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Composite hemangioendothelioma (CHE) is a rare locally aggressive vascular neoplasm of intermediate malignancy. We describe a 2-year-old patient diagnosed with this tumor. Careful documentation of patients with CHE is crucial to better define the prognosis and treatment of this entity.
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Introducción: El hemangioendotelioma retiforme es una neoplasia de grado intermedio o potencialmente maligna, su incidencia es entre la segunda y cuarta década de la vida, más frecuente 2:1 en mujeres, la etiología es incierta, se manifiesta generalmente como lesión nodular o en forma de placa en tronco o extremidades. El diagnóstico es por histopatología e inmunohistoquímica, su tratamiento es resección de la lesión, con una recurrencia del 60% posterior al manejo quirúrgico. Caso clínico: Se describe un caso atípico de hemangioendotelioma retiforme en tórax, en una paciente de sexo femenino de 43 años, su padecimiento inicia con aumento de volumen de 6 meses en axila derecha, acompañándose de dolor y limitación de la movilidad. Se realiza tomografía de tórax con reporte de tumoración del musculo pectoral de 83 mm. Se realiza exéresis de tumoración con reporte histopatológico de: hemangioendotelioma retiforme e inmunohistoquímica positiva a CD34.
Background: Retiform hemangioendothelioma is an intermediate grade or potentially malignant neoplasm, its incidence is between the second and fourth decade of life, more frequent 2:1 in women. Etiology is uncertain, it generally manifests as a nodular or plaque-shaped lesion on the trunk or extremities, the diagnosis is made by histopathology and immunohistochemistry, the treatment is resection of the lesion, presenting a recurrence of 60%. Clinical case: A case of retiform hemangioendothelioma is describes, a 43-year-old female began her condition with a 6-month increase in volume in the right armpit, with pain and limited mobility. A chest tomography was performed with a report of 83 mm pectoral muscle tumor, a tumor excision was performed with a histopathological report of: retiform hemangioendothelioma and CD34 positive immunohistochemistry.
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Hemangioendothelioma is a malignant vascular tumor, according to ISSVA classification of vascular tumors. This patient presented an epithelioid hemangioendothelioma; this type of tumor can exhibit significant local destruction, sometimes requiring limb amputation. With deferred Mohs micrographic surgery and reconstructive surgery with multiple conventional and microsurgical techniques, partial or total amputation of the hand was avoided.
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RESUMEN El hemangioendotelioma epiteloide hepático (HEH) es un tumor vascular, de etiología no aclarada, extraordinariamente infrecuente. La ausencia de características clínicas, analíticas y radiológicas especificas dificulta su correcto diagnóstico. El tratamiento del HEH depende del tamaño y localización tumoral, la extensión extrahepática y la condición médica del paciente. Entre las posibles opciones se encuentra el trasplante hepático, que obtiene unos buenos resultados clínicos, aunque el riesgo de recidiva no es despreciable. Presentamos un nuevo caso de HEH tratado mediante trasplante hepático.
ABSTRACT Hepatic epithelioid hemangioendothelioma (HEHE) is an extremely rare vascular tumor of unclear etiology. The diagnosis is difficult due to the absence of specific clinical characteristics, laboratory tests results and radiological findings. The management of HEHE depends on tumor size, location, extrahepatic extension, and patients' medical status. Liver transplantation is one of the possible options with good clinical results, although the risk of recurrence is not negligible. We present a new case of HEHE managed with liver transplantation.
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Introduction: Epithelioid hemangioendothelioma is a rare vascular tumor with an epithelioid and histiocytoid appearance. Intestinal intussusception can manifest as chronic abdominal pain, representing only 1-5% of intestinal obstructions in adults. Case report: 65-year-old female who is attended with chronic abdominal pain. We performed a computed tomography showing the incursion of the ileum into the right colon. She was taken to the operating table, with the finding of ileo-colic intestinal intussusception due to small bowel tumor, with subsequent anatomopathological results of epithelioid hemangioendothelioma. Conclusions: The diagnosis and management process with an appropriate postoperative outcome is described.
