Hemangioendotelioma Retiforme en Tórax. Reporte de un caso

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.

Trasplante hepático por hemangioendotelioma hepático difuso / Liver transplantation for diffuse epithelioid hemangioendothelioma

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.

Epitheloid hemangioendothelioma of the urinary bladder: A case report of a rare entity

Epithelioid hemangioendothelioma (EHE) is a rare, often multifocal, intermediate-grade vascular sarcoma arising from the endothelial cells. Their behavior is intermediate between hemangioma and conventional angiosarcoma. Their rarity and unpredictable clinical behavior result in our still limited understanding and treatment options for this variety of tumors. Molecular diagnostic tools like the presence of WWTR1-CAMTA1 fusion have been developed to aid this challenging diagnosis. There is no definitive consensus on the management of patients with this disease and no commonly accepted treatment strategies have been found to be effective, particularly in the metastatic setting. When possible, surgery with clear margins should be considered in case of localized disease. Here, we not only present such a rare case of EHE of the urinary bladder but also provide a comprehensive discussion on the present treatment modalities offered, as found through a thorough search of the published studies. Finally, more studies are required to establish a standard of care for this rare entity

Progressive kaposiform hemangioendothelioma and sirolimus-related severe thrombocytopenia

Kaposiform hemangioendothelioma is a locally invasive tumor and we were unable to find any previous reports of multifocal progression. Sirolimus, a mammalian target of rapamycin inhibitor, has been widely used to treat kaposiform hemangioendothelioma. Herein, we report a case of multifocal progressive kaposiform hemangioendothelioma, wherein sirolimus treatment caused severe thrombocytopenia. A 12-year-old East Asian girl presented with indurated dark-purple masses on her back. The patient had received three surgical interventions following the first appearance of the masses in 2012 and subsequent reappearances in 2014 and 2016. Kaposiform hemangioendothelioma was diagnosed based on radiological and pathological findings. Two more masses appeared in the following year. The patient was treated with oral sirolimus (2.5 mg/ m2/day) and developed grade 3 thrombocytopenia 8 days later. The patient was uneventfully relieved 5 days later after the withdrawal of sirolimus and the administration of appropriate medications. This rare case indicated that kaposiform hemangioendothelioma could be progressive with local metastatic characteristics in children. Besides, the severe sirolimus-induced complication highlights the importance of serum drug level monitoring during treatment. Physicians should be extremely cautious while treating kaposiform hemangioendothelioma patients with sirolimus.

Diagnostic value of contrast-enhanced ultrasound in hepatic epithelioid hemangioendothelioma / 中华肝脏病杂志

Objective: To investigate the features of contrast-enhanced ultrasound (CEUS) in hepatic epithelioid hemangioendothelioma (HEHE) in order to improve the preoperative diagnosis rate. Methods: CEUS images of 32 pathologically-proven cases of hepatic epithelioid hemangioendothelioma from January 2004 to August 2021 were collected. Lesions were analyzed to observe the features of enhancement mode, enhancement intensity, and distinct enhancement phases. Results: Among the 32 cases, one had a solitary lesion, 29 had multiple lesions, and two had diffuse-type lesions. Contrast-enhanced ultrasound revealed a total of 42 lesions in 32 cases. In terms of arterial phase enhancement, 18 lesions had overall enhancement, six lesions had uneven dendritic enhancement, 16 lesions had rim-like enhancement, and two lesions had just slight peripheral spot enhancement around the lesions. Among the three cases, there were multiple lesions that had overall enhancement and ring enhancement. In terms of the enhancement phase, 20 lesions showed "fast progression", 20 lesions showed "same progression", and two lesions showed "slow progression". During the late arterial or early portal venous phases with rapid washout, all lesions manifested as hypoechoic. With peaked enhanced intensity, 11 lesions had a lower enhancement intensity than the surrounding normal liver parenchyma; 11 lesions had the same enhancement degree as the surrounding normal liver parenchyma; and 20 lesions had a higher enhancement degree than the surrounding normal liver parenchyma. All 16 ring-enhancing lesions had marked hyperenhancement. In the typical enhancing lesions, four showed hyperenhancement, five showed low enhancement, and nine showed isoenhancement. In the dendrite-enhancing lesions, there were two isoenhancing and four hypoenhancing. Contrast-enhanced ultrasound delineated the boundaries of all lesions more clearly than two-dimensional ultrasound. Conclusion: Contrast-enhanced ultrasound has certain value in the diagnosis of hepatic epithelioid hemangioendothelioma.

