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3. Szavay PO, Wermes C, Fuchs J, Schrappe M, Flemming P, von Schweinitz D: Effective treatment of infantile choriocarcinoma in the liver with chemotherapy and surgical resection: a case report. J Pediatr Surg; 2000 Jul;35(7):1134-5
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  • [Title] Effective treatment of infantile choriocarcinoma in the liver with chemotherapy and surgical resection: a case report.
  • Infantile choriocarcinoma of the liver is an extremely rare entity, and outcome has been fatal in almost all published cases.
  • To the authors' knowledge, this is the first report on successful treatment with preoperative chemotherapy.
  • A 10-week-old girl presented with a large liver tumor, ovarian cysts, cardiac insufficiency, progressive hemolytic anemia, and thrombocytopenia.
  • Ultrasound scan and magnetic resonance tomography (MRT) showed the typical pattern of infantile hemangioendothelioma.
  • An emergency laparotomy was performed because of increasing cardiac insufficiency with ligation of the right hepatic artery, tumor biopsy, and subtotal resection of the ovarian cysts.
  • Histology findings showed a choriocarcinoma of the liver and corpus luteum cysts of the ovaries.
  • Chemotherapy was initiated with etoposide and cisplatin.
  • When x-ray examination showed development of lung metastases, chemotherapy was intensified with etoposide, cisplatin, and ifosfamid according to the German Study Group of Extracranial Nontesticular Malignant Germ Cell Tumors in Childhood and Adolescence (MAKEI-96).
  • After the fourth course, a complete tumor resection was achieved by an extended right hemihepatectomy with adjuvant chemotherapy being administered after the operation.
  • The authors' experience shows that chemotherapy is effective for preoperative tumor reduction.
  • [MeSH-major] Choriocarcinoma / drug therapy. Choriocarcinoma / surgery. Liver Neoplasms / drug therapy. Liver Neoplasms / surgery
  • [MeSH-minor] Combined Modality Therapy. Female. Humans. Infant, Newborn. Remission Induction

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  • (PMID = 10917316.001).
  • [ISSN] 0022-3468
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] UNITED STATES
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4. 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.
  • We describe two patients with primary EHE of the liver and review 34 cases previously published in Japan and compare them with those in Western countries.
  • 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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5. 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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  • [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.
  • 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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6. Sharif K, English M, Ramani P, Alberti D, Otte JB, McKiernan P, Gosseye S, Jenney M, de Ville de Goyet J: Management of hepatic epithelioid haemangio-endothelioma in children: what option? Br J Cancer; 2004 Apr 19;90(8):1498-501
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  • [Title] Management of hepatic epithelioid haemangio-endothelioma in children: what option?
  • Hepatic epithelioid haemangio-endothelioma (HEHE) is an endothelium-derived tumour of low-to-medium grade malignancy.
  • It is predominantly seen in adults and is unresponsive to chemotherapy.
  • Liver transplantation is an accepted indication when the tumour is unresectable.
  • Hepatic epithelioid haemangio-endothelioma is very rare in children and results after transplantation are not reported.
  • The first had successful resection followed by chemotherapy and is alive, without disease 3 years after diagnosis.
  • Two children who had progressive disease with ifosfamide-based chemotherapy have had a reduction in clinical symptoms and stabilisation of disease up to 18 and 24 months after the use of platinum-based chemotherapy.
  • Ifosfamide-based chemotherapy was not effective.
  • Further studies are necessary to confirm if HEHE progression in children may be influenced by platinum-based chemotherapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Hemangioendothelioma / drug therapy. Liver Neoplasms / drug therapy
  • [MeSH-minor] Adolescent. Antineoplastic Agents, Alkylating / therapeutic use. Child. Child, Preschool. Female. Humans. Ifosfamide / therapeutic use. Liver Transplantation. Male. Neoplasm Recurrence, Local. Prognosis. Retrospective Studies

