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1. Raphael C, Hudson E, Williams L, Lester JF, Savage PM: Successful treatment of metastatic hepatic epithelioid hemangioendothelioma with thalidomide: a case report. J Med Case Rep; 2010;4:413
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Successful treatment of metastatic hepatic epithelioid hemangioendothelioma with thalidomide: a case report.
  • INTRODUCTION: Hepatic epithelioid hemangioendothelioma is a rare malignancy arising from the vascular endothelial cells within the liver.
  • Historically, the disease is characterized as being poorly responsive to both chemotherapy and radiotherapy, with liver resection or transplantation the treatment of choice when feasible.
  • For patients with advanced disease, reports of long-term therapeutic benefits from conventional cytotoxic treatments are very limited.
  • Owing to the rarity of this malignancy, there is no structured therapeutic research, but a small number of cases have been reported to respond well to treatment with inhibitors of angiogenesis.
  • Thalidomide was originally developed as an anti-emetic but is a potent inhibitor of vascular neogenesis, and could offer potential in the treatment of hepatic epithelioid hemangioendothelioma by blocking the proliferation of the malignant vascular endothelial cells.
  • CASE PRESENTATION: We describe the case of a Caucasian British woman who presented at the age of 53 years with a hepatic mass, malignant lymphadenopathy and pulmonary metastases, which were confirmed as hepatic epithelioid hemangioendothelioma on biopsy.
  • After unproductive treatment with interferon, our patient was started on thalidomide 400 mg daily.
  • She has been successfully managed on this therapy for the past seven years, and has remained asymptomatic, with radiologically stable disease and minimal treatment-related side effects.
  • CONCLUSION: At present, there is no standard therapy for advanced hepatic epithelioid hemangioendothelioma.
  • Our case supports the role for thalidomide and potentially other inhibitors of vascular neogenesis in the treatment of patients with metastatic hepatic epithelioid hemangioendothelioma.

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  • (PMID = 21176188.001).
  • [ISSN] 1752-1947
  • [Journal-full-title] Journal of medical case reports
  • [ISO-abbreviation] J Med Case Rep
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC3022673
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2. Emamaullee JA, Edgar R, Toso C, Thiesen A, Bain V, Bigam D, Kneteman N, Shapiro AM: Vascular endothelial growth factor expression in hepatic epithelioid hemangioendothelioma: Implications for treatment and surgical management. Liver Transpl; 2010 Feb;16(2):191-7
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  • [Title] Vascular endothelial growth factor expression in hepatic epithelioid hemangioendothelioma: Implications for treatment and surgical management.
  • Epithelioid hemangioendothelioma (EHE) is a low-grade, malignant vascular tumor that most commonly presents within the liver.
  • Patients with hepatic EHE are often candidates for liver transplantation as the disease is usually multifocal at diagnosis.
  • Although these patients achieve excellent early outcomes post-transplant, there are very few data regarding tumor markers that can further direct chemotherapy in hepatic EHE to prevent recurrent disease.
  • These data suggest that anti-VEGF chemotherapeutic agents will be of use in patients with hepatic EHE, particularly as a means of reducing the tumor volume prior to resection, as a means of treating unresectable or metastatic disease, or as an adjuvant therapy in the setting of liver transplantation.
  • [MeSH-major] Hemangioendothelioma, Epithelioid / metabolism. Hemangioendothelioma, Epithelioid / surgery. Liver Neoplasms / metabolism. Liver Neoplasms / surgery. Liver Transplantation. Vascular Endothelial Growth Factor A / metabolism
  • [MeSH-minor] Adolescent. Adult. Angiogenesis Inhibitors / therapeutic use. Biomarkers, Tumor / metabolism. Biopsy. Disease-Free Survival. Female. Humans. Immunohistochemistry. Male. Middle Aged. Neoplasm Recurrence, Local / metabolism. Neoplasm Recurrence, Local / pathology. Neovascularization, Pathologic / drug therapy. Neovascularization, Pathologic / metabolism. Neovascularization, Pathologic / pathology. Retrospective Studies. Vascular Endothelial Growth Factor Receptor-1 / metabolism. Vascular Endothelial Growth Factor Receptor-2 / metabolism

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  • (PMID = 20104492.001).
  • [ISSN] 1527-6473
  • [Journal-full-title] Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society
  • [ISO-abbreviation] Liver Transpl.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Biomarkers, Tumor; 0 / VEGFA protein, human; 0 / Vascular Endothelial Growth Factor A; EC 2.7.10.1 / FLT1 protein, human; EC 2.7.10.1 / Vascular Endothelial Growth Factor Receptor-1; EC 2.7.10.1 / Vascular Endothelial Growth Factor Receptor-2
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3. Mehrabi A, Kashfi A, Fonouni H, Schemmer P, Schmied BM, Hallscheidt P, Schirmacher P, Weitz J, Friess H, Buchler MW, Schmidt J: Primary malignant hepatic epithelioid hemangioendothelioma: a comprehensive review of the literature with emphasis on the surgical therapy. Cancer; 2006 Nov 1;107(9):2108-21
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  • [Title] Primary malignant hepatic epithelioid hemangioendothelioma: a comprehensive review of the literature with emphasis on the surgical therapy.
  • Malignant hepatic epithelioid hemangioendothelioma (HEH) is a rare malignant tumor of vascular origin with unknown etiology and a variable natural course.
  • The authors present a comprehensive review of the literature on HEH with a focus on clinical outcome after different therapeutic strategies.
  • The reviewed parameters included demographic data, clinical manifestations, therapeutic modalities, and clinical outcome.
  • Most patients presented with multifocal tumor that involved both lobes of the liver.
  • Lung, peritoneum, lymph nodes, and bone were the most common sites of extrahepatic involvement at the time of diagnosis.
  • The most common management has been liver transplantation (LTx) (44.8% of patients), followed by no treatment (24.8% of patients), chemotherapy or radiotherapy (21% of patients), and liver resection (LRx) (9.4% of patients).
  • The 1-year and 5-year patient survival rates were 96% and 54.5%, respectively, after LTx; 39.3% and 4.5%, respectively, after no treatment, 73.3% and 30%, respectively, after chemotherapy or radiotherapy; and 100% and 75%, respectively, after LRx.
  • LRx has been the treatment of choice in patients with resectable HEH.
  • However, LTx has been proposed as the treatment of choice because of the hepatic multicentricity of HEH.
  • The role of different adjuvant therapies for patients with HEH remains to be determined.
  • [MeSH-major] Hemangioendothelioma, Epithelioid / diagnosis. Hemangioendothelioma, Epithelioid / surgery. Liver Neoplasms / diagnosis. Liver Neoplasms / surgery
  • [MeSH-minor] Adult. Combined Modality Therapy. Diagnosis, Differential. Female. Hepatectomy. Humans. Liver Transplantation. Male. Neoplasm Metastasis. Survival Rate

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  • [Copyright] (c) 2006 American Cancer Society.
  • (PMID = 17019735.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 101
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4. Grotz TE, Nagorney D, Donohue J, Que F, Kendrick M, Farnell M, Harmsen S, Mulligan D, Nguyen J, Rosen C, Reid-Lombardo KM: Hepatic epithelioid haemangioendothelioma: is transplantation the only treatment option? HPB (Oxford); 2010 Oct;12(8):546-53
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  • [Title] Hepatic epithelioid haemangioendothelioma: is transplantation the only treatment option?
  • BACKGROUND: Hepatic epithelioid haemangioendothelioma (HEH) is a rare vascular neoplasm with unpredictable clinical behaviour.
  • AIM: To compare overall survival (OS) and disease-free survival (DFS) between liver resection (LR) and orthotopic liver transplantation (OLT) for the treatment of HEH.
  • RESULTS: Median age was 46 years with a female predominance of 2:1.
  • Treatment included LR (n= 11), OLT (n= 11), chemotherapy (n= 5) and no treatment (n= 3).
  • LR was associated with a 1-, 3- and 5-year OS of 100%, 86% and 86% and a DFS of 78%, 62% and 62%, respectively.
  • OLT was associated with a 1-, 3- and 5-year OS of 91%, 73% and 73% and a DFS 64%, 46% and 46%, respectively.
  • Important predictors of a favourable OS and DFS were largest tumour ≤ 10 cm and multifocal disease with ≤ 10 nodules.
  • CONCLUSION: LR and OLT achieve comparable results in the treatment of HEH.
  • Metastases may not be a contraindication to surgical treatment.
  • [MeSH-major] Hemangioendothelioma, Epithelioid / surgery. Hepatectomy. Liver Neoplasms / surgery. Liver Transplantation
  • [MeSH-minor] Adult. Aged. Antineoplastic Agents / therapeutic use. Chi-Square Distribution. Disease-Free Survival. Female. Humans. Kaplan-Meier Estimate. Male. Middle Aged. Palliative Care. Proportional Hazards Models. Retrospective Studies. Risk Assessment. Risk Factors. Survival Rate. Time Factors. Treatment Outcome. United States. Young Adult

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  • [Copyright] © 2010 International Hepato-Pancreato-Biliary Association.
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  • (PMID = 20887322.001).
  • [ISSN] 1477-2574
  • [Journal-full-title] HPB : the official journal of the International Hepato Pancreato Biliary Association
  • [ISO-abbreviation] HPB (Oxford)
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Multicenter Study
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Other-IDs] NLM/ PMC2997660
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5. Uchimura K, Nakamuta M, Osoegawa M, Takeaki S, Nishi H, Iwamoto H, Enjoji M, Nawata H: Hepatic epithelioid hemangioendothelioma. J Clin Gastroenterol; 2001 May-Jun;32(5):431-4
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  • [Title] Hepatic epithelioid hemangioendothelioma.
  • Epithelioid hemangioendothelioma (EHE) is a rare neoplasm of vascular origin with unpredictable malignant potential.
  • The clinical aspects of EHE and relevant treatment results in Japanese patients were similar to those found in Western countries, the exception being the matter of difference in incidence between genders.
  • Although this tumor type has been reported to be more frequent among women, there was no significant difference in incidence between men and women.
  • Hepatic EHE generally behaves as a low-grade malignant tumor with a slow progression phenotype; however, this disease seems to be resistant to chemotherapy and to be lethal in some cases.
  • Surgical resection or liver transplantation is recommended after rapid diagnosis by radiologic examination and histologic findings, including positive staining of tumor cells for factor VIII-related antigen.
  • [MeSH-major] Hemangioendothelioma, Epithelioid. Liver Neoplasms
  • [MeSH-minor] Adult. Fatal Outcome. Humans. Male. Middle Aged. Tomography, X-Ray Computed