Introducción: El hemangioendotelioma epitelioide es un tumor vascular poco frecuente de aspecto epitelioide e histiocitoide. La intususcepción intestinal suele ser causa de dolor abdominal crónico y corresponde al 1-5% de las obstrucciones intestinales en el adulto. Caso clínico: Mujer de 65 años que acude con dolor abdominal crónico. Se realiza tomografía computarizada y se observa incursión del íleon en el colon derecho. Es llevada a mesa operatoria con hallazgo de intususcepción intestinal ileocólica por tumoración de intestino delgado, con resultado anatomopatológico de hemangioendotelioma epitelioide. Conclusiones: Se describe el proceso de diagnóstico y manejo, con apropiado desenlace posoperatorio.
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BACKGROUND: Kaposiform hemangioendothelioma (KHE) is a locally aggressive and potentially lethal vascular tumor of infancy. Current consensus recommendations include the use of vincristine and/or systemic steroids as first-line treatment. Mammalian target of rapamycin (mTOR) inhibitors represent a promising therapy for patients with KHE. The goal of our study is to critically assess the existing literature on outcomes of patients with KHE treated with mTOR inhibitors. METHODS: We conducted a literature search from 1 January 2000, to 30 April 2022. Articles reporting outcomes of patients treated with mTOR inhibitors for KHE were included. Descriptive statistics were used to describe and summarize the outcomes of the treatment. RESULTS: We included 327 patients with a mean age at diagnosis of 9.1 months (SD ± 9). Patients were treated with an mTOR inhibitor for a mean of 15.2 months (SD ± 4.1). A total of 315 (96.3%) patients had positive outcomes including improvement of the tumor size, symptoms and/or laboratory parameters in 227 (85%) and complete remission in 38 (12%). Seven (2%) patients did not respond to treatment and seven (2%) died of sepsis (4), Kasabach-Merritt phenomenon complications (1), cardiac and liver failure due to ductus arteriosus (1), or metastatic disease (1). CONCLUSION: This systematic review supports the efficacy and safety of mTOR inhibitors for KHE. Their use resulted in positive outcomes in terms of decreased symptoms, reduction in tumor size and improvement in biochemical parameters with a mortality rate of 2%. According to these findings, we suggest revised consensus treatment guidelines for KHE with mTOR inhibitors potentially considered first-line therapy.
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Hemangioendotelioma , Síndrome de Kasabach-Merritt , Sarcoma de Kaposi , Humanos , Lactente , Síndrome de Kasabach-Merritt/diagnóstico , Sirolimo/uso terapêutico , Inibidores de MTOR , Hemangioendotelioma/diagnóstico , Sarcoma de Kaposi/complicações , Serina-Treonina Quinases TOR/uso terapêuticoRESUMO
El hemangioendotelioma epiteloide hepático (HEHE) es un tumor vascular raro de menor malignidad que el hemangiosarcoma. En los poco frecuentes casos unilobulares, puede indicarse hepatectomía parcial con riesgo de recurrencia agresiva; en enfermedad hepática extensa, incluso con compromiso extrahepático, el trasplante hepático ha resultado efectivo. Las metástasis son más frecuentes en pulmón,peritoneo, ganglios linfáticos, bazo y sistema nervioso. Se presenta el caso de un adolescente asintomático con HEHE con metástasis pulmonares y compromiso ganglionar abdominal que recibió trasplante hepático con evolución favorable.
Hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular tumor of less malignancy than hemangiosarcoma. In the rare unilobar cases, partial hepatectomy may be indicated with risk of aggressive recurrence; in extensive liver disease, even with extrahepatic involvement, liver transplantation has been performed successfully. Metastases are more common in the lung, peritoneum, lymph nodes, spleen, and nervous system. We present the case of an asymptomatic adolescent with HEHE with lung metastases and abdominal lymph node involvement who received a liver transplant with a favorable outcome.
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Humanos , Masculino , Adolescente , Transplante de Fígado , Hemangioendotelioma Epitelioide/cirurgia , Hemangioendotelioma Epitelioide/diagnóstico , Hemangioendotelioma Epitelioide/patologia , Neoplasias Hepáticas/cirurgia , Neoplasias Hepáticas/patologia , Neoplasias Pulmonares/cirurgia , Resultado do TratamentoRESUMO
Hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular tumor of less malignancy than hemangiosarcoma. In the rare unilobar cases, partial hepatectomy may be indicated with risk of aggressive recurrence; in extensive liver disease, even with extrahepatic involvement, liver transplantation has been performed successfully. Metastases are more common in the lung, peritoneum, lymph nodes, spleen, and nervous system. We present the case of an asymptomatic adolescent with HEHE with lung metastases and abdominal lymph node involvement who received a liver transplant with a favorable outcome.
