Fibroma traumático em região anterior da mandíbula: relato de caso clínico / Traumatic fibroma in the anterior region of the mandible: clinical case report

Objetivo: O objetivo deste estudo foi relatar o caso clínico de uma paciente do sexo feminino, 40 anos, feoderma, ASA I, com lesão nodular na região papilar entre os elementos incisivo lateral e canino inferiores apresentando características de base séssil, sólida e fibrosa. Materiais e Métodos: Foi realizada uma biópsia excisional da lesão, e a peça cirúrgica foi coletada em um recipiente contendo formol 10% para exame histopatológico e confirmação para o diagnóstico de fibroma. Resultados: O exame histopatológico confirmou o diagnóstico de fibroma. No pós-operatório, a região cirúrgica foi de início acompanhada semanalmente e, posteriormente, em intervalos mensais a partir da quarta semana, com prognóstico favorável. Conclusão: O diagnóstico preciso do fibroma é fundamental para garantir o melhor tratamento possível. Este caso clínico destaca a importância da biópsia excecional e do acompanhamento pós-operatório adequado para assegurar uma recuperação satisfatória do paciente.(AU)

Objective: The objective of this study was to report a clinical case of a 40-year-old female patient with a nodular lesion in the papillary region between elements lower lateral incisor and canine presenting features of a sessile, solid, and fibrous base. Materials and Methods: An excisional biopsy of the lesion was performed, and the surgical specimen was collected in a container containing 10% formalin for histopathological examination and confirmation of the fibroma diagnosis. Results: The histopathological examination confirmed the diagnosis of fibroma. In the postoperative period, the surgical region was initially monitored weekly and subsequently at monthly intervals from the fourth week, with a favorable prognosis. Conclusion: Accurate diagnosis of fibroma is essential to ensure the best possible treatment. This clinical case highlights the importance of excisional biopsy and appropriate postoperative follow-up to ensure a satisfactory patient recovery.(AU)

Fibroma of tendon sheath: a clinicopathological and genetic analysis of 134 cases / 中华病理学杂志

Objective: To investigate the clinicopathological features, immunophenotypes and molecular genetics of fibroma of tendon sheath (FTS). Methods: One hundred and thirty-four cases of FTS or tenosynovial fibroma diagnosed in the Department of Pathology, West China Hospital, Sichuan University, Chengdu, China from January 2008 to April 2019 were selected. The clinical and histologic features of these cases were retrospectively reviewed. Immunohistochemistry, fluorescence in situ hybridization (FISH) and reverse transcription-polymerase chain reaction (RT-PCR) were performed on the above cases. Results: There were a total of 134 cases of FTS, including 67 males and 67 females. The patients' median age was 38 years (ranged from 2 to 85 years). The median tumor size was 1.8 cm (ranged from 0.1 to 6.8 cm). The most common site was the upper extremity (76/134, 57%). Follow-up data was available in 28 cases and there was no detectable recurrence. Classic FTS (114 cases) were well-defined and hypocellular. A few spindle-shaped fibroblasts were scattered in the dense collagenous sclerotic stroma. Characteristically elongated slit-like spaces or thin-walled vessels were observed. Most of cellular FTSs (20 cases) were well-defined and the area with increased cellularity of the spindle cells coexisted with classic FTS. There were occasional mitotic figures, but no atypical mitotic figures. Immunohistochemistry was performed in 8 cases of classic FTS and most cases were positive for SMA (5/8). Immunohistochemistry was also performed in 13 cases of cellular FTS and showed 100% positive rate for SMA. FISH was conducted on 20 cases of cellular FTS and 32 cases of classical FTS. USP6 gene rearrangement was found in 11/20 of cellular FTS. Among 12 cases of CFTS with nodular fasciitis (NF)-like morphological feature, 7 cases showed USP6 gene rearrangement. The rearrangement proportion of USP6 gene in cellular FTS without NF-like morphological features was 4/8. By contrast, 3% (1/32) of the classic FTS showed USP6 gene rearrangement. RT-PCR was performed in those cases with detected USP6 gene rearrangement and sufficient tissue samples for RT-PCR. The MYH9-USP6 fusion gene was detected in 1 case (1/8) of the cellular FTSs, while no target fusion partner was detected in the classic FTS. Conclusions: FTS is a relatively rare benign fibroblastic or myofibroblastic tumor. Our study and recent literature find that some of the classic FTS also show USP6 gene rearrangements, suggesting that classical FTS and cellular FTS are likely to be at different stages of the same disease (spectrum). FISH for USP6 gene rearrangement may be used as an important auxiliary diagnostic tool in distinguishing FTS from other tumors.

