El análisis inmunohistoquímico para el diagnóstico del tumor fibroso solitario/hemangiopericitoma / Immunohistochemical analysis for the diagnosis of solitary fibrous tumor/hemangiopericytoma

Resumen El tumor fibroso solitario/ hemangiopericitoma (TFS/HP) es un tumor extraaxial de origen mesenquimático de infrecuente observación, que usualmente se confunde con el meningioma, del cual puede ser clínica y radiológicamente indistinguible. El análisis molecular con la detección de la expresión nuclear STAT6 (signal transducer and activator of transcription 6) o la fusión NAB2-STAT6 (NGFI-A binding protein 2) es recomendable para confirmar el diagnóstico. Presentamos 3 casos clínicos, 2 mujeres y 1 varón, con diagnóstico anatomopatológico de meningioma meningotelial en el primer caso; y los casos 2 y 3 con sospecha radiológica de meningioma. La revisión anatomopatológica con estudio molecular permitió certificar el diagnóstico de TFS/ HP. Para el diagnóstico diferencial entre TFS/HP meníngeo y meningioma, se recomienda buscar la expresión de STAT6 como primer paso o la fusión NAB2-STAT6. La revisión de las muestras de biopsia debe estar garantizada en todos los pacientes, inclusive en aquellas que fueron estudiadas en Servicios de Patología Nivel 3.

Abstract The solitary fibrous tumor/ hemangiopericytoma (TFS/HP) is a rare mesenchymal extraaxial tumour. TFS/HP can sometimes be difficult to distinguish from other extra-axial tumors like meningioma, which can be clinically and radiologically indistinguishable. Molecular analysis with STAT6 (signal transducer and activator of transcription 6) nuclear expression or NAB2-STAT6 (NGFI-A binding protein 2) fusion is recommended to confirm the diagnosis. We present 3 cases, 2 women and 1 male, with pathological diagnosis of meningothelial meningioma in the first case; cases 2 and 3 with radiological suspicion of meningioma. The pathological review with molecular study certified the diagnosis of TFS/HP. For differential diagnosis between meningeal TFS/HP and meningioma, it is recommended to look for STAT6 expression as a first step, or NAB2-STAT6 fusion in order to confirm TFS/HP. The review of biopsy samples must be guaranteed in all patients, including those who were studied in Pathology Services Level 3.

Glomangiopericitoma: reporte de un caso / Glomangiopericytoma: a case report

El glomangiopericitoma es un tumor nasosinusal extremadamente raro, más frecuente en pacientes de edad avanzada y en mujeres. Si bien su etiopatogenia es desconocida, se reconocen ciertos factores predisponentes tales como hipertensión arterial, embarazo, trauma y uso de corticoides. Para su diagnóstico, resulta necesario recurrir tanto a estudio por imágenes como a la histología y técnicas de inmunohistoquímica. Microscópicamente se caracteriza por un prominente crecimiento perivascular de células uniformes ovales o fusiformes, dispuestas en fascículos cortos intercalados con capilares de diámetros variables ramificados en "asta de ciervo" y presenta inmuno-rreactividad positiva para actina, factor XIII-A y vimentina principalmente. Clínicamente este tumor presenta un comportamiento generalmente benigno, pero con una elevada tasa de recurrencia. Presentamos el caso de una mujer de 71 años, con antecedentes de hipertensión arterial, que acudió a nuestro centro por rinorrea purulenta y epistaxis unilateral derecha de 5 años de evolución. Al examen se observa masa polipoidea en fosa nasal derecha con abundante vascularización, sin otros hallazgos al examen físico. La lesión es resecada en su totalidad mediante cirugía endoscópica. El estudio histológico e inmunohistoquímico son compatibles con glomangiopericitoma. La paciente evoluciona con remisión de su sintomatología y a los dos meses desde la resección no ha presentado evidencias de recurrencia.