El hemangioendotelioma epiteloide hepático (HEHE) es un tumor vascular raro de menor malignidad que el hemangiosarcoma. En los poco frecuentes casos unilobulares, puede indicarse hepatectomía parcial con riesgo de recurrencia agresiva; en enfermedad hepática extensa, incluso con compromiso extrahepático, el trasplante hepático ha resultado efectivo. Las metástasis son más frecuentes en pulmón, peritoneo, ganglios linfáticos, bazo y sistema nervioso. Se presenta el caso de un adolescente asintomático con HEHE con metástasis pulmonares y compromiso ganglionar abdominal que recibió trasplante hepático con evolución favorable.
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Hemangioendotelioma Epitelioide , Neoplasias Hepáticas , Transplante de Fígado , Neoplasias Pulmonares , Adolescente , Hemangioendotelioma Epitelioide/diagnóstico , Hemangioendotelioma Epitelioide/patologia , Hemangioendotelioma Epitelioide/cirurgia , Humanos , Neoplasias Hepáticas/patologia , Neoplasias Hepáticas/cirurgia , Neoplasias Pulmonares/cirurgia , Resultado do TratamentoRESUMO
Pseudomyogenic hemangioendothelioma (PMH) can be a challenge for diagnosis and might be confused with other tumors, such as epithelioid sarcoma. Here we present a case and a systematic review of the literature to identify and discuss PMH treatment in primary bone involvement. A 25-year-old woman was referred for bone pain (10/10) in the left lower limb. Magnetic resonance imaging (MRI) showed multiple bone lesions (left femur, tibia, patella, ankle, and foot) with well-defined borders without signs of local aggressiveness. Positron Emission Tomography-Computed Tomography (PET-CT) showed multiple metabolic musculoskeletal lesions in the left lower limb. A CT scan-guided biopsy was performed. Histological and immunohistochemical findings confirmed the diagnosis of PMH. After treatment with intravenous pamidronate (90 mg/monthly), the patient had clinical improvement, mild pain 2/10 without the use of non-steroidal anti-inflammatory drugs or opiates. Follow-up was assessed by MRI and PET-CT. PET-CT showed metabolic resolution of most of the bone and muscular lesions and a significant improvement of the femoral lesion. MRI showed that the lesions in the left femur, tibia, and foot had a marked decrease in size without intravenous post-contrast enhancement and smaller lesions had disappeared. After a 3-year follow-up, PET-CT showed no metabolically active images. Literature review identified 31 records including 58 clinical cases of PMH with primary bone involvement and treatment description for qualitative analysis. Most lesions (69%) were treated by local excision or curettage. In addition, amputations were performed in a significant percentage of cases (20.7%). In the last years, mTOR inhibitors (n = 7) and anti-resorptive treatments (n = 4) were considered as alternative treatment options, especially in multifocal lesions.
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Hemangioendotelioma , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Adulto , Osso e Ossos/patologia , Feminino , Hemangioendotelioma/patologia , Hemangioendotelioma/cirurgia , Humanos , Imageamento por Ressonância Magnética , Pamidronato/uso terapêuticoRESUMO
Epitheloid hemangioendothelioma is a very rare tumor, with a variable presentation and unpredictable clinical behavior. The etiology and the triggering risk factors have not been specified. Unlike other primary liver tumors, it does not arise in the background of chronic liver disease. The approach is challenging due to the spectrum of possibilities and the need for immunohistochemistry to establish the definitive diagnosis. The information available so far is limited due to the few published cases, this favors that the therapeutic options are few or that there is insufficient evidence to standardize them when the lesion is not resectable.
El hemangioendotelioma epiteloide es un tumor muy infrecuente, con una presentación variable y un comportamiento clínico impredecible. No se han precisado la etiología ni los factores de riesgo desencadenantes. A diferencia de otros tumores hepáticos primarios, no surge en el contexto de una enfermedad crónica del hígado. El abordaje es un reto debido al espectro de posibilidades y la necesidad de inmunohistoquímica para establecer el diagnóstico definitivo. La información disponible hasta el momento es limitada por los escasos casos publicados, lo que favorece que las opciones terapéuticas sean pocas o no exista la evidencia suficiente para estandarizarlas cuando la lesión no sea resecable.
