Infantile (congenital) anaplastic intracranial solitary fibrous tumor/hemangiopericytoma—A case report with brief literature review

Solitary fibrous tumor/hemangiopericytoma (SFT/HPC) is a rare primary central nervous system (CNS) tumor, included in the World Health Organization (WHO) 2016 classification. Very few cases have been described in the literature so far, especially the infantile type. It is a mesenchymal tumor of the fibroblastic type, characterized by the fusion of NAB 2 and STAT 6 genes. A 10-month-old boy presented to our neurosurgery department with complaints of increasing head circumference since 1 month of age. The magnetic resonance imaging (MRI) showed a space-occupying lesion measuring 8.2 cm × 7 cm × 6.9 cm in the fronto-temporo-parietal region with a clinical diagnosis of glioma/atypical teratoid rhabdoid tumor (ATRT). The microscopy revealed a spindle cell tumor arranged in a patternless pattern with variable cellularity, increased mitosis, and areas of coagulative necrosis. The immunohistochemistry showed vimentin, CD 34, STAT6, CD99 positivity whereas Glial fibrillary acidic protein, Epithelial membrane antigen, and S-100 negativity. Hence, a diagnosis of anaplastic SFT/HPC (grade-III) was rendered. The patient improved after gross total resection (GTR). The primary intracranial congenital SFT/HPC are extremely rare, often a clinico-radiologically misdiagnosed entity. Thus, the immunohistochemistry/molecular study in addition to histology is mandatory for accurate diagnosis.

Solitary fibrous tumor: Two cases report with a focus on the differential diagnosis / Tumor Fibroso Solitário: Relato de dois casos com foco no diagnóstico diferencial

Solitary fibrous tumor (SFT) is a neoplasm of fibroblastic/myofibroblastic origin with intermediate biological behavior. We report here two cases of SFT affecting an unusual anatomical site in 58-year-old and 40-year-old female patients and discuss the differential diagnosis of this lesion. In case 01, the lesion showed the clinical appearance of an asymptomatic "blister" with normal color, rubbery consistency, measuring 0.3 cm, and affected the lower lip; while in case 02, a symptomatic red nodular lesion with a soft consistency and measuring 0.5 cm affected the floor of the mouth. Excisional biopsies were performed. Microscopically, two well-delimited benign neoplasms were observed, exhibiting the proliferation of ovoid to spindle-shaped mesenchymal cells, vascular spaces with staghorn arrangement, and the absence of mitosis figures. Immunohistochemistry was performed in case 01 to assist in the diagnosis. Weak and diffuse immunostaining was observed for α-SMA and intense and diffuse immunopositivity for Bcl-2 and CD34. Based on histopathological and immunohistochemical features, a diagnosis of SFT was rendered in both cases. The low occurrence and nonspecific clinical features of oral SFT may make its clinical diagnosis difficult. Also, morphological and immunohistochemical are essential for differential diagnosis with other mesenchymal neoplasms.(AU)

O tumor fibroso solitário (TFS) é uma neoplasia de origem fibroblástica/miofibroblastica com comportamento biológico intermediário. Nesse artigo relatamos dois casos de TFS afetando sítios anatômicos incomuns em pacientes do sexo feminino de 58 anos e 40 anos e discutir os seus diagnósticos. No caso 01 clinicamente a lesão apresentou um aspecto de "bolha" assintomática, coloração normal da mucosa, consistência borrachoide medindo 0,3 cm, em região de lábio inferior, enquanto que no caso 02, como uma lesão sintomática, vermelha, nodular com consistência mole e medindo 0,5 cm afetando o assoalho bucal. As biópsias excecionais foram realizadas. Microscopicamente, observamos duas lesões neoplásicas bem delimitadas exibindo uma proliferação de células mesenquimais variando de ovoides a fusiformes, vasos sanguíneos em formato de "chifre de veado", com ausência de figuras de mitoses. No caso 01 foi realizado análise imuno-histoquímica para auxiliar no diagnóstico. Foi observado uma marcação fraca e difusa de α-SMA e uma intensa e difusa imunopositividade para o Bcl-2 e CD34.Baseado nos achados histopatológicos e imuno-histoquímicos o diagnóstico de TFS foi estabelecido em ambos os casos. A baixa ocorrência e os achados clínicos inespecíficos do TFS oral podem dificultar o diagnóstico clínico. Além disso, as análises morfológicas e imuno-histoquimicas são essenciais para realização do diagnóstico diferencial com outras neoplasias mesenquimais.(AU)_

