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.

Peripheral fibroma obstructing the eruption pathway.

Reactive hyperplasias comprise a group of fibrous connective tissue lesions that commonly occur in oral mucosa secondary to injury. Peripheral fibroma is also a type of reactive hyperplasia. Peripheral fibroma is a reactive hyperplastic mass that occurs on the gingiva and is derived from connective tissue of the submucosa or periodontal ligament. It may occur at any age, although it does have a predilection for young adults. Females develop these lesions more commonly than do males. Here, we are presenting a case report of a child who is 6 years old and presented to us with a swelling in the lower anterior region which was providing discomfort to the child and also was obstructing the eruption pathway of the permanent central incisor in the region. The lesion was surgically excised.

Lipofibromatosis accompanied by several congenital anomalies, report of a rare case.

Lipofibromatosis is a rare tumor of infancy, which has been reported about 10 years ago, and since then very few reports have been published. This tumor has been reported in association with congenital anomalies such as macrosyndactyly, but there is no report of multiple congenital anomalies with lipofibromatosis in the English literature as far as our knowledge goes. Herein, we report a case of this tumor associated with syndactyly, bilateral complete cleft lip and palate, trigonocephaly, and atrial septal defect.

Infarto do miocárdio recorrente em um paciente com fibroelastoma papilar / Recurrent myocardial infarction in a patient with papillary fibroelastoma / Infarto de miocardio recurrente en un paciente con fibroelastoma papilar

Este relatório descreve o caso de um homem de 62 anos, admitido por infarto do miocárdio recorrente e artérias coronárias normais, causado por embolia coronária a partir de fibroelastoma papilar aórtico. Outros quadros que causam a síndrome coronariana aguda são discutidos, assim como as artérias coronárias normais. Nesse quadro clínico, faz-se necessária a avaliação cuidadosa, por meio de ecocardiograma transtorácico e transesofágico. A excisão cirúrgica do tumor é segura e curativa.

This report describes a case of a 62 year-old man admitted for recurrent myocardial infarction and normal coronary arteries, caused by coronary embolism from aortic papillary fibroelastoma. Other conditions causing acute coronary syndrome and normal coronary arteries are discussed. A careful evaluation by transthoracic and transesophageal echocardiography is required in this clinical setting. Surgical excision of the tumor is safe and curative.

Este informe describe el caso de un hombre de 62 años, admitido por infarto de miocardio recurrente y arterias coronarias normales, causado por embolia coronaria a partir de fibroelastoma papilar aórtico. Otros cuadros que causan el síndrome coronario agudo son discutidos, así como las arterias coronarias normales. En ese cuadro clínico, se hace necesaria la evaluación cuidadosa, por medio de ecocardiograma transtorácico y transesofágico. La escisión quirúrgica del tumor es segura y curativa.

Heart valve papillary fibroelastoma associated with cardioembolic cerebral events / Fibroelastoma papilífero de valva cardíaca associado a eventos cerebrais cardioembólicos

Papillary fibroelastomas of the heart valves are benign, slow-growing, rare tumors of the heart. This tumor represents a potential cause of systemic embolism, stroke, myocardial infarction and sudden death. Early diagnosis is very important, as surgical excision of these tumors can prevent cerebrovascular and cardiovascular complications. Diagnosis is usually made by transesophageal echocardiogram. We describe two cases of patients with papillary fibroelastomas causing cardioembolic cerebral events, which underwent successful surgical treatment. The authors present a brief review of the literature.

Fibroelastoma papilífero é um tumor benigno, raro e de crescimento lento, que geralmente tem origem no endocárdio das valvas cardíacas. Este tumor representa uma causa potencial de embolia sistêmica, infarto agudo do miocárdio, acidente vascular cerebral e morte súbita. O diagnóstico precoce é fundamental, já que a excisão cirúrgica pode evitar complicações cerebrovasculares e cardiovasculares. O diagnóstico geralmente é feito pelo ecocardiograma transesofágico. Neste trabalho, são descritos dois casos de pacientes com fibroelastoma papilífero de valva aórtica, causando eventos cerebrais cardioembólicos, que foram submetidos com sucesso a tratamento cirúrgico. Apresenta-se, ainda, uma sucinta revisão da literatura.

Maxillary ameloblastic fibroma: a case report

Ameloblastic fibroma is a relatively rare benign odontogenic tumor in which both the epithelial and ectomesenchymal components are neoplastic. An 8-year-old Caucasian boy was referred to the dentist for evaluation of failed eruption of the maxillary left first molar. The panoramic radiograph showed a well-circumscribed unilocular radiolucency involving an unerupted maxillary left first permanent molar. The lesion was enucleated and the material was sent for histopathologic examination. Microscopically, it was composed by cords and islands of odontogenic epithelium in a myxoid cell-rich stroma that closely resemble the dental papilla with histopathological diagnosis of ameloblastic fibroma. After 24 months of follow-up no recurrence was observed and the maxillary left first molar erupted spontaneously through the buccal mucosa and was aligned with a fixed orthodontic appliance. This case emphasized the importance of careful differential diagnosis of intraosseous oral lesions and reported a rarity of the lesion and its atypical location.