Trasplante hepático en hemangioendotelioma epiteloide hepático con metástasis pulmonares: reporte de un caso / Liver transplantation in hepatic epitheloid hemangioendothelioma and lung metastases: a case report

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.

Hepatic epithelioid hemangioendothelioma—report of three cases of a puzzling tumor

Hepatic epithelioid hemangioendothelioma (EHE) is a rare malignant vascular neoplasm with unpredictable clinical behavior. These lesions are frequently misdiagnosed owing to its non-specific symptomatology, ambiguous radiological features, and overlapping histomorphology. We report three cases of hepatic EHE, of which one was male and two were female patients. While all three patients presented with abdominal pain, the male patient gave an additional history of weight loss and was jaundiced. The radioimaging showed multiple nodules in the liver and two of the patients also had pulmonary metastasis. The biopsies of the liver nodules revealed a tumor composed of spindle, epithelioid, and stellate tumor cells, some with characteristic intracytoplasmic vacuolations/lumina surrounded by myxohyaline stroma. Some of these intracytoplasmic vacuoles/lumina showed erythrocytes, suggesting its vascular origin which was confirmed by CD31 and CD34 positivity. The article highlights the importance of histopathology and IHC in the precise diagnosis of EHE.

Kaposiform hemangioendothelioma in an unusual site: A report of two cases in children

Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor usually seen in children. It is frequently associated with Kasabach-Merritt phenomenon. Here we report two cases of KHE: the first case being an 11-month-old boy who came with massive swelling on the face and violaceous discoloration. The second case was a 7-year-old boy who presented with respiratory distress and bleeding manifestations. CT scan chest showed a large mass involving the anterior mediastinum. Histologic examination of resected masses from both these cases showed features of KHE involving subcutaneous tissue and thymus, respectively. Although cutaneous and subcutaneous location is common, thymic involvement is unusual. It is important to distinguish KHE from infantile haemangioma, tufted angioma, spindle-cell haemangioma, verrucous malformation and Kaposi sarcoma. Histologic features, supportive immunohistochemistry and the clinical profile together are helpful to differentiate KHE from other vascular lesions.

Hepatic epithelioid hemangioendothelioma: A clinocopathological correlation

Primary hepatic epithelioid hemangioendothelioma (HEHE) is a rare tumor with an incidence of <0.1 per 100,000. The clinical course is variable with variable outcomes. Due to its rarity, treatment protocols, prognostic and predictive factors are not well established underscoring the need for such a study. Pathologists’ awareness of this entity, a meticulous morphologic examination coupled with immunohistochemistry can aid in accurate diagnosis.

A case of pseudomyogenic hemangioendothelioma misdiagnosed as low-grade malignant fibrous histiocytoma and review of literature / 中南大学学报(医学版)

Pseudomyogenic hemangioendothelioma (PHE) is a rare angiogenic tumor. Histologically, the morphological characteristics of neoplastic vessels and endothelial differentiation are not obvious, and it is easy to be confused with epithelioid sarcoma, epithelioid hemangioendothelioma and myogenic tumor. PHE usually occurs in arms and legs in young people and has a significant male predominance. The tumor has a predilection for the distal extremities and its typical manifestation is multiple center invasion of a single limb, which can involve all layers of skin and subcutaneous tissues,and is often accompanied by abvious pain. Histologically, PHE is characterized by infiltrative growth of tumor. Most tumor lesions are composed of sheets and loose fascicles of plump spindle or epithelioid cells within a background of variably prominent inflammatory infiltration, which was commonly composed of neutrophils. Some cells may resemble rhabdomyoblasts, and nuclear atypia and mitosis were rare. The tumor cells generally expressed positive cytokeratin (CK), ETS-related gene (ERG), Friend leukemia virus integration 1 (FLI1) and integrase interactor 1(INI1). In some cases, the tumor cells expressed CD31. A case of a young woman was reported in this paper, who presented with a subcutaneous mass with severe pain and was chronologically misdiagnosed with herpes zoster, low-grade malignant fibrous histiocytoma and epithelioid hemangioendothelioma. In this study, the clinical and pathological features, differential diagnosis and the latest progress in therapy of PHE were analyzed based on relevant literature.