Glomangiopericytoma is an extremely rare sinonasal tumor, more common among elderly and women. Although its etiology and pathogeny are unknown, there are certain predisposing factors, such as arterial hypertension, pregnancy, trauma and corticosteroids. To diagnose this tumor, it is necessary imaging, histopathologic and inmunohistochemical studies. At the microscopic study, it is characterized by a prominent perivascular growth of oval-shaped or fusiform cells, arranged in short fascicles separated by capillary vessels of variable diameters, with staghorn-like ramifications, and lmmunohistochemistry positive mainly for actin, XIII-A factor and vimentin. Clinically, this tumor has a generally benign behavior, but with high recurrence percentage. We present the case of a 71 years old woman, with history of hypertension, who present in our center with a 5 years history of purulent rhinorrhea and right unilateral epistaxis, at the physical exam there is a polypoid mass in the right nasal fossa with rich vascularization, with no other findings. This tumor was resected entirely with endoscopic surgery. Histology and immunohistochemistry were compatible witch gomangiopericytoma. The patient evolve with remission of her symptoms and with no signs of recurrence at two-month follow-up.

Tumor fibroso solitario meníngeo / Meningeal fibrous solitary tumor

Se presenta caso de paciente masculino de 28 años, sin antecedentes médicos de importancia, con síndrome convulsivo primer evento. Se le realiza estudio de imagen donde se documenta lesión ocupativa extraaxial temporo ­ occipito ­ cerebelar derecha. Paciente es llevado a resección de tumor. Se obtiene hallazgo histopatológico de tumor fibroso solitario meningeo. Además se documenta foco irritativo cortical a este mismo nivel. El tumor fibroso solitario meningeo representa un espectro de tumores mesenquimales, agrupado actualmente por la Clasificación de la OMS como Tumor fibroso solitario/ Hemangiopericitoma grado 1. El tratamiento se basa en resección quirúrgica amplia y vigilancia a largo plazo...(AU)

Tumor fibroso solitario dérmico: presentación de caso clínico / Dermal solitary fibrous tumor: a clinical case presentation

Objetivo: analizar la importancia de una metodología protocolizada para el diagnóstico y tratamiento de patologías de baja frecuencia, como el tumor fibroso solitario, y la del diagnóstico diferencial con otras entidades. Caso clínico: se trata de una paciente con lesión subcutánea de 13 cm x 3,5 cm en región geniana izquierda. Tras efectuar estudios clínicos por imágenes y anatomopatológicos, cuyo diagnóstico presuntivo era tumor de tejido blando vascularizado, se realizó la resección de la lesión y la reconstrucción de la zona intervenida, mediante un colgajo local. El diagnóstico definitivo fue tumor fibroso solitario dérmico. Conclusiones: los datos clínicos orientan las conductas diagnósticas complementarias. Los estudios por imágenes muestran las características de la lesión, pero no la definen. La planificación del tratamiento implica la preparación del paciente, la elección de la conducta terapéutica, la evaluación de las posibles complicaciones y el seguimiento del caso.

Heart rate turbulence analysis in female patients with fibromyalgia

OBJECTIVE: Fibromyalgia is characterized by diffuse musculoskeletal pain and discomfort. There are several reports regarding autonomic nervous system dysfunction in patients with fibromyalgia. Heart rate turbulence is expressed as ventriculophasic sinus arrhythmia and has been considered to reflect cardiac autonomic activity. Heart rate turbulence has been shown to be an independent and powerful predictor of sudden cardiac death in various cardiac abnormalities. The aim of this study is to determine whether heart rate turbulence is changed in female patients with fibromyalgia compared with healthy controls. METHODS: Thirty-seven female patients (mean age, 40±11 years) with fibromyalgia, and 35 age- and sex-matched healthy female control subjects (mean age, 42±9 years) were included. Twenty-four hours of ambulatory electrocardiography recordings were collected for all subjects, and turbulence onset and turbulence slope values were automatically calculated. RESULTS: The baseline clinical characteristics of the two groups were similar. There were no significant differences in turbulence onset and turbulence slope measures between patients and control subjects (turbulence onset: −1.648±1.568% vs. −1.582±1.436%, p ϝ 0.853; turbulence slope: 12.933±5.693 ms/RR vs. 13.639±2.505 ms/RR, p ϝ 0.508). Although body mass index was negatively correlated with turbulence slope (r ϝ −0.258, p ϝ 0.046), no significant correlation was found between body mass index and turbulence onset (r ϝ 0.228, p ϝ 0.054). CONCLUSION: To the best of our knowledge, this is the first study to evaluate heart rate turbulence in patients with fibromyalgia. It appears that heart rate turbulence parameters reflecting cardiac autonomic activity are not changed in female patients with fibromyalgia. .

