Oral benign fibrous histiocytoma in a pediatric patient: case report

Benign fibrous histiocytoma (BFH) is a common mesenchymal lesion on the skin where it is better known as dermatofibroma. It mainly affects areas of the integument exposed to the sun. It can occur in the mucous membranes, being quite unusual in the oral cavity. This article presents a rare case of benign oral fibrous histiocytoma in childhood. An eleven-year-old male patient sought the stomatology clinic of the dentistry course at a reference university in northeastern Brazil, complaining of an asymptomatic increase in volume on the cheek, with an evolution of approximately two years. On intraoral examination, a lesion in the cheek mucosa was observed, measuring approximately 4 cm. The lesion had a nodular, circumscribed, reddish appearance, showing ulcerated areas covered by a yellowish pseudomembrane on its surface. The clinical diagnostic hypothesis was pleomorphic adenoma. Excisional biopsy was performed and, after morphological and immunohistochemical evaluation, the diagnosis of BFH was reached. Although it is a lesion with an excellent prognosis, BFH must be carefully analyzed for the differential diagnosis with malignant neoplasms that may have a mild appearance, such as fibrosarcoma or dermatofibrosarcoma. In addition, other benign lesions such as desmoplastic fibroblastoma, myofibroma, myopericytoma and nodular fasciitis may, in some histological sections, mimic the microscopic appearance of benign fibrous histiocytoma. In this reported case, immunohistochemical evaluation was essential to conclude the diagnosis of BFH(AU)

Transformación nodular angiomatoide esclerosante del bazo: reporte de caso / Sclerosing angiomatoid nodular transformation of the spleen: a case report

La transformación nodular angiomatoide esclerosante es una patología vascular benigna del bazo, desarrollada a partir de la pulpa roja, de etiología desconocida. Se postula que puede estar relacionada con la enfermedad por inmunoglobulina 4 y la infección por el virus de Epstein-Barr. La mayoría de los casos son asintomáticos, constituyendo hallazgos incidentales en estudios por imágenes. Presentamos el caso de un paciente masculino de 41 años con antecedentes de tiroidectomía por carcinoma papilar que consulta por fiebre. Recibió tratamiento sintomático y se realizó tomografía computarizada de abdomen por síntomas abdominales inespecíficos. La tomografía evidenció una imagen de aspecto sólido, con tenue realce periférico con el contraste que mide 62 por 52 por 51 milímetros en el polo inferior del bazo. Se realizó esplenectomía que midió 14 por 11 por 4 centímetros y pesó 284 gramos. Se identificó una formación nodular sólida, bien delimitada, con área central de aspecto fibroso, con tractos blanquecinos que delimitan áreas violáceas. La microscopía presentó nódulos coalescentes redondeados de aspecto angiomatoide, con proliferación vascular revestida por células endoteliales sin atipia, entremezclados con células ahusadas, infiltrado de linfocitos y macrófagos. El estroma entre los nódulos mostró proliferación miofibroblástica con linfocitos, plasmocitos y siderófagos. Inmunohistoquímica tuvo marcación positiva en los vasos para CD34 y CD31, sectores positivos para CD8 y negativos para CD34. Una célula positiva para inmunoglobulina 4 (IgG4) por campo de gran aumento. El estudio para Epstein-Barr por reacción en cadena de la polimerasa fue negativo. Para el diagnóstico los estudios de imagen son inespecíficos, por lo que la confirmación diagnóstica la da el estudio histopatológico. La esplenectomía es curativa sin casos reportados hasta la actualidad de transformación maligna o recidiva. No se conocen factores de riesgo y no se han comprobado factores desencadenantes, excepto la asociación de casos con IgG4 y virus de Ebstein-Barr. Por ser una entidad patológica recientemente descrita es necesario recopilar series grandes y revisar nuestros archivos, reevaluando algunos de sus diagnósticos diferenciales para lograr una mejor comprensión de la misma.