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  • (PMID = 15083175.001).
  • [ISSN] 0007-0920
  • [Journal-full-title] British journal of cancer
  • [ISO-abbreviation] Br. J. Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; UM20QQM95Y / Ifosfamide
  • [Other-IDs] NLM/ PMC2409708
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7. O'Grady JG: Treatment options for other hepatic malignancies. Liver Transpl; 2000 Nov;6(6 Suppl 2):S23-9
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  • [Title] Treatment options for other hepatic malignancies.
  • 2. Multimodality approaches involving various combinations of chemotherapy, irradiation, and surgery increasingly are being used to treat cholangiocarcinoma.
  • 3. The role of liver transplantation in the management of cholangiocarcinoma is limited by the perception that it is inappropriate to use scarce organs when 5-year survival rates are 25%.
  • 4. Liver transplantation is an important intervention in patients with tumors that remain unresectable after chemotherapy.
  • The role of liver transplantation in patients with extrahepatic disease that responds to chemotherapy is controversial.
  • Careful timing of surgery is required to avoid secondary drug resistance.
  • 5. Liver transplantation has been successfully applied to a range of rare hepatic malignancies, but small numbers preclude strong recommendations on the appropriateness of this practice.
  • [MeSH-major] Liver Neoplasms / therapy. Liver Transplantation
  • [MeSH-minor] Bile Duct Neoplasms / therapy. Bile Ducts, Intrahepatic. Cholangiocarcinoma / diagnosis. Cholangiocarcinoma / surgery. Cholangiocarcinoma / therapy. Combined Modality Therapy. Cystadenocarcinoma / therapy. Genetic Therapy. Hemangioendothelioma / therapy. Hemangiosarcoma / therapy. Hepatoblastoma / therapy. Humans. Neuroendocrine Tumors / therapy. Palliative Care

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  • (PMID = 11084081.001).
  • [ISSN] 1527-6465
  • [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
  • [Number-of-references] 26
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8. Sarialioglu F, Erbay A, Demir S: Response of infantile hepatic hemangioma to propranolol resistant to high-dose methylprednisolone and interferon-α therapy. Pediatr Blood Cancer; 2010 Dec 15;55(7):1433-4
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  • [Title] Response of infantile hepatic hemangioma to propranolol resistant to high-dose methylprednisolone and interferon-α therapy.
  • [MeSH-major] Drug Resistance, Neoplasm. Hemangioendothelioma / drug therapy. Interferon-alpha / administration & dosage. Liver Neoplasms / drug therapy. Methylprednisolone / administration & dosage. Propranolol / therapeutic use
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Humans. Infant. Male

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  • (PMID = 20981697.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Interferon-alpha; 9Y8NXQ24VQ / Propranolol; X4W7ZR7023 / Methylprednisolone
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9. 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.
  • Treatment included LR (n= 11), OLT (n= 11), chemotherapy (n= 5) and no treatment (n= 3).
  • 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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10. Walsh R, Harrington J, Beneck D, Ozkaynak MF: Congenital infantile hepatic hemangioendothelioma type II treated with orthotopic liver transplantation. J Pediatr Hematol Oncol; 2004 Feb;26(2):121-3
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  • [Title] Congenital infantile hepatic hemangioendothelioma type II treated with orthotopic liver transplantation.
  • The authors describe a 10-week-old girl with infantile hepatic hemangioendothelioma who initially presented with difficulty feeding, hepatomegaly, and multiple hemangiomas of the skin.
  • Six weeks of steroid therapy and 2 weeks of chemotherapy failed to produce clinical improvement.
  • The patient underwent split liver transplantation.
  • A definitive diagnosis of hemangioendothelioma type II was made.
  • Imaging studies cannot differentiate between hemangioendothelioma and angiosarcoma.
  • Treatment modalities for this condition remain unclear.
  • [MeSH-major] Hemangioendothelioma / congenital. Hemangioendothelioma / surgery. Liver Neoplasms / congenital. Liver Neoplasms / surgery. Liver Transplantation
  • [MeSH-minor] Female. Humans. Infant. Tomography, X-Ray Computed. Treatment Outcome


11. 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.
  • 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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12. Chen CC, Kong MS, Yang CP, Hung IJ: Hepatic hemangioendothelioma in children: analysis of thirteen cases. Acta Paediatr Taiwan; 2003 Jan-Feb;44(1):8-13
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  • [Title] Hepatic hemangioendothelioma in children: analysis of thirteen cases.
  • Hepatic hemangioendothelioma (HE) is a tumor that presents in infancy and toddler.
  • The diagnosis was made by the histology specimen or various imaging studies.
  • Abdominal ultrasonography (n = 13) showed heterogeneous and hypoechoic lesions in the liver.
  • Computed tomography (n = 11) revealed central hypointensity with peripheral enhancement after contrast of the liver masses.
  • Magnetic resonance imaging studies of the hepatic masses (n = 3) showed decreased signal intensity on T1 images and high signal intensity on T2.
  • Other management included interferon, chemotherapy, embolization and/or surgery.
  • Among the other nine patients, four patients died of sepsis, hepatic failure, disseminated intravascular coagulopathy or tumor rupture with hemorrhagic shock.
  • For its management, steroid is a first-line medication.
  • Other methods of treatment were interferon, hepatic artery embolization, chemotherapy and surgery.
  • Long term follow up is needed for the evaluation of treatment response.
  • [MeSH-major] Hemangioendothelioma / diagnosis. Liver Neoplasms / diagnosis
  • [MeSH-minor] Child, Preschool. Female. Humans. Infant. Infant, Newborn. Magnetic Resonance Imaging. Male. Tomography, X-Ray Computed