Superficial Acral Fibromyxoma: Literature Review / Fibromixoma acral superficial: Revisão da Literatura

Abstract Superficial acral fibromyxoma is a benign and rare tumor of the soft tissues. It usually manifests itself through a painless mass of slow growth that affects mainly males in the fifth decade of life. It usually affects the distal region, with a polypoid or dome-shaped appearance. The histological appearance is of a dermal mass without capsule, with spindle-shaped fibroblasts in a storiform or fasciculated pattern in the myxocollagenous stroma. The immunohistochemical evaluation of superficial acral fibromyxoma is usually positive for CD34 and CD99, with variable positivity for epithelial membrane antigen. The treatment consists of complete excision of the tumor mass. A review of the current literature on superficial acral fibromyxoma was performed, with an emphasis on the number of cases reported, location, diagnostic methods, histological characteristics, differential diagnoses and treatment. A total of 314 reported cases of superficial acral fibromyxoma with variable locations were found in the current literature, mainly in the toes (45.8%) and fingers (39.1%). It has a slightly superior incidence in men (61%) and enormous variability in the age range of occurrence. Superficial acral fibromyxoma is a single soft-tissue tumor that should enter the differential diagnosis of periungual and subungual acral lesions; the treatment consists of simple excision. More studies are needed to better understand this pathology, which was first described in 2001.

Resumo O fibromixoma acral superficial é um tumor raro de tecidos moles. Geralmente se manifesta por meio de uma massa indolor de crescimento lento que acomete principalmente adultos do sexo masculino na quinta década de vida. Ele normalmente afeta a região distal, com aparência polipoide. A aparência histológica é de uma massa dérmica sem cápsula, com fibroblastos fusiformes em estroma mixocolagenoso. A avaliação imuno-histoquímica do fibromixoma acral superficial normalmente é positiva para CD34 e CD99, com positividade variável para o antígeno epitelial de membrana. O tratamento consiste na exérese completa da massa tumoral. Foi feita uma revisão da literatura atual sobre o fibromixoma acral superficial com ênfase na quantidade de casos relatados, na localização, nos métodos diagnósticos, nas características histológicas, nos diagnósticos diferenciais, e no tratamento. Foram encontrados na literatura atual 314 casos descritos de fibromixoma acral superficial com localização variada, principalmente em pododáctilos (45,8%) e quirodáctilos (39,1%). Este tumor tem acometimento ligeiramente superior em homens (61%), e enorme variabilidade na faixa etária de acometimento. O fibromixoma acral superficial é um tumor de tecido mole único que deve entrar no diagnóstico diferencial das lesões periungueais e subungueais acrais; o tratamento consiste da exérese simples. Mais estudos são necessários para que se conheça melhor essa patologia, descrita em 2001.

Incidence of reactive hyperplastic lesions in the oral cavity: a 10 year retrospective study in Santa Catarina, Brazil / Incidência de lesões hiperplásicas reativas na cavidade bucal: estudo retrospectivo de 10 anos em Santa Catarina, Brasil

