Hemangiopericitoma en el cuello / Hemangiopericytoma in the Neck

Introducción: El hemangiopericitoma es un raro tumor mesenquimal (vascularizado y potencialmente maligno) derivado de los pericitos, que puede aparecer en cualquier parte del cuerpo; sin embargo, en el cuello se describen casos aislados. La resección quirúrgica completa constituye la piedra angular del tratamiento. Objetivo: Presentar un caso de un hemangiopericitoma en el cuello, como un caso inusual, con potencial maligno desconocido, diagnóstico y tratamiento oportuno. Presentación de caso: Paciente de sexo masculino, de 39 años de edad, sin antecedentes de enfermedad conocidos, con una masa perceptible a nivel V del cuello derecho. Estudios de imagen muestran un tumor vascularizado de aproximadamente 6 x 7 x 6 cm, entre los músculos escalenos, que fue originado en la arteria cervical profunda. Se confirmó mediante biopsia incisional el hemangiopericitoma, el cual fue tratado mediante resección tumoral completa y radioterapia adyuvante. Actualmente el paciente no tiene actividad tumoral después de su tratamiento inicial. Conclusiones: El hemangiopericitoma en el cuello es raro, el diagnóstico constituye un reto clínico e histológico, ya que, al ser poco común, su potencial maligno resulta desconocido. Aquellos tumores que tienen bajo grado de malignidad pueden ser controlados, de acuerdo a su localización y tamaño, mediante resección completa; mientras que los tumores de alto grado pueden recurrir y dar origen a metástasis. Nuestro paciente tuvo características histopatológicas con invasión capsular, lo que trajo como consecuencia un incremento del riesgo de recurrencia local. Por ese motivo, se decidió aplicar tratamiento adyuvante con radioterapia. El paciente se mantiene sin recurrencia tumoral local y a distancia después de 9 años de vigilancia médica(AU)

Introduction: Hemangiopericytoma is a rare mesenchymal tumor (vascularized and potentially malignant) derived from pericytes. It can occur anywhere in the body; however, isolated cases are described in the neck. Complete surgical resection is the cornerstone of treatment. Objective: To present a case of hemangiopericytoma in the neck, as an unusual case, with unknown malignant potential, as well as its timely diagnosis and treatment. Case presentation: A 39-year-old male patient, with no known history of disease, had a noticeable mass at the V level of the right neck. Imaging studies showed a vascularized tumor of approximately 6 x 7 x 6 cm, between the scalene muscles, which originated in the deep cervical artery. Hemangiopericytoma was confirmed by incisional biopsy, as well as treated by complete tumor resection and with adjuvant radiotherapy. Currently, the patient has no tumor activity after his initial treatment. Conclusions: Hemangiopericytoma in the neck is rare. Its diagnosis is a clinical and histologic challenge because, being uncommon, its malignant potential is unknown. Those tumors with low-grade malignancy can be controlled, according to their location and size, by complete resection; while high-grade tumors may recur and give rise to metastases. Our patient had histopathologic features with capsular invasion, which resulted in an increased risk of local recurrence. For this reason, adjuvant treatment with radiotherapy was decided to be applied. The patient remains without local or distant tumor recurrence after 9 years of medical surveillance(AU)

Recurrent meningeal malignant tumor: Assessment of differences in the solitary fibrous tumor/hemangiopericytoma spectrum through a case report / Tumor maligno recurrente de origen meníngeo: evaluación de las diferencias de la entidad tumor fibroso solitario/hemangiopericitoma mediante un caso clínico

Solitary fibrous tumors (SFTs) are neoplasms that grow from mesenchymal fusiform cells. In the central nervous system, meninges are the common origin of these neoplasms. Although literature reports mostly SFT as benign neoplasm, malignancy data have been described in recurrences or metastatic lesions. Definitive diagnosis includes immunohistochemical profiles assessing cellular positivity for CD34, vimentin, CD99 and Bcl-2. Recent studies have demonstrated NAB2-STAT6 gene fusion as a distinct molecular feature of SFT with overexpression of the fusion protein NAB2-STAT6 in nuclei of these cells. Since several years, pathologists have grouped SFT and hemangiopericytomas (HPC) as different phenotypes of the same entity although both neoplasms do not share numerous features. This article, based on a case of a recurrent malignant SFT, aims to emphasize differences in the SFT/HPC spectrum due to the diagnostic, therapeutic and prognostic implications (AU)