Tumor fibroso solitário renal: relato de caso clínico / Solitary fibrous tumor of the kidney: a case report

We describe a case report of a rare cancer affecting the kidney of 63 years of age patient who underwent imaging studies from ultrasound, computed tomography, and magnetic resonance imaging, due to complaints: left back pain and urinary obstruction. Sonographic and radiological features of the mass found suggested the diagnosis of a malignant tumor of parenchymal origin. The pathological and immunohistochemical studies of the resected anatomical specimen, however, diagnosed a rare benign variant of solitary fibrous tumor originating in the inferior pole of the left kidney...

Descrevemos o relato de caso de uma rara neoplasia acometendo o rim de um paciente de 63 anos de idade que foi submetido a estudos de imagem por ultrassonografia, tomografia computadorizada e ressonância magnética nuclear devido às queixas apresentadas: dor lombar à esquerda e alterações miccionais sugestivas de obstrução infravesical. As características ultrassonográficas e radiológicas da massa encontrada sugeriam o diagnóstico de uma neoplasia maligna de origem parenquimatosa. O estudo anatomopatológico e imuno-histoquímico da peça anatômica ressecada, entretanto, concluiu o quadro como variante benigna do raro tumor fibroso solitário originário do polo inferior do rim esquerdo...

Hemangiopericitoma: reporte de 3 casos / Hemangiopericytoma: a report of 3 cases and a literature review

Introduction: The hemangiopericytoma (HPC) is a rare vascular tumor that can be potentially malignant. It can be found in any part of the body but usually in the lower extremities or the retroperitoneum. Because its potential malign nature, it's necessary to perform oncological resections when are operating on. Methods: Three cases are presented. Results: One of them was located at the sacrococcigeal space, being the second reported case in the international literature. The other cases were in cervical and adrenal localization.

Introducción: El hemangiopericitoma (HPC) es un tumor vascular raro y potencialmente maligno que puede localizarse en cualquier parte del cuerpo, con mayor frecuencia en extremidades inferiores y retrope-ritoneo. Su potencial malignidad da la necesidad de realizar resecciones oncológicas al operarlos. Material y Método: Se presentan tres casos tratados por nuestro grupo. Resultados: Uno de ellos tiene localización sacra siendo, hasta donde sabemos, el segundo en ser publicado. Otro es de localización suprarrenal y el tercero es cervical.

Oncogenic osteomalacia: loss of hypophosphatemia might be the key to avoid misdiagnosis / Osteomalacia oncogênica: a perda de fosfatemia pode ser a chave para evitar o diagnóstico incorreto

Diagnosing oncogenic osteomalacia is still a challenge. The disorder is characterized by osteomalacia caused by renal phosphate wasting and low serum concentration of 1,25-dihydroxyvitamin D3 occurring in the presence of a tumor that produces high levels of fibroblast growth factor 23. However, it is possible that the disease is much more misdiagnosed than rare. We present the case of a 42-year-old man with a long-term history of undiagnosed progressive muscle weakness. His laboratory results mainly showed low serum phosphate. Surgical removal of a nasal hemangiopericytoma that had been diagnosed five years earlier, brought him to a symptom-free condition. Even though knowing the underlying etiology would explain his osteomalacia, the patient sought medical help from countless physicians for five consecutive years, and only after adequate treatment a rewarding outcome was achieved. Arq Bras Endocrinol Metab. 2012;56(8):570-3.