Abstract Introduction Reactive hyperplastic lesions develop in response to a chronic injury simulating an exuberant tissue repair response. They represent some of the most common oral lesions including inflammatory fibrous hyperplasia, oral pyogenic granuloma, giant cell fibroma, peripheral ossifying fibroma, and peripheral giant cell lesions. Objective The incidence of those lesions was investigated in an oral pathology service, and the clinical characteristics, associated etiological factors, concordance between the clinical and histopathological diagnostic was determined. Methods A total of 2400 patient records were screened from 2006 to 2016. Clinical features were recorded from biopsy reports and patients' files. Results A total of 534 cases of reactive hyperplastic lesions were retrieved and retrospectively studied, representing 22.25% of all diagnoses. The most frequent lesion was inflammatory fibrous hyperplasia (72.09%), followed by oral pyogenic granuloma (11.79%), giant cell fibroma (7.30%), peripheral ossifying fibroma (5.24%), and peripheral giant cell lesions (3.55%). Females were predominantly affected (74.19%), the gingiva and alveolar ridge were the predominant anatomical site (32.89%), and chronic traumatism was presented as the main etiological factor. The age widely ranges from the 1st decade of life to the 7th. Clinically, the reactive hyperplastic lesions consisted of small lesions (0.5-2 cm) and shared a strong likeness in color to the oral mucosa. The concordance between the clinical and histopathological diagnostic was high (82.5%). Conclusion Reactive hyperplastic lesions had a high incidence among oral pathologies. The understanding of their clinical features helps to achieve a clearer clinical and etiological diagnosis, and the knowledge of factors related to their development. This may contribute to adequate treatment and positive prognosis.

Resumo Introdução As lesões hiperplásicas reativas se desenvolvem em resposta a uma lesão crônica que estimula uma resposta acentuada de reparo tecidual. Elas representam uma das lesões orais mais comuns, inclusive hiperplasia fibrosa inflamatória, granuloma piogênico oral, fibroma de células gigantes, fibroma periférico ossificante e lesão periférica de células gigantes. Objetivo A incidência dessas lesões foi investigada em um serviço de patologia bucal e as características clínicas, os fatores etiológicos associados e a concordância entre os diagnósticos clínico e histopatológico foram determinados. Método Foram selecionados 2.400 registros de pacientes entre 2006 e 2016. As características clínicas foram registradas a partir de laudos de biópsia e dos prontuários dos pacientes. Resultados Um total de 534 casos de lesões hiperplásicas reativas foram recuperados e retrospectivamente estudados, representando 22,25% de todos os diagnósticos. A lesão mais frequente foi hiperplasia fibrosa inflamatória (72,09%), seguida por granuloma piogênico oral (11,79%), fibroma de células gigantes, (7,30%), fibroma periférico ossificante (5,24%) e lesão periférica de células gigantes (3,55%). O sexo feminino foi predominante (74,19%), a gengiva e a crista alveolar foram o local anatômico predominante (32,89%) e o traumatismo crônico foi demonstrado como o principal fator etiológico. A idade variou desde a 1ª década de vida até a 7ª. Clinicamente, as LHR consistiram em pequenas lesões (0,5 a 2 cm) que apresentaram uma forte semelhança de cor com a mucosa oral. A concordância entre o diagnóstico clínico e histopatológico foi alta (82,5%). Conclusão As lesões hiperplásicas reativas apresentaram alta incidência entre as patologias bucais. A compreensão das características clínicas ajuda na realização de um diagnóstico clínico e etiológico mais claro, bem como determinar os fatores relacionados ao seu desenvolvimento. Dessa forma contribui para um tratamento adequado e um prognóstico positivo.

Tumor desmoides cervical: reporte de caso / Cervical desmoid tumor: case report

El tumor desmoides es un tumor benigno raro, de origen fibroblastico no inflamatorio, algunas veces referido como fibromatosis no agresiva. Su etiología aún no está completamente clara, sin embargo, se asocia habitualmente a trauma previo y/o procedimientos quirúrgicos. La ubicación más frecuente es abdominal, el cual posee características imagenologicas claras, al igual que su presentación musculo esquelética plantar. La presentación fuera de estos lugares es infrecuente y difícil de diagnosticar. Presentamos un caso de un tumor desmoides cervical que genero un gran desafío diagnóstico, identificando sus claves diagnósticas y realizando una revisión de la bibliografía al respecto para esta ubicación.

Desmoid tumours are a rare benign tumour of fibroblastic non inflammatory origin, sometimes referred as non aggressive fibromatosis.The etiology is not yet completely clear, however, it is usually associated with previous trauma and / or surgical procedures. The most frequent location is in the abdomen, which has typical images characteristics, as well as its skeletal muscle presentation at the plantar level. The presentation outside these places is infrequent and difficult to diagnose. We present a case of a cervical desmoid tumour that generated a great diagnostic challenge, identifying its key imaging characteristics and performing a literature review of the bibliography regarding this location.