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  • (PMID = 11319317.001).
  • [ISSN] 0192-0790
  • [Journal-full-title] Journal of clinical gastroenterology
  • [ISO-abbreviation] J. Clin. Gastroenterol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 7
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6. Gupta R, Mathur SR, Gupta SD, Durgapal P, Iyer VK, Das CJ, Shalimar, Acharya SK: Hepatic epithelioid hemangioendothelioma: A diagnostic pitfall in aspiration cytology. Cytojournal; 2010;6:25

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Hepatic epithelioid hemangioendothelioma: A diagnostic pitfall in aspiration cytology.
  • Hepatic epithelioid hemangioendothelioma (EH) is a rare vascular neoplasm.
  • An accurate radiologic diagnosis is usually difficult due to the presence of multiple nodules, simulating metastatic carcinoma.
  • Though histologic features of this tumor are well described, cytologic reports of hepatic EH are very few in the available literature.
  • The diagnosis was made on core biopsy assisted by immunohistochemistry, which showed characteristic features of EH.
  • He is doing well 14 months after diagnosis, without surgical excision or chemotherapy.
  • An accurate diagnosis of hepatic EH on aspiration cytology requires an adequate specimen and awareness of its cytologic features, including discohesive atypical cells with intracytoplasmic lumina and intranuclear inclusions.
  • Since this tumor is usually unresectable but has a favorable prognosis as compared to hepatocellular carcinoma, a correct diagnosis is essential for appropriate management and prognostication.

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  • (PMID = 20165548.001).
  • [ISSN] 1742-6413
  • [Journal-full-title] CytoJournal
  • [ISO-abbreviation] Cytojournal
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] India
  • [Other-IDs] NLM/ PMC2822180
  • [Keywords] NOTNLM ; Aspiration cytology / epithelioid hemangioendothelioma (EH) / histopathology / immunohistochemistry / liver
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7. Neves N, Lima-Rodrigues F, Ribeiro-Silva M, Cacho-Rodrigues P, Eloy C, Paiva ME, Pinto R: Epithelioid hemangioendothelioma presenting as a vertebral fracture. Acta Reumatol Port; 2010 Jul-Sep;35(3):370-4
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  • [Title] Epithelioid hemangioendothelioma presenting as a vertebral fracture.
  • Epithelioid hemagioendothelioma (EH) is a rare vascular tumor with an intermediate biological behavior between hemangioma and angiosarcoma.
  • Vertebral location is even more rare, and because the number of reported cases of EH is small and the follow-up periods short, the best surgical treatment, the role of radiotherapy and chemotherapy, as well as the definitive prognosis are still not established.
  • Treatment included vertebrectomy, with complete excision of the lesion, spinal canal decompression and vertebral stabilization.
  • Anatomopathological study revealed an epithelioid vascular neoplasm with low mitotic index, and tumor cells reactive to vimentin, CD31 and CD34, leading to the diagnosis of Grade I Epithelioid Hemangioendothelioma.
  • Because of the wide resection achieved and the low aggressiveness of the lesion, no adjuvant radio or chemotherapy was undertaken, and at 6 years follow-up there are no signs of recurrence or metastasis.
  • [MeSH-major] Hemangioendothelioma, Epithelioid / complications. Lumbar Vertebrae / injuries. Spinal Fractures / etiology

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  • (PMID = 20975643.001).
  • [ISSN] 0303-464X
  • [Journal-full-title] Acta reumatológica portuguesa
  • [ISO-abbreviation] Acta Reumatol Port
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Portugal
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8. Naqvi J, Ordonez NG, Luna MA, Williams MD, Weber RS, El-Naggar AK: Epithelioid hemangioendothelioma of the head and neck: role of podoplanin in the differential diagnosis. Head Neck Pathol; 2008 Mar;2(1):25-30
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  • [Title] Epithelioid hemangioendothelioma of the head and neck: role of podoplanin in the differential diagnosis.
  • Epithelioid hemangioendothelioma is an uncommon vascular tumor of soft tissue and bone that may rarely occur in the liver, lung and the head and neck.
  • We present five new cases of epithelioid hemangioendothelioma of the head and neck region diagnosed and managed in one institution in order to define the phenotypic characteristics, podoplanin immunohistochemical staining and the biological outcome.
  • The lesions were found in the gingiva, submandibular region soft tissue, nasal cavity and tongue, and ranged in size from 0.7 to 2.5 cm.
  • All tumors manifested infiltrative cords and nests of epithelioid cells with occasional spindle morphology in a myxoid stroma.
  • Immunohistochemical analysis of vascular and epithelial markers showed strong and uniform cytoplasmic reactivity for podoplanin and variable intensity and staining of CD31 and lack of cytokeratin staining in tumor cells.
  • Surgical treatment included simple and wide local excisions.
  • Of the three patients with follow-up, one developed lymph node metastasis and one had no evidence of disease 10 months after surgery.
  • The patient with multiple recurrences and LN metastases was additionally treated with chemotherapy and is under consideration for radiation therapy.
  • Hemangioendothelioma of the head and neck is:.
  • (1) a low-grade malignancy with a tendency for local recurrence and regional lymph node metastasis, (2) complete excision with negative margins is the treatment of choice for localized disease and (3) podoplanin may be useful in differentiating epithelioid hemangioendothelioma from non-vascular tumors.
  • [MeSH-major] Head and Neck Neoplasms / diagnosis. Hemangioendothelioma, Epithelioid / diagnosis. Membrane Glycoproteins / metabolism
  • [MeSH-minor] Adolescent. Aged. Biomarkers, Tumor / metabolism. Child, Preschool. Combined Modality Therapy. Diagnosis, Differential. Female. Humans. Male. Middle Aged. Treatment Outcome

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  • (PMID = 20614338.001).
  • [ISSN] 1936-0568
  • [Journal-full-title] Head and neck pathology
  • [ISO-abbreviation] Head Neck Pathol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Membrane Glycoproteins; 0 / PDPN protein, human
  • [Other-IDs] NLM/ PMC2807604
  • [Keywords] NOTNLM ; Biological outcome / Differential diagnoses / Epithelioid hemangioendothelioma / Immunohistochemistry / Podoplanin
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9. Celikel C, Yumuk PF, Basaran G, Yildizeli B, Kodalli N, Ahiskali R: Epithelioid hemangioendothelioma with multiple organ involvement. APMIS; 2007 Jul;115(7):881-8
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  • [Title] Epithelioid hemangioendothelioma with multiple organ involvement.
  • Epithelioid hemangioendothelioma is a rare vascular neoplasm of uncertain malignant potential.
  • Various reports document metastatic or concurrent epithelioid hemangioendothelioma in several sites, most commonly with combined lung and liver involvement.
  • The concurrent involvement of multiple sites at presentation may cause diagnostic problems because epithelioid hemangioendothelioma can mimic other neoplastic processes.
  • Although it is a chemo-resistant disease, chemotherapy is usually advised for patients with metastatic or concurrent involvement.
  • Here we document the presentation, treatment, and outcome of two cases with concurrent involvement of the lung and liver.
  • [MeSH-major] Hemangioendothelioma, Epithelioid / radiography. Liver Neoplasms / radiography. Lung Neoplasms / radiography
  • [MeSH-minor] Female. Humans. Male. Middle Aged. Treatment Outcome

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  • (PMID = 17614859.001).
  • [ISSN] 0903-4641
  • [Journal-full-title] APMIS : acta pathologica, microbiologica, et immunologica Scandinavica
  • [ISO-abbreviation] APMIS
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Denmark
  • [Number-of-references] 30
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10. Ashraf S, Ashraf HM, Mamoon N, Luqman M: Epithelioid hemangioendothelioma of the liver. J Coll Physicians Surg Pak; 2007 May;17(5):280-2
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Epithelioid hemangioendothelioma of the liver.
  • Epithelioid hemangioendothelioma is an intermediate grade malignant neoplasm of vascular origin.
  • The tumor involves the liver and lungs, but other organs are affected too.
  • The key to the diagnosis is identification of cells of endothelial origin containing Factor VIII R antigen.
  • Surgical resection of isolated lesions is the treatment of choice; with unpredictable results reported for chemotherapy, radiotherapy, and resection of multiple lesions.
  • [MeSH-major] Hemangioendothelioma, Epithelioid / diagnosis. Hemangioendothelioma, Epithelioid / surgery. Liver Neoplasms / diagnosis. Liver Neoplasms / surgery