Los tumores fibrosos solitarios (TFS) son neoplasias que crecen a partir de células mesenquimales y las meninges constituyen su origen preferente en el sistema nervioso central. Aunque la literatura relaciona la mayoría de los TFS como neoplasias benignas, se describen datos de malignidad en recidivas tumorales o lesiones metastásicas. El diagnóstico definitivo incluye el perfil inmunohistoquímico, que evalúa la positividad celular para CD34, vimentina, CD99 y Bcl-2. Estudios recientes han demostrado la fusión del gen NAB2-STAT6 como una característica molecular distintiva de los TFS, con sobreexpresión de la proteína de fusión NAB2-STAT6 en los núcleos de las células. Los patólogos han agrupado los TFS y los hemangiopericitomas como diferentes fenotipos de una misma entidad, aunque ambas neoplasias no comparten numerosas características. Este artículo, basado en un caso de una lesión maligna recurrente, tiene como objetivo enfatizar las diferencias en el espectro SFT/hemangiopericitoma por sus implicaciones diagnósticas, terapéuticas y pronósticas (AU)

Recurrent meningeal malignant tumor: Assessment of differences in the solitary fibrous tumor/hemangiopericytoma spectrum through a case report.

Solitary fibrous tumors (SFTs) are neoplasms that grow from mesenchymal fusiform cells. In the central nervous system, meninges are the common origin of these neoplasms. Although literature reports mostly SFT as benign neoplasm, malignancy data have been described in recurrences or metastatic lesions. Definitive diagnosis includes immunohistochemical profiles assessing cellular positivity for CD34, vimentin, CD99 and Bcl-2. Recent studies have demonstrated NAB2-STAT6 gene fusion as a distinct molecular feature of SFT with overexpression of the fusion protein NAB2-STAT6 in nuclei of these cells. Since several years, pathologists have grouped SFT and hemangiopericytomas (HPC) as different phenotypes of the same entity although both neoplasms do not share numerous features. This article, based on a case of a recurrent malignant SFT, aims to emphasize differences in the SFT/HPC spectrum due to the diagnostic, therapeutic and prognostic implications.

[Fat-forming solitary fibrous tumor with NAB2/ STAT6 gene fusion. Case report of genial location and literature review]. / Tumor fibroso solitario variedad lipomatosa con fusión génica NAB2/STAT6. Estudio de un caso de localización geniana y revisión de la literatura.

The lipomatous variety solitary fibrous tumor is a soft tissue neoplasm composed of mature adipose tissue and hemangiopericytoma areas. A 53-year-old man consulted for facial asymmetry and maxillofacial magnetic resonance imaging showed a cystic lesion, 3 x 2 cm in size, in front of the anterior wall of the maxillary sinus. Histologically, there were dense spindle cells expressing CD34, CD99, Bcl-2, and STAT6, myxoid zones, hemangiopericytomatous blood vessels, and S100 positive adipocytes. NAB2/STAT6 gene fusion was revealed by RT-PCR. The main differential diagnosis was raised with the spindle cell lipoma and malignant variant of the lipomatous solitary fibrous tumor. STAT6 overexpression and NAB2/STAT6 gene fusion are specific for lipomatous solitary fibrous tumor and the presence of lipoblasts and atypical lipomatous tumor areas suggests malignancy. These tumors located in the head and neck region have a benign biological behavior.

Tumor fibroso solitario variedad lipomatosa con fusión génica NAB2/STAT6. Estudio de un caso de localización geniana y revisión de la literatura / Fat-forming solitary fibrous tumor with NAB2/ STAT6 gene fusion. Case report of genial location and literature review

El tumor fibroso solitario variedad lipomatosa es una neoplasia de partes blandas compuesta por tejido adiposo maduro y áreas de hemangiopericitoma. Un varón de 53 años se presentó clínicamente con asimetría facial. La resonancia magnética maxilofacial demostró una lesión quística, de 3 x 2 cm, por delante de la pared anterior del seno maxilar. Histológicamente, había áreas fusocelulares densas que expresaban CD34, CD99, Bcl-2 y STAT6, zonas mixoides, vasos sanguíneos hemangiopericitomatosos y adipocitos S100 positivos. La fusión génica NAB2/STAT6 fue revelada mediante RT-PCR. El principal diagnóstico diferencial se planteó con el lipoma de células fusiformes y la variante maligna del tumor fibroso solitario lipomatoso. La sobreexpresión de STAT6 y la fusión génica NAB2-STAT6 son específicas del tumor fibroso solitario lipomatoso y la presencia de lipoblastos y áreas de tumor lipomatoso atípico sugiere malignidad. Estos tumores en cabeza y cuello tienen un comportamiento biológico benigno.(AU)