Accidente cerebrovascular embólico secundario a fibroelastoma papilar de válvula mitral / Embolic stroke secondary to a mitral valve fibroelastoma: case report

Embolic stroke secondary to cardiac tumors is uncommon. However, 25-30% of cardiac tumors may cause systemic emboli. We report a 29-year-old male consulting for a sudden episode of aphasia and right hemiparesis, compatible with infarct of the left middle cerebral artery territory. Transthoracic echocardiography reported an ovoid tumor of 8 × 7 × 7 mm in relation to the sub valvular apparatus of the mitral valve. After neurologic stabilization, surgical treatment was performed. Approached by median sternotomy and in cardiopulmonary bypass, the mitral valve was explored. A macroscopic tumor consistent with a papillary fibroelastoma curled in sub valvular chordae was found. It was deployed and resected from its base, while the anterior mitral leaflet was preserved intact. Histopathological examination confirmed the intraoperative macroscopic diagnosis. The patient recovered uneventfully postoperatively and was discharged on the fifth day after surgery. He currently is in functional capacity I without cardiovascular symptoms at five years follow-up.

Fibroma de Células Gigantes en Lactante Mayor / Giant Cell Fibroma in Older Infant. A Case Report

RESUMEN: El fibroma de células gigantes es considerado un tumor benigno no neoplásico de la mucosa oral. Este aparece en las primeras tres décadas de la vida, siendo relativamente raro en pacientes pediátricos. Puede encontrarse principalmente en la encía mandibular, mostrando predilección por el sexo femenino. Clínicamente se presenta como un crecimiento indoloro, de base sésil o pediculado, que generalmente se confunde con otras lesiones de tipo fibrosas como los fibromas de irritación. Histológicamente, se distingue por presentar fibroblastos estrellados con la presencia de células gigantes multinucleadas cerca de la lámina del epitelio. Presentamos el caso de una paciente femenino de un año de edad la cual presenta crecimiento nodular indoloro en relación con una superficie del paladar de 51 y 61. Teniendo en cuenta el tamaño y la ubicación de la lesión, se realizó escisión, biopsia y se envió para análisis histopatológico que confirmó la lesión como fibroma de células gigantes.

ABSTRACT: The giant cell fibroma is a benign nonneoplastic fibrous tumor of the oral mucosa. It occurs in the first three decades of life and is relatively rare in pediatric patients. It can be found predominantly in the mandibular gingiva, showing predilection for females. Clinically it presents as a painless, sessile, or pedunculated growth which is usually mistaken for other fibrous lesions like irritation fibroids. Histologically it is distinguished by the presence of stellated fibroblasts along with multinucleated giant cells near the epithelial sheet. We present a case where a one-year-old female patient presented with a painless nodular growth in relation to a palatesurface of 51 and 61. Considering the size and location of the lesion, excision and biopsy were performed and sent for histopathological analysis which confirmed the lesion as giant cell fibroma.

Outcomes after surgical resection of primary non-myxoma cardiac tumors

Abstract Objective: Primary cardiac tumors are rare lesions with different histological type. We reviewed our 17 years of experience in the surgical treatment and clinical results of primary non-myxoma cardiac tumors. Methods: Between July 2000 and February 2017, 21 patients with primary cardiac tumor were surgically treated in our institution. The tumors were categorized as benign non-myxomas and malignants. Data including the demographic characteristics, details of the tumor histology and grading, cardiac medical and surgical history, surgical procedure of the patients were obtained from the hospital database. Results: Eleven patients were diagnosed with benign non-myxoma tumor (male/female:7/4), ranging in age from 10 days to 74 years (mean age 30.9±26.5 years). Papillary fibroelastoma was the most frequent type (63.6%). There were two early deaths in benign group (all were rhabdomyoma), and mortality rate was 18%. The mean follow-up period was 69.3±58.7 months (range, 3 to 178 months). All survivals in benign group were free of tumor-related symptoms and tumor relapses. Ten patients were diagnosed with malignant tumor (sarcoma/lymphoma:8/2, male/female:3/7), ranging in age from 14 years to 73 years (mean age 44.7±18.9 years). Total resection could be done in only three (30%) patients. The mean follow-up period was 18.7±24.8 months (range, 0-78 months). Six patients died in the first 10 months. Conclusion: Complete resection of the cardiac tumors, whenever possible, is the main goal of surgery. Surgical resection of benign cardiac tumors is safe, usually curative and provides excellent long-term prognosis. On the contrary, malignant cardiac tumors still remain highly lethal.

