Case of metastatic epithelioid haemangioendothelioma from an unknown primary site associated with membranous nephropathy and hypercoagulable state.

We report a rare case of metastatic epithelioid haemangioendothelioma from an unknown primary site presenting with axillary lymph node metastases. The patient also had a new-onset membranous glomerulonephritis and thromboembolism, which we postulate were paraneoplastic. The pathogenesis of this rare cancer, the risk of misdiagnosis and membranous glomerulonephritis as a paraneoplastic syndrome are discussed.

MYC Amplification in Epithelioid Angiosarcoma of the Urinary Bladder and Prostate Following Prostate Radiotherapy: A Case Report with a Novel Molecular Alteration.

Epithelioid angiosarcoma is a rare variant of angiosarcoma. Radiation-associated epithelioid angiosarcoma of the urinary bladder and prostate is an exceedingly rare tumor and there are only 8 cases of epithelioid angiosarcoma of the urinary bladder and prostate associated with previous radiotherapy in the literature. To the best of our knowledge, MYC gene amplification has not been previously reported in epithelioid angiosarcoma of the urinary bladder and prostate following radiotherapy, although it is observed in radiation-associated angiosarcoma of other anatomic sites. Here we report the first case of epithelioid angiosarcoma of the urinary bladder and prostate with MYC gene amplification detected by fluorescence in situ hybridization (FISH) analysis in a 70-year-old male patient 10 years after receiving radiation and hormonal therapy for prostate cancer.

An incidental hepatic epithelioid hemangioendothelioma in a patient with chronic hepatitis: lost in the maze.

Hepatic epithelioid angioendotheliomas (HEHE) are rare vascular tumors with low to intermediate malignancy. Imaging plays an important role in the diagnosis of HEHE. However, imaging characterization of HEHE still remains challenging, since HEHE is commonly misdiagnosed as hepatocellular carcinoma, cholangiocarcinoma or metastases.

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.

Intravenous Ketorolac Infusion for Intractable Pleuritic Pain Secondary to Metastatic Epithelioid Hemangioendothelioma.

Background: Epithelioid hemangioendothelioma (EHE) patients can experience severe pain. Nonsteroidal anti-inflammatory drugs, including ketorolac tromethamine, can effectively treat cancer-related pain, provide an opioid-sparing effect, and may be particularly effective for EHE pain. There are limited data describing prolonged (>5 days) continuous intravenous (IV) ketorolac infusion for cancer-related pain and no data on its use in EHE. Case Description: A 67-year-old woman with metastatic hepatic EHE suffered from chronic intractable pleuritic pain unresponsive to trials of nonopioid, opioid, adjuvant medications, and nonpharmacological interventions. In the hospital, continuous IV ketorolac infusion at 3.8 mg/hour (91.2 mg/day) effectively managed pain. With thorough monitoring, the patient was discharged on continuous IV ketorolac infusion at 3 mg/hour (72 mg/day). Infusion continued for 79 days without clinical or laboratory evidence of ketorolac toxicity. Conclusion: Ketorolac tromethamine as a long-term infusion is a potentially viable analgesic for patients with intractable EHE-related pain unresponsive to standard therapies.

Shortness of Breath in a 38-Year-Old Woman With Pulmonary and Hepatic Nodules.

CASE PRESENTATION: A 38-year-old African American woman with a history of menometrorrhagia on previous estrogen therapy and a previously biopsied benign thyroid nodule with recent interval enlargement presented with symptoms of shortness of breath on exertion, an intermittent nonproductive cough, and right upper quadrant abdominal pain for 1 year. She denied wheezing, hemoptysis, fevers, night sweats, or unintentional weight loss. Socially, the patient was a lifelong nonsmoker and denied alcohol or drug use. Travel history was not significant, and she had no contributory occupational, environmental, or animal exposures. Recent cancer screening that included Papanicolaou smear and mammography were negative for neoplasia. Vital signs were normal, and ambulatory pulse oximetry did not demonstrate evidence of oxygen desaturation. Physical examination demonstrated normal respiratory effort, diffuse vesicular breath sounds, and a soft abdomen without hepatomegaly or right upper quadrant tenderness.

Epithelioid Hemangioendothelioma of the Lower Limb, Discovered by a Claudication.