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  • (PMID = 12800377.001).
  • [ISSN] 1608-8115
  • [Journal-full-title] Acta paediatrica Taiwanica = Taiwan er ke yi xue hui za zhi
  • [ISO-abbreviation] Acta Paediatr Taiwan
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] China (Republic : 1949- )
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13. 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
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  • [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.
  • Its growth can lead to hepatic failure, extrahepatic metastasis and death.
  • 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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14. Makin E, Davenport M: Fetal and neonatal liver tumours. Early Hum Dev; 2010 Oct;86(10):637-42
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  • [Title] Fetal and neonatal liver tumours.
  • Liver tumours are rare in children and account for about 5% of all tumours in the fetus and newborn.
  • While the diagnosis can be suspected antenatally (by ultrasound and MR scan), a precise diagnosis is often difficult due to the complexity of the tumours.
  • Treatment options may include watchful waiting, surgical resection, hepatic artery embolisation/ligation and chemotherapy.
  • [MeSH-major] Fetal Diseases / diagnosis. Hamartoma / diagnosis. Hemangioendothelioma / diagnosis. Hepatoblastoma / diagnosis. Liver Neoplasms / diagnosis. Mesoderm / pathology
  • [MeSH-minor] Female. Humans. Infant, Newborn. Liver / surgery. Magnetic Resonance Imaging. Male. Practice Guidelines as Topic. Ultrasonography, Prenatal. Watchful Waiting

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  • [Copyright] Copyright © 2010 Elsevier Ltd. All rights reserved.
  • (PMID = 20956063.001).
  • [ISSN] 1872-6232
  • [Journal-full-title] Early human development
  • [ISO-abbreviation] Early Hum. Dev.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Ireland
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15. Haberal M, Ozcay F, Sevmis S, Karakayali H, Moray G, Torgay A, Coskun M, Demirhan B, Sarialioglu F, Arslan G: Liver transplant in an infant with bilateral cystic neuroblastoma complicated by hepatic metastases and life-threatening consumption coagulopathy. Pediatr Transplant; 2008 May;12(3):358-62
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  • [Title] Liver transplant in an infant with bilateral cystic neuroblastoma complicated by hepatic metastases and life-threatening consumption coagulopathy.
  • Here we report a patient with stage-4 bilateral cystic adrenal neuroblastomas with disseminated liver metastases and consumption coagulopathy who underwent liver transplant.
  • Our patient was initially diagnosed with infantile hepatic hemangioendothelioma and bleeding into the adrenal glands secondary to consumption coagulopathy (Kasabach-Merritt syndrome).
  • Liver transplant was performed as a life-saving procedure under this diagnosis.
  • We discuss this unique patient because of the diagnostic pitfalls of this rare disease and the successful clinical outcome after LT and subsequent chemotherapy for neuroblastoma.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Disseminated Intravascular Coagulation / therapy. Liver Neoplasms / pathology. Liver Transplantation / methods. Neuroblastoma / pathology
  • [MeSH-minor] Adrenal Glands / pathology. Antineoplastic Agents / therapeutic use. Female. Humans. Infant. Living Donors. Neoplasm Metastasis. Treatment Outcome


16. 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
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  • [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.
  • We describe a case of a young healthy adult male who was found to have multiple hepatic masses on radiologic investigations.
  • 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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17. Maisonnette F, Hardemann S, Abita T, Durand-Fontanier S, Valleix D, Descottes B: [Hepatic haemangioendothelioma: case report and review of literature]. Ann Chir; 2006 Feb;131(2):115-7
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  • [Title] [Hepatic haemangioendothelioma: case report and review of literature].
  • We discovered many hepatic lesions.
  • After reexamination of histology we discovered hepatic haemangioendothelioma.
  • The patient died 18 month after diagnosis although chemotherapy.
  • [MeSH-major] Hemangioendothelioma. Liver Neoplasms