Fibroelastoma papilar cardíaco: estudio retrospectivo: Presentación clínica y resultados quirúrgicos / Fibroelastoma papilar cardíaco: estudio retrospectivo: Presentación clínica y resultados quirúrgicos

Los fibroelastomas papilares cardíacos son pequeños tumores benignos con potencial embolígeno. Con la incorporación de la ecocardiografía y la mejoría en la resolución de las imágenes, el diagnóstico clínico es cada vez más frecuente, y en series recientes su frecuencia supera a la del mixoma cardíaco. Nuestro objetivo fue analizar las características de una serie de casos de fibroelastoma papilar cardíaco con confirmación histológica operados en nuestro hospital. Analizamos retrospectivamente los tumores cardíacos y las características clínicas de los pacientes operados desde junio de 1992 a febrero de 2017. De 108 operados, 18 presentaron fibroelastoma papilar. La edad media del grupo con fibroelastoma papilar fue 58 años (22-77); 10 eran varones. Las localizaciones más frecuentes fueron la válvula aórtica (7) y la válvula mitral (5). Ninguno presentó disfunción valvular significativa. Por ecografía transesofágica, el tamaño (diámetro mayor) fue 13.33 ± 5.55 mm (6.6-28.0). Cuatro pacientes eran sintomáticos; uno presentaba disnea, otro aleteo auricular, dos con fibroelastoma en válvula aórtica habían sufrido un accidente cerebrovascular. Los restantes, asintomáticos, fueron intervenidos preventivamente. En 15 pacientes el tumor se extirpó sin necesidad de reemplazo valvular. No hubo mortalidad quirúrgica ni recidiva tumoral en el seguimiento de 2.6 años. Se concluye que los fibroelastomas papilares cardíacos pueden ser extirpados con buenos resultados a mediano plazo y, en su mayoría, con preservación valvular. Sin embargo, no existen aún estudios aleatorizados que avalen la escisión quirúrgica en pacientes asintomáticos. Por el momento, esta última depende de la experiencia del grupo quirúrgico tratante.

Papillary ibroelastomas are small benign intracardiac tumors known for their embolic potential. Since the introduction of echocardiography with improved resolution and transesophageal imaging techniques, they are being increasingly detected in clinical practice. In recent series, papillary fibroelastoma is considered the most frequent benign tumor of the heart. Our objective was to analyze characteristics and midterm surgical outcome of histologically-confirmed cases of papillary fibroelastoma. We conducted a retrospective study on patients with cardiac tumors submitted to surgical excision between June 1992 and February 2017. Out of 108 patients, 18 had papillary fibroelastomas. Their mean age was 58 years (22-77); 10 were men. The most frequent localizations were the aortic valve (7) and the mitral valve (5). None had significant valvular dysfunction. By transesophageal echocardiography, the tumor size (larger diameter) was 13.33 ± 5.55 mm (6.6-28.0). Two patients, both with tumor in the aortic valve, had suffered a stroke; other two had dyspnoea and atrial flutter, respectively. The remaining 14 patients were asymptomatic and their tumors were incidental findings. In 15 patients the valve was preserved. There was neither surgical mortality nor recurrence after 2.6 years of follow-up. In conclusion, most papillary fibroelastomas can be surgically removed with valve preservation and favorable clinical outcome. However, until the results of randomized trials support the decision, an aggressive surgical approach in asymptomatic patients needs to be defined in the context of surgical expertise.

Histochemical and immunohistochemical differences between solitary oral fibroma and fibrous papule of the face

Abstract: Background: The morphological similarities between fibrous papules of the face and multiple sporadic oral fibromas were mentioned long ago and a relationship between them has been reported in the literature. Objective: The aim of this study was to evaluate the participation of mast cells, elastin and collagen in a series of oral fibromas and fibrous papules of the face in order to better understand the possible role of these factors in fibrosis and the formation of these lesions. Methods: Thirty cases of oral fibroma involving the buccal mucosa and 30 cases of fibrous papules of the face were selected. Tissue samples were submitted to picrosirius red staining and immunohistochemistry using anti-elastin and anti-tryptase antibodies. Results: The percentage of tryptase-positive mast cells and expression of elastin were higher in cases of fibrous papules of the face (p < 0.05). In contrast, a higher intensity of collagen deposition was observed in oral fibromas. The results showed mast cell accumulation and higher elastin synthesis in fibrous papules of the face, and mast cell accumulation with higher collagen fiber synthesis in oral fibromas. Conclusion: These findings support the hypothesis that mast cells influence the development and growth of these lesions through different mechanisms.