The lipomatous variety solitary fibrous tumor is a soft tissue neoplasm composed of mature adipose tissue and hemangiopericytoma areas. A 53-year-old man consulted for facial asymmetry and maxillofacial magnetic resonance imaging showed a cystic lesion, 3 x 2 cm in size, in front of the anterior wall of the maxillary sinus. Histologically, there were dense spindle cells expressing CD34, CD99, Bcl-2, and STAT6, myxoid zones, hemangiopericytomatous blood vessels, and S100 positive adipocytes. NAB2/STAT6 gene fusion was revealed by RT-PCR. The main differential diagnosis was raised with the spindle cell lipoma and malignant variant of the lipomatous solitary fibrous tumor. STAT6 overexpression and NAB2/STAT6 gene fusion are specific for lipomatous solitary fibrous tumor and the presence of lipoblasts and atypical lipomatous tumor areas suggests malignancy. These tumors located in the head and neck region have a benign biological behavior.(AU)

Resultados en el tratamiento de los hemangiopericitomas intracraneanos. Serie de casos / Results in the treatment of intracranial hemangiopericytomas. Case series

Objetivo Los hemangiopericitomas intracraneanos (HPC) son tumores de muy baja frecuencia, alta recidiva local y riesgo de metástasis extracraneal. El objetivo del siguiente trabajo es presentar los resultados en el tratamiento de los HPC en nuestra institución en los últimos 20 años y realizar una revisión de la literatura del tema. Material y métodos Se realizó un estudio retrospectivo que incluyó a los pacientes con diagnóstico de tumor fibroso solitario/hemangiopericitoma (TFS/HPC) intracraneanos intervenidos quirúrgicamente en el periodo 1997-2017. Se incluyeron pacientes con inmunomarcación positiva para STAT-6 y grado histológico II-III. Se recabaron datos demográficos, características tumorales, tratamiento y sobrevida de estos pacientes. Resultados Un total de 19 pacientes cumplieron los criterios de inclusión. La mediana de seguimiento fue de 96 meses (12-230). La mortalidad fue del 21% (n = 4). El 57,9% presentaron al menos una recidiva tumoral (n = 11) (con una recurrencia del 6, del 67 y del 90% a 1, 5 y 10 años, respectivamente). Cinco pacientes presentaron metástasis extracraneal. Tuvieron mayor sobrevida los pacientes con tumores < 6 cm (p < 0,05). Conclusiones Se presentó una serie de pacientes operados de TFS/HPC según los nuevos criterios de la OMS. El tamaño es un factor predictor de sobrevida. Actualmente no existen criterios validados de resección quirúrgica en esta patología. Una clasificación con orientación quirúrgica sería de utilidad en el futuro (AU)

Objective Intracranial hemangiopericytoma (HPC) is a rare central nervous system tumor characterized by its low incidence, high rate of local recurrence and risk of metastasis. The main objectives of this paper are two: to show the results in the treatment of HPC in our institution in the last 20 years and to make a review of the literature on this topic. Methods Retrospective review that includes patients diagnosed with intracranial solitary fibrous tumor/hemangiopericytoma (SFT/HPC) that have undergone surgery from 1997 to 2017. It includes patients that had nuclear expression of STAT-6 (detected by immunohistochemistry) and grade II/III histopathological diagnosis (defined by the World Health Organization in 2016). We collected demographic data, tumor characteristics, treatment and survival of these patients. Results A total of 19 patients fulfilled inclusion criteria. The median follow up was 96 months (12-230). The mortality rate was 21% (n = 4). 57.9% of patients presented at least one tumor recurrence (n = 11) (recurrences of 6%, 67% y 90% at 1, 5 and 10 years). Five patients presented extracranial metastasis. Patients with tumors < 6 cm had greater survival (P < .05). Conclusions A series of patients undergoing SFT/HPC were presented according to the new WHO criteria. Size is a predictor of survival. Currently there are no validated criteria for surgical resection in this pathology. A classification with surgical guidance would be useful (AU)

Results in the treatment of intracranial hemangiopericytomas. Case series. / Resultados en el tratamiento de los hemangiopericitomas intracraneanos. Serie de casos.