A osteomalacia oncogênica é um diagnóstico clínico desafiador, caracterizado pela perda renal de fosfato e baixos níveis de 1,25-di-hidroxivitamina D3, ocorrendo na presença de um tumor produtor de altos níveis de fator de crescimento de fibroblasto 23. No entanto, é possível que se trate muito mais de uma falha de diagnóstico clínico do que propriamente uma doença rara. Os autores relatam o caso de um homem de 42 anos com histórico de fraqueza muscular progressiva por cinco anos e restrição à cadeira de rodas, sem diagnóstico. Seus exames laboratoriais evidenciavam baixos níveis de fósforo. A remoção cirúrgica de um hemangiopericitoma detectado previamente em cavidade nasal levou à resolução completa dos sintomas. Os autores enfatizam que, mesmo com a etiologia já evidenciada, o paciente consultou diversos clínicos no decorrer dos cinco anos até que fossem instituídos o diagnóstico e o tratamento adequados. Arq Bras Endocrinol Metab. 2012;56(8):570-3.

Tumor fibroso solitário maligno de extremidades / Malignant solitary fibrous tumour of the extremities

Tumor fibroso solitário extrapleural (TFS) é neoplasia mesenquimal rara, sendo que há menos de 40 casos descritos com localização em extremidades até o presente momento. Acomete preferencialmente pacientes na quinta década e não tem predileção por sexo. Relatamos um caso com os aspectos clínicos, radiológicos, histológicos e imunoistoquímicos característicos, discutindo a importância do diagnóstico diferencial com outros sarcomas.Pouco é conhecido sobre o comportamento biológico do TFS. Fatores de risco para menor sobrevida livre de metástases incluem: margens cirúrgicas comprometidas, tamanho maior que 10 cm e histologia maligna. Excisão completa é o tratamento recomendado.

Extrapleural solitary fibrous tumour (SFT) is a rare mesenchymal neoplasm, with less than 40 cases reported affecting the extremities so far. This tumour affects preferentially middle-aged patients (median: 50 years) and shows no predilection for sex. We report a case with typical clinical, radiologic, histopathologic and immunohistochemical features, discussing the importance of differential diagnosis of other sarcomas. Little is known about the biological behavior of SFT. Risk factors for shorter metastasis-free survival include impaired surgical margins, tumor size greater than 10 cm and malignant histology. Complete excision is the recommended treatment.

Miopericitoma em fossa nasal / Myopericytoma in nasal cavity

Introdução: Os miopericitomas representam cerca de 1 % dos tumores vasculares, sendo relativamente comum na região de cabeça e pescoço, 25% dos casos, e raro nas cavidades nasais e paranasais. Objetivo: Descrever um caso de miopericitoma em fossa nasal. Relato do Caso: Apresentamos um caso de paciente adulta, do sexo feminino, com queixas de obstrução nasal, dor em região de fossas nasais e epistaxe eventual em fossa nasal direita, apresentando massa angiomatosa e facilmente sangrante, não pulsátil, ocupando toda fossa nasal direita. Comentários Finais: Os mopericitomas são tumores vasculares incomuns, raramente localizados na cavidade nasal e nos seios paranasais. Devem ser incluídos no diagnóstico diferencial das massas bem delimitadas, vasculares e de crescimento lento à tomografia computadorizada.

Introduction: The myopericytomas represent about 1% of the vascular tumors, is relatively common in the region of head and neck, 25% of the cases, and uncommon in the nasal and paranasal cavities. Objective: To describe one case of myopericytoma in nasal cavity. Case Report: We present a case of an adult patient, of the female sex, with complaints of nasal obstruction, pain in the nasal cavities region and eventual epistaxis in the right nasal cavity, which present an angiomatous and easily bleeding, non-pulsatile mass occupying all the right nasal cavity. Final Considerations: The myopericytomas are uncommon vascular tumors, rarely located in the nasal cavity and in the paranasal sinuses. They must be included in the differential diagnosis of the well delimited, vascular and slow growth masses upon computed tomography.