Myxofibrosarcoma - Case report

Abstract Myxofibrosarcoma is recognized as a malignant neoplasm of fibroblastic origin with increased prevalence in the elderly, presenting as nodules or tumors that may extend to the dermis and skeletal muscle, preferably in the lower limbs. Histologically it is characterized by a proliferation of spindle cells in a myxoid stroma. Myxofibrosarcoma has a high potential for local recurrence and metastasis, mainly when it presents a high or intermediate histological grade. We report the case of an eighty-four year old patient with a difficult diagnosis of a highly aggressive tumor.

Two friends with eroded nodules on the ears: atypical fibroxanthoma case report

AbstractAtypical fibroxanthoma is an uncommon mesenchymal tumor that manifests clinically as a reddish papule or nodule in sun-exposed areas of the body. The clinical presentation is not specific and histology and immunohistochemistry are both necessary for a correct diagnosis. Surgery is the gold standard of therapy. Recurrence and metastasis should be excluded with a follow-up at 6 months, since this tumor should nowadays be considered a medium-grade neoplasm, rather than low-grade as previously believed. We report the case of two friends who came to our hospital during the same period, complaining of very similar lesions. After biopsy and immunohistochemical examination, a diagnosis of atypical fibroxanthoma in both cases was formulated.

Tumor fibromixoma lipoesclerosante óseo: Presentación de un caso y revisión de la literatura / Bone liposclerosing myxofibrous tumor: Case presentation and literature review

El tumor fibromixoma lipoesclerosante óseo (TFMLE) fue descrito inicialmente por Ragsdale en 1986, como una lesión fibroósea polimórfica de hueso con una mezcla de elementos histológicos incluyendo lipoma, fibroxantoma, mixoma, mixofibroma, necrosis grasa, osificación isquémica, áreas de displasia fibrosa y rara vez cartílago o cambios quísticos. La localización más frecuentemente descrita es en la región intertrocantérica del fémur. Es un hallazgo en pacientes asintomáticos o bien estar asociado a fractura. Radiológicamente es una lesión lítica, geográfica, de margen bien definido y habitualmente esclerótico, en algunos casos puede observarse mineralización en el interior de la lesión o un cierto grado de expansión en el contorno. Se ha descrito la estrecha relación del TFMLE con la displasia fibrosa por las características histológicas y la presencia de una mutación Gsα, otra hipótesis de la etiología de la lesión incluye la reacción de la displasia fibrosa a la fatiga por estrés.

The bone liposclerosing myxofibrous tumor (LSMFT) was initially described by Ragsdale in 1986 as a polymorphic fibroosseous bone lesion with a mix of histologic elements that include lipoma, fibroxanthoma, myxoma, myxofibroma, fat necrosis, ischemic ossification, areas of fibrous dysplasia, and infrequent presence of cartilage or cystic changes. The most frequently reported location is the intertrochanteric area of the femur. Radiologically it is a lytic, geographic lesion, with well-defined margins and usually sclerotic. In some cases findings include mineralization inside the lesion or a certain degree of expansion to the contour. The close relationship between LSMFT and fibrous dysplasia has been described based on the histologic characteristics and the presence of the Gsα mutation. Another hypothesis of the etiology of the lesion is the reaction of fibrous dysplasia to stress.

Storiform collagenoma: case report / Colagenoma estoriforme: relato de caso

Storiform collagenoma is a rare tumor, which originates from the proliferation of fibroblasts that show increased production of type-I collagen. It is usually found in the face, neck and extremities, but it can also appear in the trunk, scalp and, less frequently, in the oral mucosa and the nail bed. It affects both sexes, with a slight female predominance. It may be solitary or multiple, the latter being an important marker for Cowden syndrome. It presents as a painless, solid nodular tumor that is slow-growing. It must be considered in the differential diagnosis of other well-circumscribed skin lesions, such as dermatofibroma, pleomorphic fibroma, sclerotic lipoma, fibrolipoma, giant cell collagenoma, benign fibrous histiocytoma, intradermal Spitz nevus and giant cell angiohistiocytoma.

