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1.
Medicine (Baltimore) ; 99(13): e19651, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32221091

RESUMO

RATIONALE: Intracranial solitary fibrous tumors (SFTs) and hemangiopericytomas (HPCs) are rare spindle cell tumors of mesenchymal origin that include benign and malignant neoplasms. PATIENT CONCERNS: We present a 66-year-old male with a 5-year history of headache and dizziness, with left progressive sensorineural hearing loss over 1 month. DIAGNOSES: WHO grade II SFT/HPC originating from the internal auditory canal in the left cerebellopontine angle. INTERVENTIONS: surgical resection. OUTCOMES: No local recurrence or metastases were observed in the follow-up 3 months after the surgery. LESSONS: Intracranial SFTs/HPCs are rare mesenchymal neoplasms that are challenging to manage. If the imaging characteristics of tumor are not typical, clinicians should depend on tissue biopsy and immunohistochemistry to make a definitive diagnosis.


Assuntos
Neoplasias Cerebelares/patologia , Ângulo Cerebelopontino/patologia , Hemangiopericitoma/patologia , Tumores Fibrosos Solitários/patologia , Idoso , Neoplasias Cerebelares/diagnóstico , Neoplasias Cerebelares/cirurgia , Diagnóstico Diferencial , Tontura/etiologia , Cefaleia/etiologia , Perda Auditiva/etiologia , Hemangiopericitoma/diagnóstico , Hemangiopericitoma/cirurgia , Humanos , Masculino , Gradação de Tumores , Neuroma Acústico/diagnóstico , Tumores Fibrosos Solitários/diagnóstico , Tumores Fibrosos Solitários/cirurgia
2.
Neurocirugía (Soc. Luso-Esp. Neurocir.) ; 31(1): 14-23, ene.-feb. 2020. tab, ilus, graf
Artigo em Inglês | IBECS | ID: ibc-190368

RESUMO

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


El hemangiopericitoma y el tumor fibroso solitario son enfermedades del sistema nervioso central (SNC) con una incidencia baja. Estos tumores también pueden tener algunas características como una tendencia a recurrir localmente y hacer metástasis. La OMS, en su última clasificación de tumores del SNC, integró ambos tumores en una nueva entidad: tumor fibroso solitario/hemangiopericitoma (SFT/HPC), pero uno de los problemas actuales pendientes es demostrar la efectividad de la cirugía y la radioterapia (RT) complementaria, y estandarizar el uso de la misma. Revisamos todos los datos clínicos de nuestro hospital en un período de 10 años, y encontramos 10 pacientes con SFT/HPC. Se evaluaron los tamaños de los tumores, las ubicaciones y las características radiológicas. Los registros quirúrgicos de cada paciente se evaluaron para determinar las características macroscópicas y el alcance de la resección del tumor (EOR). También comparamos los resultados en los pacientes que utilizan o no RT como tratamiento complementario. El seguimiento medio fue de 74,8 meses, con un rango de 12 y 210 meses. La población incluía 3 varones (30%) y 7 mujeres (70%). La localización más frecuente fue la convexidad cerebral (30%). Las distribuciones de las localizaciones restantes del tumor fueron columna cervical y lumbar, sacro, intraventricular, torcular, ala esfenoidal e intraorbitario. La radioterapia postoperatoria (EBRT) se administró en 7 pacientes (70%), los criterios para tratarlos fueron una resección parcial o un grado histológico de la OMS II y III. Dos pacientes desarrollaron recidivas locales a los 12 y 19 meses después de la cirugía inicial. Un paciente se sometió a 2 cirugías, y el otro a 4 cirugías. La tasa media de supervivencia libre de recidiva (RFS) fue de 15,5 meses. Se encontraron metástasis a distancia en 4 pacientes durante el período de seguimiento. Tres de los 10 pacientes fallecieron durante el período de seguimiento. La tasa de supervivencia general a 5 años fue del 66%, y la supervivencia media global fue de 76 meses. Una resección segura y completa en la primera cirugía es el factor pronóstico más importante. Consideramos que la RT complementaria puede ser útil, incluso en casos de resección completa en SFT/HPC de bajo grado. Sería interesante definir previamente la diferenciación entre el SFT/HPC y los meningiomas para las estrategias de tratamiento