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Hemangioendotelioma Epitelioide , Neoplasias Hepáticas , Neoplasias Vasculares , Hemangioendotelioma Epitelioide/diagnóstico , Hemangioendotelioma Epitelioide/cirurgia , Humanos , Imuno-Histoquímica , Neoplasias Hepáticas/diagnósticoRESUMO
INTRODUCCIÓN: El hemangioendotelioma kaposiforme (HEK) es un tumor vascular poco frecuente caracterizado por una invasión local agresiva y un síndrome de atrapamiento de plaquetas conocido como fenómeno de Kasabach-Merritt. Aunque muchos casos de HEK se tratan con éxito con control local o quimioterapia de baja intensidad, otros son resistentes y se cuenta con pocas opciones terapéuticas. El objetivo de este reporte es mostrar la experiencia del tratamiento con sirolimus por vía oral en un paciente pediátrico con HEK asociado a fenómeno de Kasabach-Merritt refractario al tratamiento de primera línea, quien mostró excelente respuesta al tratamiento. CASO CLÍNICO: Paciente de sexo masculino de 3 meses con un HEK refractario al manejo de primera línea (corticoides, propranolol, vincristina), sin posibilidad de hacer control local, por lo que se decide terapia combinada con sirolimus, presentando control local y resolución de la coagulopatía desde la primera semana de iniciado el manejo y con resolución de la malformación vascular después de 12 meses de seguimiento. CONCLUSIONES: Aunque no existen pautas claras para el tratamiento del HEK refractario en la edad pediátrica, la evidencia actual demuestra que el sirolimus es un medicamento eficaz que puede ser considerado como opción terapéutica de primera línea en estos pacientes. BACKGROUND: Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor characterized by aggressive local invasion and a platelet entrapment syndrome known as the Kasabach-Merritt phenomenon. Although many cases of KHE are successfully treated with local control or low-intensity chemotherapy, some cases are often resistant, with few therapeutic options available. Here, we report a pediatric patient with KHE associated with Kasabach-Merritt phenomenon refractory to first-line treatment, who demonstrated excellent response to treatment. CASE REPORT: We present the case of a 3-month-old male patient with a KHE refractory to first-line treatment (vincristine, corticosteroids, propranolol), without possibility of local control treatment. Therefore, combined therapy with sirolimus was decided, presenting local control and resolution of the coagulopathy from the first week after starting the management and with resolution of vascular malformation after 12 months of follow-up. CONCLUSIONS: Although there are no clear guidelines for the treatment of refractory KHE in the pediatric population, current evidence demonstrate that sirolimus is an effective option that could be considered as a first-line treatment in such patients.
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Hemangioendotelioma , Síndrome de Kasabach-Merritt , Sarcoma de Kaposi , Criança , Hemangioendotelioma/diagnóstico , Hemangioendotelioma/tratamento farmacológico , Humanos , Lactente , Síndrome de Kasabach-Merritt/diagnóstico , Síndrome de Kasabach-Merritt/tratamento farmacológico , MasculinoRESUMO
Resumen Introducción: El hemangioendotelioma kaposiforme (HEK) es un tumor vascular poco frecuente caracterizado por una invasión local agresiva y un síndrome de atrapamiento de plaquetas conocido como fenómeno de Kasabach-Merritt. Aunque muchos casos de HEK se tratan con éxito con control local o quimioterapia de baja intensidad, otros son resistentes y se cuenta con pocas opciones terapéuticas. El objetivo de este reporte es mostrar la experiencia del tratamiento con sirolimus por vía oral en un paciente pediátrico con HEK asociado a fenómeno de Kasabach-Merritt refractario al tratamiento de primera línea, quien mostró excelente respuesta al tratamiento. Caso clínico: Paciente de sexo masculino de 3 meses con un HEK refractario al manejo de primera línea (corticoides, propranolol, vincristina), sin posibilidad de hacer control local, por lo que se decide terapia combinada con sirolimus, presentando control local y resolución de la coagulopatía desde la primera semana de iniciado el manejo y con resolución de la malformación vascular después de 12 meses de seguimiento. Conclusiones: Aunque no existen pautas claras para el tratamiento del HEK refractario en la edad pediátrica, la evidencia actual demuestra que el sirolimus es un medicamento eficaz que puede ser considerado como opción terapéutica de primera línea en estos pacientes.