Fibroma ameloblástico é um tumor odontogênico benigno relativamente raro, em que ambos os componentes epiteliais e ectomesenquimais são neoplásicos. Menino de oito anos de idade, branco, foi encaminhado ao dentista para avaliar a falha na erupção do primeiro molar maxilar do lado esquerdo. A radiografia panorâmica revelou imagem radiolúcida, unilocular, bem circunscrita, envolvendo o primeiro molar permanente maxilar esquerdo incluso. A lesão foi enucleada e o material encaminhado para avaliação histopatológica. Microscopicamente, era composta de ilhas e cordões de epitélio odontogênico num estroma mixóide rico em células, que se assemelhava à papila dentária, com diagnóstico histopatológico de fibroma amelobástico. Após 24 meses de acompanhamento, nenhuma recorrência foi observada e o primeiro molar permanente maxilar irrompeu espontaneamente através da mucosa bucal e foi alinhado com aparelho ortodôntico fixo. Esse caso enfatiza a importância do cuidadoso diagnóstico diferencial das lesões orais intra-ósseas e relato de lesão rara e sua localização atípica.

Fibroelastoma papilífero do ventrículo esquerdo / Fibroelastoma papilar del ventrículo izquierdo / Left ventricular papillary fibroelastoma

Avaliamos o caso de uma paciente portadora de fibroelastoma papilífero (FEP) que apresentou embolização para membro superior direito. A paciente foi submetida à embolectomia percutânea, com retirada do fragmento. O diagnóstico foi confirmado por ecocardiograma transtorácico e exame anatomopatológico. Optou-se pelo tratamento clínico conservador e acompanhamento da paciente, que mostrou boa evolução e não teve recorrência do quadro até o momento. Aproveitamos esse raro e interessante caso na intenção de revisar a literatura vigente e discutir a melhor conduta terapêutica.

We evaluated the case of a patient with Papillary Fibroelastoma (PFE) that presented embolization to the upper right limb. The patient was submitted to percutaneous embolectomy, with fragment removal. The diagnosis was confirmed by transthoracic echocardiogram and anatomopathological analysis of the fragment. The patient chose to undergo the conservative clinical treatment and the follow-up has shown good evolution with no disease recurrence to date. We decided to use this rare and interesting case with the objective of reviewing the current literature and discuss the best therapeutic management.

Evaluamos el caso de una paciente portadora de fibroelastoma papilar (FEP) que presentó embolización para miembro superior derecho. La paciente fue sometida a embolectomía percutánea, con retirada del fragmento. El diagnóstico fue confirmado por ecocardiograma transtoracico y examen anatomopatológico. Se optó por el tratamiento clínico conservador y seguimiento de la paciente, que mostró la buena evolución y no tuvo recurrencia del cuadro hasta el momento. Aprovechamos este raro e interesante caso en la intención de revisar la literatura vigente y discutir la mejor conducta terapéutica.

Caso para diagnóstico / Case for diagnosis

Fibromatose hialina juvenil e hialinose sistêmica infantil são desordens autossômicas recessivas, raras da infância. À histologia, há depósito de material hialino na derme e subcutâneo. As características clínicas principais são: lesões pápulo-nodulares, hipertrofia gengival, contratura articular, lesões ósseas osteolíticas e retardo no crescimento. Mutações no mesmo gene foram identificadas nas duas condições, sugerindo que sejam espectros da mesma doença.

Juvenile hyaline fibromatosis and infantile systemic hyalinosis are rare autossomal recessive disorders with onset in infancy or early childhood. Histological examination shows deposit of hyaline material in the dermis and subcutaneous tissue. Clinical features include papulonodular skin lesions, gingival hypertrophy, flexion contractures of joints, osteolytic bone lesions and stunted growth. Mutations in the same gene were detected in both conditions, suggesting that they may be variants of the same disorder.

Fibroelastoma valvular aórtico como causa de accidente cerebrovascular embólico: reporte de un caso / Aortic valve fibroelastoma as a cause of an embolic cerebrovascular accident: a case report

Los tumores cardíacos son una causa rara de accidente cerebrovascular embólico. Comunicamos el caso de una paciente de 65 años quien debuta su historia con un accidente cerebrovascular. El estudio de fuente embólica con ecocardiografía transesofágica demostró un fibroelastoma de la válvula aórtica en el borde libre del velo no coronariano. El tumor fue extraído mediante circulación extracorpórea. El estudio anatomopatológico confirmó el diagnóstico y la paciente se encuentra en capacidad funcional I y sin síntomas neurológicos a 19 meses de seguimiento.