Tumor fibroso solitario (TFS) parafaríngeo: reporte de caso y revisión de literatura

El espacio parafaríngeo es una región donde puede surgir un amplio espectro de tumores, que son poco frecuentes y comprenden solo el 0,5 al 1,5% de las neoplasias de cabeza y cuello. Generalmente, son hallazgos y suelen presentarse como masas asintomáticas. Incluye neoplasias primarias, extensión directa de regiones adyacentes y enfermedad metastásica. La literatura describe múltiples patologías e histologías que pueden surgir de esta zona, entre ellas, tenemos los tumores fibrosos solitarios, que son un espectro de neoplasias mesenquimatosas fibroblásticas de histogénesis incierta, que generalmente involucran la pleura. Son lesiones muy poco frecuentes y su localización parafaríngea es excepcional, siendo su primer reporte en la literatura en 1993. Se presenta el caso de un tumor fibroso solitario del espacio parafaríngeo evaluado en nuestro centro, con su enfrentamiento diagnóstico, resolución quirúrgica y seguimiento respectivo, con el objetivo de presentar esta patología poco frecuente a nuestros colegas y contribuir al conocimiento médico.

The parapharyngeal space is a region where a wide spectrum of tumors can arise, which are rare and comprise only 0.5% to 1.5% of head and neck neoplasms. They are generally findings and usually present as asymptomatic masses. Includes primary neoplasms, direct extension from adjacent regions, and metastatic disease. The literature describes multiple pathologies and histologies that can arise from this area, among them, we have solitary fibrous tumors, which are a spectrum of fibroblastic mesenchymal neoplasms of uncertain histogenesis, which are generally the pleura. It is a very rare lesion and its parapharyngeal location is exceptional, being its first report in the literature in 1993. We present the case of a solitary fibrous tumor of the parapharyngeal space evaluated in our center, with its diagnos- tic confrontation, surgical resolution and respective follow-up, with the aim of presenting this rare pathology to our colleagues and contributing to medical knowledge.

Tumor fibroso solitario de próstata: informe de caso y revisión de la literatura / Solitary fibrous tumor of the prostate: case report and literature review

El tumor fibroso solitario (TFS) es una neoplasia mesenquimatosa de tipo fibroblástico que, a pesar de ser localizado principalmente en pleura, se ha observado en otros órganos como la próstata. Por su parte, el tumor fibroso solitario de la próstata es una neoplasia de baja incidencia, crecimiento lento y potencial maligno incierto, que generalmente se compone de células fusiformes de apariencia citológicamente benignas, dispuestas en una arquitectura desorganizada, mezcladas con colágeno y pequeños vasos sanguíneos. Establecer su diagnóstico se ha vuelto más reproducible desde la identificación de la fusión de los genes NAB2-STAT6 por biología molecular, que lleva a la sobreexpresión de STAT6 por inmunohistoquímica, el cual es un marcador muy sensible y específico para TFS. Presentamos el caso clínico de un paciente que debutó con síntomas de compresión vesical, en quien se identificó una masa con epicentro en la próstata que infiltraba la vejiga y llegaba a la pared rectal, y que luego de estudios de patología, inmunohistoquímica y pruebas moleculares se clasificó como un TFS de la próstata, finalmente tratado con cistoprostatectomía radical más derivación urinaria

Solitary fibrous tumor (SFT) is a mesenchymal neoplasm of fibroblastic type, which despite being located mainly in the pleura, has been observed in other organs such as the prostate. On the other hand, solitary fibrous tumor of the prostate is a rare neoplasm, slow growing, and of uncertain malignant potential, which is generally composed of spindle cells of cytologically benign appearance, arranged in a disorganized architecture, mixed with collagen and small blood vessels. Establishing its diagnosis has become more reproducible since the identification of the NAB2-STAT6 gene fusion by molecular biology, leading to the overexpression of STAT6 by immunohistochemistry, a very sensitive and specific marker for SFT. We present a clinical report of a patient who consulted with symptoms of bladder compression, in whom a mass was identified with the epicenter in the prostate infiltrating into the bladder and reaching the rectal wall. Following histopathology study, immunohistochemistry and molecular tests it was classified as a SFT of the prostate, finally treated with radical cystoprostatectomy plus urinary shunt