Assuntos
Humanos , Masculino , Feminino , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Hemangiopericitoma/radioterapia , Hemangiopericitoma/cirurgia , Neoplasias do Sistema Nervoso Central/radioterapia , Neoplasias do Sistema Nervoso Central/cirurgia , Taxa de Sobrevida , Estudos Retrospectivos , Hemangiopericitoma/diagnóstico por imagem , Eletrofisiologia/métodos , Hemangiopericitoma/patologia , Diagnóstico Diferencial , Período Pós-Operatório
3.
Medicine (Baltimore) ; 98(47): e17888, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31764783

RESUMO

RATIONALE: Hemangiopericytoma (HPC) is a rare pediatric neoplasm with a high risk of bleeding, aggressive growth and high early relapse rates. Surgical excision remains the mainstream treatment, while the functions of chemotherapy and radiotherapy remain controversial. In particular, an infantile giant extracranial HPC located in the forehead has never been reported. PATIENT CONCERNS: A 3-day-old girl was delivered normally with a giant tumor localized mainly in the right frontal region. The surface of the mass was filled with vascularity. DIAGNOSIS: According to the results of imaging and pathological examinations, the diagnosis was HPC grade II. INTERVENTIONS: Gross total resection of the tumor and the invading partial frontal bone followed by skin scalp reconstruction was carried out without any blood transfusion. OUTCOMES: No recurrence was identified during 5 years of follow-up. And better outcomes can be achieved without adjuvant therapy. LESSONS: Multimodality imaging and a collaborative multidisciplinary approach are indispensable for the successful surgical management of infantile HPC, especially for giant tumors and their potential risk of life-threatening bleeding. Gross total resection is the optimal option for infantile HPC, and even without adjuvant therapy, it achieves better outcomes.


Assuntos
Neoplasias de Cabeça e Pescoço , Hemangiopericitoma , Neoplasias de Tecidos Moles , Pré-Escolar , Feminino , Seguimentos , Testa , Neoplasias de Cabeça e Pescoço/patologia , Neoplasias de Cabeça e Pescoço/cirurgia , Hemangiopericitoma/patologia , Hemangiopericitoma/cirurgia , Humanos , Recém-Nascido , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/cirurgia , Fatores de Tempo , Carga Tumoral
5.
In Vivo ; 33(6): 2141-2145, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31662549

RESUMO

BACKGROUND/AIM: Extralobar pulmonary sequestration (EPS) is an unusual congenital defect characterized by the presence of non-functioning lung tissue receiving arterial supply from the systemic arteries. Primary hemangiopericytoma (HPC) is an uncommon potentially malignant tumor of vascular origin that usually involves the soft tissue of the extremities or retroperitoneum, but extremely rarely affects the lung. We present the rare case of a primary pulmonary HPC arising in an EPS. CASE REPORT: A 65-year-old woman, with dyspnea and pleuritic chest pain, was referred for further investigation. Radiological evaluation demonstrated a well-circumscribed mass above the right hemidiaphragm, receiving its arterial supply from the descending thoracic aorta. The patient underwent a right posterolateral thoracotomy and a middle lobectomy. The intraoperative finding was a well-encapsulated solid mass. The histological evaluation described HPC. RESULTS: The patient remains fit and healthy. CONCLUSION: Pulmonary HPC can arise in EPS. Surgical excision is the treatment of choice.


Assuntos
Sequestro Broncopulmonar/complicações , Sequestro Broncopulmonar/diagnóstico , Hemangiopericitoma/diagnóstico , Hemangiopericitoma/etiologia , Idoso , Biomarcadores , Biópsia , Sequestro Broncopulmonar/cirurgia , Diagnóstico Diferencial , Feminino , Hemangiopericitoma/cirurgia , Humanos , Avaliação de Sintomas , Tomografia Computadorizada por Raios X , Resultado do Tratamento
6.
Arch Esp Urol ; 72(7): 705-709, 2019 Sep.
Artigo em Espanhol | MEDLINE | ID: mdl-31475682

RESUMO

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.