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  • (PMID = 16246294.001).
  • [ISSN] 0003-3944
  • [Journal-full-title] Annales de chirurgie
  • [ISO-abbreviation] Ann Chir
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Number-of-references] 11
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18. Berber E, Ari E, Herceg N, Siperstein A: Laparoscopic radiofrequency thermal ablation for unusual hepatic tumors: operative indications and outcomes. Surg Endosc; 2005 Dec;19(12):1613-7
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  • [Title] Laparoscopic radiofrequency thermal ablation for unusual hepatic tumors: operative indications and outcomes.
  • BACKGROUND: There is increasing experience with laparoscopic radiofrequency ablation for the treatment of patients with hepatic metastasis from colorectal and neuroendocrine cancer and those with hepatocellular cancer.
  • Little is known about the outcomes for patients with other tumor types.
  • METHODS: Between January 1996 and March 2005, 517 patients with 1,500 primary and metastatic liver tumors underwent laparoscopic radiofrequency ablation.
  • Among these, 53 patients (10%) had cancers other than the colorectal, neuroendocrine, or hepatocellular types including sarcoma (n = 18), breast cancer (n = 10), esophagus cancer (n = 4), melanoma (n = 4), lung cancer (n = 3), ovarian cancer (n = 2), pancreas cancer (n = 2), unknown primary cancer (n = 2), cholangiocarcinoma (n = 2), rectal squamous cancer (n = 2), renal cancer (n = 2), papillary thyroid cancer (n = 1), and hemangioendothelioma (n = 1).
  • Unlike the criteria for treatment of the more usual tumor types, these patients had a diagnosis of liver-exclusive disease, as diagnosed by preoperative imaging.
  • They also had failed chemotherapy.
  • RESULTS: The 53 patients underwent ablation of 192 lesions, with 8 patients undergoing repeat treatment.
  • Complications included one postoperative hemorrhage, one liver abscess, and one wound infection.
  • CONCLUSION: Laparoscopic radiofrequency ablation can safely and effectively treat hepatic metastasis of these unusual tumor types.
  • The authors believe that this heterogeneous group of patients, selected for their unusual presentation of liver-exclusive disease, may benefit from cytoreduction of their tumor by laparoscopic radiofrequency ablation when other treatment methods have failed.
  • [MeSH-major] Catheter Ablation / methods. Laparoscopy. Liver Neoplasms / secondary. Liver Neoplasms / surgery
  • [MeSH-minor] Female. Humans. Male. Middle Aged. Survival Rate. Treatment Outcome

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  • (PMID = 16247574.001).
  • [ISSN] 1432-2218
  • [Journal-full-title] Surgical endoscopy
  • [ISO-abbreviation] Surg Endosc
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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19. 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.
  • The purpose of this study was to analyze the expression of the angiogenic factor vascular endothelial growth factor (VEGF) and its receptors in hepatic EHE.
  • Six patients with hepatic EHE were assessed for liver transplantation at our center.
  • Pathology specimens of primary and recurrent EHE were analyzed by hematoxylin and eosin staining and by immunofluorescence for VEGF, fetal liver kinase 1 (Flk-1), and fms-related tyrosine kinase 1 (Flt-1) expression.
  • Five patients underwent liver transplantation, and 1 patient underwent liver resection.
  • In 1 patient with recurrent hepatic EHE post-liver transplantation, a progressive increase in the VEGF fluorescence intensity and distribution was observed.
  • In conclusion, in this series, VEGF expression was observed in all hepatic EHE specimens analyzed.
  • 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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20. 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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  • [Title] Clinical outcomes of surgical resections for primary liver sarcoma in adults: results from a single centre.
  • BACKGROUND: Primary hepatic sarcoma is a rare tumour with a poor prognosis.
  • METHODS: From 1997 to 2002 eight patients had liver resection for primary sarcoma of the liver at our institution.
  • 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.
  • CONCLUSIONS: The majority of patients with primary liver sarcoma present with right upper quadrant pain, fever and a liver mass.
  • 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-major] Liver Neoplasms / surgery. Sarcoma / surgery
  • [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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21. Stringer MD: The role of liver transplantation in the management of paediatric liver tumours. Ann R Coll Surg Engl; 2007 Jan;89(1):12-21
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  • [Title] The role of liver transplantation in the management of paediatric liver tumours.
  • In recent years, considerable progress has been made in the treatment of children with hepatoblastoma largely due to effective pre-operative chemotherapy.
  • Total hepatectomy and liver transplantation has emerged as an effective treatment for the small proportion of children with unresectable hepatoblastoma limited to the liver.
  • In contrast, the results of liver transplantation in children with hepatocellular cancer remain poor because these tumours are usually advanced with evidence of major vascular invasion and/or extrahepatic spread at the time of presentation.
  • An exception is those children in whom the hepatocellular carcinoma is detected during surveillance of chronic liver disease - they typically have smaller tumours and frequently have a good prognosis after liver transplantation.
  • The role of liver transplantation in children with other primary hepatic malignancies remains uncertain because experience is very limited.
  • Liver transplantation is rarely needed in the management of children with benign liver tumours but, if other treatments have failed, it can be a life-saving intervention.
  • [MeSH-major] Liver Neoplasms / surgery. Liver Transplantation / methods
  • [MeSH-minor] Carcinoma, Hepatocellular / mortality. Carcinoma, Hepatocellular / surgery. Child. Hamartoma / mortality. Hamartoma / surgery. Hemangioendothelioma, Epithelioid. Hepatoblastoma / mortality. Hepatoblastoma / surgery. Humans. Liver Diseases / mortality. Liver Diseases / surgery. Neoplasms, Muscle Tissue / mortality. Neoplasms, Muscle Tissue / surgery. Sarcoma / mortality. Sarcoma / surgery. Survival Analysis. Treatment Outcome