Tumor fibroso solitario gigante de pleura / Giant solitary fibrous tumor of the Pleura

Resumen Objetivo: Reportar el caso de una masa gigante en hemitórax izquierdo de 19 cm de diámetro en un paciente de 59 años que debutó con disnea, tos y dolor torácico, confirmándose por estudio imagenológico. Materiales y Método: Registro clínico de un paciente al cual se le diagnostica tumor fibroso solitario de pleura, siendo intervenido quirúrgicamente para exéresis de la lesión. Resultados: Se realiza toracotomía posterolateral izquierda para exéresis de tumor gigante, requiriendo además, resección de diafragma y pericardiectomía parcial con evolución favorable. Discusión: El tumor fibroso solitario es una neoplasia rara derivada del mesénquima que afecta más comúnmente a la pleura, típicamente bien circunscrita, pediculada, con vasos dentro del pedículo tumoral, pudiendo llegar a ser de gran tamaño, siendo considerados gigantes cuando tienen más de 15 cm de diámetro. Conclusión: El diagnóstico correcto es de vital importancia, ya que con la resección quirúrgica es potencialmente curable. El tratamiento quirúrgico puede efectuarse por toracotomía o videotoracoscopia, dependiendo del tamaño del tumor. A pesar del comportamiento benigno, requiere seguimiento a largo plazo debido a la tendencia a la recidiva.

Aim: To report the case of a 19 cm diameter giant mass in the left hemithorax in a 59-year-old patient who presented with dyspnea, cough and chest pain, confirmed by imaging study. Materials and Method: Clinical record of a patient who was diagnosed with a solitary fibrous tumor of the pleura, undergoing surgery to excise the lesion. Results: A left posterolateral thoracotomy was performed to excise the giant tumor, also requiring resection of the diaphragm and partial pericardiectomy with favorable evolution. Discussion: The solitary fibrous tumor is a rare neoplasm derived from the mesenchyme that most commonly affects the pleura, typically well circumscribed, pedunculated, with vessels within the tumor pedicle, and can become large, being considered giant when they are larger than 15 cm diameter. Conclusión: The correct diagnosis is of vital importance, since surgical resection it a potentially curable treatment. Surgical treatment can be performed by thoracotomy or videothoracoscopy, depending on the size of the tumor. Despite the benign behavior, it requires long-term follow-up due to the tendency to recur.

Characteristics and management of solitary fibrous tumor in lacrimal drainage system / 国际眼科杂志(Guoji Yanke Zazhi)

@#Solitary fibrous tumor is a rare mesenchymal tumor originating from fibroblasts. It was first reported in the pleura. In recent years, more and more cases of solitary fibroma in the extra pleural area have been reported. It has been reported that the tumor can occur in almost all sites. The structure and function of the lacrimal drainage system has its particularity, and there are still few reports about solitary fibroma of the lacrimal drainage system. Therefore, this article summarizes the existing reports of solitary fibroma of the lacrimal drainage system to analyse its clinical characteristics and treatment.

A case report of huge solitary fibrous tumor of kidney / 中华泌尿外科杂志

Solitary fibrous tumor is a rare mesenchymal tumor associated with NAB2-STAT6 fusion gene, which is rarely seen in kidney. A 16-year-old boy was hospitalized because of left back pain for more than 3 years. Abdominal CT/MRI identified a huge space-occupying lesion in the left kidney. Laparoscopic radical left nephrectomy was performed initially. Nevertheless, laparoscopic-to-open procedure was adopted due to the huge size of the tumor. The pathological diagnosis was renal solitary fibrous tumor. The symptoms of the patient disappeared and no recurrence was observed at the 2-month follow-up after the surgery.