Abstract Background: Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor characterized by aggressive local invasion and a platelet entrapment syndrome known as the Kasabach-Merritt phenomenon. Although many cases of KHE are successfully treated with local control or low-intensity chemotherapy, some cases are often resistant, with few therapeutic options available. Here, we report a pediatric patient with KHE associated with Kasabach-Merritt phenomenon refractory to first-line treatment, who demonstrated excellent response to treatment. Case report: We present the case of a 3-month-old male patient with a KHE refractory to first-line treatment (vincristine, corticosteroids, propranolol), without possibility of local control treatment. Therefore, combined therapy with sirolimus was decided, presenting local control and resolution of the coagulopathy from the first week after starting the management and with resolution of vascular malformation after 12 months of follow-up. Conclusions: Although there are no clear guidelines for the treatment of refractory KHE in the pediatric population, current evidence demonstrate that sirolimus is an effective option that could be considered as a first-line treatment in such patients.
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Hepatic epithelioid hemangioendothelioma (HEHE) is a vascular tumor with a low incidence rate. We report a case of a 26-year-old man who was referred to our hospital with a misdiagnosis of liver cirrhosis. On physical examination, ascites was noted. Chest and abdominal computer tomography scans showed coalescent lesions involving the peripheral liver with heterogeneous contrast enhancement and portal vein dilation due to portal hypertension. Extrahepatic metastasis was not observed. The biopsy with immunohistochemical stains suggested HEHE (Factor VIII, CD31, and CD34). This report describes an uncommon case of HEHE with non-cirrhotic portal hypertension.
El hemangioendotelioma epitelioide hepático (HEHE) es un tumor vascular con una tasa de incidencia baja. Presentamos el caso de un varón de 26 años que fue remitido a nuestro hospital con un diagnóstico erróneo de cirrosis hepática. En el examen físico se observó ascitis. La tomografía computarizada de tórax y abdomen mostró lesiones coalescentes que afectaban al hígado periférico con realce heterogéneo de contraste y dilatación de la vena porta por hipertensión portal. No se observó metástasis extrahepática. La biopsia con tinciones inmunohistoquímicas sugirió HEHE (Factor VIII, CD31 y CD34). Este informe describe un caso poco común de HEHE con hipertensión portal no cirrótica.
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Kaposiform hemangioendothelioma is a rare neoplasm with intermediate malignant behavior, mainly affecting infants and children. Involvement head and neck is uncommon, and there are only four cases reported in the oral cavity and oropharynx. Microscopically, it is characterized by a vascular proliferation permeated by spindle-to-ovoid cells resembling Kaposi sarcoma. Immunohistochemically, the tumor is positive for CD31, CD34 and negative for D2-40. Herein we present a rare case of intraoral Kaposiform hemangioendothelioma in a 10-year-old boy.
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Hemangioendotelioma/diagnóstico , Síndrome de Kasabach-Merritt/diagnóstico , Neoplasias Bucais/diagnóstico , Sarcoma de Kaposi/diagnóstico , Biomarcadores Tumorais/análise , Criança , Diagnóstico Diferencial , Hemangioendotelioma/patologia , Hemangioendotelioma/cirurgia , Humanos , Síndrome de Kasabach-Merritt/patologia , Síndrome de Kasabach-Merritt/cirurgia , Masculino , Neoplasias Bucais/patologia , Neoplasias Bucais/cirurgia , Radiografia Panorâmica , Sarcoma de Kaposi/patologia , Sarcoma de Kaposi/cirurgiaRESUMO
Introdução: O hemangioendotelioma cutâneo kaposiforme (HEK) é um tumor vascular raro localmente agressivo, visto principalmente em recém-nascidos e crianças. Tem prevalência de 0,91 casos por 100.000 crianças, mais comum nas extremidades. O tratamento de escolha é a ressecção total, todavia muitas vezes não é possível devido à extensão da lesão e associação ao fenômeno de Kasabach-Merritt. Objetivos: Descrever a evolução de caso raro de tumor na região plantar de criança, correlacionando os achados radiológicos, cirúrgicos e histopatológicos. Métodos: Os autores relatam o caso de menino admitido aos cinco anos de idade com lesão cutânea plantar dolorosa recidivada. No exame de ressonância magnética (RMN) apresentava lesão na região plantar posterior medindo 3cmx2cm, superficial à fáscia plantar, no exame de biópsia revelou hemangioendotelioma kaposiforme, sem associação com o fenômeno de Kasabach- Merritt. Foi submetido a procedimento cirúrgico para exérese, apresentou recidiva após seis meses. Foi realizada nova ressecção ampla, reconstrução com retalho plantar e enxerto de pele parcial, obtendo-se margens livres, sem recidiva no seguimento de 15 anos. Conclusão: Os achados clínicos sugeriam fibromatose plantar, a RMN auxiliou na delimitação do tumor, o exame histopatológico com imunohistoquímica confirmaram o diagnóstico de hemangioendotelioma cutâneo kaposiforme. Realizou-se ressecção até a fáscia com recidiva, sendo necessária reabordagem e ressecção até o periósteo com a reconstrução com retalho plantar e enxerto de pele, sem recidiva no seguimento de 15 anos. Os autores chamam a atenção para a ressecção ampla de margens profundas e laterais para controle do crescimento tumoral.
