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.

Angiolymphoid hyperplasia with eosinophilia: A report of a rare entity in an elderly male

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon benign proliferation of blood vessels of uncertain etiology. It primarily affects the head‑and‑neck region. Histologically, it is characterized by the prominent proliferation of plump endothelial cells, and accompanying eosinophilic and lymphocytic infiltration. Herein, we report the case of ALHE in a 65‑year‑old male.

Uterine Epithelioid Leiomyosarcoma Arising Within Leiomyoma in A Postmenopausal Woman: A Case Report

@#Uterine leiomyosarcoma is a malignant smooth muscle tumour of the uterus. It is rare and accounting for less than 2% of cases in malignant gynaepathology. To date, only a few reported cases of leiomyosarcoma arising from leiomyoma documented in the literature. We shared an uncommon occurrence of leiomyosarcoma arising from leiomyoma. Presented herein is a case of a ‘rare epithelioid subtype’ of leiomyosarcoma arising from a leiomyoma in a postmenopausal woman. We highlighted the importance of recognizing the possibilities of this event to allow for a timely diagnosis of leiomyosarcoma and to provide insights on management of patients presented with clinically presumed fibroid.

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.

Spitzoid melanocytic tumors / 中华皮肤科杂志

Clinical and histological features of Spitz nevi, atypical Spitz tumors and spitzoid melanoma overlap each other, making their identification challenging. Combined with clinical and histological features of spitzoid melanocytic tumors, this review summarizes research progress in their immunohistochemical features and application of fluorescence in situ hybridization in their identification.

Primary adrenal epithelioid sarcoma: a case report / 中华泌尿外科杂志

Adrenal epithelioid sarcoma is very rare in clinic. A case of epithelioid sarcoma of the right adrenal gland was reported in this paper. After physical examination, the patient was found to have a mass in the right adrenal area and underwent right adrenalectomy. The postoperative pathological diagnosis was right adrenal epithelioid sarcoma. Two months after adrenalectomy, positron emission tomography computed tomography(PET/CT) noted recurrence at the tumor bed and multiple metastases.The patient underwent chemotherapy combined with immunotherapy. After 16 months of follow-up, the disease was stable.

Multi-slice spiral CT manifestations of perivascular epithelioid cell tumors / 中国基层医药

Objective:To investigate CT manifestations of perivascular epithelioid cell tumors.Methods:The multi-slice spiral CT manifestations in 11 patients with perivascular epithelioid cell tumors which were pathologically confirmed in the First Affiliated Hospital of Shantou University Medical College between October 2012 and August 2021 were retrospectively analyzed.Results:Perivascular epithelioid cell tumors were located in the kidney of eight patients, in the right lobe of the liver of two patients, and in the descending colon of one patient. The largest tumor, approximately 20.0 cm × 26.0 cm × 11.0 cm, was located in the kidney. The smallest tumor, 2.4 cm × 2.6 cm × 3.4 cm, was located in the colon. CT plain scans showed uniform density without bleeding, calcification, or fat in two patients, and non-uniform density with fatty change in two patients. Contrast-enhanced CT images showed non-uniform enhancement in the arterial phase in 10 patients and uniform enhancement in one patient. Contrast-enhanced CT images showed decreased degrees of enhancement of the lesions in 11 patients from the portal venous phase to the delayed phase and dilated and tortuous vascular shadows in 11 patients.Conclusion:The multi-slice spiral CT manifestations of perivascular epithelioid cell tumors are characteristic and the multi-slice spiral CT is of value for the diagnosis of perivascular epithelioid cell tumors.

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.

Epithelioid hemangioma of the ascending aorta: A rare case

Cardiac epithelioid hemangioma is extremely rare. Currently, there are only a few described cases of intraluminal hemangiomas of the aorta and the aortic valve and no described cases with extraluminal epithelioid hemangiomas of the ascending aorta. We now present a case of epithelioid hemangioma of the ascending aorta that was an incidental finding during the coronary artery bypass and successfully resected.