Tumor fibroso solitario de la pleura / Solitary fibrous tumor of the pleura

El tumor fibroso solitario de la pleura (TFSP) representa una neoplasia benigna de evolución habitualmente silente, hallazgo incidental y heterogeneidad en su presentación. Raramente se malignizan y la sintomatología se relaciona fundamentalmente con el compromiso por compresión de las estructuras vecinas. Su tratamiento es la cirugía. Se describe el caso de un paciente masculino de 49 años con antecedentes de hipertensión arterial, diabetes mellitus y obesidad que consultó al Servicio de urgencias por disnea. Se solicitó tomografía de tórax que evidenció voluminosa masa de densidad de partes blandas en hemitórax izquierdo con desplazamiento del mediastino y descenso del diafragma del mismo lado. La fibrobroncoscopia no evidenció lesión endoluminal. Fue evaluado por Cirugía del Tórax. Se realizó abordaje de la masa mediante toracotomía postero lateral izquierda. El estudio anatomopatológico informó formación tumoral de 25 × 16 × 13 cm, peso de 1905 gr y en la microscopía se describió proliferación celular fibroblástica, células fusiformes de núcleos pequeños ovoides y escaso citoplasma dispuestas en haces desordenados con colágeno interpuesto. La inmunohistoquímica informó vimentina y CD34 positivos. Se realizó el diagnóstico de tumor fibroso solitario de la pleura. La evolución posterior fue buena.

The solitary fibrous tumor of the pleura (SFTP) represents a benign neoplasm of commonly silent evolution, incidental finding and heterogeneous presentation. It rarely becomes malignant and symptoms mainly result from the involvement of neighboring structures due to compression. It is treated with surgery. We describe the case of a 49-year-old male patient with history of arterial hypertension, diabetes mellitus and obesity who consulted the Emergency Services because he was experiencing dyspnea. We requested chest tomography that showed a voluminous soft tissue density mass in the left hemithorax with mediastinal shift and decreased diaphragm on the same side. The fibrobronchoscopy didn't show endoluminal lesion. The patient was evaluated by the Thorax Surgery staff. The mass was treated by means of left posterolateral thorachotomy. The anatomopathological study reported the formation of a tumor of 25 × 16 ×13 cm and 1905 gr, and the microscopy described fibroblast cell proliferation, spindle cells of small ovoid nuclei and very little cytoplasm placed in disorganized bundles with collagen interposition. The immunohistochemistry disclosed positive vimentin and CD34. The patient was diagnosed with solitary fibrous tumor of the pleura, with good subsequent evolution.

Tumor fibroso solitario gigante de pleura. Serie de casos / Giant Solitary Fibrous Tumor of the Pleura. Case Series

Resumen Introducción. El tumor fibroso solitario de pleura (TFSP) es una neoplasia poco frecuente, con aproximadamente 1.000 casos reportados en la literatura mundial. La aproximación diagnóstica inicial se realiza con estudios imagenológicos. Métodos. De forma retrospectiva, se recopilaron cuatro casos de pacientes con TFSP gigante operados en nuestra institución. Se describen las características sociodemográficas, clínicas, imagenológicas, macroscópicas y microscópicas de cada caso. Resultados. Todos los pacientes de la serie cursaron con manifestaciones clínicas, con un promedio de 23,75 meses de evolución. El 50% de los tumores se localizaron en la cavidad pleural derecha y el 50% en la izquierda. En tomografía computarizada (TC) de tórax, los cuatro casos se presentaron como una masa sólida, de densidad heterogénea, con diámetros mayores entre 17 y 22 cm y contornos variables (lisos en tres casos y lobulados en un paciente). Se observaron calcificaciones intratumorales en dos casos y derrame pleural en tres pacientes. En cirugía, todas las masas presentaron pedículos. El análisis histológico e inmuno-químico confirmó la naturaleza benigna de tres casos y malignidad en una de las neoplasias. Conclusiones. Los TFSP generalmente son benignos y de buen pronóstico. Sin embargo, entre 10 y 20% de esos tumores son malignos. Las imágenes diagnósticas pueden sugerir el diagnóstico de TFSP, pero la confirmación de la naturaleza de la lesión debe realizarse con el análisis histopatológico de toda la pieza quirúrgica.

Abstract Introduction. Solitary fibrous tumor of the pleura (SFTP) is a rare neoplasm, with ~1.000 cases reported in the worldwide literature. The initial diagnostic approach is performed by imaging studies. Methods. Retrospectively, we collected four cases of patients with giant SFTP operated in our institution. The sociodemographic, clinical, imaging, macroscopic, and microscopic characteristics of each case are described. Results. All the patients in the series had clinical manifestations, with an average of 23.75 months of evolution. 50% of the tumors were located in the right pleural cavity and 50% in the left. In chest computed tomography (CT), the four cases presented as a solid mass, of heterogeneous density, with greater diameters between 17 and 22 cm, and variable contours (smooth in three cases and lobulated in one patient). Intratumoral calcifications were observed in two cases and pleural effusion in three patients. In surgery, all masses presented pedicles. The histological and immunochemical analysis confirmed the benign nature of three cases and malignancy in one of them. Conclusions. SFTPs are usually benign and have a good prognosis. However, between 10 and 20% of these tumors are malignant. Diagnostic images may suggest the diagnosis of SFTP, but confirmation of the nature of the lesion should be made with the histopathological analysis of the entire surgical specimen.