O colagenoma estoriforme é um tumor raro originado a partir da proliferação de fibroblastos com produção aumentada de colágeno tipo I. É encontrado mais frequentemente na face, pescoço e extremidades, podendo aparecer no tronco, couro cabeludo e, raramente, na mucosa oral e leito subungueal. Afeta ambos os gêneros, com discreta predominância em mulheres. Pode ser solitário ou múltiplo, sendo que, neste caso, é um importante indicador da presença de síndrome de Cowden. Apresenta-se como tumor sólido, nodular, de crescimento lento e indolor. Deve constar como diagnóstico diferencial de tumores cutâneos bem delimitados, como dermatofibroma, fibroma pleomórfico, lipoma esclerótico, fibrolipoma, colagenoma de células gigantes, histiocitoma fibroso benigno, nevo de Spitz intradérmico e angioistiocitoma de células gigantes.

Mortality and Embolic Potential of Cardiac Tumors / Mortalidade e Potencial Embólico dos Tumores Cardíacos

Background: Cardiac tumors are rare, mostly benign with high embolic potential. Objectives: To correlate the histological type of cardiac masses with their embolic potential, implantation site and long term follow up in patients undergoing surgery. Methods: Between January 1986 and December 2011, we retrospectively analyzed 185 consecutive patients who underwent excision of intracardiac mass (119 females, mean age 48±20 years). In 145 patients, the left atrium was the origin site. 72% were asymptomatic and prior embolization was often observed (19.8%). The diagnosis was established by echocardiography, magnetic resonance and histological examination. Results: Most tumors were located in the left side of the heart. Myxoma was the most common (72.6%), followed by fibromas (6.9%), thrombi (6.4%) and sarcomas (6.4%). Ranging from 0.6cm to 15cm (mean 4.6 ± 2.5cm) 37 (19.8%) patients had prior embolization, stroke 10.2%, coronary 4.8%, peripheral 4.3% 5.4% of hospital death, with a predominance of malignant tumors (40% p < 0.0001). The histological type was a predictor of mortality (rhabdomyomas and sarcomas p = 0.002) and embolic event (sarcoma, lipoma and fibroelastoma p = 0.006), but not recurrence. Tumor size, atrial fibrillation, cavity and valve impairment were not associated with the embolic event. During follow-up (mean 80±63 months), there were 2 deaths (1.1%) and two recurrences 1 and 11 years after the operation, to the same cavity. Conclusion: Most tumors were located in the left side of the heart. The histological type was predictor of death and preoperative embolic event, while the implantation site carries no relation with mortality or to embolic event. .

Fundamento: Os tumores do coração são infrequentes, em sua maioria benignos e com alto potencial embólico. Objetivo: Correlacionar o tipo histológico do tumor cardíaco com seu potencial embólico, com o sítio de implantação e analisar a evolução tardia destes pacientes submetidos à cirurgia. Métodos: No período de dezembro de 1986 a setembro de 2011 foram retrospectivamente analisados 186 pacientes operados (119 do sexo feminino e idade média de 48 ± 20 anos). Foram 145 tumores de átrio esquerdo (77%), 72% dos pacientes assintomáticos e 19,8% com embolização prévia. O diagnóstico foi confirmado por ecocardiograma, ressonância magnética e exame histológico. Resultados: A maioria dos tumores situava-se nas câmaras esquerdas. O mixoma foi o mais frequente (72,6%), seguido dos fibromas (6,9%), trombos (6,4%) e sarcomas (6,4%). Seus tamanhos variaram de 0,6cm a 15 cm (média de 4,6 ± 2,5cm). Houve 37 embolizações prévias à operação (10,2% AVC, 4,8% IAM e 4,3% periférica). Foram 5,4% de óbito hospitalar, com predomínio nos tumores malignos (40% p < 0,0001). O tipo histológico foi preditor de mortalidade (rabdomioma e sarcomas p = 0,002) e de evento embólico (sarcomas, fibroelastoma e lipoma p = 0,006), porém não de recidiva. O tamanho tumoral, a fibrilação atrial, a cavidade e valva acometida não apresentaram relação com o evento embólico. Durante o seguimento (média de 80 ± 63 meses), houve 2 óbitos (1,1%) e duas recidivas tumorais 1 e 11 anos após a operação, ambas para a mesma cavidade. Conclusão: O tipo histológico foi preditor de óbito e de evento embólico pré-operatório, enquanto o sítio de implantação não. .

