RESUMO
Epithelioid haemangioendothelioma (EH) is a rare malignant vascular tumour occurring mainly in the liver and lungs, with bones being a rare site and primarily seen in the adult population. This case presents a male patient in his 40s who presented to the outpatient department with a chief issue of a painless swelling over the inguinal region for 4 months, gradually increasing in size, along with a history of a gradually enlarging, painless mass on his left knee over the past 5 years. Despite occasional discomfort during physical activities, the mass exhibited no associated trauma, fever, weight loss or systemic symptoms. Physical examination revealed a firm mass on the left knee and a matted lymph nodal swelling in the left inguinal region. Subsequent imaging studies identified multiple soft tissue lesions, osseous involvement and pulmonary metastases, suggestive of multicentric haemangioendothelioma. The patient underwent surgical excision of the inguinal mass and fixation of a pathological fracture in the left femur. He is currently undergoing chemotherapy and is scheduled for regular follow-up appointments. This case underscores the importance of thorough diagnostic evaluation and multidisciplinary management in complex oncological conditions like multicentric haemangioendothelioma.
Assuntos
Hemangioendotelioma Epitelioide , Humanos , Masculino , Hemangioendotelioma Epitelioide/diagnóstico , Hemangioendotelioma Epitelioide/cirurgia , Hemangioendotelioma Epitelioide/diagnóstico por imagem , Adulto , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/diagnóstico por imagem , Neoplasias de Tecidos Moles/terapia , Diagnóstico Diferencial , Neoplasias Pulmonares/diagnóstico , Fraturas Espontâneas/diagnóstico por imagem , Fraturas Espontâneas/etiologia , Fraturas Espontâneas/cirurgia , Neoplasias Ósseas/diagnósticoRESUMO
PURPOSE: Hepatic epithelioid hemangioendothelioma (HEHE) is a rare tumor with currently no established standard of care. This international multicenter retrospective study assesses the use of percutaneous irreversible electroporation (IRE) as an ablative tool to treat HEHE and provides a clinical overview of the current management and role of IRE in HEHE treatment. MATERIAL AND METHODS: Between 2017 and 2023, 14 patients with 47 HEHE tumors were treated with percutaneous IRE using CT-scan guidance in 23 procedures. Baseline patient and tumor characteristics were evaluated. Primary outcome measures included safety and effectiveness, analyzed using Common Terminology Criteria for Adverse Events (CTCAE) and treatment response by mRECIST criteria. Secondary outcome measures included technical success, post-treatment tumor sizes and length of hospital stay. Technical success was defined as complete ablation with an adequate ablative margin (intentional tumor free ablation margin > 5 mm). RESULTS: IRE treatment resulted in technical success in all tumors. Following a median follow-up of 15 months, 30 tumors demonstrated a complete response according to mRECIST criteria. The average tumor size pre-treatment was 25.8 mm, accompanied by an average reduction in tumor size by 7.5 mm. In 38 out of 47 tumors, there was no evidence of local recurrence. In nine tumors, residual tumor was present. There were no cases of progressive disease. Median length of hospital stay was one day. Only one grade 3 CTCAE event occurred, a pneumothorax requiring chest tube placement. CONCLUSION: The current study provides evidence that IRE is a safe and efficacious minimally invasive treatment option for HEHE.
Assuntos
Eletroporação , Hemangioendotelioma Epitelioide , Neoplasias Hepáticas , Tomografia Computadorizada por Raios X , Humanos , Masculino , Feminino , Neoplasias Hepáticas/terapia , Neoplasias Hepáticas/diagnóstico por imagem , Neoplasias Hepáticas/cirurgia , Neoplasias Hepáticas/patologia , Pessoa de Meia-Idade , Estudos Retrospectivos , Hemangioendotelioma Epitelioide/diagnóstico por imagem , Hemangioendotelioma Epitelioide/cirurgia , Hemangioendotelioma Epitelioide/terapia , Adulto , Eletroporação/métodos , Idoso , Resultado do Tratamento , Adulto JovemRESUMO
BACKGROUND: Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor predominantly arising in soft tissue. We report a rare case of thoracic spinal EHE with pulmonary metastasis. METHODS: Case report and systematic review of spinal EHE. RESULTS: A 36-year-old man presented with bilateral lower extremity weakness, progressive paresthesia, and urinary incontinence. He underwent open surgical resection of the tumor and decompression of the spinal cord, with subsequent improvement in neurologic function. Systematic review identified 84 cases of spinal EHE, 73 of which were primary, and 14 of which developed extra-spinal metastases. CONCLUSION: EHE is an exceedingly rare tumor that may present with a wide swath of clinical symptoms. At present, no guidelines or formal treatment recommendations have been established. Surgical debulking has demonstrated efficacy as a front-line treatment, particularly in the setting of compressive neurologic dysfunction; data regarding adjuvant chemoradiation are less consistently reported, mandating further study.