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  • (PMID = 17553327.001).
  • [ISSN] 1022-386X
  • [Journal-full-title] Journal of the College of Physicians and Surgeons--Pakistan : JCPSP
  • [ISO-abbreviation] J Coll Physicians Surg Pak
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Pakistan
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11. Mehrabi A, Kashfi A, Schemmer P, Sauer P, Encke J, Fonouni H, Friess H, Weitz J, Schmidt J, Büchler MW, Kraus TW: Surgical treatment of primary hepatic epithelioid hemangioendothelioma. Transplantation; 2005 Sep 27;80(1 Suppl):S109-12
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Surgical treatment of primary hepatic epithelioid hemangioendothelioma.
  • Epithelioid hemangioendothelioma is a very rare tumor of vascular origin.
  • It can develop in different tissues such as soft tissue, lung, or liver.
  • Hepatic epithelioid hemangioendothelioma (HEH) mostly affects females.
  • The malignant potential of HEH often remains unclear in the individual patient.
  • It can range from benign hemangioma to malignant hemangioendotheliosarcoma.
  • All patients in our series with confirmed histological HEH did not show extrahepatic extension and consequently underwent surgical treatment.
  • One patient died while she was on the waiting list for LTx due to rapid tumor progression.
  • No adjuvant chemotherapy was applied.
  • Until now, no recurrence of local tumor or distant metastases could be observed during follow-up in our series.
  • Early detection and surgical intervention in case of HEH can potentially offer curative treatment.
  • The treatment of first choice appears to be radical liver resection.
  • In our view, LTx represents a potentially important option for patients with a nonresectable tumor.
  • Despite the long waiting time, its often unclear dignity, and a proven progressive growth pattern, living related LTx also plays a potentially important role.
  • The role of adjuvant therapy currently remains unclear.
  • [MeSH-major] Hemangioendothelioma, Epithelioid / surgery. Liver Neoplasms / surgery
  • [MeSH-minor] Adult. Female. Humans. Middle Aged. Retrospective Studies. Survivors. Treatment Outcome

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  • (PMID = 16286886.001).
  • [ISSN] 0041-1337
  • [Journal-full-title] Transplantation
  • [ISO-abbreviation] Transplantation
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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12. Kassam A, Mandel K: Metastatic hepatic epithelioid hemangioendothelioma in a teenage girl. J Pediatr Hematol Oncol; 2008 Jul;30(7):550-2
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Metastatic hepatic epithelioid hemangioendothelioma in a teenage girl.
  • SUMMARY: Hepatic epithelioid hemangioendothelioma (HEHE) is a rare malignant tumor characterized by its epithelioid structure and vascular endothelium origin.
  • The clinical course of HEHE is variable, ranging from long-term survival without treatment to a rapidly progressive course with a fatal outcome.
  • As a consequence, no standard treatment has been determined.
  • We present a case of HEHE occurring in a 13-year-old girl, in which a novel treatment approach using antiangiogenic therapy was tried and was successful in slowing the progression of the disease.
  • [MeSH-major] Hemangioendothelioma, Epithelioid / secondary. Liver Neoplasms / drug therapy. Lung Neoplasms / secondary
  • [MeSH-minor] Adolescent. Angiogenesis Inhibitors / administration & dosage. Angiogenesis Inhibitors / therapeutic use. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Carboplatin / administration & dosage. Celecoxib. Cisplatin / administration & dosage. Disease Progression. Doxorubicin / administration & dosage. Drug Therapy, Combination. Female. Humans. Interferon-alpha / therapeutic use. Liver Failure / etiology. Liver Failure / surgery. Liver Transplantation / ethics. Neovascularization, Pathologic / drug therapy. Neovascularization, Pathologic / etiology. Pyrazoles / administration & dosage. Pyrazoles / therapeutic use. Recombinant Proteins. Sulfonamides / administration & dosage. Sulfonamides / therapeutic use. Thalidomide / administration & dosage. Thalidomide / therapeutic use. Vinblastine / administration & dosage. Vinblastine / therapeutic use

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  • Hazardous Substances Data Bank. CIS-DIAMINEDICHLOROPLATINUM .
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  • (PMID = 18797205.001).
  • [ISSN] 1536-3678
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Interferon-alpha; 0 / Pyrazoles; 0 / Recombinant Proteins; 0 / Sulfonamides; 4Z8R6ORS6L / Thalidomide; 5V9KLZ54CY / Vinblastine; 76543-88-9 / interferon alfa-2a; 80168379AG / Doxorubicin; BG3F62OND5 / Carboplatin; JCX84Q7J1L / Celecoxib; Q20Q21Q62J / Cisplatin
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13. Calabrò L, Di Giacomo AM, Altomonte M, Fonsatti E, Mazzei MA, Volterrani L, Miracco C, Maio M: Primary hepatic epithelioid hemangioendothelioma progressively responsive to interferon-alpha: is there room for novel anti-angiogenetic treatments? J Exp Clin Cancer Res; 2007 Mar;26(1):145-50
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary hepatic epithelioid hemangioendothelioma progressively responsive to interferon-alpha: is there room for novel anti-angiogenetic treatments?
  • Primary hepatic epithelioid hemangioendothelioma (HEH) is a rare, low-grade malignant neoplasm of endothelial origin, with an unpredictable clinical course and prognosis.
  • No standard therapeutic strategies are still available for HEH, due to the infrequency of the disease and to its variable natural history that limit the identification of the most effective treatment.
  • In the absence of metastatic disease, surgical resection or liver transplantation represent the treatment of choice for HEH, while several antineoplastic agents have been proposed in the presence of metastatic nonresectable disesase.
  • Herein, we describe the biological characterization and the clinical course of a primary HEH progressively responsive to treatment with intermediate doses of interferon-alpha (IFN)-alpha2a.
  • [MeSH-major] Angiogenesis Inhibitors / therapeutic use. Hemangioendothelioma, Epithelioid / drug therapy. Interferon-alpha / therapeutic use. Liver Neoplasms / drug therapy. Neovascularization, Pathologic / prevention & control
  • [MeSH-minor] Antigens, CD / analysis. Antigens, CD31 / analysis. Antigens, CD34 / analysis. Female. Humans. Immunohistochemistry. Middle Aged. Receptors, Cell Surface / analysis. Recombinant Proteins. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 17550144.001).
  • [ISSN] 0392-9078
  • [Journal-full-title] Journal of experimental & clinical cancer research : CR
  • [ISO-abbreviation] J. Exp. Clin. Cancer Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Antigens, CD; 0 / Antigens, CD31; 0 / Antigens, CD34; 0 / ENG protein, human; 0 / Interferon-alpha; 0 / Receptors, Cell Surface; 0 / Recombinant Proteins; 76543-88-9 / interferon alfa-2a
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14. Mascarenhas RC, Sanghvi AN, Friedlander L, Geyer SJ, Beasley HS, Van Thiel DH: Thalidomide inhibits the growth and progression of hepatic epithelioid hemangioendothelioma. Oncology; 2004;67(5-6):471-5
Hazardous Substances Data Bank. THALIDOMIDE .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Thalidomide inhibits the growth and progression of hepatic epithelioid hemangioendothelioma.
  • Hepatic epithelioid hemangioendothelioma (HEH) is a rare vascular tumor of the liver with an unpredictable malignant potential.
  • Surgical resection or liver transplantation is the treatment of choice if metastasis is not identified.
  • We report the case of a 52-year-old patient with HEH metastatic to the lungs who was successfully treated with oral thalidomide therapy.
  • [MeSH-major] Angiogenesis Inhibitors / therapeutic use. Antineoplastic Agents / therapeutic use. Hemangioendothelioma, Epithelioid / drug therapy. Hemangioendothelioma, Epithelioid / pathology. Liver Neoplasms / drug therapy. Liver Neoplasms / pathology. Thalidomide / therapeutic use
  • [MeSH-minor] Disease Progression. Drug Administration Schedule. Humans. Male. Middle Aged. Tomography, X-Ray Computed. Treatment Outcome

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  • [Copyright] Copyright (c) 2004 S. Karger AG, Basel
  • (PMID = 15714004.001).
  • [ISSN] 0030-2414
  • [Journal-full-title] Oncology
  • [ISO-abbreviation] Oncology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Antineoplastic Agents; 4Z8R6ORS6L / Thalidomide
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15. Chartier A, Barbier C, Thumerelle C, Santos C, Desfachelles AS, Devisme L, Deschildre A: [Hypertrophic pulmonary osteo-arthropathy revealed by a pulmonary epithelioid hemangioendothelioma in a 15-year-old girl]. Arch Pediatr; 2003 Jul;10(7):626-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Hypertrophic pulmonary osteo-arthropathy revealed by a pulmonary epithelioid hemangioendothelioma in a 15-year-old girl].
  • Pulmonary epithelioid hemangioendothelioma is a rare vascular tumor of the lung, that may be revealed by hypertrophic pulmonary osteo-arthropathy.
  • A hypertrophic pulmonary osteo-arthropathy due to a paraneoplastic syndrome associated with lung epithelioid hemangioendothelioma was diagnosed.
  • Despite surgical resection and chemotherapy, the patient died 6 months later.
  • Diagnosis is confirmed by long bones proliferative periostitis on standard X-ray examination.
  • Pulmonary epithelioid hemangioendothelioma is a rare vascular malignant tumor, that spreads from the vascular pulmonary or hepatic tissues.
  • The prognosis is variable, depending on the malignancy of the tumor.
  • In the absence of a chronic cardiorespiratory disease, the diagnosis of a pulmonary hypertrophic osteo-arthropathy in childhood should prompt the search for a tumoral cause.
  • [MeSH-major] Hemangioendothelioma, Epithelioid / complications. Hemangioendothelioma, Epithelioid / diagnosis. Lung Neoplasms / complications. Lung Neoplasms / diagnosis. Osteoarthropathy, Primary Hypertrophic / etiology
  • [MeSH-minor] Adolescent. Diagnosis, Differential. Female. Humans

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  • (PMID = 12907072.001).
  • [ISSN] 0929-693X
  • [Journal-full-title] Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie
  • [ISO-abbreviation] Arch Pediatr
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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16. Rosenthal DI, Treat ME, Mankin HJ, Rosenberg AE, Jennings CL: Treatment of epithelioid hemangioendothelioma of bone using a novel combined approach. Skeletal Radiol; 2001 Apr;30(4):219-22
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  • [Title] Treatment of epithelioid hemangioendothelioma of bone using a novel combined approach.
  • Radiofrequency (RF) treatment has been used for a variety of malignant and benign conditions.
  • However, treatment of a bone malignancy has yet to be reported.
  • The authors present a 21-year old woman with multifocal epithelioid hemangioendothelioma (EH) treated by a combination of surgical excision, chemotherapy and four RF ablations.
  • Follow-up radiographs of the RF-treated sites reveal no evidence of recurrent disease 71, 58, 49 and 33 months, respectively, after treatment.
  • [MeSH-major] Bone Neoplasms / therapy. Hemangioendothelioma, Epithelioid / therapy
  • [MeSH-minor] Adult. Antineoplastic Agents / therapeutic use. Catheter Ablation. Combined Modality Therapy. Female. Foot Bones. Humans. Interferon-alpha / therapeutic use. Leg Bones

