixomas são os tumores cardíacos mais comuns na idade adulta e os rabdomiomas, os mais comuns da população pediátrica. O fibroelastoma papilífero (FEP) é um tumor benigno do coração, relativamente raro, correspondendo a aproximadamente 8 por cento dos tumores cardíacos. São os que mais comumente acometem as valvas cardíacas . No passado, consistiam de achados de necropsia ou eram encontrados em procedimentos cirúrgicos ao acaso. O diagnóstico in vivo era esporádico². Com o aprimoramento das técnicas de ecocardiografia, o FEP tem sido diagnosticado com maior freqüência. São, geralmente, descritos como uma massa móvel, pedunculada, bem delimitada e com predileção pelo endocárdio valvar. A proposta terapêutica, quando pedunculados, é a ressecção cirúrgica, visando a prevenção de fenômenos embólicos cerebrais, pulmonares, coronarianos ou periféricos1,3. Serão apresentados cinco casos diagnosticados em nossa instituição, no período de agosto de 1995 a junho de 2004.

Fibroma esclerótico cutáneo: a propósito de 11 observaciones / Cutaneous sclerotic fibroma: an 11 cases observations

El fibroma esclerótico cutáneo es un tumor benigno bien circunscrito, con un patrón histológico característico. Presentamos 11 casos de este tipo particular de fibroma solitario en pacientes que no presentan otros signos de enfermedad de Cowden. Analizamos retrospectivamente durante un período de 10 años todos los casos esporádicos de fibromas escleróticos cutáneos diagnosticados en nuestra institución, a fin de contribuir al conocimiento clinicopatológico de esta neoplasia poco frecuente. Se realiza una revisión bilbiográfica del tema teniendo en cuenta sus aspectos históricos, clínicos, histológicos, inmunohistoquímicos y diagnósticos diferenciales. Sus características clínicas, microscópicas y perfil inmunohistoquímico establecen que es una neoplasia proliferativa con patrón histológico específico, aunque algunos autores lo consideran el estadio final de otro tumor o lesión esclerosante (AU)

Gastric fibromyxoma, a distinct entity of pure fibroblastic tumor--an ultrastructural study.

A rare case of fibromyxoma arising in the stomach of a 78-year-old male is presented. A well-defined, encapsulated tumor measuring 27x17x8 cm showed a lobular growth in the submucosa and muscularis propria, with no association with the propria mucosa. Histologically, the tumor was characterized by a diffuse, widely spaced proliferation of small spindle, stellate and round cells with dendritic outlines with fibromyxoid stroma. Neither cellular atypia nor mitotic figures were observed. Immunohistochemically, the tumor was positive for vimentin and CD34 but negative for CD117, alpha-smooth muscle actin, muscle actin, desmin and S-100 protein. Ultrastructurally, the tumor cells had irregularly shaped nuclei and cytoplasm containing moderate amounts of mitochondria, rough endoplasmic reticulum, subplasmalemmal densities, and short interdigitating processes. These findings indicated the fibroblastic nature of the tumor. The patient was well without recurrence 20 months after surgery. It is important to recognize that gastric fibromyxoma is a distinct entity and to include it in the differential diagnosis of gastric mesenchymal tumors, as the tumors show a different clinical behavior and as limited surgery with local excision is possible.

Histochemical and ultrastructural study of an elastofibroma dorsi coexisting with a high grade spindle cell sarcoma.

Elastofibroma dorsi is a pseudotumoral fibroproliferative lesion characterized by polymorphic fiber-like deposits of elastinophilic material. Several theories have been reported explaining the pathogenesis of elastofibroma. Recent cytogenetic studies have demonstrated chromosomal instability in elastofibromas, not normally observed in non-neoplastic tissues. These chromosomal defects are commonly observed in aggressive fibromatosis too. Such clinical observations suggest a multistage pathogenetic mechanism for the onset of elastofibroma. This study, using histochemical, immunohistochemical staining techniques, and ultrastructural examination, describes the detection of an otherwise typical elastofibroma contextual to a high grade sarcoma. Hence, the coexistence of elastofibroma and high-grade sarcoma may suggest a causal link between the two pathological entities. The results obtained suggest that the coexistence of the two pathological entities is conceivably coincidental.

Intracellular collagen and fibronexus in fibromatosis and other fibroblastic tumors.

Myofibroblasts are mesenchymal cells with combined function and structure for contraction and collagen synthesis. They are found in reparative responses, nodular fasciitis, fibromatosis, and myofibroblastic sarcoma. Ultrastructurally, myofibroblasts are characterized by a specialized cell surface structure called the fibronexus (FNX). In addition, intracellular collagen fibers (ICF) have been described in nodular fasciitis and fibromatosis, but their origin and nature are still controversial. The aim of the present work was, first, to assess the frequency of FNX and ICF in proliferative myofibroblastic conditions compared to diverse mesenchymal tumors with spindle-shaped cells, and, second, to determine what kind of organelles contain ICF and if they are related to phagocytosis or cell synthesis. Forty-two cases of aggressive fibromatosis and 11 of nodular fasciitis (group A) were compared to 82 spindle-cell mesenchymal tumors of diverse nature (group B) by electron microscopy study. The presence and frequency of FNX and ICF was compared in both groups, and the organelles containing ICF were recorded. FNX and ICF were constantly found in group A (69.8 and 84.9%, respectively), and rarely in group B (0 and 5.12%, respectively). Most frequently ICF were contained in tunnels and phagolysosomes, but also were found in Golgi vesicles and cisternae of rough endoplasmic reticulum. In the majority of cases (75%), ICF were similar to collagen fibers of the extracellular space, but in some cases (22.5%), they were in dissimilar stages of fibrogenesis. Fibromatosis and nodular fasciitis are characterized by proliferation of myofibroblasts and constantly show FNX and ICF. These structures are rarely found in other mesenchymal tumors. The ICF are found in organelles of digestion and also in others related to synthesis and transport.

Factores de riesgo de la enfermedad de Dupuytren: revisión sistemática de la evidencia científica / Risk factors for Dupuytren's disease: systematic review of the scientific evidence

Objetivo. La revisión sistemática de la evidencia científica es un método estructurado de recuperación y análisis de la bibliografía existente sobre un determinado problema de interés. Material y método. Este artículo analiza los factores de riesgo, población, raza, género, ocupación laboral, habito enólico, diabetes, tabaquismo, epilepsia y origen genético que, según la doctrina científica, se consideran vinculados, o que predisponen en la aparición y posterior desarrollo de la enfermedad de Dupuytren. Se han consultado, al menos, dos bases de datos distintas, y ampliado la búsqueda con una revisión manual de la bibliografía. Resultados y conclusiones. Se resumen estos trabajos, y se discuten sus resultados y conclusiones. La población oriunda del norte de Europa, el factor hereditario, el género masculino y el tabaco son los factores que proporcionan el mayor grado de evidencia científica en la aparición y desarrollo de la enfermedad de Dupuytren (AU)