Introduction: Kaposiform cutaneous hemangioendothelioma (HEK) is a rare locally aggressive vascular tumor, seen mainly in newborns and children. It has a prevalence of 0.91 cases per 100,000 children, being most common in the extremities. The treatment of choice is total resection; however, it is often not possible due to the lesion's extent and association with the Kasabach-Merritt phenomenon. Objectives: To describe the evolution of a rare tumor in the plantar region of a child, correlating the radiological, surgical, and histopathological findings. Methods: The authors report the case of a boy admitted at the age of five with a recurrent painful plantar skin lesion. In the magnetic resonance examination (NMR), he presented a lesion in the posterior plantar region measuring 3cmx2cm, superficial to the plantar fascia. In the biopsy examination, he revealed kaposiform hemangioendothelioma without association with the Kasabach-Merritt phenomenon. He underwent a surgical procedure for excision and presented recurrence after six months. A new broad resection, reconstruction with a plantar flap, and partial skin graft were performed, obtaining free margins, with no recurrence in the 15-year follow-up. Conclusion: Clinical findings suggested plantar fibromatosis, NMR helped in delimiting the tumor, and histopathological examination with immunohistochemistry confirmed the diagnosis of kaposiform cutaneous hemangioendothelioma. Resection was performed up to the fascia with recurrence, requiring re-approach and resection to the periosteum with reconstruction using a plantar flap and skin graft, without recurrence after 15 years. The authors call attention to the wide resection of deep and lateral margins to control tumor growth.
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ABSTRACT The osteoblastoma-like tumor is a rare condition with limited information about its treatment in the current medical literature. The tumor histologically resembles osteoblastoma, although the imaging features are similar to those seen in primary vascular lesions. Due to the uncertainty in the biological behavior of this tumor and because it is an unusual diagnosis, treatment can be aggressive, such as amputation, en bloc resection, and/or chemotherapy. This work reports a rare case of a patient with multicentric osteoblastoma-like in the craniofacial region, treated aggressively with total resection of the lesions.
RESUMEN El tipo osteoblastoma es una afección poco común y la literatura médica actual tiene información limitada sobre su tratamiento. Es histológicamente similar al osteoblastoma, aunque las características de las imágenes son similares a las que se observan en las lesiones vasculares primarias. Por la incertidumbre de su comportamiento biológico y por tratarse de un diagnóstico poco habitual, el tratamiento puede ser agresivo, con amputación, resección en bloque y/o quimioterapia. Este trabajo reporta un caso raro de osteoblastoma multicéntrico en la región craneofacial, tratado de manera agresiva con resección total de las lesiones.
RESUMO O osteoblastoma-like é uma condição rara, e a literatura médica atual tem informações limitadas sobre seu tratamento. Ele se assemelha histologicamente ao osteoblastoma, embora as características imaginológicas sejam semelhantes às observadas nas lesões vasculares primárias. Devido à incerteza do seu comportamento biológico e por se tratar de um diagnóstico incomum, o tratamento pode ser agressivo, com amputação, ressecção em bloco e/ou quimioterapia. Este trabalho relata um caso raro de osteoblastoma-like multicêntrico em região craniofacial, tratado de forma agressiva com ressecção total das lesões.