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  • (PMID = 17316514.001).
  • [ISSN] 1478-7083
  • [Journal-full-title] Annals of the Royal College of Surgeons of England
  • [ISO-abbreviation] Ann R Coll Surg Engl
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 71
  • [Other-IDs] NLM/ PMC1963524
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22. Szymik-Kantorowicz S, Partyka L, Dembinska-Kiec A, Zdzienicka A: Vascular endothelial growth factor in monitoring therapy of hepatic haemangioendothelioma. Med Pediatr Oncol; 2003 Mar;40(3):196-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Vascular endothelial growth factor in monitoring therapy of hepatic haemangioendothelioma.
  • [MeSH-major] Angiogenesis Inhibitors / therapeutic use. Endothelial Growth Factors / blood. Hemangioendothelioma / blood. Hemangioendothelioma / drug therapy. Intercellular Signaling Peptides and Proteins / blood. Interferon-alpha / therapeutic use. Liver Neoplasms / blood. Liver Neoplasms / drug therapy. Lymphokines / blood
  • [MeSH-minor] Biomarkers / blood. Drug Monitoring. Humans. Infant. Vascular Endothelial Growth Factor A. Vascular Endothelial Growth Factors

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  • (PMID = 12518353.001).
  • [ISSN] 0098-1532
  • [Journal-full-title] Medical and pediatric oncology
  • [ISO-abbreviation] Med. Pediatr. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Biomarkers; 0 / Endothelial Growth Factors; 0 / Intercellular Signaling Peptides and Proteins; 0 / Interferon-alpha; 0 / Lymphokines; 0 / Vascular Endothelial Growth Factor A; 0 / Vascular Endothelial Growth Factors
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25. 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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  • [Title] Liver transplantation for non-hepatocellular carcinoma malignancy: Indications, limitations, and analysis of the current literature.
  • Orthotopic liver transplantation (OLT) is currently incorporated into the treatment regimens for specific nonhepatocellular malignancies.
  • Hepatic involvement from neuroendocrine tumors can be treated with OLT when metastases are unresectable or for palliation of medically uncontrollable symptoms.
  • Hepatic epithelioid hemangioendothelioma is a rare tumor of vascular origin.
  • Hepatoblastoma is the most common primary hepatic malignancy in children.
  • 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%.
  • Fibrolamellar hepatocellular carcinoma is a distinct liver malignancy best treated by surgical resection.
  • 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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27. Sevinir B, Ozkan TB: Infantile hepatic hemangioendothelioma: clinical presentation and treatment. Turk J Gastroenterol; 2007 Sep;18(3):182-7
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  • [Title] Infantile hepatic hemangioendothelioma: clinical presentation and treatment.
  • BACKGROUND/AIMS: Hepatic hemangioendotheliomas are rare tumors in childhood.
  • METHODS: This retrospective analysis included eight patients with infantile hepatic hemangioendothelioma.
  • RESULTS: The median age at diagnosis was 24 days (age range: 1 to 70 days) and the female/male ratio was 5/3.
  • Four cases had single hepatic tumors while the others had multiple.
  • These lesions were located equally in the right and left hepatic lobes, and three babies had bilobar involvement.
  • Most of the multifocal hepatic tumors were associated with skin hemangiomas.
  • Treatment options were assessed individually.
  • Systemic prednisolone therapy (2 mg/kg/d) was commenced in six patients.
  • One boy with Kasabach-Merritt syndrome did not respond to this therapy.
  • Interferon-alpha (1 million units (MU)/m2/day) was started, and the daily dose of the drug was increased up to 10 MU/m2, administered 3 times per week, until clinical improvement was achieved.
  • CONCLUSIONS: The treatment approaches depend on the center's experience.
  • A multidisciplinary approach is required for the best treatment option.
  • [MeSH-major] Hemangioendothelioma / diagnosis. Hemangioendothelioma / therapy. Liver Neoplasms / diagnosis. Liver Neoplasms / therapy
  • [MeSH-minor] Abdomen. Alanine Transaminase / metabolism. Aspartate Aminotransferases / metabolism. Dilatation, Pathologic / etiology. Female. Glucocorticoids / therapeutic use. Hemangioma / diagnosis. Hepatomegaly / etiology. Humans. Immunologic Factors / therapeutic use. Infant. Infant, Newborn. Interferon-alpha / therapeutic use. Male. Neoplasms, Multiple Primary. Prednisolone / therapeutic use. Respiratory Distress Syndrome, Newborn / etiology. Retrospective Studies. Skin Neoplasms / diagnosis. Turkey / epidemiology