Epithelioid hemangioendothelioma (EHE) is a rare case of a tumor with different clinical behaviors and a difficult anatomopathological diagnosis. The diagnosis of EHE is usually confirmed by postoperative histopathologic examination. Actually, it is a challenge to put a correct diagnosis and to propose aggressive treatment. We report a case of an EHE of the left lower limb discovered in a 53-year-old claudicant woman. Surgical resection, arterial, and venous bypass were performed. The histology demonstrated EHE with a low mitotic index, emerging for the femoral vein.

Epithelioid Hemangioendothelioma of the Ulnar Artery Presenting with Neuropathy.

We present a rare case of epithelioid hemangioendothelioma arising from the wall of ulnar artery in distal forearm. The presentation was interesting in a 34-year-old man, with progressively worsening symptoms of ulnar neuropathy. A mass was seen arising from the ulnar artery on imaging with ultrasound and magnetic resonance imaging. Soft tissue epithelioid hemangioendothelioma in extremities almost always arise from the veins. Existing literature do not have elaborated imaging findings of epithelioid hemangioendothelioma arising from the arterial wall. The aim of this paper is to briefly review the interesting presentation and imaging features of this rare entity. Knowledge of such vascular tumor would avoid the mishap during surgery. Our case will add an interesting presentation of such rare pathology to the existing literature.

A 49-Year-Old Man With Cough and Hand, Wrist, and Knee Pain.

CASE PRESENTATION: A 49-year-old man was sent by his primary care physician to the rheumatology clinic with complaints of several months of bilateral lower extremity swelling. The swelling migrated from both ankles up to his knees. Presenting symptoms consisted of bilateral knee pain as well as bilateral wrist and hand pain with swelling. Pulmonary symptoms consisted of a nagging productive cough of several months. He also complained of significant weight loss: 50 pounds over 12 months. He was a never smoker. The examination was notable for bilateral knee effusions. Radiographs of his wrists, hands, and knee were obtained, along with a chest radiograph. He was then referred to a pulmonologist for further workup.

Recurrent upper limb neuropathies secondary to an epithelioid haemangioendothelioma - A rare mimic of nerve tumours.

Epithelioid haemangioendothelioma (EHE) is an uncommon type of vascular tumour with intermediate malignant potential, classified as a sarcoma which occasionally involves neurovascular structures and can mimic nerve sheath tumours. EHE is difficult to distinguish from other nerve sheath tumours based on imaging, including MRI, and biopsy is often required for diagnosis. Diagnosis of EHE from biopsy often requires the use of vascular immunohistochemical stains. We present a case of left upper limb neurovascular bundle EHE presenting with proximal ulnar nerve neuropathy and subsequent median nerve neuropathy and liver, lungs and bone metastases. The tumour had been identified 20 years prior with a similar presentation of upper limb weakness and sensory disturbance, yet following surgical excision it was misdiagnosed as inflammatory fibrous tissue. Treatment with propranolol has resulted in disease stability and surgical debulking resulted in improved upper limb function. The use of beta-adrenergic receptor antagonists in EHE and other sarcomas have been shown to increase T-cell infiltration and decrease immunosuppressive PD-1 expression in neoplastic cells.

Acute ST-segment elevation myocardial infarction due to extrinsic compression of left coronary artery from pulmonary epithelioid hemangioendothelioma: A case report.

RATIONALE: Acute myocardial infarction is usually caused by coronary atherosclerotic plaque disruption (rupture or erosion), also including other uncommon etiologies. Pulmonary epithelioid hemangioendothelioma (PEH) is a rare low to intermediate malignant vascular tumor originating from vascular endothelial cells. Here, we report a rare case of acute ST-segment elevation myocardial infarction (STEMI) due to extrinsic compression of left coronary artery from PEH. PATIENT CONCERNS: A 63-year-old woman with pulmonary nodules received left pulmonary nodulectomy, and the pathological examination indicated PEH. Five months after the pulmonary nodulectomy, the patient was admitted due to progressive dyspnea. DIAGNOSIS: Electrocardiography showed the obvious ST-segment elevation in the leads I, aVL, and V1-3, and laboratory tests revealed the elevated level of cardiac troponin T. Emergent coronary angiography and the contrast-enhanced computed tomography scan conformed STEMI due to extrinsic compression of left coronary artery from PEH. INTERVENTIONS: The patient did not undergo further therapy after the pulmonary nodulectomy. During the present hospitalization, she received basic life support and nutritional support treatment. OUTCOMES: The patient deteriorated rapidly into multi-organ failure and eventually died. LESSONS: Acute STEMI could be caused by extrinsic compression of the coronary artery from the mass effects of PEH, and active therapy and close follow-up should be considered for patients with PEH.