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.

Tumor fibroso solitario hepático gigante. Informe de caso en la Argentina / Giant solitary fibrous tumors of the liver. A case report in Argentina

RESUMEN Los tumores fibrosos solitarios hepáticos, una neoplasia poco frecuente, son tumores benignos del tejido submesotelial, pero con un potencial maligno no definido. Suelen presentarse asintomáticos, pero pueden debutar como una masa abdominal palpable y con síntomas de plenitud. Por imágenes pueden ser confundidos con otros tumores y está indicado el tratamiento quirúrgico. Presentamos el caso de una paciente de 70 años resuelto por hepatectomía lateral izquierda, y realizamos una revisión bibliográfica del tema.

ABSTRACT Solitary fibrous tumors of the liver are uncommon benign tumors originating from the submesothelial tissue with non-well-defined malignant potential. Most cases present as asymptomatic, some cases show abdominal bloating and a palpable mass. They may mimic other tumors on imaging tests and surgery is indicated. We report a case of a 70-year-old female patient treated with left lateral liver resection with literature review of the condition.

A Case of Solitary Fibrous Tumor of Subungual Region

Solitary fibrous tumor (SFT) is a relatively uncommon mesenchymal neoplasm that usually arises in the pleura, but also has been reported in numerous extrapleural locations, including cutaneous site. The skin lesion presents as a circumscribed nodule or tumor, mainly on the head and neck. A 41-year-old male presented with 6 months history of nail lesion without symptom on the left third finger. The lesion is slightly yellowish discoloration with subungual erythematous nodule and distal onycholysis. Biopsy specimen from the nail lesion showed the spindle cells form patternless pattern with hypercellular and hypocellular area. And small blood vessels and dilated vascular spaces were present. The result of special stain for specimen showed that positive for CD34, Bcl-2, and CD99 but negative for S-100, FactorXIIIa, and smooth muscle action. Recognition of this uncommon location of SFT is important because of possible confusion with other subungual tumors, including glomus tumor, fibroma and other fibrohistiocytic tumors like dermatofibrosarcoma protuberans, superficial acral fibromyxoma and cellular digital fibroma. Here in, we report a case of SFT of subungual region. We think this case is interesting because of uncommon location and may be helpful to more understand the character of this disease.

Clinical imaging analysis of 12 cases of orbital solitary fibrous tumor / 国际眼科杂志(Guoji Yanke Zazhi)

@#AIM:To investigate the characteristics of color Doppler sonography and MRI in orbital solitary fibrous tumor(SFT). <p>METHODS: It was a retrospective case series study. A total of 12 cases of orbital solitary fibrous tumors were recruited from April 2013 to August 2018 in Tianjin Medical University Eye Hospital. Color Doppler sonography and MRI plain scan and DCE-MRI were applied in all cases. <p>RESULTS: Of 12 cases, 7 cases were males and 5 were females, with the duration of symptoms ranged from 3mo-20y, with an average course of 3.2y; 6 cases were the primary cases and 6 cases were relapse cases. All lesions involved one side of the orbit, of which 8 cases were in the left orbit and 4 in the right. Of 12 cases, there were 5 tumors in intraconal space, 3 in the extraconal space, and 4 in intra and extraconal space. Well-circumscribed lesions showed oval shape in 9 cases and the left 3 were irregular. 12 cases showed hypoechoic and 2 cases heterogeneous signal on color Doppler sonography; All cases had flow signals on CDFI, and showed arterial spectrum on PW. On T1WI, all lesions demonstrated isointense. On T2WI, 5 lesions showed hypointense, 3 lesions showed isointense and 4 lesions slight hyperintense, of which 3 lesions showed heterogeneous signal. After contrast enhancement, all cases demonstrated markedly enhancement, with homogeneous enhancement in 10 cases and heterogeneous enhancement in 2 cases. The time-intensity curves(TIC)of 7 cases exhibited a rapid washout pattern, and 5 cases a rapid plateau pattern on DCE-MRI. <p>CONCLUSION: Color Doppler sonography features of orbital SFT include hypoechoic and flow signals. Heterogeneous signals on T2WI, marked enhancement, and a rapidly enhancing and slow washout pattern TIC on DCE-MRI are the typical MRI features of orbital SFT.