Assuntos
Hemangiopericitoma/cirurgia , Terapia Neoadjuvante , Sistema Urinário/cirurgia , Adulto , Idoso , Quimioterapia Adjuvante , Terapia Combinada , Hemangiopericitoma/tratamento farmacológico , Humanos , Masculino , Sarcoma
7.
Arch. esp. urol. (Ed. impr.) ; 72(7): 705-709, sept. 2019. ilus
Artigo em Espanhol | IBECS | ID: ibc-187857

RESUMO

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


Objective: Hemangiopericytoma is a tumor of vascular origin. It is very rare in the prostate. They are generally aggressive tumors. Currently, the treatment ofchoice 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


Assuntos
Humanos , Masculino , Adulto , Idoso , Hemangiopericitoma/tratamento farmacológico , Hemangiopericitoma/cirurgia , Neoplasias da Próstata/tratamento farmacológico , Neoplasias da Próstata/cirurgia , Neoplasias Renais/tratamento farmacológico , Neoplasias Renais/cirurgia , Imagem por Ressonância Magnética , Quimioterapia Adjuvante
9.
J Cancer Res Ther ; 15(3): 729-732, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31169253

RESUMO

In 1942, Stout and Murray first used the term hemangiopericytoma to describe a tumor which is distinguished histologically from other types of vascular neoplasm characterized by proliferation of pericytes. It is a rare neoplasm that was originally described as a vascular tumor derived from the pericytes. They account for 2%-3% of all soft tissue sarcomas in humans and they occur mainly in the musculoskeletal system. About 15%-30% of all hemangiopericytomas occur in the head and neck region. Hemangiopericytoma of supraglottis is very rare neoplasm with only nine cases reported in literature and ours is the tenth case overall and first case in pediatric age group. Herein, we are presenting an extremely rare case report of hemangiopericytoma of supraglottis in a 6-year-old male child who presented with stridor followed by which tracheostomy was done. Later, the patient was treated initially with radiotherapy followed by surgery, i.e., laryngectomy in view of residual disease postcurative radiotherapy. Hence, hemangiopericytoma is a very rare tumor overall and can present in pediatric age group and can be one most important differential diagnosis because many patients in this age group, stridor most commonly occurs due to the infectious causes such as influenza virus and diphtheria-induced croup, i.e., laryngotracheobronchitis.


Assuntos
Glote/patologia , Hemangiopericitoma/diagnóstico , Biópsia , Criança , Diagnóstico Diferencial , Hemangiopericitoma/cirurgia , Humanos , Imuno-Histoquímica , Radiografia , Tomografia Computadorizada por Raios X , Resultado do Tratamento
10.
J Neurooncol ; 143(3): 457-464, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31054100

RESUMO

BACKGROUND: Prior studies have highlighted infratentorial tumor location as a prognostic factor for solitary fibrous tumor (SFT) and hemangiopericytoma (HPC) of the central nervous system (CNS), and spinal location is considered a positive prognostic factor for other tumors of the CNS. While SFT/HPC of the CNS is known to frequently arise from the spinal meninges, there are no case series that report outcomes for spinally located CNS tumors, and their prognosis in relation to intracranial and other CNS-located tumors is unknown. OBJECTIVE: To investigate outcomes for patients with SFT/HPC of the spinal meninges. METHODS: The Surveillance, Epidemiology, and End-Results Program was used to identify patients with SFT/HPC within the CNS from 1993-2015. We retrospectively analyzed the relationship between tumor location (spinal vs. Brain and other CNS) and survival. RESULTS: We identified 551 cases of CNS SFT/HPC, 64 (11.6%) of which were primary tumors of the spinal meninges. Spinal tumors were more likely than brain and other CNS tumors to be SFT vs. HPC (37.5 vs. 12%, p < 0.001), benign (42.2 vs. 20.3%, p < 0.001), and less than 5 cm (53.1 vs. 35.7%, p < 0.001). The 10-year survival rates for spinal and brain/other CNS tumors were 85 and 58%, respectively. Median survival time was significantly longer for spinal tumors (median survival not reached vs. 138 months, p = 0.03, HR = 0.41 [95% CI 0.18-0.94]). On multivariable analysis, spinal tumor location was associated with improved survival over tumors located in the brain and other CNS (HR = 0.36 [95% CI 0.15-0.89], p = 0.03). CONCLUSION: Spinal tumor location is associated with improved survival in patients with SFT/HPC of the CNS. Larger institutional studies are necessary to characterize the relationship between tumor location and other relevant factors such as presentation and amenability to gross-total resection and adjuvant radiotherapy. Future studies exploring optimal management of spinally located tumors are also needed.