Epithelioid haemangioma of bone: A series of four cases with a revision of this contentious entity

Epithelioid hemangioma of bone is a rare and locally aggressive vascular neoplasm of bone associated with a good prognosis. Because of its worrisome histomorphologic features and aggressive clinicoradiologic findings, at times with multifocal presentation, they tend to simulate malignant tumors. We report a series of four cases of epithelioid hemangioma of bone with their clinicopathologic characteristics. All had adjacent soft tissue involvement and two had multifocal bone disease. Microscopically, all cases had a tumor in lobular configuration, composed of epithelioid endothelial cells with the formation of well-formed vessels or grew in solid sheets. The tumor cells lacked significant cytologic atypia, necrosis, and increased mitosis. All cases were immunohistochemically positive for vascular markers CD34, CD31, ERG1, whereas negative for CK. Two of the cases were treated with excision, and the other two underwent curettage. None had local recurrence or metastasis on follow-up. This study highlights the importance of recognizing histomorphological and clinicoradiological features for distinguishing epithelioid hemangiomas from malignant vascular neoplasms of bone because of their distinct therapeutic implications and clinical outcomes.

Sarcoma epitelioide de tipo proximal de vulva

El sarcoma epitelioide es un tipo raro de tumor mesenquimal. El sarcoma epitelioide de tipo proximal de vulva es un tumor maligno extremadamente raro de los tejidos blandos, con frecuentes recurrencias por su comportamiento agresivo. Debido a que sus características son similares a otras lesiones benignas, esto lleva a retrasos diagnósticos y terapéuticos. Las características histológicas de este tumor incluyen células epitelioides prominentes y aparición de rasgos rabdoides con marcadas atipias nucleares. Debido a su baja incidencia, no existen algoritmos diagnósticos basados en la evidencia ni recomendaciones publicadas para el tratamiento. El pronóstico es generalmente malo. Se presenta un caso de sarcoma epitelioide de tipo proximal de vulva.

Epithelioid sarcoma is a rare type of mesenchymal tumor. Proximal-type epithelioid sarcoma of the vulva is an extremely rare malignant tumor of the soft tissues with frequent recurrences due to its aggressive behaviour. Because its characteristics are similar to other benign lesions, this leads to diagnostic and therapeutic delays. Histologic features of this tumor include prominent epithelioid cells and the appearance of rhabdoid features with marked nuclear atypia. Due to its low incidence, there are no evidence-based diagnostic algorithms or published recommendations for treatment. The prognosis is generally poor. A case of proximal type epithelioid sarcoma of the vulva is presented.

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 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.

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.

Clinical diagnosis and treatment of liver perivascular epithelioid cell neoplasms / 中华肝胆外科杂志

Objective:To analyze the clinical features and prognosis of liver perivascular epithelioid cell neoplasms (PEComa).Methods:The clinical data of 12 patients with liver PEComa diagnosed by pathology at the First Affiliated Hospital of Xi 'an Jiaotong University from October 2011 to November 2021 were retrospectively analyzed. There were 1 male and 11 females, with a median age of 44 (range 20 to 63) years old. The clinical manifestations, laboratory examinations, imaging features, treatment methods, postoperative pathological features and treatment outcomes of these patients were collected and analysed. Postoperative follow-up by telephone or patient's follow-up records.Results:Among the 12 patients with hepatic PEComa, 8 patients (66.7%) were asymptomatic and 11 patients (91.7%) had a single lesion. All patients underwent surgical treatment, including local tumor resection in 10 patients (83.3%) and extended hemihepatectomy in 2 patients (16.7%). Enhanced CT showed the lesion to be a quasi-round homogeneous low-density mass, enhanced in arterial phase with hepatic artery branches in the lesion, and decrease in enhancement degrees in portal vein phase and delayed phase. Postoperative pathology of the lesions in all the 12 patients was benign. Immunohistochemical results showed that the positive rates of melanoma cell markers HMB45, Melan-A and smooth muscle actin were 100.0%(12/12), 83.3%(10/12) and 91.7%(11/12) respectively. The median follow-up period was 27 months, and no recurrence or metastasis was found.Conclusion:Hepatic PEComa occurred commonly in women with obscure symptoms. The lesion was mainly single and it had no correlation with hepatitis. It is easily confused with primary liver cancer and liver metastasis on medical imagings. PEComa expressed markers of both melanocyte and smooth muscle cell, and radical surgical resection resulted in good results.