OBJECTIVE: Intracranial hemangiopericytoma (HPC) is a rare central nervous system tumor characterized by its low incidence, high rate of local recurrence and risk of metastasis. The main objectives of this paper are two: to show the results in the treatment of HPC in our institution in the last 20years and to make a review of the literature on this topic. METHODS: Retrospective review that includes patients diagnosed with intracranial solitary fibrous tumor/hemangiopericytoma (SFT/HPC) that have undergone surgery from 1997 to 2017. It includes patients that had nuclear expression of STAT-6 (detected by immunohistochemistry) and gradeII/III histopathological diagnosis (defined by the World Health Organization in 2016). We collected demographic data, tumor characteristics, treatment and survival of these patients. RESULTS: A total of 19 patients fulfilled inclusion criteria. The median follow up was 96 months (12-230). The mortality rate was 21% (n=4). 57.9% of patients presented at least one tumor recurrence (n=11) (recurrences of 6%, 67% y 90% at 1, 5 and 10years). Five patients presented extracranial metastasis. Patients with tumors <6cm had greater survival (P<.05). CONCLUSIONS: A series of patients undergoing SFT/HPC were presented according to the new WHO criteria. Size is a predictor of survival. Currently there are no validated criteria for surgical resection in this pathology. A classification with surgical guidance would be useful.

Hipoglucemia tumoral dependiente y no dependiente de células ß-pancreáticas, un reto diagnóstico / Tumor and non-tumor mediated hypoglycemia by pancreatic beta cells, a diagnostic challenge

La hipoglucemia es una urgencia médica frecuente que en la mayoría de los casos es secundaria al uso de fármacos hipoglucemiantes, orales o inyectados, indicados en pacientes con diabetes mellitus. No obstante, puede presentarse en forma espontánea y severa relacionándose con múltiples condiciones clínicas, incluyendo las neoplasias. Ante una hipoglucemia de origen paraneoplásico se deben reconocer los mecanismos fisiopatológicos que la generan y establecer el diagnóstico oportuno y preciso para disminuir las complicaciones propias de este síndrome clínico. Presentamos dos pacientes con cuadro de hipoglucemia refractaria al manejo médico inicial, de aparición similar con patologías diferentes. El primer caso corresponde a un paciente con insulinoma y el segundo con un hemangiopericitoma.

Hypoglycemia is a common medical emergency which is mostly secondary to the use of oral or injected hypoglycemic drugs indicated in patients with diabetes mellitus. However, it can present spontaneously and severely in relation to multiple clinical conditions, including neoplasms. When faced with hypoglycemia associated with paraneoplastic disorders, the pathophysiological mechanisms of hypoglycemia must be recognized and a timely and accurate diagnosis must be established in order to diminish complications inherent to this clinical syndrome. We herein present two patients with hypoglycemia refractory to initial medical management, sharing similar appearance with other pathologies. The first case corresponds to a patient with an insulinoma and the second to a patient with a hemangiopericytoma.

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.

Hemangiopericytoma/Solitary Fibrous Tumor in the central nervous system. Experience with surgery and radiotherapy as a complementary treatment: A 10-year analysis of a heterogeneous series in a single tertiary center.

Hemangiopericytoma and Solitary Fibrous Tumor are tumors with low incidence. They have a tendency to recur locally and to metastasize. The WHO integrated both tumors into a new entity but one of the pending issues is to demonstrate the effectiveness of surgery plus complementary radiotherapy (RT) and standardize the use of it. We reviewed the data from 10 years. We assessed pathologic and radiologic characteristics. The operation records were evaluated to determine the features and extent of tumor resection. We compared the outcomes in patients using or not RT. The mean follow-up was 74.8 months, with a range of 12 and 210 months. The population included 3 males (30%) and 7 females (70%). The most common location was brain convexity (30%), the remaining were cervical and lumbar spine, sacrum, intraventricular, torcular, sphenoid ridge and intraorbital. Postoperative external beam radiotherapy was delivered in 7 patients (70%), the criteria were a partial resection or WHO II and III histological grades. 2 patients developed local recurrences at 12 and 19 months after initial surgery. 1 patient underwent 2 surgeries, and the other, 4 surgeries. The mean recurrence free survival rate was 15.5 months. Distant metastases were found in 4 patients. 3 of the 10 patients died. Five-year overall survival rate was 66% and mean overall survival was 76 months. A safe and complete resection in the first surgery is the most important prognostic factor. Complementary RT can be helpful, even in cases of complete resection in WHO low-grade.