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  • (PMID = 17891692.001).
  • [ISSN] 2148-5607
  • [Journal-full-title] The Turkish journal of gastroenterology : the official journal of Turkish Society of Gastroenterology
  • [ISO-abbreviation] Turk J Gastroenterol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Turkey
  • [Chemical-registry-number] 0 / Glucocorticoids; 0 / Immunologic Factors; 0 / Interferon-alpha; 9PHQ9Y1OLM / Prednisolone; EC 2.6.1.1 / Aspartate Aminotransferases; EC 2.6.1.2 / Alanine Transaminase
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28. Hertl M, Cosimi AB: Liver transplantation for malignancy. Oncologist; 2005 Apr;10(4):269-81
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  • [Title] Liver transplantation for malignancy.
  • Liver transplantation for hepatic malignancies has emerged from an exotic and desperate approach to a well-documented and proven treatment modality for these unfortunate patients.
  • Currently, <10% of all liver transplants performed are for hepatocellular cancer (HCC).
  • Similarly, liver transplantation for HCC in the adult population yields good results for patients whose tumor masses do not exceed the Milan criteria.
  • It remains to be determined whether patients with more extensive tumors can be reliably selected to benefit from the procedure.
  • Epitheloid hemangioendothelioma is also an appropriate indication for liver transplantation, unlike angiosarcoma.
  • Metastatic liver disease is not an indication for liver transplantation, with the exception of cases in which the primary is a neuroendocrine tumor, for which liver transplantation can result in long-term survival and even cure in a number of patients.
  • And finally, while gallbladder cancers are never an indication for liver transplantation, rare cases of cholangiocellular cancer might qualify if aggressive combination therapies, including chemotherapy and radiotherapy followed by OLT, are carried through.
  • Survival in these selected patients can approach that for patients with cholestatic liver disease.
  • [MeSH-major] Bile Duct Neoplasms / surgery. Carcinoma, Hepatocellular / surgery. Liver Neoplasms / surgery. Liver Transplantation
  • [MeSH-minor] Cholangiocarcinoma / mortality. Cholangiocarcinoma / pathology. Cholangiocarcinoma / surgery. Hemangioendothelioma, Epithelioid / mortality. Hemangioendothelioma, Epithelioid / pathology. Hemangioendothelioma, Epithelioid / surgery. Hepatoblastoma / mortality. Hepatoblastoma / pathology. Hepatoblastoma / surgery. Humans. Medical Oncology / trends. Neoplasm Metastasis. Patient Selection. Survival Rate. Treatment Outcome. Waiting Lists