Assuntos
Hemangiopericitoma/mortalidade , Tumores Fibrosos Solitários/mortalidade , Neoplasias da Coluna Vertebral/mortalidade , Neoplasias da Coluna Vertebral/patologia , Feminino , Seguimentos , Hemangiopericitoma/patologia , Hemangiopericitoma/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Programa de SEER , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/cirurgia , Neoplasias da Coluna Vertebral/cirurgia , Taxa de Sobrevida
11.
J Am Anim Hosp Assoc ; 55(4): 194-200, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31099606

RESUMO

This retrospective study evaluated the postoperative outcome and clinical parameters associated with prognosis in 167 client-owned dogs with 167 hemangiopericytomas. Parameters that were reviewed for an association with long-term outcome included signalment, clinical history, results of staging tests, tumor and surgical variables, and administration of adjunctive therapy. History of previous surgery, type of surgery performed, status of surgical margins, tumor location, and whether adjunctive therapy was performed were associated with tumor recurrence. The distal forelimb was the most common location reported overall (46/167 [27.5%]). Dogs with tumors located at the tail/perineum had the fastest recurrence rate, with a median disease-free interval of ∼16 mo (505 days). Tumor grade alone was not associated with recurrence (P = .069), but when analyzing tumor grade and margin, low-grade tumors with dirty margins had a significantly shorter time to recurrence than low-grade tumors with either clean or narrow margins. Tumor location should be considered when assessing the treatment plan and follow-up recommendations for any hemangiopericytomas. Aggressive initial surgical treatment is recommended when possible to reduce the chance of local tumor recurrence.


Assuntos
Doenças do Cão/cirurgia , Hemangiopericitoma/veterinária , Neoplasias Esplênicas/veterinária , Animais , Terapia Combinada , Intervalo Livre de Doença , Doenças do Cão/patologia , Cães , Hemangiopericitoma/patologia , Hemangiopericitoma/cirurgia , Estudos Retrospectivos , Esplenectomia/veterinária , Neoplasias Esplênicas/patologia , Neoplasias Esplênicas/cirurgia , Resultado do Tratamento
12.
Prog Neurol Surg ; 34: 223-231, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31096251

RESUMO

Leksell stereotactic radiosurgery has proven to be effective for less common tumors encountered in the brain, including hemangiomas of the orbit or cavernous sinus, recurrent hemangiopericytomas, and both sporadic hemangioblastomas as well as those encountered in the context of von Hippel-Lindau (VHL) disease. While all three tumors are responsive to single-session radiosurgery, hemangiomas and hemangiopericytomas are the most likely to demonstrate tumor regression. Hemangiopericytomas that recur after initial resection can be lower grade or anaplastic and have both higher local as well as distant recurrence risks. Sporadic hemangioblastomas undergo Leksell radiosurgery at the time of recurrence after initial surgery. In the context of VHL, growing or recurrent tumors are treated with tumor control rates exceeding 90%. Tumor control improves with higher dose delivery, typically >15 Gy at the margin. Dose-limiting structures may include the optic apparatus for hemangiomas and brain stem locations for hemangioblastomas.


Assuntos
Neoplasias Encefálicas/radioterapia , Hemangioblastoma/radioterapia , Hemangioma/radioterapia , Hemangiopericitoma/radioterapia , Recidiva Local de Neoplasia/radioterapia , Radiocirurgia/métodos , Doença de von Hippel-Lindau/radioterapia , Neoplasias Encefálicas/cirurgia , Hemangioblastoma/cirurgia , Hemangioma/cirurgia , Hemangiopericitoma/cirurgia , Humanos , Recidiva Local de Neoplasia/cirurgia , Doença de von Hippel-Lindau/cirurgia
13.
No Shinkei Geka ; 47(3): 329-334, 2019 Mar.
Artigo em Japonês | MEDLINE | ID: mdl-30940785

