[Pulmonary Epithelioid Hemangioendothelioma Requiring Differentiation from Metastatic Lung Tumor:Report of a Case].

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.

Pleural epithelioid hemangioendothelioma in a 39-Year-old female: a case report.

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.

Image-guided percutaneous ablation of hepatic epithelioid hemangioendothelioma.

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.

Primary pulmonary epithelioid hemangioendothelioma.

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare tumour of vascular origin with low to intermediate malignancy. Typical radiological finding on CT is multiple small nodules in bilateral lungs, and some will have punctate calcifications and pleural thickening. The diagnosis of PEH is confirmed by histopathological findings and positive immunohistochemistry staining. We report a case of a woman in her 50s with a medical history of lung adenocarcinoma. Later, regular chest CT during a routine cancer follow-up revealed multiple small pulmonary nodules and increased sizes of these nodules on serial images, initially misdiagnosed as multiple lung metastases. The histopathological diagnosis was made on a pulmonary wedge resection. Finally, PEH was diagnosed on the basis of positive immunohistochemical staining for CD31, ERF and TFE3. In the current study, the clinicopathological features and review of the literature were investigated. Our case highlights the importance of a histological diagnosis to avoid misdiagnosis.

A case of complex hemangioendothelioma of the liver in an infant.

A physical examination of a 9-month-old female infant presenting with vomiting and diarrhea revealed tenderness in the right upper abdomen and heightened abdominal muscle tone. Abdominal ultrasonography identified an irregular hypoechoic area within the right lobe of the liver. While a subsequent enhanced CT examination disclosed a well-defined lesion exhibiting internal focal calcification and delayed heterogeneous enhancement. Subsequently, she underwent surgical resection, and postoperative pathology revealed areas of epithelioid hemangioendothelioma and cavernous hemangioma. Immunohistochemistry demonstrated positive expression of CD34, CD31, FLI-1, and F-VIII. The pathologic diagnosis was confirmed as composite hemangioendothelioma (CHE).

An extremely rare case of pulmonary epithelioid hemangioendothelioma.

Epithelioid emangioendothelioma (EHE) is a rare tumor originating from vascular endothelial cells, which belongs to malignant vascular tumors with low to moderate differentiation and potential metastatic ability and its incidence rate is less than one in a million. We herein report a rare case of EHE of pulmonary origin and a review of the literature concerning the clinical and pathological features of this disease. The patient underwent left upper lobectomy to completely extirpate the lesion by video-assisted thoracic surgery. Our findings suggest the difficulty of making a diagnosis before surgery and that more cases need to be reported in order to facilitate the preoperative diagnosis of such a rare tumor.

NR1D1::MAML1 epithelioid and spindle cell sarcoma mimicking pseudomyogenic hemangioendothelioma in core biopsy: A case report and review of the literature.

Epithelioid and spindle cell sarcomas with NR1D1::MAML1/2 gene fusions are rare and emerging entities. Only six cases of NR1D1-rearranged mesenchymal tumors have previously been reported in the literature; they are often characterized by an epithelioid morphology, at least focal pseudogland formation, prominent cytoplasmic vacuoles, and focal to diffuse immunohistochemical expression of keratin. We herein report the first case of an NR1D1::MAML1 epithelioid and spindle cell sarcoma with dual immunohistochemical expression of ERG and FOSB, mimicking a pseudomyogenic hemangioendothelioma (PHE) on core biopsy. The sarcoma arose in the left forearm of a 64-year-old man. Initial biopsy showed a mesenchymal neoplasm composed of epithelioid and spindle cells dispersed in myxoid stroma with scattered stromal neutrophils. The morphologic features, combined with the dual immunohistochemical expression of ERG and FOSB, initially mimicked PHE, representing an important potential diagnostic pitfall. The patient subsequently underwent a radical resection, which showed a much more diffuse epithelioid appearance with nested architecture and pseudogland formation. Next-generation sequencing was performed on the resection specimen, which revealed an NR1D1::MAML1 gene fusion, confirming the final diagnosis. Given the fully malignant potential of this tumor, knowledge and recognition of this rare entity are essential to ensure proper management, prevent misdiagnosis, and further characterize the clinical course of this emerging entity. Comprehensive molecular testing can help to identify these rare tumors and exclude the possibility of epithelioid mimics, including PHE.

A Rare Case of Primary Adrenal Epithelioid Angiosarcoma.