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  • (PMID = 11392296.001).
  • [ISSN] 0364-2348
  • [Journal-full-title] Skeletal radiology
  • [ISO-abbreviation] Skeletal Radiol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Interferon-alpha
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17. Schilling G, Schuch G, Panse JP, Sterneck M, Bokemeyer C: Activity of lenalidomide in metastatic hepatic epithelioid hemangioendothelioma (HEH): A case report. J Clin Oncol; 2009 May 20;27(15_suppl):e21527

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Activity of lenalidomide in metastatic hepatic epithelioid hemangioendothelioma (HEH): A case report.
  • : e21527 Background: HEH is a rare tumor of the liver with an unpredictable malignant potential.
  • Systemic therapy, however, is not standardized.
  • Small cohorts have been treated with interferon, chemotherapy and angiogenesis inhibitors such as thalidomide with various results.
  • Biopsy revealed a tumor with predominant epithelioid cells, positive for CD31 and CD34 and negative for CD117, HHV8, AFP and CEA, classified as a HEH.
  • Disease progressed during first line treatment with adriamycin and ifosfamide (2 cycles given).
  • Because of the remaining extrahepatic lesions it is planned to continue lenalidomide therapy in combination with the immunosuppressant rapamycine.
  • CONCLUSIONS: We report the first case of successful therapy with lenalidomide in HEH.

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  • (PMID = 27963457.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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18. Mucha K, Foroncewicz B, Zieniewicz K, Nyckowski P, Krawczyk M, Cyganek A, Paczek L: Patient with liver epithelioid hemangioendothelioma treated by transplantation: 3 years' observation. Transplant Proc; 2006 Jan-Feb;38(1):231-3
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  • [Title] Patient with liver epithelioid hemangioendothelioma treated by transplantation: 3 years' observation.
  • Epithelioid hemangioendothelioma (EHE) is a rare neoplasm of vascular origin, but unknown etiology that occurs in the liver, lungs and other organs.
  • Its hepatic form (HEHE) generally behaves as a low-grade malignant tumor with a slowly progressive phenotype.
  • Surgical resection or liver transplantation (OLT) has been recommended after diagnosis.
  • The histopathological diagnosis from a needle biopsy was carcinoma cholangiogenes desmoplasticum.
  • For 2 years the patient was treated with chemotherapy combinations.
  • To explain the lack of efficacy of chemotherapy, a laparoscopic biopsy was performed and HEHE diagnosed.
  • After 3 years observation the patient presented with good liver function and no signs of tumor recurrence.
  • We concluded that immunohistochemical staining for characteristic endothelial cell markers may facilitate the correct diagnosis of HEHE.
  • After diagnosis, OLT followed by immunosuppressive therapy, consisting of basiliximab, corticoids, low doses of tacrolimus and temporary administration of rapamycin, may be safe and effective.
  • [MeSH-major] Hemangioendothelioma, Epithelioid / surgery. Liver Neoplasms / surgery. Liver Transplantation / methods
  • [MeSH-minor] Adult. Female. Follow-Up Studies. Humans. Treatment Outcome


19. Sumrall A, Fredericks R, Berthold A, Shumaker G: Lenalidomide stops progression of multifocal epithelioid hemangioendothelioma including intracranial disease. J Neurooncol; 2010 Apr;97(2):275-7
Hazardous Substances Data Bank. THALIDOMIDE .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Lenalidomide stops progression of multifocal epithelioid hemangioendothelioma including intracranial disease.
  • Epithelioid Hemangioendothelioma (EH) is a rare soft-tissue tumor which may present as an isolated tumor or can spread to affect internal organs.
  • The course of EH varies, based on the tissue of origin.
  • This case report describes a young woman who developed cutaneous EH with concurrent intracranial disease during pregnancy.
  • Even after several courses of chemotherapy and radiotherapy, the patient developed multifocal disease including pulmonary, skeletal, and liver disease.
  • She now exhibits stable disease after approximately 6 years of therapy with lenalidomide.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Brain Neoplasms / drug therapy. Hemangioendothelioma, Epithelioid / drug therapy. Thalidomide / analogs & derivatives
  • [MeSH-minor] Adult. Clinical Trials as Topic. Female. Humans. Magnetic Resonance Imaging. Tomography, X-Ray Computed

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  • [Cites] Br J Cancer. 2004 Mar 8;90(5):955-61 [14997189.001]
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  • (PMID = 19898746.001).
  • [ISSN] 1573-7373
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 4Z8R6ORS6L / Thalidomide; F0P408N6V4 / lenalidomide
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20. Coulibaly B, Tasei AM, Payan-Defais MJ, Bouvier C, Trousse D, Doddoli C, Figarella-Branger D: [Pulmonary epithelioid haemangioendothelioma: two different clinical courses]. Rev Mal Respir; 2008 Sep;25(7):867-70
MedlinePlus Health Information. consumer health - Lung Cancer.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Pulmonary epithelioid haemangioendothelioma: two different clinical courses].
  • In both cases, chest radiography and thoracic CT scans showed multiple, bilateral pulmonary nodules, bronchial endoscopy was normal and surgical biopsy revealed epithelioid haemangioendothelioma.
  • It is a rare primary pulmonary tumour which is usually found in soft tissue, bone or liver.
  • Epithelioid haemangioendothelioma is a vascular tumour usually affecting women less than 40 years of age.
  • Chemotherapy and radiotherapy are generally ineffective and surgery is sometimes impossible because of the multifocal lesions.
  • Epithelioid haemangioendothelioma is considered a sarcoma without precise histological prognostic criteria.
  • [MeSH-major] Hemangioendothelioma, Epithelioid. Lung Neoplasms
  • [MeSH-minor] Biopsy. Female. Follow-Up Studies. Humans. Lung / pathology. Male. Middle Aged. Radiography, Thoracic. Time Factors. Tomography, X-Ray Computed. Treatment Outcome. Young Adult

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  • (PMID = 18946414.001).
  • [ISSN] 0761-8425
  • [Journal-full-title] Revue des maladies respiratoires
  • [ISO-abbreviation] Rev Mal Respir
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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21. Aquilina K, Lim C, Kamel MH, Marks CJ, O'Sullivan MG, Keohane C: Epithelioid hemangioendothelioma of the spine. Report of two cases. J Neurosurg Spine; 2005 Nov;3(5):393-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Epithelioid hemangioendothelioma of the spine. Report of two cases.
  • Epithelioid hemangioendothelioma (EH) is a rare tumor of vascular origin.
  • In the first case the patient underwent resection of the tumor; this case represents the longest reported follow-up period for spinal EH.
  • In the second case, extensive involvement of C-2, C-3, and C-4 as well as encasement of both vertebral arteries precluded safe tumor resection, and posterior occipitocervical stabilization was performed.
  • The findings in these two cases underscore the difficulty in predicting the clinical behavior of spinal EH based solely on histological and clinical features as well as the uncertainty of the roles of surgery, chemotherapy, and radiotherapy in the oncological management of a spinal tumor for which clinical data are very limited.
  • [MeSH-major] Hemangioendothelioma, Epithelioid / surgery. Spinal Neoplasms / surgery

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  • (PMID = 16302636.001).
  • [ISSN] 1547-5654
  • [Journal-full-title] Journal of neurosurgery. Spine
  • [ISO-abbreviation] J Neurosurg Spine
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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22. Hannachi Sassi S, Mansouri D, Abbes I, Dhouib R, Driss M, Mrad K, Ben Ayed F, Ben Romdhane K: [Soft tissue epithelioid hemangioendothelioma: a case report]. Rev Chir Orthop Reparatrice Appar Mot; 2005 Nov;91(7):671-5
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  • [Title] [Soft tissue epithelioid hemangioendothelioma: a case report].
  • [Transliterated title] Hémangio-endothéliome épithélioïde des tissus mous. A propos d'un cas.
  • We report a case of epithelioid hemangioendothelioma observed in soft tissue in a 35-year-old man who presented a painful mass of the right arm which progressed in size for two years.
  • Imaging revealed a 7-cm poorly limited expansive tumor process located in the medial muscle compartment of the right arm.
  • Histological and immunohistochemical examination led to the diagnosis of soft tissue epithelioid hemangioendothelioma.
  • The tumor recurred in the right arm and the right axillary fossa with development of multiple nodules in the contralateral thoracic wall and pleural effusion.
  • Adjuvant chemotherapy and radiotherapy were performed, but the patient died at two years.
  • [MeSH-major] Hemangioendothelioma, Epithelioid. Soft Tissue Neoplasms

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  • (PMID = 16327673.001).
  • [ISSN] 0035-1040
  • [Journal-full-title] Revue de chirurgie orthopédique et réparatrice de l'appareil moteur
  • [ISO-abbreviation] Rev Chir Orthop Reparatrice Appar Mot
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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23. Lopes T, Clemente S, Feliciano A, Lourenço I, Costa A, Gil Duarte J: [Pulmonary epithelioid hemangioendothelioma - rarity, diagnosis and treatment difficulties]. Rev Port Pneumol; 2009 Nov-Dec;15(6):1167-74
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  • [Title] [Pulmonary epithelioid hemangioendothelioma - rarity, diagnosis and treatment difficulties].
  • [Transliterated title] Hemangioendotelioma epitelióide do pulmão - Raridade, dificuldades diagnósticas e terapêutica.
  • The authors report a case of a primary pulmonary epithelioid haemangioendothelioma (EHE) in a 51 year-old man, a mechanic, who complained of a dry cough followed by constitutional symptoms and dyspnoea.
  • He was prescribed tuberculosis drugs for three weeks.
  • The patient underwent three cycles of chemotherapy with carboplatin, etoposide and bevacizumab with no complications.
  • He died seven months after onset of symptoms and seven weeks after definitive diagnosis.
  • The authors wish to highlight the rarity of this pulmonary neoplasm and the importance of clinical suspicion, and the diagnosis and treatment difficulties in addition to the potential benefits of antiangiogenic drugs.
  • [MeSH-major] Hemangioendothelioma, Epithelioid. Lung Neoplasms