RESUMO

Revision of WHO guidelines in 2016 led to the classification of solitary fibrous tumours(SFTs)and haemangiopericytomas(HPCs)as a single tumor entity characterized by NAB2-STAT6 fusion. Standard-of-care treatment involves surgery, but local recurrence and distant metastasis sometimes occur. The average latency to metastasis after surgery is 99 months. A 38-year-old female patient presented with a complaint of headache. An 8×5×2cm lesion showing Gd-T1 enhancement was detected near the superior sagittal sinus. Pathological assessment following resection revealed proliferating, polymorphic, atypical tumor cells with distinct nucleoli in a "patternless pattern." Cellularity was moderate to high, and mitotic figures were observed in 15/10 high power fields. Immunohistochemically, tumor cells tested positive for STAT6, and RT-PCR revealed a NAB2-STAT6 fusion gene(exons 6 and 17, respectively), supporting a diagnosis of SFT/HPC WHO grade III. Despite postoperative radiotherapy, multiple metastases to the spleen were detected 8 months after surgery, and distal pancreatectomy with splenectomy was performed. The pathology of the splenic tumor was similar to that of the intracranial tumor. Recurrent disease in a lymph node was detected 1 month later, and local radiation therapy was administered. The patient died of cancerous peritonitis 5 months later. In this case, exceedingly rapid metastasis to the spleen occurred, despite the administration of vigorous treatment. Here, we review SFT/HPC incidence, treatment, and outcomes to better understand this rare malignancy.


Assuntos
Hemangiopericitoma , Tumores Fibrosos Solitários , Neoplasias Esplênicas/secundário , Adulto , Feminino , Hemangiopericitoma/secundário , Hemangiopericitoma/cirurgia , Humanos , Recidiva Local de Neoplasia , Proteínas Repressoras , Fator de Transcrição STAT6 , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/cirurgia
15.
World Neurosurg ; 126: e48-e56, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30716501

RESUMO

BACKGROUND: Intracranial solitary fibrous tumors (ISFTs) are rare mesenchymal neoplasms originating in the meninges and characterized by very different biologic and clinical behaviors. Benign histotypes, such as hemangiopericytomas, are now considered a cellular phenotypic variant of this heterogeneous group of rare spindle-cell tumors. Owing to their rarity and resemblance to other, more common brain tumors, ISFTs are often poorly recognized and remain a diagnostic challenge. METHODS: We describe a surgical series of 29 patients treated for ISFTs confirmed histologically and through immunohistochemistry. We attempt to provide a focus on the natural history of these pathologies and the need for tailored management. RESULTS: This was a retrospective consecutive series of 29 patients with either solitary fibrous tumor (n = 14) or hemangiopericytoma (n = 15) over a 10-year period. Mean follow-up time was 37.71 months. Recurrence rate was 42.9% for solitary fibrous tumors versus 26.7% for hemangiopericytomas. STAT6 expression was 66.7% in hemangiopericytomas versus 42.9% in SFTs. CONCLUSIONS: Histopathology and immunohistochemical staining (characterized by positive expression of mainly STAT6 but also CD34, Bcl-2 protein, and vimentin) are key in diagnosis and management of ISFTs. Although ISFTs are still considered benign lesions with very rare aggressive evolution, their clinical behavior is largely unpredictable. This study highlights the importance of relying on immunohistochemistry for a thorough definition of the management strategy.


Assuntos
Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/patologia , Tumores Fibrosos Solitários/diagnóstico , Tumores Fibrosos Solitários/patologia , Adulto , Neoplasias Encefálicas/cirurgia , Feminino , Seguimentos , Hemangiopericitoma/patologia , Hemangiopericitoma/cirurgia , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Procedimentos Neurocirúrgicos , Estudos Retrospectivos , Fator de Transcrição STAT6/biossíntese , Fator de Transcrição STAT6/genética , Tumores Fibrosos Solitários/cirurgia , Análise de Sobrevida , Resultado do Tratamento
16.
J Clin Neurosci ; 60: 142-147, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30352760