Background: Cardiac tumors are an infrequent cause of an embolic source and aortic fibroelastoma is even more rare as causative of a stroke. We report a 65 year old female with no particular clinical history admitted to the hospital with an embolic cerebrovascular accident whose embolic source study with a transesophageal echocardiogram revealed a fibroelastoma in the free edge of the non coronarian leaflet of the aortic valve. The patient was subjected to surgery by means of extracorporeal circulation and the tumor was excised. The pathological study confirmed the diagnosis. Post operative recovery was uneventful with no neurological damage and after 19 months of follow up she is in functional class I.

Dispositivo intrauterino en cavidad abdominal: presentación de un caso y revisión del tema / Intrauterine device in the abdominal cavity

Se presenta un caso de un dispositivo intrauterino (ASA de Lippes) en cavidad abdominal en una paciente a la que se le practica una histerectomía por fibromatosis uterina y que pensaba le había sido retirado hacia 15 años. Cursó asintomática e infertil hasta seis meses antes de la intervención. Se hace una revisión del tema donde se plantea la posibilidad de perforación uterina en el momento de la inserción y/o migración del dispositivo hacia el peritoneo pelviano...

Tuberous sclerosis with portal vein thrombosis, protein C and S deficiency.

A 29-year-old lady with a bad obstetric history and portal vein thrombosis, presented to the Skin OPD for facial lesions. On examination, angiofibromas on face, shagreen patch and periungual fibromas were observed. She also had dental pits and a retinal hamartoma. Investigations revealed hamartomas in the brain and kidney. Hematological work-up showed protein C and S deficiency with Factor V Leiden positivity. Except for the cutaneous symptoms, the patient did not have any clinical manifestations in other organs affected by tuberous sclerosis. A similar association of tuberous sclerosis with protein C deficiency has been reported in only one case in literature.

Fibroelastoma de valva mitral como causa de acidente vascular isquêmico transitório / Fibroelastoma of the mitral valve as a cause of transient ischemic stroke

A 44-year-old woman had a transient ischemic stroke, fibroelastoma of the mitral valve being the source of the embolus. The patient evolved with neutropenia induced by ticlopidine after 10 days of treatment. We report the major clinical features, therapeutical options, and medicamentous toxicity resulting from the use of antiplatelet drugs

Paquidermodactilia: inusual asociación con porfiria cutánea tarda en una niña / Pachydermodactyly: unusual association with porphyria cutanea tarda ina a girl

La paquidermodactilia (PDD) es una forma rara de fibromatosis superficial benigna de los dedos de la mano, que afecta a hombres jóvenes, caracterizada por engrosamiento fusiforme de tejidos blandos de las superficies dorsal y principalmente lateral de las articulaciones interfalángicas proximales de los dedos de las manos. El objetivo de este trabajo es presentar el estudio clínico e histopatológico de una niña con paquidermodactilia asociada a porfiria cutánea tarda (PCT). Según la bibliografía consultada, dicha asociación no ha sido previamente reportada

Cardiac fibroma presenting as sudden death in a six-month-old infant

Cardiac fibroma is a rare benign tumour which occurs predominantly in infancy and childhood. We present the case of a six-month-old female infant who died suddenly at home and was found at autopsy to have a large cardiac fibroma in the ventricular septum. The tumor was apparently asymptomatic although there was evidence of mild cardiac failure. Death was thought to be due to a fatal arrhythmia.

Adult filarial worm (Wuchereria bancrofti) in a benign ovarian tumour.

A case of an adult filarial worm (Wuchereria bancrofti) associated with a benign ovarian tumour and a cyst is presented. Possible reasons for the findings of a well preserved part of the worm and a foreign body granuloma in the lymphatic subendothelium are discussed.

Fístula esôfago-aórtica causada por pseudotumor inflamatório do esôfago / Aortic-esophageal fistula caused by inflammatory pseudotumor of the esophagus

Um homem de 56 anos, até entäo saudável, morreu na sala cirúrgica por hemorragia digestiva maciça, uma semana após episódio isolado de odinofagia pós prandial. O espécime cirúrgico do esôfago e da aorta mostrou um trajeto fistuloso, estenddendo-se de uma massa tumoral esofageana para a aorta. O exame histológico mostrou os achados microscópicos do chamado pseudotumor inflamatório (PTI) do esôfago. Até onde sabemos, esse e o primeiro caso relatado de PTI do esôfago com formaçäo de fístula para a aorta