BACKGROUND Primary adrenal epithelioid angiosarcoma (PAEA) is a very uncommon primary adrenal gland tumor that usually occurs around the age of 60 years and is more common among males. Owing to its rarity and histopathological features, PAEA could be misdiagnosed as adrenal cortical adenoma, adrenal cortical carcinoma, or other metastatic cancers, such as metastatic malignant melanoma and epithelioid hemangioendothelioma. CASE REPORT A 59-year-old male patient presented to our hospital with a complaint of abdominal bloating that started 2 months prior. His vital signs and the results of his physical and neurological examinations were unremarkable. A computed tomography scan showed a lobulated mass arising from the hepatic limb of the right adrenal gland but no evidence of metastasis to the chest or abdomen. The patient underwent right adrenalectomy, and the macroscopic pathological findings from a right adrenalectomy specimen revealed atypical tumor cells with an epithelioid appearance in the background of an adrenal cortical adenoma. Immunohistochemical staining was performed to confirm the diagnosis. The final diagnosis was epithelioid angiosarcoma involving the right adrenal gland with a background adrenal cortical adenoma. The patient had no postoperative complications, pain in the surgical wound, or fever. Therefore, he was discharged with a schedule for followup appointments. CONCLUSIONS PAEA may be misinterpreted as adrenal cortical carcinoma, metastatic carcinoma, or malignant melanoma radiologically and histologically. Immunohistochemical stains are essential for diagnosing PAEA. Surgery and strict monitoring are the main treatments. In addition, early diagnosis is essential for patient recovery.

[Epithelioid Hemangioendothelioma of the Upper Mediastinum:Report of a Case].

A 81-year-old woman was referred to our hospital for neck discomfort. Chest computed tomography (CT) showed a tumor in the upper mediastinum. Fluorodeoxyglucose-positron emission tomography (FDG-PET) showed the mild accumulation in the tumor. Percutaneous biopsy was performed and epithelioid hemangioendothelioma was suspected, and the surgical treatment was performed. The histological study showed polygonal and irregular cells with nuclear atypia in myxoma-like substrate compatible with epithelioid hemangioendothelioma.

Multifocal and hormone-dependent epithelioid hemangioendothelioma with osteolysis of the second cervical vertebral body: report of an unprecedented surgical approach by using autologous bone graft.

We report the case of a 28-year-old female patient who complained of extreme neck pain when giving birth to a child. Magnetic resonance imaging (MRI) of the cervical spine demonstrated an osteolytic lesion at the second cervical vertebral body (C2). In this presentation, we highlight a transoral surgical approach in order to prevent instability of this osteolytic lesion. To the best of our knowledge, this is the first time that such a route of access has been described for this tumor entity. A histopathologic examination led to the diagnosis of epithelioid hemangioendothelioma. During a follow-up period of 33 months, the patient had no complaints.

Epithelioid hemangioendothelioma - an unexpected diagnosis of a mediastinal tumor with extensive local thrombosis.

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor and the mediastinal localization is amongst the most infrequent. We present the case of a 37-year-old woman with a history of resected left thyroid tumor that presented to our department for evaluation of a left supraclavicular palpable mass in close contact with local vascular structures, and with heterogeneous contrast enhancement as described by computed tomography (CT) and magnetic resonance imaging (MRI). Considering the history of the patient, the presumptive diagnosis of thyroid tumor recurrence was established, and the patient was referred to surgical department. During procedure, we encountered important bleeding from a ruptured jugular vein branch, which we assumed to be a newly formed tumor blood vessel. After surgery (48 hours postoperatively), the patient developed important local thrombosis that encompassed the left internal jugular vein, left subclavian vein and the left brachiocephalic trunk that partially subsided after anticoagulant therapy. The histological examination revealed the presence of a vascular tumor proliferation of epithelioid endothelial cells that was characteristic of an EHE confirmed later on the immunohistochemical studies as Yes-associated protein 1-transcription factor E3 (YAP1-TFE3) subtype. In addition to the case report, some relevant information from the scarce literature data about mediastinal EHE were reviewed here.

The challenging surgical management of hepatic epithelioid hemangioendothelioma: a narrative review.

BACKGROUND AND OBJECTIVE: Hepatic epithelioid hemangioendothelioma (HEHE) is an uncommon vascular endothelial cell cancer involving the hepatic tissue with full malignant potential. HEHE diagnosis is challenging because blood tests and radiological findings are often non-pathognomonic. Therapeutic possibilities are manifold, ranging from a simple "wait and see" to surgical approaches, up to systemic therapies with vascular endothelial growth factor (VEGF) and mechanistic target of rapamycin (mTOR) inhibitors. The standardized adoption of the surgical approaches, namely resection or transplantation, still remains unclear. Aim of this review is to clarify the role of the surgical strategies for the management of HEHE. METHODS: A review of the literature was done with the intent to focus on the relevant studies exploring the role of the different surgical therapies. A search of these studies was done using the electronic database MEDLINE-PubMed. KEY CONTENT AND FINDINGS: The surgical approaches, namely resection or transplantation, represent the best option in treating well-selected patients with HEHE. Resection is indicated in the case of single lesion or oligonodular monolobar disease. Liver transplantation is indicated in the case of multifocal bilobar disease, even in the presence of resectable extrahepatic disease. The potential role of a minimally invasive approach should represent a relevant field to explore. CONCLUSIONS: The impact of surgery for the treatment of HEHE requires further studies to further clarify its relevant role. Standardized approaches are required.