Assuntos
Hemangioendotelioma Epitelioide , Procedimentos Neurocirúrgicos , Adulto , Humanos , Masculino , Descompressão Cirúrgica/métodos , Hemangioendotelioma Epitelioide/cirurgia , Hemangioendotelioma Epitelioide/diagnóstico por imagem , Neoplasias Pulmonares/cirurgia , Neoplasias Pulmonares/patologia , Procedimentos Neurocirúrgicos/métodos , Neoplasias da Medula Espinal/cirurgia , Neoplasias da Medula Espinal/diagnóstico por imagem , Neoplasias da Coluna Vertebral/cirurgia , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Vértebras Torácicas/cirurgiaAssuntos
Neoplasias Ósseas , Hemangioma , Humanos , Hemangioma/patologia , Hemangioma/cirurgia , Neoplasias Ósseas/patologia , Neoplasias Ósseas/cirurgia , Feminino , Masculino , Hemangioendotelioma Epitelioide/patologia , Hemangioendotelioma Epitelioide/cirurgia , Hemangioendotelioma Epitelioide/diagnósticoRESUMO
BACKGROUND: Epithelioid hemangioendothelioma (EHE) is a rare malignancy of vascular origin which can be primarily be seen in various tissues. EHE originating from the pleura is an even more uncommon subtype which may mimic mesothelioma and pleural carcinomatosis. The prognosis of pleural EHE is poor and there is no consensus on the optimal therapeutic approach. CASE PRESENTATION: A 39-year-old middle-eastern female presented with progressive dyspnea and left shoulder discomfort. Chest computed tomography scan revealed a left side pleural effusion and pleural thickening. Pleuroscopy was done and biopsies were taken which were positive for CD31, CD34, CK, factor 8-R-antigen, and vimentin. Patient was diagnosed with pleural epithelioid hemangioendothelioma (PEHE) and chemotherapy was started and underwent extrapleural pneumonectomy 7 months later. Unfortunately, the patient passed away 10 months after diagnosis due to disease complications. CONCLUSIONS: Once PEHE is suspected in histology it can be confirmed with immunohistochemistry. Chemotherapy, surgery or a combination of both is currently used as the treatment but the standard treatment remains a question.
Assuntos
Hemangioendotelioma Epitelioide , Derrame Pleural , Neoplasias Pleurais , Humanos , Feminino , Adulto , Hemangioendotelioma Epitelioide/diagnóstico , Hemangioendotelioma Epitelioide/patologia , Hemangioendotelioma Epitelioide/cirurgia , Neoplasias Pleurais/cirurgia , Pleura/patologia , Derrame Pleural/patologia , PrognósticoRESUMO
BACKGROUND: Pulmonary epithelioid hemangioendothelioma is a rare malignant disease, and most cases are found as multiple lung nodules, rarely as a single nodule. CASE: Computed tomography( CT) in a 71-year-old man revealed a growing 3-mm lung nodule in the left S6 after rectal cancer operation. Wedge resection was performed. A pathological examination resulted in a diagnosis of pulmonary epithelioid hemangioendothelioma based on CD31 and CD34 positivity in immunohistochemistry. CONCLUSION: When new nodules are noted on routine CT scans of other malignancies, it is essencial to make a pathological diagnosis, bearing in mind that pulmonary nodules can arise from a variety of causes.