[Urinary tract hemangiopericytoma treated with radical surgery and neoadjuvant chemotherapy. Report of two cases.] / Hemangiopericitoma en la via urinaria tratados con cirugía radical y quimioterapia neoadyuvante. A propósito de dos casos.

OBJECTIVE: Hemangiopericytoma is a tumor of vascular origin. It is very rare in the prostate. They are generally aggressive tumors. Currently, the treatment of choice is radical surgery. In the literature, there are less than 50 cases described, and neoadjuvant treatment has not been reported in any article. This treatment presents positive responses in another type of sarcomas. Our goal is to report two cases of malignant hemangiopericytoma. The first case was treated with radical surgery and the second case was treated with neoadjuvant chemotherapy before surgery. METHODS: The first case is a 40 year old male. Obstructive urinary symptoms were his first symptoms. A prostate mass with tumor characteristics was seen on ultrasound. After transrectal biopsy, he was diagnosed with hemangiopericitoma. Because the tumor size, neoadjuvant chemotherapeutic prior to radical surgery was decided. The second case is a 77 year old male with an incidental diagnosis of renal mass. After radical nephrectomy, he was diagnosed with hemangiopericitoma. He did not receive adjuvant chemotherapy. RESULTS: In the first case, after neoadjuvant therapy, tumor size was reduced significantly. A pelvic exenteration with radical cystoprostatectomy and rectal resection and Bricker type urinary diversion and colostomy were carried out. In the second case controls after radical nephrectomy were correct and he did not need any treatment. CONCLUSIONS: In urinary tumors, the prostate hemangiopericytoma is a rare entity. Currently, neoadjuvant chemotherapy is not established as treatment for these tumors. For other sarcomas neoadyuvant treatment has good response. In our case, a good result was obtained with neoadjuvant chemotheraphy before surgery. However, a greater number of cases are necessary to establish the use of neoadjuvant chemotherapy in urinary hemangiopericytomas.

OBJETIVO: El hemangiopericitoma es un tumor de origen vascular muy infrecuente en la próstata. Son tumores de características agresivas y actualmente el tratamiento de elección es la cirugía radical. En la literatura existen descritos menos de 50 casos de hemangiopericitomas, y ninguno con tratamiento neoadyuvante. Dicho tratamiento neoadyuvante está descrito en otro tipo de sarcomas con buenas respuestas. Presentamos dos casos de un hemangiopericitoma maligno, uno tratado sólo con cirugía radical y otro con neoadyuvancia quimioterápica previa a la cirugía radical.MATERIAL Y MÉTODOS: El primer caso es un varón de 40 años con síntomas urinarios obstructivos. Se diagnosticó de hemangiopericitoma tras realización de biopsia transrectal. Se decidió neoadyuvancia quimioterápica previa a la cirugía. El segundo paciente es un varón de 74 años con hallazgo accidental de masa renal. Tras nefrectomía radical se diagnosticó de hemangiopericitoma. RESULTADOS: Tras la neoadyuvancia, en el primer paciente, se realizó una cistoprostatectomía radical y una resección de recto con derivación urinaria tipo Bricker y colostomía. El segundo paciente no precisó tratamiento adyuvante tras la cirugía radical. CONCLUSIONES: El hemangiopericitoma es una entidad rara en los tumores urinarios. Actualmente la neoadyuvancia quimioterápica no está establecida como pilar del tratamiento de estos tumores, aunque en otros campos donde los sarcomas son más frecuentes, si que se objetiva mejor respuesta. En nuestro caso obtuvimos una buena respuesta con dicha neoadyuvancia previa a la cirugía, aunque es necesario un mayor seguimiento a una mayor cohorte de pacientes para establecer el uso de la quimioterapia neoadyuvante en los hemangiopericitomas urinarios.