A review of hepatic epithelioid hemangioendothelioma-Analyzing patient characteristics and treatment strategies.

BACKGROUND: Hepatic epithelioid hemangioendothelioma (HEH) is a rare vascular tumor of unknown etiology and unpredictable natural history. To date, no large-scale studies have been published evaluating this disease due to its rare occurrence. METHODS: The National Cancer Database was reviewed between 2004 and 2016 to identify patients with HEH. Univariate analysis with overall survival (OS) was performed by Cox proportional hazards model. Kaplan-Meier method was used to create OS curves and compared using the log-rank test. RESULTS: We identified 229 patients with HEH. The majority of patients were female (61.1%), white (84.3%), and had a Charlson-Deyo score of 0 (75%). Chemotherapeutic intervention was seen in 26% of the patients while 33% received surgical intervention in the form of wedge/segmental liver resection (n = 27), hepatectomy lobectomy/extended lobectomy (n = 18), and liver transplant (n = 22). Five-year survival in surgical patients was 90.5%, 66.5% and 81%, respectively (p = 0.485). Age greater than 55 years (hazard ratio [HR], 2.78; p < 0.001), Asian ethnicity compared to white (HR, 2.84; p = 0.012), and a higher Charlson-Deyo score (score 1: HR, 2.28; p < 0.001 and score ≥2: HR, 2.76; p = 0.011) were associated with worse OS. CONCLUSION: Treatment for HEH remains variable with only a third of the patients undergoing surgery. International collaboration is necessary to determine the optimal treatment for this rare disease.

Epithelioid Hemangioma of Bone: A Rare Vascular Neoplasm. A Case Report and Literature Review.

Epithelioid hemangioma (EH) of bone is a rare benign, albeit locally aggressive vascular neoplasm. It is usually solitary and involves the metaphysis or diaphysis of long tubular bones, especially in the lower extremities. Rarely it may present as multifocal lesions. The differential diagnosis includes malignant vascular bone tumors such as epithelioid hemangioendothelioma and epithelioid angiosarcoma. Clinical presentation and radiographic and histological findings are not specific and diagnosis is based mostly on immunohistochemical and molecular studies. There is no consensus regarding the optimal treatment. Curettage and bone grafting or en bloc resection are the current treatment options, however local recurrence have been reported. We present a case of multifocal EH of the distal tibia, distal fibula and hindfoot in a 38 year-old male managed with curettage, radiofrequency ablation and cement osteoplasty. The imaging features, histological findings and treatment options of this rare vascular tumor are discussed.

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.

Rare case of epithelioid hemangioendothelioma in the right innominate vein-An innovative diagnostic approach.

BACKGROUND AND AIMS: Epithelioid hemangioendothelioma is a rare malignant vascular tumor with limited literature. AIMS: We reported an innovative endovascular biopsy of the right innominate vein tumor. MATERIALS AND METHODS: Endovascular suction thrombectomy was performed with multipurpose catheter and constant negative pressure under fluoroscopic guidance. RESULTS: Epithelioid hemangioendothelioma was diagnosed preoperatively and a complete margin-free tumor resection with patch repair of the right innominate vein was achieved via sternotomy. DISCUSSION: Preoperatively diagnosis is usually not available due to lesions' location. Identifying malignant vascular tumors becomes valuable to guide the surgical treatment. CONCLUSIONS: In this case report, this innovative endovascular approach led to a rare preoperative diagnosis of EHE and subsequent margin-free resection.

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

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.

Treatment modalities and long-term outcomes of hepatic hemangioendothelioma in the United States.

BACKGROUND: We analyzed the outcomes of patients with hepatic epithelioid hemangioendothelioma (HEHE) in the United States after stratification by their most definitive treatment. METHODS: The National Cancer Data Base was used to identify patients with HEHE between 2004 and 2018. Patients were divided in four treatment groups: no surgical therapy, ablation, liver resection or liver transplantation. Demographics and clinical characteristics were compared, and Kaplan Meier functions and Cox-regression were used for unadjusted and adjusted survival analyses. RESULTS: Among a total of 334 patients, 218 (65.2%) were managed non-surgically, 74 (22.1%) underwent hepatic resections, 35 (10.4%) underwent liver transplantation and 7 (2.1%) underwent ablations. The overall median survival was 111 months (95%CI 73-149) after liver transplantation, 69 months (95%CI 45-92) after hepatic resection, 38 months (95%CI 0-78) after ablation and 80 months (95%CI 70-90) for patients managed by watchful waiting (P < 0.001). After adjustment, patients who underwent liver transplantation were found to have a better survival when compared to other therapies (Hazard Ratio: 0.61, 95% Confidence Interval: 0.38-0.97, p = 0.035). CONCLUSIONS: This study reports the outcomes of the largest cohort of patients with HEHE. The longest survival was observed after liver transplantation, followed by non-surgical management and hepatic resection. Because of selection bias, future studies to better characterize what criteria should be used for the selection of treatment modalities for HEHE are urgently needed.