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  • (PMID = 19859632.001).
  • [ISSN] 2172-6825
  • [Journal-full-title] Revista portuguesa de pneumologia
  • [ISO-abbreviation] Rev Port Pneumol
  • [Language] por
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Portugal
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24. Wedmid A, Masterson TA, Maki RG, Russo P: A case of high-risk penile epithelioid hemangioendothelioma. Nat Rev Urol; 2009 Apr;6(4):223-7
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  • [Title] A case of high-risk penile epithelioid hemangioendothelioma.
  • Peyronie disease was initially diagnosed, but the disease progressed despite steroid treatment.
  • DIAGNOSIS: Penile epithelioid hemangioendothelioma, which is a multifocal, atypical epithelioid vascular tumor.
  • MANAGEMENT: Single-agent chemotherapy using liposomal doxorubicin hydrochloride for eight cycles, followed by 63 Gy of adjuvant external beam radiation therapy to the penis fractionated over 35 treatments.
  • [MeSH-major] Hemangioendothelioma, Epithelioid / diagnosis. Penile Neoplasms / diagnosis
  • [MeSH-minor] Combined Modality Therapy / methods. Humans. Male. Middle Aged. Risk Factors

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  • (PMID = 19352397.001).
  • [ISSN] 1759-4820
  • [Journal-full-title] Nature reviews. Urology
  • [ISO-abbreviation] Nat Rev Urol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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25. Sanchez-Carpintero I, Martínez MI, Mihm MC Jr: Clinical and histopathologic observations of the action of imiquimod in an epithelioid hemangioendothelioma and Paget's mammary disease. J Am Acad Dermatol; 2006 Jul;55(1):75-9
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  • [Title] Clinical and histopathologic observations of the action of imiquimod in an epithelioid hemangioendothelioma and Paget's mammary disease.
  • An epithelioid hemangioendothelioma and Paget's disease of the breast were treated with topical imiquimod 5% cream with very impressive results.
  • In this article, in addition to describing the treatment approach, the findings in biopsy specimens of the epithelioid hemangioendothelioma and the Paget's disease before, during, and after therapy will be described.
  • Furthermore, mast cells appear to be involved in the development of regression in the vascular tumor.
  • We considered it important to describe these findings because of their possible use in developing strategies for the application of imiquimod in the treatment of other tumors in human beings.
  • [MeSH-major] Aminoquinolines / therapeutic use. Antineoplastic Agents / therapeutic use. Breast Neoplasms / drug therapy. Breast Neoplasms / pathology. Facial Neoplasms / drug therapy. Facial Neoplasms / pathology. Hemangioendothelioma, Epithelioid / drug therapy. Hemangioendothelioma, Epithelioid / pathology. Paget's Disease, Mammary / drug therapy. Paget's Disease, Mammary / pathology

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  • (PMID = 16781296.001).
  • [ISSN] 1097-6787
  • [Journal-full-title] Journal of the American Academy of Dermatology
  • [ISO-abbreviation] J. Am. Acad. Dermatol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Aminoquinolines; 0 / Antineoplastic Agents; 99011-02-6 / imiquimod
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26. André ST, Valente C, Paiva B, Pêgo A, Carvalho L, Luís AS: [Epithelioid hemangioendothelioma of the pleura - A rare presentation of a clinical case]. Rev Port Pneumol; 2010 May-Jun;16(3):477-82
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Epithelioid hemangioendothelioma of the pleura - A rare presentation of a clinical case].
  • [Transliterated title] Hemangioendotelioma epitelióide da pleura - Uma apresentação rara a propósito de um caso clínico.
  • Epithelioid Hemangioendothelioma (EHE) is a vascular tumour with rare pleural presentation.
  • A pleural -pulmonary biopsy carried out by toracotomy established the histological diagnosis of EHE of the pleura.
  • Due to the locally advanced stage of the tumour, chemotherapy with carboplatin and etoposide was prescribed and the patient died 6 months later.
  • This case confirms that pleural EHE has an aggressive behaviour, similar to an angiossarcoma, with a median survival of only a few months after diagnosis.
  • [MeSH-major] Hemangioendothelioma, Epithelioid / diagnosis. Pleural Neoplasms / diagnosis

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  • (PMID = 20635062.001).
  • [ISSN] 2172-6825
  • [Journal-full-title] Revista portuguesa de pneumologia
  • [ISO-abbreviation] Rev Port Pneumol
  • [Language] por
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Portugal
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27. Cronin P, Arenberg D: Pulmonary epithelioid hemangioendothelioma: an unusual case and a review of the literature. Chest; 2004 Feb;125(2):789-93
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pulmonary epithelioid hemangioendothelioma: an unusual case and a review of the literature.
  • We describe a case of pulmonary epithelioid hemangioendothelioma, previously known as intravascular bronchoalveolar tumor, in a 35-year-old woman with an initial diagnosis made by transbronchial biopsy.
  • Although our patient underwent thoracoscopic lung biopsy, the diagnosis was initially made on transbronchial biopsy; to our knowledge, this has not been previously described in the English-language literature.
  • This tumor can affect multiple organs.
  • There is no single effective treatment, though spontaneous regression and response to chemotherapy and interferon are reported.
  • [MeSH-major] Hemangioendothelioma, Epithelioid / pathology. Lung Neoplasms / pathology. Neoplasm Invasiveness / pathology

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  • [CommentIn] Chest. 2005 May;127(5):1870-1; author reply 1871 [15888881.001]
  • (PMID = 14769767.001).
  • [ISSN] 0012-3692
  • [Journal-full-title] Chest
  • [ISO-abbreviation] Chest
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 14
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28. Wen CC, Munarriz R, Goldstein I: Three-chamber priapism in a patient with primary epithelioid hemangioendothelioma of penis. Urology; 2004 Jul;64(1):156-8
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  • [Title] Three-chamber priapism in a patient with primary epithelioid hemangioendothelioma of penis.
  • A 58-year-old man presented with a 6-month history of painful progressive penile firmness, initially diagnosed as Peyronie's disease.
  • Cavernosal biopsies revealed epithelioid hemangioendothelioma, and the metastatic workup found hepatic and pulmonary lesions.
  • [MeSH-major] Diagnostic Errors. Hemangioendothelioma, Epithelioid / complications. Hemangioendothelioma, Epithelioid / diagnosis. Penile Neoplasms / complications. Penile Neoplasms / diagnosis. Priapism / etiology
  • [MeSH-minor] Antineoplastic Agents, Phytogenic / therapeutic use. Biopsy. False Negative Reactions. Fatal Outcome. Humans. Liver Neoplasms / drug therapy. Liver Neoplasms / secondary. Lung Neoplasms / drug therapy. Lung Neoplasms / secondary. Male. Middle Aged. Paclitaxel / therapeutic use. Penile Induration / diagnosis. Urination Disorders / etiology

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  • (PMID = 15245958.001).
  • [ISSN] 1527-9995
  • [Journal-full-title] Urology
  • [ISO-abbreviation] Urology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Phytogenic; P88XT4IS4D / Paclitaxel
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29. Marsh Rde W, Walker MH, Jacob G, Liu C: Breast implants as a possible etiology of epithelioid hemangioendothelioma and successful therapy with interferon-alpha2. Breast J; 2005 Jul-Aug;11(4):257-61
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Breast implants as a possible etiology of epithelioid hemangioendothelioma and successful therapy with interferon-alpha2.
  • Epithelioid hemangioendothelioma is a rare vascular tumor with unpredictable behavior.
  • It often presents as liver or lung nodules and is refractory to conventional chemotherapy.
  • Surgical resection and/or liver transplantation have been the mainstay of therapy, but the results are mixed and unsatisfactory.
  • The implications for other women who have had this type of implant could be significant, and the early use of IFN-alpha may be optimal.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Breast Implants / adverse effects. Hemangioendothelioma, Epithelioid / drug therapy. Hemangioendothelioma, Epithelioid / etiology. Interferon-alpha / therapeutic use. Liver Neoplasms / drug therapy. Liver Neoplasms / etiology. Lung Neoplasms / drug therapy. Lung Neoplasms / etiology. Silicone Elastomers / adverse effects


30. Deyrup AT, Tighiouart M, Montag AG, Weiss SW: Epithelioid hemangioendothelioma of soft tissue: a proposal for risk stratification based on 49 cases. Am J Surg Pathol; 2008 Jun;32(6):924-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Epithelioid hemangioendothelioma of soft tissue: a proposal for risk stratification based on 49 cases.
  • Epithelioid hemangioendothelioma (EHE) of soft tissue is a distinctive vascular tumor that has been variously considered a tumor of borderline malignancy and low-grade angiosarcoma.
  • We analyzed 49 cases of EHE to determine if a method for stratifying risk for mortality could be developed.
  • Tumors were evaluated with respect to location, size, cytologic atypia, mitotic activity, tumor cell spindling, and necrosis.
  • They developed in the head and neck (6), extremities (32), mediastinum (4), trunk (4), genitals (2), and retroperitoneum (1) and ranged in size from 0.5 to 18 cm.
  • Eleven patients (22%) had metastatic disease affecting lung (6), lymph node (4), liver (2), and bone, retroperitoneum, and soft tissue (1 each).
  • Treatment modality was known for 46 patients: 31 were treated surgically and 15 were treated with surgery and chemotherapy and/or radiation therapy.
  • Tumor site, cytologic atypia, the presence of necrosis, and tumor spindling were not significant.
  • [MeSH-major] Hemangioendothelioma, Epithelioid / diagnosis. Soft Tissue Neoplasms / diagnosis