Sclerosing angiomatoid nodular transformation is a benign vascular pathology of the spleen, developed from the red pulp, of unknown etiology; it is postulated that it may be related to IgG4 disease and Epstein-Barr virus infection. Most cases are asymptomatic, constituting incidental findings in imaging studies. We present a 41-year-old male patient with a history of thyroidectomy for papillary carcinoma who consulted for fever, received symptomatic treatment and performed a computed tomography of the abdomen for nonspecific abdominal symptoms, the same evidence in the lower pole of the spleen a solid-looking image with faint Peripheral enhancement with contrast, measures 62x 52x51 mm. A splenectomy measuring 14x 11x4 cm and weighing 284 grams was performed, identifying a solid, well-defined nodular formation, with a central fibrous-looking area, with whitish tracts that delimited purplish areas. Microscopy: rounded angiomatoid-like coalescing nodules, with vascular proliferation lined by endothelial cells without atypia, interspersed with spindle cells, infiltrated by lymphocytes and macrophages. The stroma between the nodules shows myofibroblastic proliferation with lymphocytes, plasma cells, and siderophages. Immunohistochemistry: positive labeling in vessels for CD34 and CD31, positive sectors for CD8 and negative for CD34. One IgG4 positive cell per high power field. The study for Epstein-Barr by Polymesara Chain Reaction was negative. For the diagnosis, the imaging studies are nonspecific, so the diagnostic confirmation is given by the histopathological study. Splenectomy is curative with no reported cases of malignant transformation or recurrence to date. There are no known risk factors and no triggering factors have been proven, except the association of cases with IgG4 and Ebstein-Barr virus. As it is a recently described pathological entity, it is necessary to collect large series and review our files, reevaluating some of its differential diagnoses to achieve a better understanding of it

Case for diagnosis. Atypical fibroxanthoma

Abstract Atypical fibroxanthoma is a rare superficial fibrohistiocytic tumor. Clinically, it presents itself as a painless, solitary, ulcerated, and slow-growing nodule found mainly on sun-exposed areas - especially of the head and neck - of elderly individuals. The diagnosis is based on histopathological and immunohistochemical features. Immunohistochemical staining plays a critical role for the exclusion of differential diagnoses. Since atypical fibroxanthoma has an indolent clinical course, surgical excision is adequate for treatment. We present a case of atypical fibroxanthoma on the ear of a patient.

Dermatomiofibroma lineal: reporte de un caso / Linear dermatomyofibroma: report of a case

El dermatomiofibroma es un tumor benigno infre-cuente de origen mesenquimático. Generalmente, se presenta como un tumor rosado nodular, espe-cialmente en mujeres en la década de los 30, en hombros y axila. Clínica e histológicamente puede simular otros tu-mores mesenquimáticos como el dermatofibroma , leiomioma y el dermatofibrosarcoma protuberans. Se presenta caso de dermatomiofibroma lineal, para describir sus características clínicas e histo-lógicas, necesarias para su correcto diagnóstico y enfrentamiento terapéutico.

Dermatomyofibroma is an uncommon mesen-chymal benign tumor. It usually presents in wo-men at their thirties, as a pink nodular tumor on the shoulder and axilla. Clinically and histologically it may mimic other mesenchymal tumors such as dermatofibroma, leiomyoma and dermatofibrosarcoma protube-rans. We present the case of a linear dermatomyofi-broma and describe its clinical and histological characteristics, which may help the clinician in the correct diagnosis and therapeutic approach of this rare but benign tumor.

Case for diagnosis. Multinucleated cell angiohistiocytoma

Abstract: Multinucleate cell angiohistiocytoma is a rare idiopathic benign fibrohistiocytic and vascular proliferation usually presenting as multiple asymptomatic papules, red to violaceous in colour, primarily located on the extremities of middle-aged females. This entity is probably underdiagnosed due to the lack of recognition by clinicians and pathologists. We describe a patient with a multinucleate cell angiohistiocytoma of the face, a less frequent localization, in order to increase awareness of this entity and elucidate its clinical, histopathological, and immunohistochemistry features.

Patrón dermatoscópico en arcoiris (irisado) en dermatofibroma hemosiderótico: reporte de un caso / Dermoscopic pattern in rainbow (iridescent) in hemosiderotic dermatofibroma: a case report

El patrón en arcoiris ha sido un tema controvertido, dado que en un principio se planteó como un patrón dermatoscópico específico asociado al diagnóstico de Sarcoma de Kaposi. Sin embargo, esta asociación ha sido cuestionada por diversos reportes que han evidenciado la presencia de este patrón dermatoscópico en otro tipo de lesiones, tales como, otros tumores vaculares, cicatriz hipertrófica, angioqueratoma, dermatitis por estasis, pseudo-Kaposi (acroangiodermatitis), melanoma, liquen plano, dermatofibroma hemosiderótico, entre otros. Se ha propuesto que este efecto se genera por la absorción, difracción y difusión de la luz polarizada y su interferencia con diferentes componentes vasculares y fibrosos de la dermis más que por algún hallazgo histopatológico específico. Presentamos el primer caso con registro fotográfico de dermatofibroma hemosiderótico con presencia de un 'patrón irisado' en la dermatoscopía.