Hypoglycemic syndrome in hepatic epithelioid hemangioendothelioma, successful treatment - liver transplantation from a living related donor.

Hypoglycemic syndrome occurs not only in endocrine diseases but can complicate the course of many somatic diseases and tumors of pancreatic and extra-pancreatic localization. Development of hypoglycemia in liver tumors is associated with a decrease in the volume of functioning liver tissue, increased consumption, and utilization of glucose by the tumor tissue, inhibition of gluconeogenesis and glycogenolysis, and secretion of insulin-like peptides. Hypoglycemia in liver tumors is rarely the first symptom of the disease and usually occurs in patients with large tumor sizes and symptoms of tumor intoxication. Epithelioid hemangiendothelioma of the liver is the primary malignant neoplasm from the group of mesenchymal tumors, it occurs less than in 1% of cases of all malignant neoplasms of the liver. The clinical course of epithelioid hemangiendothelioma of the liver is highly variable. There are slowly and rapidly progressing variants. The diagnosis is based on the results of histological and immunohistochemical examination of the postoperative material. In the literature there is no description of hypoglycemia in epithelioid hemangiendothelioma. We report a patient with severe hypoglycemic syndrome, which was due to an epithelioid hemangioendothelioma of the liver. Pharmacological treatment of hypoglycemia was ineffective. The presence of massive bilobar tumor made it impossible to use a liver resection. Hepatectomy with living related liver transplantation was life-saving procedure and made it possible to eliminate hypoglycemia.

Spontaneous rupture of a splenic artery aneurysm with splenic epithelioid hemangioendothelioma: a case report.

Spontaneous rupture of a splenic artery aneurysm with splenic epithelioid hemangioendothelioma is a rare condition. Splenic artery aneurysm can be complicated by rupture resulting in hypovolemic shock, which can be fatal if not treat properly. We report a case of a 50-year-old man who presented with sudden onset of left upper quadrant pain and shock. This patient underwent splenectomy with distal pancreatectomy. His pathological diagnosis showed splenic epithelioid hemangioendothelioma.

Right atrial epithelioid angiosarcoma with multiple pulmonary metastasis confirmed by multimodality imaging-guided pulmonary biopsy: A case report and literature review.

RATIONALE: Primary cardiac tumors are very rare, and angiosarcoma accounts for about 33% of all primary malignant cardiac tumors. Primary cardiac epithelioid angiosarcoma is a highly aggressive and difficult to diagnose tumor, with early systemic metastasis and poor prognosis. PATIENT CONCERNS: A 35-year-old Han male experienced sudden severe palpitation and moderate dyspnea. The patient received a whole body F-18 fluoro-deoxyglucose positron emission tomography (18F-FDG PET)/computed tomography (CT) scan, the scan showed a large mass in the right atrium (RA) and numerous pulmonary nodules in both lungs. DIAGNOSES: The patient was diagnosed as right atrial epithelioid angiosarcoma with multiple pulmonary metastasis by pulmonary biopsy through CT-guided percutaneous transthoracic fine needle aspiration. INTERVENTIONS: The patient received a cycle of chemotherapy with docetaxel and gemcitabine, followed by another cycle with epirubicin and ifosfamide. OUTCOMES: The chemotherapy was ineffective. After the two cycles, the bilateral pleural effusion steadily increased, the patient had severe dyspnea and palpitation, and died three weeks later, with an overall survival of 2.5 months. LESSONS: Primary angiosarcoma of heart is a very rare and aggressive disease, and its diagnosis and treatment are difficult. Most patients may have systemic metastasis at diagnosis, and have a very short survival without surgical resection. Hence, early diagnosis and surgical resection is extremely important to treat this disease.