Spine metastasis of intracranial hemangiopericytoma: case report of two treatments / Metástase na coluna de hemangiopericitoma intracraniano: relato de 2 tratamentos

ABSTRACT Objective: To report the use of two techniques (radiosurgery and en bloc vertebrectomy) on the same patient for the treatment of two metastases in different sites of the spine arising from intracranial hemangiopericytoma. Intracranial hemangiopericytomas are rare, comprising approximately 2.4% of meningeal tumors and <1% of all tumors of the central nervous system. Metastases to the spine are even rarer: The largest case series reported in the literature has 5 and 7 cases. Methods: A 37-year-old man diagnosed with intracranial hemangiopericytoma was referred for a metastatic lesion in T12 and underwent en bloc resection using the Tomita technique. Results: The disease evolved with a metastasis to T2 treated by radiosurgery with 1600 cGy. The patient died 1,706 days after the en bloc resection of T12 and 1324 days after the radiosurgery of T2, and no recurrence occurred in these locations due to progression of the systemic diseases (liver and central nervous system). Conclusion: This is the first case reported in the literature in which two different techniques were used to treat metastatic lesions in the spine from an intracranial hemangiopericytoma and is unique for its use of two treatments in the same patient. Level of evidence: V, case report

RESUMO Objetivo: Reportar o uso de duas técnicas (radiocirurgia e vertebrectomia em bloco) no mesmo paciente, para o tratamento de metástases de um hemangiopericitoma intracraniano em dois locais da coluna. Hemangiopericitomas intracranianos são raros, consistindo em cerca de 2,4% dos tumores da meninge e menos de 1% de todos os tumores do sistema nervoso central, e metástases para a coluna são ainda mais raras: as maiores séries de casos publicada tinham 5 e 7 casos. Métodos: Um homem de 37 anos com diagnóstico de hemangiopericitoma intracraniano foi encaminhado para resecção em bloco de lesão metastática em T12 com a técnica de Tomita. Resultados: A doença evoluiu com metástase em T2, tratada com radiocirurgia (dose de 1600 cGy). O paciente morreu 1.706 dias após a resecção em bloco de T12 e 1.324 dias após a radiocirurgia de T2, livre de recorrência nessas localizações, devido a progressão sistêmica da doença (para fígado e sistema nervoso central). Conclusão: Este é o primeiro caso na literatura no qual duas técnicas diferentes foram usadas pra tratar lesões metastáticas da coluna de hemangiopericitoma intracraniano, único pelo uso de duas técnicas de tratamento no mesmo paciente. Nível de evidência V, relato de caso.

Hemangiopericitoma primario de pulmon como hallazgo incidental. reporte de dos casos / Primary pulmonary hemangiopericytoma as an incidental finding. two case report

El hemangiopericitoma intra-pulmonar es una neoplasia poco frecuente, que se origina a partir de lo pericitos que rodean la membrana basal de capilares y vénulas dentro del parénquima pulmonar. Presentar dos casos clínicos como hallazgo incidental en su presentación conocer comportamiento y manejo de dicha neopla Paciente masculino 40 años cuadro clínico caracterizado por tos seca persistente, cansancio con dificultad para respirar con progresión a disnea rápida a pequeños esfuerzo y pérdida de peso. Paciente femenina de 37 años cuadro clínico caracterizado por dolor torácico tipo opresivo en región latero posterior de hemitórax erecho, con irradiación al hombro, radiografía de tórax póstero-anterior, se descubrió múltiples imágenes nodulares parenquimatosas bilaterales a nivel central y sub-pleural. Segundo paciente se realiza radiografía de tórax póstero-anterior se observa patrón de masa tumoral localizada en el centro del pulmón derecho, las cuales fueron confirmadas por tomografía computada de tórax. Se procedió a la recesión quirúrgica de dicha lesión para biopsia, mientras que en la paciente femenina la conducta fue más radical se practicó umonectomía derecha. Evolucionan satisfactoriamente. Reporte de biopsia y de inmunohistoquímica: emangiopericitoma primario de pulmón/tumor fibroso solitario. Ambos pacientes fueron referidos al servicio de oncología médica para terapia adyuvante.Tumor poco frecuente, su patogénesis es todavía desconocida y aunque es un tumor en apariencia y comportamiento benigno, debe tratarse como potencialmente maligno, se debe ser radical en el tratamiento y realizar un seguimiento cuidadoso, continuo y estricto del paciente(AU)