A case of epithelioid hemangioendothelioma diagnosed by bone marrow cell morphology / 中华检验医学杂志

Epithelioid hemangioendothelioma (EHE) is a rare malignant vascular tumor. Its malignancy is between benign hemangioma and highly malignant angiosarcoma. It originates from vascular endothelial cells or pre-endothelial cells. It is characterized by the proliferation of vascular endothelial cells with a skin-like or histiocyte-like appearance. The incidence of EHE is less than 1% in all vascular tumors, and it can occur in multiple parts of the body, most often in the liver, followed by simultaneous involvement of the liver and lung, the lung alone, and the bone alone. At present, there is no report of epithelioid hemangioendothelioma diagnosed by bone marrow cell morphological examination in China. In this case, abnormal cells were found through bone marrow cell morphological examination, which guided the direction of further diagnosis and treatment. And finally the patient was diagnosed as epithelioid hemangioendothelioma. The bone marrow cell morphological examination can provided an important basis for clinical diagnosis and treatment. Epithelioid hemangioendothelioma needs to be differentiated from a variety of benign and malignant angiogenic tumors, especially other types of epithelioid angiogenic tumors. At present, it has been found that the disease has characters of cytogenetic and molecular biological abnormalities. Combined with histopathological morphology and immunohistochemical examination, we can make the diagnosis and differential diagnosis.

Hemangioendotelioma kaposiforme refractario en población pediátrica: reporte de caso y revisión de la literatura / Refractory kaposiforme hemangioendothelioma in the pediatric population: case report and literature review

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.

Portal hypertension as an uncommon presentation of hepatic epithelioid hemangioendothelioma: a case report

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.

Hemangioendotelioma kaposiforme: correlação radiológica, cirúrgica e anatomopatológica / Kaposiform hemangioendothelioma: radiological, surgical and anatomopathological correlation

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.

Management and prognosis of infantile hepatic hemangioendothelioma-arteriovenous fistula complicated with heart failure in neonates / 中国新生儿科杂志

Objective:To study the clinical features and prognosis of infantile hepatic hemangioendothelioma-arteriovenous fistula (IHHE-AVF) complicated with heart failure in neonates.Method:From May 2016 to June 2020, neonates with IHHE-AVF complicated with heart failure admitted were retrospectively studied. The clinical presentation, treatment and outcomes were analyzed.Result:A total of 11 cases of IHHE-AVF complicated with heart failure were enrolled (male 5, female 6). The onset age of heart failure was 12.0 (0.0, 17.0) d. 6 cases showed IHHE on fetal ultrasound. All patients had significantly enlarged heart on chest X-ray. All patients had decreased left ventricular systolic function and pulmonary hypertension on echocardiography. All patients required respiratory support and 6 of them were intubated. 3 cases received conservative treatment (all dead). 1 case received surgery (dead). 7 cases received interventional therapy at the age of (25.6±18.5) d. 1 case was dead, and the other 6 cases were improved and discharged. All the 6 cases were followed up to 3~18 months. None of them had heart failure again. The IHHE were shrunk or completely disappeared. Coagulation function and platelet count were normal.Conclusion:The fatality rate of neonatal-onset IHHE-AVF complicated with heart failure is extremely high. Interventional therapy may be more effective than conservative therapy and surgery.

Multicentric osteoblastoma-like: report of a rare case in the craniofacial region / Osteoblastoma-like multicêntrico: relato de um caso raro na região craniofacial

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.

Retiform hemangioendothelioma: An uncommon vascular neoplasm

Retiform hemangioendothelioma is a rare vascular neoplasm of intermediate grade, the diagnosis of which can be challenging. We report a case of 35-year-old man with swelling in the postauricular region. He had undergone FNAC which had revealed blood only. Microscopic examination showed narrow, arborizing, vascular channels resembling normal rete testis. Evidence of mitoses or cytological atypia were lacking. Immunohistochemistry showed diffuse and strong staining for CD34 along with CD31 positivity. Immunostains for D240 and GLUT1 were negative. A diagnosis of retiform hemangioendothelioma was made. Histologically, it should be distinguished from Kaposiform hemangioendothelioma, Dabska tumor, epithelioid hemangioendothelioma, and angiosarcoma.