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  • (PMID = 18551749.001).
  • [ISSN] 1532-0979
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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31. Koh YC, Yoo H: Epithelioid haemangioendothelioma of the sphenoid bone. J Clin Neurosci; 2001 May;8 Suppl 1:63-6
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  • [Title] Epithelioid haemangioendothelioma of the sphenoid bone.
  • A 26-year-old woman presented with a 1-year history of progressing exophthalmos in the left eye.
  • Histopathological examination revealed typical epithelioid cell cords or nests in myxoid stroma with a positive immunoreactivity to factor VIII-related antigen.
  • Because there is no convincing data to advise radiation/chemotherapy, total resection and close follow-up may be reasonable.
  • [MeSH-major] Hemangioendothelioma, Epithelioid / pathology. Pregnancy Complications, Neoplastic / pathology. Puerperal Disorders / pathology. Skull Neoplasms / pathology. Sphenoid Bone / pathology
  • [MeSH-minor] Adult. Blood Loss, Surgical / prevention & control. Embolization, Therapeutic. Exophthalmos / etiology. Female. Humans. Neovascularization, Pathologic / etiology. Orbital Neoplasms / complications. Orbital Neoplasms / pathology. Orbital Neoplasms / surgery. Pregnancy. Vision Disorders / etiology

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  • [Copyright] Copyright 2001 Harcourt Publishers Ltd.
  • (PMID = 11386829.001).
  • [ISSN] 0967-5868
  • [Journal-full-title] Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
  • [ISO-abbreviation] J Clin Neurosci
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Scotland
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32. Kanizaj TF, Cvrlje VC, Mrzljak A, Kardum-Skelin I, Sustercić D, Segro D, Gustin D, Kocman B: Epitheloid hemangioendothelioma in patient with liver transplantation. Coll Antropol; 2010 Mar;34(1):177-80
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  • [Title] Epitheloid hemangioendothelioma in patient with liver transplantation.
  • Malignant hepatic epithelioid hemangioendothelioma (HEH) is a rare malignant tumor of vascular origin with unknown aetiology and a variable natural course.
  • At the time of diagnosis, most patients present with multifocal tumours lesions that involve both liver lobes.
  • From the therapeutic aspect, liver resection (LRx), liver transplantation (LTx), chemotherapy, radiotherapy, and/or immunotherapy have been used in the treatment of patients with HEH.
  • However, because of the rarity of this tumor and its unpredictable natural history, it is impossible to assess the effectiveness of these respective therapies.
  • In this report, our objective was to present clinical aspects, diagnostic options, therapeutic modalities, and the clinical outcome of single patient with LTx because of this rare tumor.
  • [MeSH-major] Hemangioendothelioma, Epithelioid / pathology. Hemangioendothelioma, Epithelioid / surgery. Liver Neoplasms / pathology. Liver Neoplasms / surgery. Liver Transplantation

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  • (PMID = 20432748.001).
  • [ISSN] 0350-6134
  • [Journal-full-title] Collegium antropologicum
  • [ISO-abbreviation] Coll Antropol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Croatia
  • [Chemical-registry-number] 0 / Antigens, CD31; 0 / Antigens, CD34; 0 / May-Grunwald Giemsa; T42P99266K / Methylene Blue; TDQ283MPCW / Eosine Yellowish-(YS)
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33. Coppo P, Lassoued S, Billey T, Lassoued K: Successful treatment of osteolytic epithelioid hemangioendothelioma with pamidronate. Clin Exp Rheumatol; 2005 May-Jun;23(3):400-1
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  • [Title] Successful treatment of osteolytic epithelioid hemangioendothelioma with pamidronate.
  • We describe the case of a 70-year-old man with unicentric grade 1 epitheloid hemangioendothelioma (EH) of the bone that favourably responded to intravenous pamidronate as a single agent.
  • We suggest that use of bisphosphonates should be considered in the treatment of osteolytic EH.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Bone Neoplasms / drug therapy. Diphosphonates / therapeutic use. Hemangioendothelioma / drug therapy. Osteolysis / drug therapy
  • [MeSH-minor] Aged. Disease-Free Survival. Fingers / pathology. Humans. Male. Radiography. Treatment Outcome. Wrist / pathology

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  • (PMID = 15971432.001).
  • [ISSN] 0392-856X
  • [Journal-full-title] Clinical and experimental rheumatology
  • [ISO-abbreviation] Clin. Exp. Rheumatol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Diphosphonates; OYY3447OMC / pamidronate
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34. Otte JB, de Ville de Goyet J: The contribution of transplantation to the treatment of liver tumors in children. Semin Pediatr Surg; 2005 Nov;14(4):233-8
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  • [Title] The contribution of transplantation to the treatment of liver tumors in children.
  • Major progress has been achieved during the last decades in the treatment of malignant liver tumors in children, both in chemotherapy and surgical management.
  • Chemosensitivity varies between tumor types, and radical resection remains essential to effect a cure.
  • In tumors extensively involving a normal liver, in a diffuse or multifocal manner, radical resection cannot be accomplished with a partial hepatectomy.
  • This has been the case for some instances of advanced hepatoblastoma and epithelioid hemangioendothelioma.
  • In hepatoblastoma, current experience shows that results of primary liver transplantation with neoadjuvant chemotherapy are excellent with around an 80% 5-to-10-year disease-free survival rate.
  • Epithelioid hemangioendothelioma is very rarely seen in children and may have a more malignant behavior than in adult patients, and liver transplantation may not be the best management option.
  • In nonresectable hepatocellular carcinoma (HCC) developed on an otherwise normal liver, the results of liver transplantation are similarly poor to those obtained in adult patients, except in a few highly selected series fulfilling the Milano criteria.
  • When they are symptomatic, however, indications for transplantation should be very selective regarding tumor size, multi-focality, vascular invasion and distant metastases.
  • [MeSH-minor] Carcinoma, Hepatocellular / surgery. Child. Hemangioendothelioma, Epithelioid / surgery. Hepatoblastoma / surgery. Humans

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  • (PMID = 16226698.001).
  • [ISSN] 1055-8586
  • [Journal-full-title] Seminars in pediatric surgery
  • [ISO-abbreviation] Semin. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 38
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35. Zhang PJ, Brooks JS, Goldblum JR, Yoder B, Seethala R, Pawel B, Gorman JH, Gorman RC, Huang JH, Acker M, Narula N: Primary cardiac sarcomas: a clinicopathologic analysis of a series with follow-up information in 17 patients and emphasis on long-term survival. Hum Pathol; 2008 Sep;39(9):1385-95
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  • Although cardiac sarcomas are rare in comparison to their soft tissue counterparts, they are the second most common type of primary cardiac neoplasm.
  • There were 6 angiosarcomas, 6 myxofibrosarcomas, 3 malignant peripheral nerve sheath tumors, 3 leiomyosarcomas, 2 synovial sarcomas, 1 epithelioid hemangioendothelioma, 1 chondrosarcoma, 1 osteosarcoma, and 4 poorly differentiated sarcomas.
  • Six high-grade and 1 low-grade tumors were also treated with adjuvant chemotherapy and/or radiation.
  • In 17 patients with follow-up data, 6 of 12 patients with high-grade tumor died (4 within 5 days of the initial surgery, 1 in 21 months, and 1 in 131 months), and 1 patient with moderate-grade tumor and all 4 patients with low-grade tumor were alive without evidence of disease at the end of follow-up.
  • Tumor grade appeared to be prognostically important in cardiac sarcoma.
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Infant. Male. Middle Aged. Survival Analysis

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  • (PMID = 18602663.001).
  • [ISSN] 1532-8392
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Grant] United States / NHLBI NIH HHS / HL / R01 HL063954
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ NIHMS522251; NLM/ PMC4081532
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36. Almogy G, Lieberman S, Gips M, Pappo O, Edden Y, Jurim O, Simon Slasky B, Uzieli B, Eid A: Clinical outcomes of surgical resections for primary liver sarcoma in adults: results from a single centre. Eur J Surg Oncol; 2004 May;30(4):421-7
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  • BACKGROUND: Primary hepatic sarcoma is a rare tumour with a poor prognosis.
  • The clinical characteristics, imaging findings, surgical procedures, adjuvant therapy and outcome were retrospectively reviewed.
  • There were two patients each with angiosarcoma (AS), leiomyosarcoma (LMS), and undifferentiated embryonal sarcoma (UES), one patient with epithelioid hemangioendothelioma (EHE) and one patient with malignant peripheral nerve sheath sarcoma (PNSS).
  • Preoperative diagnosis of a primary liver sarcoma was made in 7/8 cases, either by fine needle aspiration (n = 5) or angiography (n = 2).
  • Two patients developed complications and there was one death.
  • Systemic chemotherapy led to tumour regression in both patients with UES which enabled a second hepatic resection.
  • Differentiating the rare primary liver sarcoma from the much more common hepatocellular carcinoma (HCC) may aid in planning therapy.
  • Liver resection combined with adjuvant chemotherapy are the mainstays of treatment for UES in the adult.
  • [MeSH-minor] Adult. Aged. Chemotherapy, Adjuvant. Diagnosis, Differential. Female. Hepatectomy / methods. Humans. Length of Stay. Liver Function Tests. Male. Middle Aged. Retrospective Studies. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 15063896.001).
  • [ISSN] 0748-7983
  • [Journal-full-title] European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology
  • [ISO-abbreviation] Eur J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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37. Malagón HD, Valdez AM, Moran CA, Suster S: Germ cell tumors with sarcomatous components: a clinicopathologic and immunohistochemical study of 46 cases. Am J Surg Pathol; 2007 Sep;31(9):1356-62
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  • The germ cell component consisted of pure mature or immature teratoma (23 cases), teratoma mixed with other seminomatous or nonseminomatous components (17), pure seminoma (2), intratubular germ cell neoplasia (1), and yolk sac tumor (1).
  • The SC included embryonal rhabdomyosarcoma (29), angiosarcoma (6), leiomyosarcoma (4), undifferentiated sarcoma (3), myxoid liposarcoma (1), malignant peripheral nerve sheath tumor (1), malignant "triton" tumor (1), and epithelioid hemangioendothelioma (1).
  • All patients were treated by cisplatinum-based chemotherapy plus other agents followed by surgery.
  • Thirty-two of 40 patients either died of tumor (25/40; 62.5%) or were alive with advanced, progressive disease (7/40; 17.5%), and only 8/40 (20%) were alive and free of disease between 5 to 40 months (mean=18 mo).
  • [MeSH-major] Immunohistochemistry. Mediastinal Neoplasms / diagnosis. Neoplasms, Germ Cell and Embryonal / diagnosis. Ovarian Neoplasms / diagnosis. Retroperitoneal Neoplasms / diagnosis. Sarcoma / diagnosis. Testicular Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Adult. Aged. Antineoplastic Combined Chemotherapy Protocols. Disease-Free Survival. Female. Follow-Up Studies. Humans. Male. Middle Aged. Neoplasm Metastasis. Neoplasm Staging. Orchiectomy. Ovariectomy. Time Factors. Treatment Outcome