RESUMO

Hemangiopericytoma (HPC) is a rare tumor originating from pericapillary cells. Rarely found in the central nervous system, it is extremely rare in the spinal canal. Because of the low incidence of this tumor, its radiographic features and clinical manifestations have not been extensively studied and reported, therefore, it is often misdiagnosed as a schwannoma or spinal meningioma. We describe an unexpected HPC in a 35-year-old woman who was admitted to the Peking Union Medical College Hospital with a severe backache, sensory abnormalities, and muscle weakness. Magnetic resonance imaging showed an enhancing lesion at T6-7 with severe compression of the spinal cord. Gross total resection was achieved, and subsequently, a marked neurologic improvement was observed. The diagnosis of primary extradural HPC in our patient was confirmed based on postoperative histopathology and immunohistochemistry. Neither recurrence nor metastasis of the tumor was found during the 14-month follow-up, which did not include radiotherapy. To describe the demography, radiologic features, treatment, and prognosis of spinal HPC, a comprehensive literature review was performed and 105 cases of primary spinal HPC from 1958 to 2017 were collected from 39 articles. Although rare, HPC should be considered in the differential diagnosis of intraspinal lesions. Immunohistologic examination is of decisive importance in making the diagnosis. Adequate surgical resection, when feasible, is the first choice of treatment for all cases of HPC; however, the outcomes of radiotherapy and chemotherapy have yet to be determined. Individualized treatment combined with long-term follow-up for each patient is recommended.


Assuntos
Neoplasias Epidurais/diagnóstico , Hemangiopericitoma/diagnóstico , Adulto , Diagnóstico Diferencial , Neoplasias Epidurais/patologia , Neoplasias Epidurais/cirurgia , Feminino , Hemangiopericitoma/patologia , Hemangiopericitoma/cirurgia , Humanos
17.
World Neurosurg ; 123: 68-75, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30503286

RESUMO

BACKGROUND: Hemangiopericytomas (HPCs) are rare vascular tumors that resemble meningiomas on imaging and have a high rate of local recurrence and metastases. There remains a paucity of data to guide management decisions of intraspinal dissemination of HPCs in the literature, and none specifically related to anaplastic HPCs. CASE DESCRIPTION: We report a case of a 34-year-old woman with locally and distantly recurrent anaplastic HPC (World Health Organization grade III). She initially presented with tinnitus in her right ear. A well-circumscribed, contrast-enhancing lesion was identified in the right cerebellopontine angle. Treatment consisted of subtotal resection and postoperative radiation therapy (RT) to a dose of 60 Gy in 30 fractions. After a 3-year disease-free interval, 7 lesions recurred intra- and extracranially. The extracranial lesions were drop metastases of the original HPC through the cerebrospinal fluid into the spinal canal. Of note, fluorodeoxyglucose positron emission tomography (PET)/computed tomography scan was not sensitive enough to detect these new lesions. The intracranial recurrence was on the edge of the prior radiotherapy field, representing a marginal failure having received less than 50 Gy. The intracranial recurrences were treated with salvage gamma knife stereotactic radiosurgery (SRS) with local control. She underwent intradural extramedullary hemilaminectomy of a thoracic spine metastasis followed by fractionated proton beam therapy (PBT) with a boost to unresected lesions. Within a few months of PBT, she became pregnant. Pregnancy did not affect recurrence or ameliorate tumor growth. CONCLUSIONS: This case report discusses the role genetics, adjuvant RT, SRS, magnetic resonance imaging, and PET scan played in this unique clinical scenario of anaplastic HPC.


Assuntos
Neoplasias Encefálicas/patologia , Hemangiopericitoma/patologia , Neoplasias da Medula Espinal/secundário , Adulto , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/radioterapia , Neoplasias Encefálicas/cirurgia , Feminino , Hemangiopericitoma/diagnóstico por imagem , Hemangiopericitoma/radioterapia , Hemangiopericitoma/cirurgia , Humanos , Imagem por Ressonância Magnética , Recidiva Local de Neoplasia/diagnóstico por imagem , Recidiva Local de Neoplasia/radioterapia , Recidiva Local de Neoplasia/cirurgia , Procedimentos Neurocirúrgicos , Tomografia por Emissão de Pósitrons , Radiocirurgia , Neoplasias da Medula Espinal/diagnóstico por imagem , Resultado do Tratamento
18.
World Neurosurg ; 122: 459-463, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30447454

RESUMO

BACKGROUND: Hemangiopericytomas (HPCs) are rare and aggressive vascular mesenchymal tumors. Unlike meningiomas, which have a similar radiologic appearance, these tumors have a higher risk of local recurrence after resection, and distant metastasis can reach up to 23%. Metastases to the vertebral bones from an intracranial HPC are very rare, with so far only 9 cases reported in the literature. CASE DESCRIPTION: We present the case of a 46-year-old man who was surgically treated for a presumed left parieto-occipital falx meningioma in 2008. He presented 9 years later with a thoracic vertebral mass that was causing relentless pain. Reexamination of the cranial pathology allowed correction of the diagnosis performed in 2008 to a meningeal HPC, and the spinal lesion was confirmed after surgery to be a metastatic tumor. CONCLUSIONS: The literature lacks randomized controlled trials and large studies defining the natural history of HPC to draw clear recommendations for a precise management of the disease. However, en bloc resection followed by radiation therapy seems to provide the optimal treatment for a long disease-free survival.