Discussion on the treatment of 106 cases of Kaposiform hemagioendothelioma / 中华整形外科杂志

Objective@#To compare the curative effect of non-surgical treatment, early operation and advanced operation on the Kaposiform hemagioendothelioma (KHE). To explore the optimal treatment for KHE.@*Methods@#The 106 cases of KHE patients admitted by our hospital from January 2004 to December 2016 were selected as the study subjects. The age is the minimum 1d and the maximum 43 years, median age 94.5 d. According to different treatment methods, they were divided into non-surgical treatment and surgical treatment: according to the onset of the disease and the day of the operation, the early operation was performed less than three months since the onset of the disease, and the advanced operation more than 3 months. Efficacy evaluation criteria: The recovery meant that the tumor disappeared or mostly disappeared, the platelets returned to normal, and there was no recurrence more than half a year after treatment. " Effective" indicated that tumors were shrunken significantly and the platelet counting rose significantly. " Ineffective" indicated that the tumor was not shrunken or continued to increase and platelet counting did not rise or continued to decrease.The three groups of patients were followed up, and the efficacy of the three groups after treatment and the changes of platelet counting before and after treatment on day 1, 7 and 14 were compared. The two groups of early and late treatment were compared for operative and postoperative conditions.@*Results@#All patients were followed up for 6-23 months, 24 cases were effective, 12 cases were ineffective, and 5 cases died. After 7 days of treatment, the platelet counting of the three groups were 68 (31, 157)×109/L in the non-surgical treatment group, (294.75±150.59)×109/L in the early operation group, (221.48±148.71)×109/L in the late operation group, with statistical significance (P < 0.05). After 14 days of treatment, the platelet counting of the three groups were 78 (25.51, 54.5) in the non-surgical treatment group, 281 (234, 356)×109/L in the early operation group, and in the late operation group (219.67±140.95)×109/L, with statistical significance (P < 0.05). After at least half a year of follow-up, the total effective rate of clinical efficacy in the three groups was the non-surgical treatment group 62.07%, the early operation group 96.34% and the late operation group 81.82%; the difference was statistically significant between two groups (P < 0.05); There was significant difference between the early operation group and the late operation group in the amount of bleeding and operation time, the time of platelet counting and the total hospitalization time (P< 0.05).@*Conclusions@#Early surgical treatment of Kaposiform hemangioendothelioma is superior to late-operative and non-surgical treatment.Surgical treatment will be the preferred option for treatment of Kaposiform hemangioendothelioma, and examinations should be operated to potential patients for early diagnosis.

CT imaging and quantitative analysis in the differential diagnosis of hepatic epithelioid hemangioendothelioma and hepatic metastasis / 中华肝胆外科杂志

Objective To study the use of abdominal enhanced CT imaging and quantitative index analysis in the differential diagnosis of hepatic epithelioid hemangioendothelioma (HEH) and hepatic metastasis.Methods A study group of 12 patients with HEH who underwent abdominal enhanced CT scanning at the First Affiliated Hospital of Zhengzhou University from February 2014 to October 2018 was retrospectively compared with a control group of 52 patients with hepatic metastases diagnosed clinically and by imaging examinations.The general information and imaging data of these patients were collected and analyzed.Results The lesions in the 2 groups mainly presented as multiple and diffuse lesions.The diffuse lesions of HEH often fused into strips.The hepatic metastasis group showed a higher CT attenuation and TNR in the portal vein phase than the HEH group (P ＜ 0.05).The area under the ROC curves of the two indexes were 0.756 and 0.841 respectively.The centers of the lesions showed almost no or slightly homogeneous enhancement in the HEH group,while the liver metastasis group showed slightly and moderately heterogeneous enhancement,with a significant difference between the two groups (P ＜ 0.05).Female,subcapsular distribution,capsular contraction,target ring sign and lollipop sign were independent risk factors for HEH (P ＜0.05),while a high CT attenuation and TNR in the portal vein phase,elevated tumor markers and lymph node metastasis were independent risk factors for liver metastasis on logistic regression analysis (P ＜ 0.05).Conclusions CT attenuation,TNR,central enhancement features in the portal vein phase,special signs and secondary changes of lesions were helpful for the differential diagnosis between HEH and liver metastasis.

Diagnosis and treatment of hepatic vascular tumors / 临床肝胆病杂志

Hepatic vascular tumors originate from endothelial cells. Except hepatic cavernous angioma, hepatic vascular tumors tend to have a low incidence rate and atypical clinical symptoms and imaging findings, which brings great challenges to clinical diagnosis and treatment. This article reviews the classification, clinical diagnosis, intervention measures, and prognosis of hepatic vascular tumors, so as to improve the overall understanding of such diseases.