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  • (PMID = 17721191.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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38. Grossman EJ, Millis JM: Liver transplantation for non-hepatocellular carcinoma malignancy: Indications, limitations, and analysis of the current literature. Liver Transpl; 2010 Aug;16(8):930-42
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  • Orthotopic liver transplantation (OLT) is currently incorporated into the treatment regimens for specific nonhepatocellular malignancies.
  • For patients suffering from early-stage, unresectable hilar cholangiocarcinoma (CCA), OLT preceded by neoadjuvant radiotherapy has the potential to readily achieve a tumor-free margin, accomplish a radical resection, and treat underlying primary sclerosing cholangitis when present.
  • Hepatic epithelioid hemangioendothelioma is a rare tumor of vascular origin.
  • There exist subtle differences in the timing of chemotherapy between US and European centers; however, the long-term survival rate after transplantation ranges from 66% to 77%.
  • In the treatment of either primary or metastatic hepatic sarcomas, unacceptable survival and recurrence rates currently prohibit the use of OLT.
  • [MeSH-major] Liver Neoplasms / therapy. Liver Transplantation / methods
  • [MeSH-minor] Aged. Cholangiocarcinoma / therapy. Hemangioendothelioma / therapy. Hepatoblastoma / therapy. Humans. Immunosuppressive Agents / therapeutic use. Liver / pathology. Medical Oncology / methods. Middle Aged. Neoplasm Metastasis. Neuroendocrine Tumors / therapy. Sarcoma / therapy. Treatment Outcome

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  • [Copyright] (c) 2010 AASLD.
  • (PMID = 20677284.001).
  • [ISSN] 1527-6473
  • [Journal-full-title] Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society
  • [ISO-abbreviation] Liver Transpl.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunosuppressive Agents
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39. Krakauer EL, Penson RT, Truog RD, King LA, Chabner BA, Lynch TJ Jr: Sedation for intractable distress of a dying patient: acute palliative care and the principle of double effect. Oncologist; 2000;5(1):53-62
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  • The case presented is of a young man dying of recurrent epithelioid hemangioendothelioma, distressed with stridor and severe pain, whose poorly controlled symptoms were successfully treated with an infusion of propofol, titrated to provide effective comfort in the last few hours of the patient's life.
  • The tenet of double effect, which allows aggressive treatment of suffering in spite of foreseeable but unintended consequences, is reviewed.
  • The patient's parents were invited and contributed to the Rounds, providing compelling testimony to the power of the presence of clinicians at the time of death and the importance of open communication about difficult ethical issues.
  • [MeSH-major] Ethics, Medical. Hemangioendothelioma / complications. Hypnotics and Sedatives / therapeutic use. Neoplasms / complications. Pain, Intractable / drug therapy. Palliative Care. Terminal Care
  • [MeSH-minor] Adult. Caregivers. Decision Making. Family Health. Humans. Interprofessional Relations. Male. Neoplasm Recurrence, Local

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  • (PMID = 10706650.001).
  • [ISSN] 1083-7159
  • [Journal-full-title] The oncologist
  • [ISO-abbreviation] Oncologist
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / Hypnotics and Sedatives
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40. Liu M, Ba E, Zhao P, Li X: [A clinicopathological study of fifteen epithelioid angiosarcoma]. Zhonghua Bing Li Xue Za Zhi; 2002 Oct;31(5):407-10
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [A clinicopathological study of fifteen epithelioid angiosarcoma].
  • OBJECTIVE: To study the clinicopathological features of epithelioid angiosarcoma (EA) and to propose its differential diagnosis.
  • The tumors were located in the retroperitoneum (n = 3), extremities (n = 3), femur (n = 4), thyroid (n = 2), supraobit (n = 1), soft tissue of thorax (n = 1), soft tissue of neck (n = 1).
  • Histologically, the epithelioid cells were arranged diffusely or in nests with hemorrhage and focal necrosis.
  • Atypical tumor cells lined the irregular spaces and single or small nests of cells grouped around a lumen containing red blood cells.
  • FVIII, CD34, CD31,CK and EMA were positive in tumor cells and SMA was positive in pericytes.
  • Surgical resection was the treatment of choice, occasionally with adjuvant chemotherapy. Follow-up.
  • Two patients are presently alive 19 months and 7 years following diagnosis.
  • Twelve died from 2 weeks to 38 months with a median interval of 19 months after lumpectomy.
  • The differential diagnosis of EA includes metastatic carcinoma, epithelioid hemangioendothelioma, melanoma, epithelioid sarcoma etc.
  • [MeSH-major] Hemangioendothelioma, Epithelioid / diagnosis. Melanoma / diagnosis. Sarcoma / diagnosis
  • [MeSH-minor] Adult. Aged. Diagnosis, Differential. Epithelioid Cells / pathology. Female. Humans. Immunohistochemistry. Male. Middle Aged

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  • (PMID = 12485481.001).
  • [ISSN] 0529-5807
  • [Journal-full-title] Zhonghua bing li xue za zhi = Chinese journal of pathology
  • [ISO-abbreviation] Zhonghua Bing Li Xue Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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41. Bien E, Kazanowska B, Dantonello T, Adamkiewicz-Drozynska E, Balcerska A, Madziara W, Rybczynska A, Nurzynska-Flak J, Solarz E, Kurylak A, Zalewska-Szewczyk B, Krawczyk M, Izycka-Swieszewska E, Rapala M, Koscielniak E: Factors predicting survival in childhood malignant and intermediate vascular tumors : retrospective analysis of the Polish and German cooperative paediatric soft tissue sarcoma study groups and review of the literature. Ann Surg Oncol; 2010 Jul;17(7):1878-89
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  • [Title] Factors predicting survival in childhood malignant and intermediate vascular tumors : retrospective analysis of the Polish and German cooperative paediatric soft tissue sarcoma study groups and review of the literature.
  • BACKGROUND: The rarity of malignant and intermediate vascular tumors in children means that little is known about their clinical course, optimal treatment, and variables predicting survival.
  • METHODS: A total of 32 children with malignant vascular tumors (14 angiosarcomas [AS], 5 epithelioid hemangioendotheliomas, and 13 intermediate vascular tumors, including other hemangioendotheliomas plus adult-type hemangiopericytomas), registered in the German and Polish Paediatric Soft Tissue Sarcomas Study Groups, were treated following the Cooperative Weichteilsarkom Studiengruppe (CWS)-81, -86, -91, and -96 protocols.
  • RESULTS: Male sex, AS histology, tumor size >5 cm, and T2 invasiveness were independent predictors of inferior 5-year overall survival, while AS histology and T2 invasiveness were predictors of inferior 5-year event-free survival.
  • Completeness of primary tumor excision was a good prognostic factor for survival in univariate, but not multivariate, analysis.
  • Local therapy (radiotherapy and delayed surgery) were provided to the minority of patients (28% and 38%, respectively) late in the course of disease (after a mean of 9 and 6 months, respectively) and did not prevent local relapses.
  • Response to systemic treatment was poor (44%) and did not prevent local and distant relapses.
  • CONCLUSIONS: The clinical course and outcome in childhood epithelioid HE seems to be similar to intravascular tumors and less aggressive than AS.
  • An urgent need for modification of systemic therapy is needed because of the development of many metastatic and/or combined relapses and poor response to classic chemotherapy.
  • The problem of effective therapy for childhood AS is the most appaling: 13 of 14 patients died of progression despite multimodal treatment.
  • [MeSH-major] Hemangioendothelioma / mortality. Hemangiopericytoma / mortality. Hemangiosarcoma / mortality. Sarcoma / mortality. Vascular Neoplasms / mortality

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  • (PMID = 20333551.001).
  • [ISSN] 1534-4681
  • [Journal-full-title] Annals of surgical oncology
  • [ISO-abbreviation] Ann. Surg. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] United States
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42. Poggio JL, Nagorney DM, Nascimento AG, Rowland C, Kay P, Young RM, Donohue JH: Surgical treatment of adult primary hepatic sarcoma. Br J Surg; 2000 Nov;87(11):1500-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Surgical treatment of adult primary hepatic sarcoma.
  • Optimal therapeutic approaches remain unclear.
  • METHODS: Twenty consecutive adult patients who had surgical treatment for primary hepatic sarcomas were reviewed.
  • Other than one patient with primary hepatic angiosarcoma who had a history of thorium dioxide colloid (Thorotrast) exposure 23 years before diagnosis, no predisposing causes were apparent.
  • No patient received neoadjuvant chemotherapy or radiotherapy but radiotherapy was delivered intraoperatively in one patient.
  • RESULTS: Leiomyosarcoma was the most common histological type of sarcoma diagnosed (five of 20 patients), followed by malignant solitary fibrous tumour (four) and epithelioid haemangioendothelioma (three).
  • Thirteen patients developed a recurrence.
  • Distant metastases (ten patients) and intrahepatic recurrence (six) were the predominant sites of initial treatment failure.
  • Six patients received salvage chemotherapy.
  • CONCLUSION: Surgical resection is the only effective therapy for primary hepatic sarcomas at present.
  • Better adjuvant therapy is necessary, especially for high-grade malignancies, owing to the high failure rate with operation alone.
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Antineoplastic Agents / therapeutic use. Disease-Free Survival. Female. Humans. Male. Middle Aged. Neoplasm Recurrence, Local / pathology. Preoperative Care / methods. Retrospective Studies