The dermoscopic 'rainbow pattern' has been a controversial issue; it was initially proposed as a specific dermatoscopic pattern associated with Kaposi's Sarcoma. However, this has been questioned by several reports that have shown the presence of this dermoscopic pattern in a wide variety of lesions such as other vascular tumors, hypertrophic scars, angiokeratoma, stasis dermatitis, pseudo-Kaposi acroandgiodermatitis), melanoma, lichen planus, and hemosiderotic dermatofibroma, among others. It has been proposed that this effect is generated by the absorption, diffraction and diffusion of polarized light and its interference with different vascular and fibrous components of the dermis, rather than being caused by a specific histopathologic finding. We present the first photographically recorded case of hemosiderotic dermatofibroma, characterized by the presence of a rainbow pattern in dermoscopy.

Clinical and histological patterns of dermatofibroma without gross skin surface change: A comparative study with conventional dermatofibroma.

Backg round: Dermatofibroma sometimes clinically presents as a nodular lesion without gross skin surface change. Clinicopathologic features of this variant of dermatofi broma have not been evaluated. Aims: To assess clinicopathologic features of dermatofi broma presenting as a subcutaneous nodule. Methods: This study reviewed the clinical and histological features of 42 cases of subcutaneous dermatofibromas and compared them with 95 cases of conventional dermatofi broma. Results: Dermatofi broma without gross skin surface change was associated with a shorter pre-diagnosis duration than conventional dermatofi broma. Increase in size during the pre-diagnosis period was signifi cantly more frequent in the conventional type. In addition, these dermatofi bromas were more likely than the conventional type to occur in the head and neck region. Although tumor depth was deeper than in the conventional type, less than half of the dermatofi bromas without gross skin surface change were found histologically to be “subcutaneous” or “deep-penetrating dermatofi broma”. Subcutaneous extension was more frequent in these dermatofi bromas while focal stromal hyalinization and hemosiderin deposits were more common in the conventional type. Limitations: This study is a retrospective, single center design. Conclusion: The present study suggests that dermatofi broma without gross skin surface change is a variant type with distinct clinical and histological features that distinguish them from conventional dermatofi broma.

Treatment of oral hemangioma with sclerotherapy: case report / Tratamento de hemangioma oral com escleroterapia: relato de caso

Hemangiomas are benign neoplasms that are common in the head and neck, but relatively rare in the oral cavity. They can cause esthetic and functional impairment, depending on location. The most common site is the upper lip, but they can occur in other areas, such as the tongue, buccal mucosa and palate. Treatment is primarily dependent on correct diagnosis of the lesion and on its anatomic location. The purpose of this article is to provide a description of a case of a hemangioma on the upper lip, treated by therapeutic sclerosis with monoethanolamine oleate (Ethamolin®), covering clinical characteristics and methods for diagnosing these lesions. Precise diagnosis and appropriate therapeutic management resulted in satisfactory esthetic and functional results, with total regression of the lesion and no signs of relapse at 1-year follow-up...

O hemangioma é uma neoplasia benigna comum na região de cabeça e pescoço, e é relativamente rara na cavidade oral, podendo causar prejuízo estético e funcional a depender da sua localização. Sua localização mais frequente é o lábio superior, mas pode ocorrer em outras regiões, como língua, mucosa jugal e palato. O seu tratamento depende, principalmente, do correto diagnostico da lesão, bem como da localização anatômica da mesma. A proposta deste artigo é relatar um caso de hemangioma em lábio superior tratado com esclerose terapêutica com oleato de monoetanolamina (Ethamolin®), considerando as características clínicas e os métodos de diagnóstico desta lesão. Por meio de um diagnóstico preciso e uma conduta terapêutica adequada, o caso apresenta-se com um acompanhamento de um ano, sem sinais de recidiva, e com um resultado estético funcional satisfatório, com regressão total da lesão...

Benign fibrous histiocytoma of the maxilla: A case report and review of literature.

Benign fibrous histiocytoma (BFH) is a mesenchymal tumor which commonly affects the skin of extremities. Histologically, BFH of soft tissues and bone shows similar features, but BFH of the bone is rare with less than 100 cases reported worldwide and usually it involves tibia, femur, and pelvic bone. As far as the oral cavity is concerned, majority of the BFHs are found in the soft tissues of the buccal mucosa, gingiva, lower and upper lips, soft palate, and floor of the mouth. Occurrence of this tumor in the jaw bones is extremely rare as only one case of the maxilla and six cases of the mandible have been reported so far . Hence, the purpose of this article is to report the second case of BFH of the maxilla in a 23-year-old female patient and to review the literature of this entity affecting the jaws.