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INTRODUCTION AND OBJECTIVES: Infantile hepatic hemangioendothelioma (IHHE) is a benign liver tumor, associated with hypothyroidism and vascular malformations along the skin, brain, digestive tract and other organs. Here, we determined a single-center patient cohort by evaluating the effectiveness and safety of propranolol and sirolimus for the treatment of IHHE. PATIENTS AND METHODS: We performed a monocentric and observational study, based on clinical data obtained from 20 cases of IHHE treated with oral propranolol and sirolimus at the Shanghai Children's Medical Center (SCMC), between December 2017 and April 2019. All cases were confirmed by abdominal enhanced CT examination (18/20, 90%) and sustained decrease of alpha fetoprotein (AFP) (2/20, 10%). Propranolol treatment was standardized as once a day at 1.0mg/kg for patients younger than 2 months, and twice a day at 1.0mg/kg (per dose) for patients older than 2 months. Sirolimus was used to treat refractory IHHE patients after 6 months of propranolol treatment, and initial dosing was at 0.8mg/m2 body surface per dose, administered every 12h. Upon treatment, abdominal ultrasound scanning was regularly performed to evaluate any therapeutic effects. All children were followed up for 6-22 months (mean value of 12.75 months). The clinical manifestations and therapeutic effects, including complications during drug management, were reviewed after periodic follow-up. RESULTS: The effective rate of propranolol for the treatment of children with IHHE was 85% (17/20). In most cases, the AFP levels gradually decreased into the normal range. A complete response (CR) was achieved in 3 cases, partial response (PR) for 14 cases, progressive disease (PD) for 2 cases and stable disease (SD) was only detected once. Lesions decreased in two PD patients after administration of oral sirolimus. No serious adverse reactions were observed. CONCLUSION: This study indicates that both propranolol and sirolimus were effective drugs for the treatment of children with IHHE at SCMC.
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Antineoplásicos/administração & dosagem , Hemangioendotelioma/tratamento farmacológico , Neoplasias Hepáticas/tratamento farmacológico , Propranolol/administração & dosagem , Sirolimo/administração & dosagem , Administração Oral , Antineoplásicos/efeitos adversos , Pré-Escolar , China , Feminino , Hemangioendotelioma/sangue , Hemangioendotelioma/diagnóstico por imagem , Hemangioendotelioma/patologia , Humanos , Lactente , Recém-Nascido , Neoplasias Hepáticas/sangue , Neoplasias Hepáticas/diagnóstico por imagem , Neoplasias Hepáticas/patologia , Masculino , Propranolol/efeitos adversos , Sirolimo/efeitos adversos , Fatores de Tempo , Resultado do Tratamento , alfa-Fetoproteínas/metabolismoRESUMO
El hemangioendotelioma hepático del lactante es una patología rara y más aún que su forma de presentación tenga complicaciones graves y el paciente fallezca. Se presenta el caso clínico de una lactante menor de 3 meses de edad que ingresó al Servicio Desconcentrado Hospital Pediátrico Dr. Agustín Zubillaga con diagnóstico de intoxicación herbácea (hierbabuena) e íleo metabólico. La hepatomegalia fue el signo más llamativo en la exploración clínica asociado a valores elevados de alfafetoproteína sérica. Los hallazgos ecosonográficos y tomográficos reportaron múltiples lesiones redondeadas que impresionaron metástasis hepáticas. La paciente fallece a los 23 días de iniciada la enfermedad actual siendo el diagnóstico postmortem hemangioendotelioma cavernoso gigante hepático, Se concluye que en general el diagnóstico de esta enfermedad se puede plantear sin una confirmación histológica, particularmente en lactantes menores, cuando los hallazgos clínicos, analíticos e imagenológicos son muy sugestivos aun cuando hay excepciones como lo presentado en este caso clínico(AU)