Tumores nasossinusais raros: série de casos e revisão de literatura / Rare nasosinusal tumors: case series and literature review

Os tumores nasossinusais são patologias pouco freqüentes na prática clínica. Aproximadamente 0,8 por cento de todos os cânceres humanos localizam-se nessa região. Apesar de rara, a neoplasia nasossinusal manifesta-se habitualmente através de sintomas inespecíficos e comuns a inúmeras patologias inflamatórias. Este estudo se propõe a descrever uma série de casos de tumores nasossinusais não-epiteliais raros, incluindo estesioneuroblastoma, granuloma central de células gigantes, plasmocitoma extramedular, hemangiopericitoma sinonasal, neurofibroma e fibroma cemento-ossificante, diagnosticados no Hospital Geral de Fortaleza, SESA/SUS. Faz-se uma breve revisão de literatura de cada patologia, salientando-se a necessidade do diagnóstico anatomopatológico preciso para condução adequada de cada caso.

Tumors of the nasal cavity and paranasal sinuses are unusual pathologies found in clinical practice. Approximately 0.8 percent of all human cancers are located in this area. Despite being rare, nasosinusal neoplasms usually manifest through nonspecific symptoms that are common to numerous inflammatory pathologies. The aim of this study is to describe a series of rare nasosinusal tumors, including esthesioneuroblastomas, central giant cell granulomas, extramedullary plasmocytomas, nasosinusal hemangiopericytomas, neurofibromas and cemento-ossifying fibromas, diagnosed at the Fortaleza General Hospital. We, hereby, briefly review each of the aforementioned pathologies, stressing the need for a precise histological diagnosis for proper treatment in each case.

Hemangiopericitoma da mama / Hemangiopericytoma of the breast

Hemangiopericitoma é uma incomum neoplasia mesenquimal, ricamente vascularizada, compostapor células indiferenciadas, descrita em várias localizações do corpo humano e raramente namama. Na maioria dos casos, possui comportamento biológico benigno. Relata-se um caso de hemangiopericitomade mama feminina, enfatizando aspectos clínicos e anatomopatológicos típicosdesta neoplasia.

Hemangiopericytoma is an uncommon richly vascular mesenchymal neoplasm, composed of undifferentiatedcells that has been described in various sites of the human body, but only rarely in the breast. Themajority of cases has a benign biological behavior. We report one case of hemangiopericytoma of femalebreast emphasizing typical clinical and anatomopathological features of this neoplasm.

Angiomatous meningioma: a diagnostic dilemma.

Angiomatous meningioma accounts for 2.1% of all meningiomas. It has features of a typical benign meningioma with many small or large vascular channels which may predominate over its meningothelial elements. We present here a series of three cases of angiomatous meningioma, which posed diagnostic difficulty to clinicians, radiologists, and pathologists. All the three cases showed a tumor entirely composed of thin-walled vascular channels and cells with bland morphology in the background. The diagnosis was confirmed by immunohistochemistry. We present series of three cases to highlight the histomorphological features of this uncommon variant of meningioma that could help in distinguishing it from hemangioblastoma and hemangiopericytoma.

Malignant hemangiopericytoma in the gluteal region: a rare entity.

We present here a case of malignant hemangiopericytoma in a 40-year-old female who presented with a history of slowly growing mass in left gluteal region for about 1.5 years. She was evaluated and a mass of 10 x 7.5 x 5.5 cm 3 was found on computed tomography. The tumor was resected and was sent for histopathological evaluation. Grossly the tumor was encapsulated and on cut section it was nodular. Microscopy showed spindle cells forming cords and sheets separated by thin delicate stroma consisting largely of thin-walled vessels revealed by reticulin stain. Pleomorphism and mitotic figures were seen. It was diagnosed as a case of malignant hemangiopericytoma of gluteal region. The patient underwent radiotherapy and did apparently well. She is on regular follow-up because long-term follow-up is essential in all cases as recurrence can occur several years after treatment.

Cytomorphologic findings of hemangiopericytoma of the meninges: a case report.