Intrapulmonary hemangiopericitoma potentially is a rare malignancy, which originates from the pericytes that surround the basal membrane of the capillaries and venules in lung parenchyma. The aim is to resent two cases as an incidental finding in its presentation and also know the behavior and management of this neoplasm. A male patient of 40 years with clinical picture haracterized by persistent dry cough, fatigue with shortness of breath with rapid progression to small effort dyspnea and weight loss and a female patient aged 37 clinical picture characterized by chest pain ppressive kind in lateral region posterior right chest, radiating to the shoulder in the chest film, multiple nodular parenchymal bilateral central and sub-pleural. The second patient so poster anterior chest radiograph where a pattern of tumor mass located in the center of the right lung, which was confirmed by computed tomography of the chest, is observed is performed. In the patient he proceeded to the surgical recession of the injury to biopsy, while in the female patient's behavior was more radical right neumonectomy was practical. They are evolving satisfactorily. Report biopsy and mmunohistochemistry for primary lung: Hemangiopericitoma / SFT. Both patients were referred to medical oncology service for adjuvant therapy. Is a rare tumor, its pathogenesis is still unknown and although a tumor in appearance and benign behavior, should be treated as malignant potential, must be radical in treatment and careful monitoring, continuous and strict patient(AU)

Endoscopic management of sinonasal hemangiopericytoma: case report and literature review

ABSTRACT Introduction: Hemangiopericytoma is a rare vascular tumor of the sinonasal region, associated with epistaxis and nasal obstruction as the main symptoms. When located in this region, it has special clinical characteristics that differentiate it from others. Case presentation: The following paper reports the case of a 43-year-old female patient presenting with right nasal obstruction and 6 months of evolution associated with mucopurulent rhinorrhea and recurrent right side epistaxis. Physical examination showed a right obstructive mass originating from the cribriform plate. Computed tomography of the paranasal sinuses revealed a complete blockage of the right nasal cavity by a homogeneous content, with soft tissue density and no evident contrast enhancement. The lesion extended superiorly to the cribriform plate but without intracranial or orbital extension. The patient was treated with endoscopic surgery and anatomopathological study revealed sinonasal hemangiopericytoma. The patient had complete remission and subsequent 3-year follow-up without recurrence. Conclusion: The recommended treatment for hemangiopericytoma is total surgical excision with free margins. The results are generally good and the risk of recurrence seems to be associated with incomplete tumor excision. A literature review is presented and its main characteristics are discussed.

RESUMEN Introducción: El hemangiopericitoma es un tumor vascular poco frecuente en la región nasosinusal que se asocia con epistaxis y obstrucción nasal como principales síntomas. Cuando se presenta en esta región, tiene características clínicas especiales que lo hace diferente de otras localizaciones. Presentación del caso: A continuación se presenta el caso de una paciente de 43 años de edad, con síntomas de obstrucción en fosa nasal derecha y 6 meses de evolución asociados con rinorrea mucopurulenta y epistaxis recurrente del lado derecho. El examen físico mostró una masa obstructiva en el lado derecho con origen en la placa cribriforme. Una tomografía computarizada de los senos paranasales reveló un bloqueo completo de la cavidad nasal derecha por un contenido homogéneo, con densidad de partes blandas, sin captación de contraste evidente. La lesión se extendía hacia el lado superior de la placa cribriforme, sin extensión intracraneal u orbitaria. La paciente fue tratada con cirugía endoscópica y el estudio anatomopatológico reveló un hemangiopericitoma sinonasal. La paciente tuvo remisión completa y se realizó seguimiento por 3 años sin recurrencia. Conclusión: El tratamiento recomendado para el hemangiopericitoma es la escisión quirúrgica total con márgenes libres, cuyos resultados son generalmente buenos. El riesgo de recurrencia parece estar asociado con una escisión tumoral incompleta. Se presenta una revisión de literatura, así como comentarios sobre sus características principales.

Solitary fibrous tumour/haemangiopericytoma of the spinal cord. / Tumor fibroso solitario/hemangiopericitoma de la médula espinal.

Solitary fibrous tumours/haemangiopericytomas (SFT/HPC) are fibroblastic mesenchymal neoplasms that harbour a genetic fusion of NAB2/STAT6. There are few reported cases in the central nervous system (CNS), of which spinal tumours are minority. We present a case of a 63-year-old woman with progressive paraparesis and a sensory level of T6. On the MRI we detected an intradural extramedullary lesion on T9-T10. We performed a laminectomy with an intraoperative ultrasound study in which we observed a heterogeneous lesion with an infiltrating pattern affecting the medulla at several points, and resection of the lesion. Immunohistological findings revealed positivity for vimentin, CD34 and STAT6; Ki-67 was 2%. A final diagnosis of SFT/HPC with SFT phenotype was made. SFT/HPC of the CNS is a rare neoplasm that should be taken into account in the differential diagnosis of these kinds of lesions, as clinical and MRI findings often lead to errors in diagnostic approach. The support of intraoperative ultrasound can assist in surgical decision-making.