Primary solitary fibrous tumour of the prostate: A case report and literature review

@#Introduction: Solitary fibrous tumour (SFT) is a rare mesenchymal tumour with intermediate malignant potential. Although this tumour arises in several sites, prostatic SFT is an extremely rare neoplasm and may prove confusing owing to the lack of clinical experience because of tumour rarity. The diagnosis may be further difficult because SFTs can manifest positive immunoreactivity for CD34 and progesterone receptor, which are known markers of prostatic stromal tumours. Herein, we describe a case of prostatic SFT that was difficult to differentiate from a prostatic stromal tumour of uncertain malignant potential because of positive immunoreactivity to CD34 and progesterone receptor. Case Report: A 40-year-old Japanese man presented with lower abdominal pain. Computed tomography revealed a prostatic mass; furthermore, prostate core needle biopsy revealed proliferating bland spindle cells, without necrosis or prominent mitoses. Tumour cells were positive for CD34 and progesterone receptor on immunohistochemical analysis; thus, a prostatic stromal tumour of uncertain malignant potential was initially suspected. However, as the tumour cells showed positive immunoreactivity for STAT6, the final diagnosis was an SFT of the prostate. The patient underwent tumour resection, and at the 6-month postoperative follow-up, neither local recurrence nor distant metastasis occurred. Conclusion: For an accurate diagnosis of an SFT of the prostate, STAT6 immunohistochemistry should be conducted for all mesenchymal tumours of the prostate. When STAT6 immunohistochemical analysis is unfeasible, pathologists should be aware that the morphological and immunohistochemical characteristics of SFT variable from case to case and diagnose with combined analysis of several immunohistochemical markers.

Solitary Fibrous Tumour of Liver: A Case Report and Literature Review

Solitary fibrous tumour is an uncommon mesenchymal neoplasm diagnosis of which is difficult because of non-specific clinical and radiological features. A 76 year old male presented with complaints of nausea and vomiting. Physical examination revealed firm mass in the right hypochondrium and epigastrium. CT scan revealed a heterogeneous, lobed and some vascularized mass of size 17.6 x 12.8 cm, situated in the right lobe. After surgery the mass was found to measure 19 cm x 13 cm x 10 cm. Histopathological examination found the tumour cells to be strongly positive for vimentin and CD34, confirming our diagnosis. Microscopic examination revealed haphazard arrangement of spindle cells and collagen bundles between the tumour cells with mild nuclear pleomorphism, and the mitotic count was 0-1/10 high power field. The post-operative course was uncomplicated and the patient was discharged home. After 16 months of follow up examinations, there was no recurrence of disease, local or distant metastasis and the patient remained asymptomatic. The clinical presentation and the radiological imaging can be very non-specific. Suggestive features on CT scan are a single, large, well-circumscribed, heterogeneously enhancing hepatic mass.

Tumor fibroso solitario gigante de pleura en paciente joven / Giant Solitary Fibrous Tumor of the Pleura in Young Patient / Tumor fibroso solitário gigante de pleura em paciente jovem

Resumen Introducción : el tumor fibroso solitario de pleura (TFSP) es una patología poco frecuente, corresponde a menos del 5 % de los tumores primarios de pleura, siendo aún más raros los tumores mayores de 15 cm y aquellos en pacientes menores de 35 años, ya que el pico de incidencia es entre los 60 a 70 años con un solo caso reportado en Colombia. Presentación del caso : paciente femenina de 33 años, sin exposición a asbesto o cigarrillo. Consultó por cuadro de disnea de moderados esfuerzos y dolor dorsal derecho. La tomografía axial computarizada (TAC) contrastada de tórax evidenció una gran masa sólida de contornos lobulados que comprometía el 70 % del hemitórax derecho de origen extrapulmonar y que comprimía la aurícula derecha, la inmunohistoquímica de la biopsia percutánea clasificó la lesión como tumor fibroso solitario. Fue llevada a toracotomía posterolateral derecha extendida, donde se encontró un tumor dependiente de la pleura parietal derecha resecado en su totalidad. Histopatología e inmunohistoquímica del producto de resección quirúrgica, compatibles con tumor fibroso solitario de pleura, tamaño 30 x 23 x 16 cm sin características de malignidad. La evolución posoperatoria fue satisfactoria, con un egreso hospitalario temprano. Discusión : en una paciente joven, el TFSP gigante es inusual, posterior a su diagnóstico, y a pesar de tener algunas características que sugerían malignidad: tamaño y origen en la pleura parietal, la resección quirúrgica fue el tratamiento indicado para este tumor, con posterior confirmación histopatológica compatible con tumor benigno.