29. 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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  • [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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30. Nord KM, Kandel J, Lefkowitch JH, Lobritto SJ, Morel KD, North PE, Garzon MC: Multiple cutaneous infantile hemangiomas associated with hepatic angiosarcoma: case report and review of the literature. Pediatrics; 2006 Sep;118(3):e907-13
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  • [Title] Multiple cutaneous infantile hemangiomas associated with hepatic angiosarcoma: case report and review of the literature.
  • Multiple cutaneous hemangiomas can be associated with internal hemangiomas, with the liver being the most common site.
  • Here we report a case of a premature female neonate who presented with cardiac failure at birth and had typical-appearing infantile hemangiomas on the skin in association with vascular lesions in the liver.
  • Her clinical presentation was felt to be consistent with cutaneous and hepatic infantile hemangiomas.
  • After failure to respond to systemic steroids and chemotherapy, she underwent liver transplantation.
  • Histopathologic evaluation of the liver revealed a diagnosis of type 2 infantile hepatic hemangioendothelioma (regarded as synonymous with angiosarcoma) rather than benign infantile hemangioma of the liver.
  • We report this case and a review of the literature on pediatric angiosarcoma of the liver associated with cutaneous infantile hemangiomas.
  • [MeSH-major] Hemangioma / pathology. Hemangiosarcoma / pathology. Liver Neoplasms / pathology. Skin Neoplasms / pathology
  • [MeSH-minor] Female. Heart Defects, Congenital. Humans. Infant, Newborn. Liver Transplantation

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  • [ErratumIn] Pediatrics. 2007 Jun;119(6):1271
  • (PMID = 16880251.001).
  • [ISSN] 1098-4275
  • [Journal-full-title] Pediatrics
  • [ISO-abbreviation] Pediatrics
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 27
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31. Wiederkehr JC, Coelho IM, Avilla SG, e Silva EM, Schuller S, Ouno DD, Wiederkehr BA, Polimeni M: Prevalence of posttransplantation lymphoproliferative disease in pediatric liver transplant recipients. Transplant Proc; 2010 Mar;42(2):521-2
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  • [Title] Prevalence of posttransplantation lymphoproliferative disease in pediatric liver transplant recipients.
  • OBJECTIVE: The objective of this study was to identify the incidence of posttransplantation lymphoproliferative disease (PTLD) among children within 1 year after liver transplantation.
  • METHODS: This retrospective review analyzed information in medical charts of pediatric (younger than 18 years of age) recipients of liver transplants between September 2000 and December 2007.
  • RESULTS: Seventy-one patients underwent a liver transplantation and 7 (9.85%) developed PTLD.
  • Indications that led the children to have their transplantation were 1 case of hemangioendothelioma, 1 case of autoimmune hepatic cirrhosis, 1 case of alpha-1-antitrypsin deficiency, and 4 cases of biliary atresia.
  • The median time from the first symptoms to the initial treatment was 9.7 days.
  • The standard treatment was withdrawal of immunosuppression and close observation of tacrolimus levels and liver function tests associated with antiviral drugs and chemotherapy.
  • [MeSH-major] Liver Transplantation / adverse effects. Lymphoproliferative Disorders / epidemiology. Postoperative Complications / epidemiology
  • [MeSH-minor] Biliary Atresia / surgery. Child. Child, Preschool. Epstein-Barr Virus Infections / surgery. Female. Humans. Immunosuppressive Agents / therapeutic use. Infant. Liver Diseases / classification. Liver Diseases / surgery. Male. Prevalence. Retrospective Studies. Splenomegaly / etiology. Tacrolimus / therapeutic use. alpha 1-Antitrypsin Deficiency / surgery

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  • [Copyright] Copyright (c) 2010 Elsevier Inc. All rights reserved.
  • (PMID = 20304183.001).
  • [ISSN] 1873-2623
  • [Journal-full-title] Transplantation proceedings
  • [ISO-abbreviation] Transplant. Proc.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunosuppressive Agents; WM0HAQ4WNM / Tacrolimus
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32. Biecker E, Fischer HP, Strunk H, Sauerbruch T: Benign hepatic tumours. Z Gastroenterol; 2003 Feb;41(2):191-200
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  • [Title] Benign hepatic tumours.
  • Benign hepatic tumours include a broad spectrum of regenerative and true neoplastic processes.
  • Due to advances in imaging procedures like MRI, Cf-scan and ultrasound as well as progress in immunohistochemistry, the appropriate diagnosis is made ina high percentage of patients without laparotomy and resection.
  • Most important in clinical practice is the differential diagnosis of focal nodular hyperplasia and hepatocellular adenoma because of the risk of rupture and bleeding in the latter.
  • Cavernous haemangioma, the most common benign hepatic tumour, rarely needs treatment.
  • The diagnosis of nodular regenerative hyperplasia is often missed and patients present with secondary complications and signs of portal hypertension that necessitate treatment.
  • The main problem in angiomyolipoma is to distinguish it from malignant processes which do require treatment.
  • Because of its clinical presentation, inflammatory pseudotumour is also sometimes confused with a malignant tumour.
  • Therapeutic options are drug therapy or surgical resection.
  • [MeSH-major] Liver Diseases / diagnosis. Liver Neoplasms / diagnosis
  • [MeSH-minor] Adenoma, Liver Cell / diagnosis. Adenoma, Liver Cell / pathology. Adult. Aged. Diagnostic Imaging. Focal Nodular Hyperplasia / diagnosis. Focal Nodular Hyperplasia / pathology. Granuloma, Plasma Cell / diagnosis. Granuloma, Plasma Cell / pathology. Hemangioendothelioma / diagnosis. Hemangioendothelioma / pathology. Hemangioma, Cavernous / diagnosis. Hemangioma, Cavernous / pathology. Humans. Infant. Liver / pathology. Liver Regeneration / physiology. Middle Aged