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  • (PMID = 11091236.001).
  • [ISSN] 0007-1323
  • [Journal-full-title] The British journal of surgery
  • [ISO-abbreviation] Br J Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] ENGLAND
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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43. Radzikowska E, Szczepulska-Wójcik E, Chabowski M, Oniszh K, Langfort R, Roszkowski K: Pulmonary epithelioid haemangioendothelioma--interferon 2-alpha treatment--case report. Pneumonol Alergol Pol; 2008;76(4):281-5
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  • [Title] Pulmonary epithelioid haemangioendothelioma--interferon 2-alpha treatment--case report.
  • Chest Computer Tomography scanning showed partially calcified nodules (up to 1 cm in diameter) located in the middle and base areas of both lungs.
  • Lung specimens displayed intraalveolar and intravascular growth of neoplastic cells.
  • Pulmonary epithelioid haemangioendothelioma was diagnosed.
  • Interferon alpha treatment was introduced.
  • During the therapy, a slight regression of pulmonary changes was noticed and since then stabilization of the disease was observed.
  • [MeSH-major] Antineoplastic Agents / administration & dosage. Hemangioendothelioma, Epithelioid / drug therapy. Hemangioendothelioma, Epithelioid / radiography. Interferon-alpha / administration & dosage. Lung Neoplasms / drug therapy. Lung Neoplasms / radiography
  • [MeSH-minor] Antigens, CD3 / analysis. Antigens, CD44 / analysis. Female. Humans. Immunohistochemistry. Middle Aged. Radiography, Thoracic. Recombinant Proteins. Treatment Outcome. von Willebrand Factor / analysis

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  • (PMID = 18785134.001).
  • [ISSN] 0867-7077
  • [Journal-full-title] Pneumonologia i alergologia polska
  • [ISO-abbreviation] Pneumonol Alergol Pol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Poland
  • [Chemical-registry-number] 0 / Antigens, CD3; 0 / Antigens, CD44; 0 / Antineoplastic Agents; 0 / Interferon-alpha; 0 / Recombinant Proteins; 0 / von Willebrand Factor; 99210-65-8 / interferon alfa-2b
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44. Díaz R, Segura A, Calderero V, Cervera I, Aparicio J, Jordá MV, Pellín L: Central nervous system metastases of a pulmonary epitheloid haemangioendothelioma. Eur Respir J; 2004 Mar;23(3):483-6
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  • [Title] Central nervous system metastases of a pulmonary epitheloid haemangioendothelioma.
  • The case of a 55-yr-old male with a right pleural effusion and multiple bilateral nodules is reported.
  • A diagnostic thoracothomy was necessary to obtain a definitive histological diagnosis.
  • His general condition remained poor and the patient died 18 months after the initial diagnosis.
  • The final diagnosis was pulmonary epitheloid haemangioendothelioma with synchronous central nervous system dissemination, the first time the authors believe that association has been reported.
  • Little is known of the prognosis and treatment of these tumours, due to their rarity.
  • Treatment should include surgical resection if possible; chemotherapy appears to have little effect.
  • [MeSH-major] Brain Neoplasms / secondary. Hemangioendothelioma, Epithelioid / secondary. Lung Neoplasms / pathology
  • [MeSH-minor] Brain / pathology. Humans. Immunohistochemistry. Lung / pathology. Male. Middle Aged. Pleural Effusion, Malignant / chemistry

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  • (PMID = 15065842.001).
  • [ISSN] 0903-1936
  • [Journal-full-title] The European respiratory journal
  • [ISO-abbreviation] Eur. Respir. J.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Denmark
  • [Number-of-references] 17
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45. Belmont L, Zemoura L, Couderc LJ: Pulmonary epithelioid haemangioendothelioma and bevacizumab. J Thorac Oncol; 2008 May;3(5):557-8
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  • [Title] Pulmonary epithelioid haemangioendothelioma and bevacizumab.
  • [MeSH-major] Angiogenesis Inhibitors / administration & dosage. Antibodies, Monoclonal / administration & dosage. Hemangioendothelioma, Epithelioid / drug therapy. Lung Neoplasms / drug therapy
  • [MeSH-minor] Adult. Antibodies, Monoclonal, Humanized. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bevacizumab. Humans. Male. Tomography, X-Ray Computed

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  • [CommentIn] J Thorac Oncol. 2010 Jul;5(7):1107-8 [20581581.001]
  • [CommentIn] J Thorac Oncol. 2011 Mar;6(3):651-2 [21317750.001]
  • (PMID = 18449015.001).
  • [ISSN] 1556-1380
  • [Journal-full-title] Journal of thoracic oncology : official publication of the International Association for the Study of Lung Cancer
  • [ISO-abbreviation] J Thorac Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 2S9ZZM9Q9V / Bevacizumab
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46. Kelly H, O'Neil BH: Response of epithelioid haemangioendothelioma to liposomal doxorubicin. Lancet Oncol; 2005 Oct;6(10):813-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Response of epithelioid haemangioendothelioma to liposomal doxorubicin.
  • [MeSH-major] Doxorubicin / administration & dosage. Doxorubicin / therapeutic use. Hemangioendothelioma, Epithelioid / drug therapy. Liposomes. Liver Neoplasms / drug therapy

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  • (PMID = 16198988.001).
  • [ISSN] 1470-2045
  • [Journal-full-title] The Lancet. Oncology
  • [ISO-abbreviation] Lancet Oncol.
  • [Language] eng
  • [Grant] United States / NCRR NIH HHS / RR / 1-K12-RR017667
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 0 / Liposomes; 80168379AG / Doxorubicin
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47. Kim YH, Mishima M, Miyagawa-Hayashino A: Treatment of pulmonary epithelioid hemangioendothelioma with bevacizumab. J Thorac Oncol; 2010 Jul;5(7):1107-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Treatment of pulmonary epithelioid hemangioendothelioma with bevacizumab.
  • [MeSH-major] Angiogenesis Inhibitors / therapeutic use. Antibodies, Monoclonal / therapeutic use. Hemangioendothelioma, Epithelioid / drug therapy. Lung Neoplasms / drug therapy
  • [MeSH-minor] Adult. Antibodies, Monoclonal, Humanized. Bevacizumab. Humans. Male. Tomography, X-Ray Computed. Vascular Endothelial Growth Factor A / immunology. Vascular Endothelial Growth Factor A / metabolism

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  • [CommentIn] J Thorac Oncol. 2011 Mar;6(3):651-2 [21317750.001]
  • [CommentOn] J Thorac Oncol. 2008 May;3(5):557-8 [18449015.001]
  • (PMID = 20581581.001).
  • [ISSN] 1556-1380
  • [Journal-full-title] Journal of thoracic oncology : official publication of the International Association for the Study of Lung Cancer
  • [ISO-abbreviation] J Thorac Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Comment; Letter
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / VEGFA protein, human; 0 / Vascular Endothelial Growth Factor A; 2S9ZZM9Q9V / Bevacizumab
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48. Kamat AM, Plager C, Tamboli P, Tran N, Pettaway CA: Metastatic epithelioid hemangioendothelioma of the penis managed with surgery and interferon-alpha. J Urol; 2004 May;171(5):1886-7
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  • [Title] Metastatic epithelioid hemangioendothelioma of the penis managed with surgery and interferon-alpha.
  • [MeSH-major] Hemangioendothelioma / drug therapy. Hemangioendothelioma / surgery. Interferon-alpha / therapeutic use. Penile Neoplasms / drug therapy. Penile Neoplasms / surgery
  • [MeSH-minor] Combined Modality Therapy. Follow-Up Studies. Humans. Male. Middle Aged

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  • (PMID = 15076299.001).
  • [ISSN] 0022-5347
  • [Journal-full-title] The Journal of urology
  • [ISO-abbreviation] J. Urol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Interferon-alpha
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49. Kumar P, Ladas GP, Judson I, Nicholson AG: Epithelioid hemangioendothelioma and other vascular mediastinal tumors: a role for alpha-2a interferon? Ann Thorac Surg; 2003 Aug;76(2):653; author reply 653
Genetic Alliance. consumer health - Hemangioendothelioma.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Epithelioid hemangioendothelioma and other vascular mediastinal tumors: a role for alpha-2a interferon?
  • [MeSH-major] Hemangioendothelioma, Epithelioid / diagnosis. Hemangioendothelioma, Epithelioid / drug therapy. Interferon-alpha / administration & dosage. Mediastinal Neoplasms / diagnosis. Mediastinal Neoplasms / drug therapy
  • [MeSH-minor] Adult. Combined Modality Therapy. Dose-Response Relationship, Drug. Drug Administration Schedule. Follow-Up Studies. Humans. Male. Recombinant Proteins. Severity of Illness Index. Treatment Outcome

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  • [CommentOn] Ann Thorac Surg. 2002 Aug;74(2):567-9 [12173847.001]
  • (PMID = 12902133.001).
  • [ISSN] 0003-4975
  • [Journal-full-title] The Annals of thoracic surgery
  • [ISO-abbreviation] Ann. Thorac. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Comment; Letter
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Interferon-alpha; 0 / Recombinant Proteins; 76543-88-9 / interferon alfa-2a
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