Abstract Introduction : The solitary fibrous tumor of the pleura (SFTP) is an uncommon pathology. It corresponds to less than 5 % of the primary tumors of the pleura. Even much rare is to find one solitary fibrous tumor, of more than 15 cm in patients younger than 35 years since the incidence peak is between 60 to 70 years. There has been only one case reported in Colombia. Case presentation : The subject of study was a 33-year-old female patient, no exposure to asbestos or cigarette. The chief complaints of the patient were mild dyspnea and right dorsal pain. Thoracic contrast-enhanced computerized tomography showed a large solid mass of lobulated contours that compromised 70 % of the right hemithorax, of extrapulmonary origin compressing the right atrium. Immunohistochemistry of the percutaneous biopsy demonstrated a solitary fibrous tumor. The patient underwent an extended right posterolateral thoracotomy. We found and completely resected a tumor hanging on the right parietal pleura. Histopathology and immunohistochemistry of the product of the surgical resection were compatible with a solitary fibrous tumor of the pleura, 30 x 23 x 16cm in size, and no malignancy. The evolution after surgery was satisfactory with an early hospital discharge. Discussion : In a young patient, a giant TFSP is unusual. After diagnosis and despite having some characteristics that suggested malignancy: its size and origin in the parietal pleura, surgical resection was the appropriate procedure for this tumor, with subsequent histopathological confirmation compatible with a benign tumor.

Resumo Introdução : o tumor fibroso solitário de pleura (TFSP) é uma patologia pouco frequente, corresponde a menos do 5 % dos tumores primários de pleura, sendo ainda mais estranhos os tumores maiores de 15 cm e aqueles em pacientes menores de 35 anos, pois o pico de incidência é entre os 60 a 70 anos com um só caso reportado na Colômbia. Apresentação de caso : paciente feminina de 33 anos, sem exposição a asbesto ou cigarro. Consultou por quadro de dispneia de moderados esforços e dor dorsal direita. A tomografia axial computadorizada (TAC) contrastada de tórax evidenciou uma grande massa sólida de contornos lobulados que comprometia o 70 % do hemitórax direito de origem extrapulmonar e que comprimia a aurícula direita, imuno-histoquímica da biopsia percutânea classificou a lesão como tumor fibroso solitário. Foi levada à toracotomia posterolateral direita estendida, encontrando um tumor dependente da pleura parietal direita ressecado em sua totalidade. Histopatologia e imuno-histoquímica do produto de ressecção cirúrgica, compatíveis com tumor fibroso solitário de pleura, tamanho 30 x 23 x 16cm sem características de malignidade. A evolução pós-cirúrgica foi satisfatória, conseguindo um egresso hospitalar precoce. Discussão : em uma paciente jovem, o TFSP gigante é inusual, posterior a seu diagnóstico e apesar de ter algumas características que sugeriam malignidade; tamanho e origem na pleura parietal, a ressecção cirúrgica foi o tratamento indicado para este tumor, com posterior confirmação hispatológica compatível com tumor benigno.

Sindrome de Doege Potter tumor fibroso solitario endotoracico Hipoglicemiante ¿cuando y cómo lo diagnostico ? y ¿Cual es mi conducta final?: Presentación de un caso y revisión de la literatura / "doege - potter syndrome", solitary fibrous tumor intrathoracic hypoglycemic when and as the diagnosis? and what is my final behavior?: report of a case and review of the literature