Intracranial Fibroxanthoma in an Infant: a Case Report

A primary fibroxanthoma of the central nervous system is very rare. We present a case of an infantile fibroxanthoma that arose from the cranial dura mater in a six-month-old girl with US, MRI and PET/CT features that mimicked a meningioma. The tumor appeared as a large, well-circumscribed echogenic mass in the right parieto-occipital area on US. The tumor was seen as isoattenuated to slightly hypoattenuated on pre-contrast CT scan and as hypometabolic on PET/CT. As seen on T2-weighted image, the mass was heterogeneously hyperintense to the gray matter. The mass was isointense on T1-weighted image and homogeneously strongly enhanced on contrast enhanced T1-weighted image.

Oral fibro-histiocytic lesions: immuno-histological investigation and review of an unusual presentation in the tongue

A right sided mid-tongue growth in a 14-year-old boy diagnosed initially as Pyogenic Granuloma was found subsequently to be a Benign Fibrous Histiocytoma. This lesion had three seeming recurrences with divergent histologic and clinical appearances within a short period of time. Although there was eventual consensus on the morphogenesis and benign histologic nature of the lesion this caused diagnostic and management problems and a potential prognostic dilemma. This report on a unique and aggressive head and neck lesion, possibly the third documented case ill the tongue, reviews the bewildering spectrum of clinical and immuno- histologic manifestations of fibrous histiocytic lesions. Wide excision with close follow up is recommended

Benign fibrous histiocytoma of bone: a case report.

Benign fibrous histiocytoma (BFH) occurs commonly in soft tissues with very few cases of BFH of bone on record. We report a case of BFH of tibia in fifty year old male who presented with supracondylar fracture femur due to trivial fall and a brief review of literature.

False Positive and False Negative FDG-PET Scans in Various Thoracic Diseases

Fluorodeoxyglucose (FDG)-positron emission tomography (PET) is being used more and more to differentiate benign from malignant focal lesions and it has been shown to be more efficacious than conventional chest computed tomography (CT). However, FDG is not a cancer-specific agent, and false positive findings in benign diseases have been reported. Infectious diseases (mycobacterial, fungal, bacterial infection), sarcoidosis, radiation pneumonitis and post-operative surgical conditions have shown intense uptake on PET scan. On the other hand, tumors with low glycolytic activity such as adenomas, bronchioloalveolar carcinomas, carcinoid tumors, low grade lymphomas and small sized tumors have revealed false negative findings on PET scan. Furthermore, in diseases located near the physiologic uptake sites (heart, bladder, kidney, and liver), FDG-PET should be complemented with other imaging modalities to confirm results and to minimize false negative findings. Familiarity with these false positive and negative findings will help radiologists interpret PET scans more accurately and also will help to determine the significance of the findings. In this review, we illustrate false positive and negative findings of PET scan in a variety of diseases.

Histiocitoma fibroso benigno profundo: achados na tomografia computadorizada e histologia / Deep benign fibrous histiocytoma: computed tomography and histology findings

Apresentamos imagens de tomografia computadorizada de um paciente de 83 anos de idade com histiocitoma fibroso benigno profundo, na face lateral da perna esquerda, com evolução de longa data. A tomografia computadorizada observamos massa bem delimitada, ovalada, e que apresentava intenso realce periférico com uso do meio de contraste iodado. Este é um tumor de partes moles pouco freqüente e com raros relatos dos seus aspectos de imagem.

Fibrohistiocitoma benigno ósseo na coluna lombar / Benign fibrous histiocytoma of the lumbar spine

CONTEXTO: O fibrohistiocitoma benigno intra-ósseo é uma neoplasia rara, caracterizada por proliferação fibroblástica e fibras colágenas. A dor na região lombar é uma queixa freqüente em muitos pacientes, mas, na maioria das vezes está relacionada com causa muscular ou degenerativa. RELATO DE CASO: Relata-se aqui o caso de um paciente com diagnóstico de fibrohistiocitoma benigno ósseo na coluna lombar que apresentava queixa de lombalgia há cinco meses antes do diagnóstico. Foi submetida à biópsia aberta transpedicular na quarta vértebra lombar ,e, posteriormente realizada curetagem da lesão via anterior retroperitonial e artrodese vertebral de L3-L5 com enxerto de fíbula. CONCLUSAO: Apesar de raro, o fibrohistiocitoma benigno intra-ósseo deve ser uma das hipóteses quando observamos uma lesão primária na coluna vertebral.