Hemangiopericytomas (HPCs) are tumors, constituting 2.5%, of soft tissue neoplasms. Meningeal hemangiopericytomas are rare non meningothelial mesenchymal tumors of the dura. They were classified as angioblastic meningiomas because of their similarity to meningiomas. However, these tumors are now regarded as distinct entities, akin to hemangiopericytoma elsewhere in the body. Few reports have addressed the fine needle aspiration (FNA) cytology of HPC. We present the cytological findings of one such rare case ofprimary meningeal hemangiopericytoma which metastasized to the lymph node. A 47 years male presented with cervical lymphadenopathy of 2 months duration. He was operated twice in the last 4 years and diagnosed atypical meningioma both times. The patient now also had spinal metastasis producing nerve root compression. Aspiration cytology of the node revealed cellular aspirate demonstrating round to oval cells with ample cytoplasm, round nuclei and inconspicuous nuclei. They showed a characteristic ferning out of blood vessels. Occasional mitosis was present. A diagnosis of malignant tumor of vascular origin was offered. Lymph node biopsy showed a characteristic histological picture of hemangiopericytoma described in other soft tissues. The cytology of this tumor is characteristic, but the rarity of the lesion, especially in metastatic sites makes diagnosis difficult unless a high index of suspicion is present. The recognition of the distinct cytological findings makes this possible. The case documents the role of FNA cytology in confirming HPC.

Unilateral Renal Haemangiopericytoma: Report of a Rare Tumour

RESUMEN: Los pericitos son células musculares lisas modificadas de los tubos capilares circundantes los cuales cambian el diámetro del lumen vascular. En este artículo relatamos un caso dehemangiopericitoma renal presente en una mujer de 78 años, quien manifestaba dolor abdominal. La ultrasonografía determinó una lesión sólida en la tercera porción del riñón izquierdo. La exploración abdominal de CT reveló una masa tumoral. Debido a la alta sospecha de neoplasia maligna, la paciente fue sometida a nefrectomía radical izquierda. La evaluación histopatológica confirmó el hemangiopericitoma, a través de inmunohistoquímica. La paciente sobrevive cinco años después de la nefrectomía radical.

Bilateral metastatic renal hemangiopericytoma ten years after primary intracranial lesion

We report a case of bilateral metastatic renal hemangiopericytoma. A 37-year-old Caucasian male presented in 1993 with intracranial hemangiopericytoma. Subsequent metastatic disease noted years later include bilateral renal hemangiopericytoma 10 years after initial presentation. To our knowledge, this is only the second reported case of bilateral metastatic renal hemangiopericytoma.

Hemangiopericitoma intracraneal: reporte de un caso / Intracranial hemangiopericytoma: report of a case

El Hemangiopericitoma es un tumor infrecuente del sistema nervioso central que simula ser un Meningioma en apariencia y sintomatología, pero que se comporta con gran agresividad presentando alta tendencia a la recidiva en resecciones quirúrgicas limitadas y a las metástasis dentro y fuera del sistema nervioso. Presentamos el caso de un paciente masculino de 28 años de edad con un Hemangiopericitoma frontal izquierdo adherido a la falx cerebral, con historia de cefalea intensa de un año de evolución, nauseas y vómitos de instauración progresiva, acompañado de trastornos de la personalidad y sin déficit neurológico focal, quien presenta en estudios imagenológicos LOE sugestivo de meningioma de la Falx cerebri y lóbulo frontal izquierdo.

Hemangiopericitoma nasal: aportación de un nuevo caso pediatrico y revisión bibliografica / Haemangiopericytoma nasal

Los hemangiopericitomas son tumores infrecuentes en la edad pediatrica y tienen baja incidencia en la región cervico facial. El objetivo de este trabajo es presentar un caso de hemangiopericitoma nasal en una niña de doce años y revisar la bibliografia. El cuadro clínico se caracterizó por la obstrucción nasal, en lugar de epistaxis, que es lo que con mayor frecuencia domina la sintomatologia. Se le practicó cirugia endonasal encontrandose como resultado buen margen quirúrgico. Durante las revisiones realizadas no se ha observado recurrencia local ni metastasis a los 16 meses posteriores a la cirugia. Se concluye que la histologia de apariencia benigna no excluye la posibilidad de recurrencia y continúa siendo un desafio el diagnóstico, tratamiento y pronóstico del hemangiopericitoma.