Infantile hemangioendothelioma is a rare, usually benign, disease. We present a case of a 3-month-old infant who was admitted in the Servicio Desconcentrado Hospital Pediátrico Dr. Agustín Zubillaga with the diagnosis of herbaceous poisoning (peppermint) and metabolic ileus. Hepatomegaly was the most characteristic sign in clinical examination and was associated with elevated levels of alpha-fetoprotein. Echographic and tomographic findings revealed multiple rounded lesions that impressed liver metastases. The patient died 23 days after admission and the postmortem diagnosis showed giant hepatic cavernous hemangioendothelioma. Diagnosis of hemangioendothelioma usually can be done without histopathological confirmation, especially in infants, when clinical, laboratory and imaging features are very suggestive of this disease, however there are exceptions as the one presented in this case(AU)
Assuntos
Humanos , Feminino , Lactente , alfa-Fetoproteínas/análise , Hemangioendotelioma Epitelioide/patologia , Hepatomegalia/etiologia , Neoplasias Hepáticas , Pediatria , Tomografia , Ultrassonografia , Metástase NeoplásicaRESUMO
Epithelioid hemangioendothelioma (EHE) is a malignant vascular tumor that usually affects the liver, lung, bone and deep soft tissues of the extremities or trunk. To our knowledge, only 3 cases in the parotid gland have been reported to date. We report a case of a 62-year-old woman who presented with a 1-year history of a slow-growing, painless mass over the left mandibular angle. Imaging studies showed a 2cm mass over the left parotid gland with peripheral calcifications. The patient underwent a superficial parotidectomy. Sections displayed neoplastic epithelioid cells with cytoplasmic vacuoles containing erythrocytes, surrounded by a myxohyaline stroma. Immunohistochemistry was positive for CD31, CD34, ERG, and factor VIII, but negative for cytokeratin AE1/AE3, CK7, EMA, SMA, and S100. The findings were those of an EHE involving the parotid gland. This case demonstrates an EHE in a rare location and emphasizes the need to consider this tumor when diagnosing uncommon soft tissue tumors of salivary glands.
Assuntos
Hemangioendotelioma Epitelioide/diagnóstico , Neoplasias Parotídeas/diagnóstico , Biomarcadores Tumorais/análise , Diagnóstico Diferencial , Feminino , Hemangioendotelioma Epitelioide/química , Hemangioendotelioma Epitelioide/patologia , Hemangioendotelioma Epitelioide/cirurgia , Hemangiossarcoma/diagnóstico , Humanos , Melanoma/diagnóstico , Pessoa de Meia-Idade , Proteínas de Neoplasias/análise , Neoplasias Parotídeas/química , Neoplasias Parotídeas/patologia , Neoplasias Parotídeas/cirurgia , Neoplasias de Tecidos Moles/diagnóstico , Tomografia Computadorizada por Raios XRESUMO
Se presenta el caso de una niña de 4 meses de edad que ingresa a la Unidad de Cuidados Intensivos (UCI) por presentar el Síndrome de Kasabach-Merritt asociado a un hemangioendotelioma kaposiforme. Luego de un mes de recibir tratamiento con vincristina, corticoides, propanolol y ácido acetilsalicílico, continúa con evolución desfavorable, incrementándose el tamaño de la lesión. Los exámenes de imágenes (tomografia axial computarizada y resonancia magnética nuclear) muestran compromiso de músculos intercostales, pleura, parénquima pulmonar y trombosis de venas cervicales. Posteriormente, llega a presentar cianosis y dificultad respiratoria por obstrucción de vías aéreas, requiriendo intubación y ventilación mecánica. Se inicia sirolimus, y en la segunda semana de tratamiento se evidencia mejoría de la lesión y de la analítica. Se brinda el alta hospitalaria a los 42 días de terapia con sirolimus, tratamiento que continúa recibiendo hasta un año después.
A 4-month girl was admitted to Pediatric Intensive Care Unit (PICU) due to a Kasabach-Merritt Syndrome associated to Kaposiform hemangioendothelioma. After 1-month of treatment with vincristine, steroids, propranolol, and acetylsalicylic acid; her progress was not good, and lesions became larger. Imaging studies (computed tomography and nuclear magnetic resonance) showed involvement of intercostal muscles, pleura, lung parenchyma, and cervical venous thrombosis. Afterwards, the patient developed cyanosis and dyspnea due to an upper airway obstruction, requiring intubation and ventilation support. Sirolimus was started and, on the second week of treatment, both lesion and laboratory parameters improved. The patient was discharged after 42 days of sirolimus therapy. One year later, she is still receiving this drug.