Assuntos
Neoplasias Encefálicas/patologia , Hemangiopericitoma/cirurgia , Neoplasias Meníngeas/cirurgia , Neoplasias da Coluna Vertebral/secundário , Coluna Vertebral/cirurgia , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/cirurgia , Hemangiopericitoma/diagnóstico , Humanos , Masculino , Neoplasias Meníngeas/diagnóstico , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/diagnóstico , Recidiva Local de Neoplasia/patologia , Neoplasias da Coluna Vertebral/diagnóstico , Neoplasias da Coluna Vertebral/patologia , Coluna Vertebral/patologia , Vértebras Torácicas/patologia
19.
Ophthalmic Plast Reconstr Surg ; 35(2): 148-154, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30371551

RESUMO

PURPOSE: A risk assessment score for metastasis based on age, tumor size, and mitotic figures has been suggested for nonorbital solitary fibrous tumor (SFT)/hemangiopericytoma. The authors herein examine the clinicopathological features of recurrent and metastatic orbital SFT and evaluate the existing risk assessment score for orbital SFT. METHODS: The American Society of Ophthalmic Plastic and Reconstructive Surgery Oncology Database was queried for patients with recurrent or malignant orbital hemangiopericytoma/SFT. The medical records were reviewed for clinical and pathologic findings, treatments, and outcomes. RESULTS: Eight patients from 3 institutions were identified with recurrent orbital hemangiopericytoma/SFT. Median age at diagnosis was 59 years, and 4 patients were women. The mean size of tumor was 2.1 ± 1.1 cm. All patients were initially treated with surgery and experienced local recurrence after a median of 4 (range 0.5-10) years. Five patients were treated with orbital radiation. Two patients also developed distant metastases and eventually died of their disease. Median Ki-67 was 5% (range 1-65%) and 5 mitotic figures/10 high-power fields (range 2-30). The previously described risk stratification model for nonorbital SFT did not correlate with the propensity to develop metastases in this cohort; however, both patients with distant metastasis had > 4 mitotic figures /10 high-power fields. CONCLUSIONS: In this cohort of recurrent orbital hemangiopericytoma/SFT, median time to recurrence was 4 years underscoring the importance of careful continued follow-up. The current risk stratification models have limited use for orbital lesions, mostly due to the fact that orbital SFTs are smaller than even the smallest size criteria in this risk assessment model.


Assuntos
Hemangiopericitoma/patologia , Recidiva Local de Neoplasia/patologia , Neoplasias Orbitárias/patologia , Tumores Fibrosos Solitários/patologia , Adulto , Idoso , Diagnóstico Diferencial , Feminino , Seguimentos , Hemangiopericitoma/cirurgia , Humanos , Incidência , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/epidemiologia , Procedimentos Cirúrgicos Oftalmológicos , Neoplasias Orbitárias/cirurgia , Estudos Retrospectivos , Tumores Fibrosos Solitários/cirurgia , Fatores de Tempo , Estados Unidos/epidemiologia
20.
Br J Neurosurg ; 33(4): 439-441, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28830250

RESUMO

Hemangiopericytoma is a rare mesenchymal tumor originating from capillary pericytes, known as Zimmermann pericytes. The adult form is not uncommon and generally malignant but tumor is found rarely in children. Here we describe an intracranial hemangiopericytoma in a preterm newborn whose had the tumor resected successfully shortly after birth.


Assuntos
Neoplasias Encefálicas/cirurgia , Hemangiopericitoma/cirurgia , Neoplasias Encefálicas/diagnóstico por imagem , Craniotomia/métodos , Feminino , Hemangiopericitoma/diagnóstico por imagem , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Tomografia Computadorizada por Raios X , Resultado do Tratamento
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