Fibromatose digital infantil: relato de caso / Infantile digital fibromatosis: case report

Introdução: A fibromatose digital infantil é uma proliferação nodular, assintomática, rara e benigna do tecido fibroso, que ocorre quase exclusivamente na região dorsal e lateral dos dedos das mãos e pés. O artigo relata um caso de fibromatose digital infantil, também conhecida como tumor de Reye. Relato de Caso: Trata-se de um caso diagnosticado por meio de exames clínico, de imagem e histopatológico. O paciente apresentou-se ao Setor de Ortopedia do Hospital, queixando-se de uma lesão nodular, eritematosa, indolor, no segundo pododáctilo do pé esquerdo, existente havia quatro anos. Durante o exame físico, notava-se uma deformidade no II pododáctilo, causada por uma lesão nodular, eritematosa, indolor, de aproximadamente 1,5 cm, que não acarretava alterações funcionais. O exame de ultrassom revelou a presença de uma imagem nodular sólida, hipoecogênica, envolvendo o tendão do extensor do II pododáctilo na falange média. O diagnóstico inicial era de fibroma ou sinovioma. Pelas características clínicas da lesão, por seu tempo de evolução e pelos achados de imagem, a equipe optou por uma biópsia. No entanto, devido ao pequeno tamanho da lesão, sendo a biópsia aberta, realizou-se a exérese cirúrgica. O exame histopatológico confirmou o diagnóstico de fibromatose digital infantil. Conclusão: Esse tumor constitui uma entidade clínica rara, que deve ser diferenciada de outras lesões encontradas nos dedos das mãos e dos pés. O diagnóstico correto raramente é feito antes da operação, devido, principalmente, à falha em reconhecer essa entidade. Por essa razão, é essencial considerar essa lesão em diagnósticos diferenciais.

Introduction: Infantile digital fibromatosis, also known as Reye tumor, is a rare, asymptomatic, benign nodular proliferation of fibrous tissue, which occurs almost exclusively in the dorsolateral region of the fingers and toes. This article reports a case of infantile digital fibromatosis. Case report: This case was diagnosed by clinical, imaging, and histopathological examination. The patient presented at the rthopedic Department of our hospital, with a 4-year history of a painless, erythematous nodular lesion on the econd toe of the left foot. On physical examination, a deformity of the second toe caused by a nodular, erythematous, painless lesion of approximately 1.5 cm diameter was noted; the lesion did not result in functional changes. Ultrasound examination revealed a solid, hypoechoic nodule involving the extensor tendon in the middle phalanx of the second toe. The initial diagnosis was fibroma or synovioma. Due to the clinical characteristics of the lesion, its evolution, and the imaging findings, the team chose to perform a biopsy. However, due to the small size of the lesion, upon open biopsy, surgical excision was performed. Histopathological examination confirmed the diagnosis of infantile digital fibromatosis. Conclusion: Infantile digital fibromatosis is a rare clinical entity, which should be differentiated from other lesions found in the fingers and toes. The correct diagnosis is rarely made pre-operatively, due mainly to a failure to recognize this entity. For this reason, it is essential to onsider this lesion in the differential diagnosis of digital nodules.

Superficial Acral Fibromyxoma involving the nail's apparatus. Case report and literature review

Superficial Acral Fibromyxoma is a rare tumor of soft tissues. It is a relatively new entity described in 2001 by Fetsch et al. It probably represents a fibrohistiocytic tumor with less than 170 described cases. We bring a new case of SAF on the 5th toe of the right foot, in a 43-year-old woman. After surgical excision with safety margins which included the nail apparatus, it has not recurred (22 months of follow up). We carried out a review of the location of all SAF published up to the present day.