Glomangiopericytoma: an unusual type of nasosinusal tumor / Glomangiopericitoma: tipo incomum de tumor nasossinusal

ABSTRACT Glomangiopericytomas are soft tissue tumors showing distinct perivascular myoid differentiation in sinonasal region that correspond to less than 0.5% of neoplasms in this region. We report the case of a 39-year-old patient with intranasal tumor of hemangiopericytoid pattern and immunohistochemistry compatible with glomangiopericytoma. We opted for external and endonasal surgical treatment, with preoperative embolization. Glomangiopericytomas are uncommon and are characterized by frequent recurrence, but metastases are rare. Generally painless, they present with unilateral nasal obstruction and/or epistaxis, with a polypoid, reddish and friable mass, and the diagnosis can be confirmed by histopathological and immunohistochemical examination.

RESUMO Glomangiopericitomas são tumores de partes moles que apresentam diferenciação mioide perivascular distinta na região sinunasal e correspondem a menos de 0,5% das neoplasias dessa região. Relatamos o caso de um paciente de 39 anos de idade com tumoração intranasal de proliferação celular de padrão hemangiopericitoide e imuno-histoquímico compatível com glomangiopericitoma. Optou-se por tratamento cirúrgico externo e endonasal, com embolização pré-operatória. Os glomangiopericitomas são incomuns e caracterizam-se pela recorrência frequente, sendo raras as metástases. Geralmente indolores, apresentam-se com obstrução nasal unilateral e/ou epistaxe, com massa de aspecto polipoide, avermelhada e friável. O diagnóstico pode ser confirmado pelo exame histopatológico e imuno-histoquímico.

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)

Hemangiopericitoma cerebral en paciente adolescente: reporte de un caso y revisión de literatura / Cerebral hemangiopericytoma in adolescent patient: case report and literature review

Introducción: En 1942 Stout y Murray describieron un tumor extraneural compuesto por una proliferación de vasos sanguíneos con endotelio normal rodeados de células neoplásicas que presumiblemente surgían de los Pericitos. La Neoplasia fue llamada Hemangiopericitoma. Se trata de un tumor agresivo, más frecuente en adultos. En los niños son extremadamente raros, solo 11 casos han sido reportados en la literatura. Se originan de la transformación maligna de los Pericitos de Zimmerman. Descripción del caso: Presentamos el caso de una adolescente de 16 años, con antecedente de convulsiones generalizadas en el año 2009, detectándose en el 2014 lesión ocupante de espacio parieto-occipital derecha, la cual es extirpada, informándose como meningioma. Evoluciona con recidiva tumoral 3 meses más tarde, evaluándose por inmunomarcación nueva muestra de lesión, con la que se arriba al diagnóstico de hemangiopericitoma. Conclusión: El Hemangiopericitoma cerebral es una patología rara, de muy baja prevalencia, y de gran similitud clínica e imagenológica con los meningiomas. Incluso genera gran cantidad de diagnósticos erróneos con la histopatología convencional. Por todo lo antes mencionado, es muy importante tener presente esta patología a la hora de pensar en diagnósticos diferenciales de meningiomas, siendo fundamental la inmunomarcación para confirmar uno u otro diagnóstico.

Introduction: In 1942, Stout and Murray described an extraneural tumor composed of a proliferation of blood capillaries with normal endothelium and surrounded by neoplastic cells, which presumably arose from pericytes. The neoplasm was thus labeled an hemangiopericytoma. This aggressive tumor is more common in adults than in children, in whom it is extremely rare, with only 11 cases reported in the literature. It stems from the malignant transformation of pericytes of Zimmerman. Case report: We present the case of a 16-year old teen with a history of generalized seizures in 2009, in whom a spaceoccupying parieto-occipital lesion was detected and removed in 2014, at which time it was diagnosed as a meningioma. However, upon tumor recurrence three months later, further immuno-staining revealed the lesion to be a hemangiopericytoma. Conclusion: Cerebral hemangiopericytomas have a very low prevalence and high degree of clinical and imaging similarity with meningiomas. This similarity frequently leads to misdiagnosis with conventional histopathology. For this reason, it is crucial to remember this pathology in the differential diagnosis of a meningioma, so that appropriate immuno-staining is performed to either confirm or rule out its presence.