Assuntos
Hemangioendotelioma Epitelioide , Neoplasias Pulmonares , Nódulos Pulmonares Múltiplos , Neoplasias de Tecido Conjuntivo , Neoplasias Cutâneas , Masculino , Humanos , Idoso , Neoplasias Pulmonares/diagnóstico por imagem , Neoplasias Pulmonares/cirurgia , Hemangioendotelioma Epitelioide/diagnóstico por imagem , Hemangioendotelioma Epitelioide/cirurgia , Pulmão/patologia , Nódulos Pulmonares Múltiplos/cirurgia , Tomografia Computadorizada por Raios X , Neoplasias de Tecido Conjuntivo/patologia , Neoplasias Cutâneas/patologiaRESUMO
PURPOSE: To investigate the feasibility, safety, and clinical outcomes of image-guided thermal ablation in patients with hepatic epithelioid hemangioendothelioma (HEHE). MATERIALS AND METHODS: This was a multicenter retrospective investigation of 18 patients (43.9 years [SD ± 14.8]; 6 men) who underwent image-guided thermal ablation for HEHE between January 2013 and February 2023. A total of 31 ablation sessions (24 involving microwave ablation and 7 involving radiofrequency ablation) were evaluated. The rates of technical success, adverse events, and outcomes were assessed. The KaplanâMeier method was used to estimate progression-free survival (PFS) and overall survival (OS) rates. The risk factors affecting PFS were investigated using Cox proportional hazard regression analysis. RESULTS: The technical success rate was 93.5% (29/31). No major adverse events occurred. Local tumor progression occurred after 2 sessions (6.5%, 2/31), and intrahepatic distant metastasis occurred after 16 sessions (51.6%, 16/31). During the medium follow-up time of 37.2 months (range, 3-117 months), the OS and PFS rates were 87.6% and 62.2%, respectively, at 1 year; 75.5% and 37.4%, respectively, at 3 years; and 75.5% and 37.4%, respectively, at 5 years. The median OS and PFS were 90.5 months (95% CI: 68.1-112.8) and 23.8 months (95% CI: 15.4-32.2), respectively. According to the multivariate analysis, a larger tumor size (P = .026) was associated with shorter PFS. CONCLUSIONS: Image-guided thermal ablation is a feasible and safe treatment option for patients with HEHE that resulted in local tumor control and a favorable long-term prognosis.
Assuntos
Estudos de Viabilidade , Hemangioendotelioma Epitelioide , Neoplasias Hepáticas , Intervalo Livre de Progressão , Humanos , Masculino , Neoplasias Hepáticas/cirurgia , Neoplasias Hepáticas/patologia , Neoplasias Hepáticas/mortalidade , Neoplasias Hepáticas/diagnóstico por imagem , Feminino , Pessoa de Meia-Idade , Hemangioendotelioma Epitelioide/cirurgia , Hemangioendotelioma Epitelioide/patologia , Hemangioendotelioma Epitelioide/mortalidade , Hemangioendotelioma Epitelioide/diagnóstico por imagem , Estudos Retrospectivos , Adulto , Fatores de Risco , Fatores de Tempo , Ablação por Radiofrequência/efeitos adversos , Ablação por Radiofrequência/mortalidade , Micro-Ondas/uso terapêutico , Micro-Ondas/efeitos adversos , Progressão da Doença , Adulto Jovem , Idoso , China , Resultado do TratamentoRESUMO
PURPOSE: Disease control and survival following percutaneous ablation of hepatic epithelioid hemangioendothelioma (EHE) was studied retrospectively. METHODS: Six patients underwent 16 image-guided ablation procedures to treat 35 liver tumors from 2015 to 2022 (17 microwave ablation, 9 irreversible electroporation, 8 cryoablation, and 1 radiofrequency ablation). Technical success, local progression, intrahepatic progression, distant progression, overall survival, and adverse events were assessed. RESULTS: Four of six (67%) patients were treatment naïve prior to ablation. The mean length of imaging follow-up from first ablation procedure was 43.0 ± 31.2 months. Thirty-three of 35 (94.3%) ablated tumors did not progress locally. Three of 6 patients (50%) had new intrahepatic progression and underwent repeat ablation or systemic treatment. No extrahepatic progression was observed. One patient died from EHE 2.7 years after initial diagnosis. No severe adverse events occurred. CONCLUSION: Percutaneous ablation is feasible, often in a staged fashion, and may provide favorable intermediate to long-term disease control for patients with hepatic EHE.
Assuntos
Ablação por Cateter , Criocirurgia , Hemangioendotelioma Epitelioide , Neoplasias Hepáticas , Humanos , Hemangioendotelioma Epitelioide/diagnóstico por imagem , Hemangioendotelioma Epitelioide/cirurgia , Estudos Retrospectivos , Neoplasias Hepáticas/diagnóstico por imagem , Neoplasias Hepáticas/cirurgia , Neoplasias Hepáticas/patologia , Criocirurgia/métodos , Ablação por Cateter/métodos , Resultado do TratamentoRESUMO
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.
Assuntos
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
Os autores apresentam um caso raro de hemangioendotelioma epitelióide hepático multicêntrico de uma paciente oligossintomática, tratada inicialmente com ressecção e alcoolização. Na sua recidiva, foi submetida à radioablação. Revisando a literatura, os autores concluem que a ressecção permanece como o principal tratamento, associada ou não a métodos ablativos, e que o transplante tem indicação na doença multinodular bilateral.