El Síndrome de "Doege - Potter" es una entidad clínica rara con incidencia baja de difícil diagnóstico, poco conocida alrededor del mundo descrita en 1930, el cual consiste en un tumor intratorácico asociado a hipoglicemi asintomática. El objetivo del presente artículo es exponer un caso de "Síndrome de Doege-Potter", diagnosticado y tratado en el Centro de Especialidades Clínico - Quirúrgicas Jesús Obrero, en un paciente masculino de 55 años; tras la revisión sintomatológica, semiológica/topográfica, estudios imagenologicos, anatomopatologicos, y la revisión sistemática de la literatura internacional se llegó al diagnóstico final. Además se enfocara no solo conocimientos históricos, etiológicos y fisiopatológicos, sino medios diagnósticos estandarizados, que implica tomar en cuenta en esta patología una vez diagnosticada, para poder direccionar el tratamiento más adecuado según los hallazgos y el estado del paciente; siendo esta una manera de aportar en el levantamiento epidemiológico y casuístico de esta variedad rara y poco frecuente patología torácica a nivel mundial.

"Doege - Potter" syndrome is a rare clinical entity with low incidence of difficult diagnosis, little-known around the world described in 1930, which consists of an intrathoracic tumor associated with symptomatic hypoglycemia. The aim of this article is to expose a case of "syndrome of Doege-Potter", diagnosed and treated in the Centre of specialties Clinical - surgical Jesús Obrero, in a 55-year-old male patient; after reviewing symptomatology, / topographic, semiological studies imaging, pathological, and the systematic review of the international literature became the final diagnosis. In addition, focuses not only etiological, historical knowledge and physiopathological, but standardized diagnostic means, which implies taking into account in this disease diagnosed once, to be able to address the most appropriate treatment according to the findings and the patient's condition; this being a way to bring in the epidemiological and case lifting of this rare variety and frequent short thoracic pathology worldwide.

A Case of Solitary Fibrous Tumor of Hard Palate / 대한이비인후과학회지

Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm first described and often encountered in the pleura. It has also been documented in a variety of extrapleural sites including the abdominal cavity, respiratory tract, lung, breast, and rarely in the head and neck region. SFT in the hard palate is an extremely unusual location. We present a rare case of SFT that presented as a palate mass, which was suggested as a mesenchymal cell neoplasm by preoperative punch biopsy, and finally diagnosed as STF after successful surgical excision. SFTs should be considered as a differential diagnosis of palate benign tumor when preoperative pathologic findings show mesenchymal neoplasms of fibroblastic or myofibroblastic origin. Possible malignant variants should be distinguished by evaluating the histological parameters, including high cellularity, frequent mitotic activity, nuclear pleomorphism, and presence of necrosis.

Solitary fibrous tumor/hemangiopericytoma of central nervous system: a clinicopathological analysis of 60 cases / 中华病理学杂志

Objective@#To investigate the clinical manifestations, imaging features, clinicopathologic features, and differential diagnosis of solitary fibrous tumors/anginoblastomas (SFT/HPCs) originating in the central nervous system.@*Methods@#Sixty cases of SFT/HPCs originating in the central nervous system were collected at Nanjing Jinling Hospital, from January 1, 2008 to December 31, 2016. The clinical data, imaging data, histomorphologic changes and immunohistochemical finding were analyzed in the sixty cases.@*Results@#The 60 cases included 26 males and 34 females, aged 14 to 85 (median 49) years. The main clinical manifestations were headache, dizziness with nausea and vomiting. Radiologically, the tumors were large, enhancing, solid and cystic masses attached to the dura. Histopathologically, the neoplasms were composed of spindle cells with oval nuclei, inconspicuous nucleoli and moderate amount of eosinophilic cytoplasm arranged in fascicles with areas of hyalinized stroma, myxoid changes and a staghorn vascular pattern. Immunohistochemically, tumor cells of all cases were positive for vimentin (100.0%, 60/60), STAT6 (98.3%, 59/60), CD34 (61.7%, 37/60), and the tumor cells were typically positive for CD99, bcl-2, EMA and SSTR2 as well.Negative for S-100 protein, SOX10, E-cadherin, GFAP. Ki-67 index ranged from 1% to 50%. Forty cases were followed up for 6 to 82 months with average of 40 months, 30 patients were alive and 10 patients died.@*Conclusions@#Central nervous system SFT/HPCs can be aggressive and relapses may occur several years after diagnosis. STAT6 is highly sensitive and specific for the diagnosis. Complete tumor resection is optional treatment followed by radiotherapy and chemotherapy. There is a correlation between the prognosis and the location of the disease, the histological grade, Ki-67 index, and fusion gene variants.