CT, magnetic resonance imaging and clinicopathological characteristics of perivascular epithelioid cell tumor in children / 中华实用儿科临床杂志

Objective:To investigate the CT, magnetic resonance imaging(MRI) and clinicopathological cha-racteristics of perivascular epithelioid cell tumors (PEComas) in children, and improve the diagnostic accuracy of PEComas.Methods:The CT, MRI and clinicopathological characteristics of 6 children confirmed with PEComas by histopathology caming from Beijing Children′s Hospital, Capital Medical University during March 2019 and April 2022 were retrospectively analyzed.Results:The patients aged from 1.7 to 11.9 years old (with mean age of 7.6 years old). The male to female ratio was 1∶2.Of the 6 solitary tumors identified, 2 cases were found in the kidney, 2 cases in the abdominal cavity, 1 case in the pelvic cavity, and 1 case in the groin.The solitary tumors had well-defined borders.Five solitary tumors were shaped like an ellipse and 1 solitary tumor was multinodular-shaped.Lace-like or nodular changes were observed at tumor margins(3 cases). The maximum diameters of the tumors ranged from 4.8 to 15.9 cm (mean value: 9.3 cm). Five tumors showed peripheral invasion and 2 cases had lymph node metastasis.Two patients were complicated with tuberous sclerosis.One case suffered from tumor recurrence after operation.The tumors (6 cases) were isodensity or low density on the plain CT scan, and gradual heterogeneous enhancement was observed after injection of the contrast agent.A majority of the tumors had tortuous (5 cases) and thickened blood vessels inside, and a few of them (2 cases) underwent gravel-like calcification.Patch-like or nodule-like high signal intensity could be seen in the center of the tumors (3 cases) on MRI T1WI and T2WI, while the peripheral part of the lesions showed iso-signal intensity (equal to muscle). On diffusion weighted imaging(DWI) (b=800 s/mm 2), the lesions exhibited restricted diffusion at the peripheral part with significant enhancement, and iso-signal intensity at the central part with none or slight enhancement.Histological classification results revealed that 1 of 6 cases was a benign PEComa and 5 cases were malignant PEComas.The expression rates of Melan-A and SMA were 100.0% and 83.3%, respectively. Conclusions:The CT, MRI and pathological manifestations of PEComas in children have some distinct features.PEComas are often an oval-like mass with clear borders and nodular margins in the abdominal and pelvic cavity, and tortuous and thickened blood vessels can be seen in the tumor.The periphery of the tumor is the main region to be enhanced by the contrast agent.Local invasion and distant metastasis can be observed.Most of the tumors are malignant in pathology.Both melanocyte and muscle cell markers can be found in the expression of immunohistochemistry.

Recurrence of melanoma in the scar after excised Spitz nevus in a 17-year-old child

Abstract Melanoma in childhood is rare and its diagnosis is more difficult than in adults, as it often presents histologic features overlapping with the Spitz nevus. The authors report the case of a 17-year old boy who was first diagnosed with Spitz nevus, however, the final diagnosis made after the excision of the tumor arising in the scar was changed to melanoma. The case in this present study emphasizes the importance of the differential diagnosis of skin tumors in young 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.

Epithelioid inflammatory myofibroblastic sarcoma: the youngest case reported

Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare variant of the inflammatory myofibroblastic tumor. It has an aggressive clinical course and a high rate of recurrence. EIMS primarily affects children and young adults. Hereby, we report this entity in a 4-month-old infant who presented with an abdominal mass. Imaging studies revealed a large hypodense mesentery-based lesion involving the right half and mid-region of the abdomen. The mass with an attached segment of the small bowel was excised in toto. Grossly, a large encapsulated tumor was identified arising from the mesentery of the small bowel. The histological examination showed a tumor consisting of epithelioid to spindle cells loosely arranged in a myxoid background with numerous blood vessels and lymphoplasmacytic inflammatory infiltrate. On immunohistochemistry, the tumor cells showed positivity for ALK1 (nuclear), desmin, SMA, CD68, and focal positivity for CD30. A final diagnosis of EIMS of the small intestine was rendered. To the best of our knowledge, this case is the youngest reported case in literature.