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  • (PMID = 12650132.001).
  • [ISSN] 0044-2771
  • [Journal-full-title] Zeitschrift für Gastroenterologie
  • [ISO-abbreviation] Z Gastroenterol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 138
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33. Ekinci S, Karnak I, Tanyel FC, Senocak ME, Kutluk T, Büyükpamukçu M, Büyükpamukçu N: Hepatic lobectomies in children: experience of a center in the light of changing management of malignant liver tumors. Pediatr Surg Int; 2006 Mar;22(3):228-32
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  • [Title] Hepatic lobectomies in children: experience of a center in the light of changing management of malignant liver tumors.
  • Hepatic resection is the main treatment modality for hepatic tumors in childhood.
  • The aim of this study is to report our experience in hepatic lobectomy, which is relatively rare procedure in childhood.
  • Medical records of 25 patients who underwent hepatic lobectomy between January 1977 and June 2002 were reviewed retrospectively.
  • Age, gender, diagnosis, physical examination findings, results of preoperative laboratory investigations, radiological examination, resectability criteria, preoperative biopsies, chemotherapies, radiotherapies, postoperative pathological results, incisions, operation technique, intraoperative transfusions, drains used, antibiotic prophylaxes, and intraoperative and postoperative complications were evaluated for all patients.
  • Out of 25 patients with hepatic tumor seven patients with hepatoblastoma and four patients with hepatocellular carcinoma were given 5.7 +/- 0.3 cycles of chemotherapy before the operation.
  • Pathological examination of resected tumors revealed hepatoblastoma (n=11), mesenchymal hamartoma (n = 5), hepatocellular carcinoma (n = 4), hemangioendothelioma (n=1), malignant mesenchymal tumor (n = 1), hemangioma (n = 1), cyst adenoma (n = 1), and metastasis of cellular mesoblastic nephroma (n = 1).
  • Hepatic lobectomy is a major operation, which is feasible yielding curative results in children.
  • Safe hepatic resections with acceptable blood loss can be performed by a technique relying on good anatomic dissection and surgical control.
  • [MeSH-major] Hepatectomy / methods. Liver Neoplasms / surgery
  • [MeSH-minor] Child, Preschool. Diagnosis, Differential. Female. Follow-Up Studies. Humans. Infant. Magnetic Resonance Imaging. Male. Retrospective Studies. Tomography, X-Ray Computed. Treatment Outcome

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  • [ErratumIn] Pediatr Surg Int. 2006 Aug;22(8):695
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  • (PMID = 16395609.001).
  • [ISSN] 0179-0358
  • [Journal-full-title] Pediatric surgery international
  • [ISO-abbreviation] Pediatr. Surg. Int.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
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34. 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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  • [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.
  • In three patients, liver transplantation (LTx) was performed (two cadaveric and one living related).
  • No adjuvant chemotherapy was applied.
  • 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.
  • Despite the long waiting time, its often unclear dignity, and a proven progressive growth pattern, living related LTx also plays a potentially important role.
  • Long-term survival of patients with HEH is significantly higher compared to other hepatic malignancies.
  • 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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35. 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


36. Kalpatthi R, Germak J, Mizelle K, Yeager N: Thyroid abnormalities in infantile hepatic hemangioendothelioma. Pediatr Blood Cancer; 2007 Dec;49(7):1021-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Thyroid abnormalities in infantile hepatic hemangioendothelioma.
  • We report an infant with hepatic hemangioendothelioma (HAE) associated with compensated hypothyroidism.
  • The hepatic lesions regressed with steroid therapy and his thyroid function normalized with high doses of thyroxine supplement.
  • [MeSH-major] Hemangioendothelioma / complications. Hypothyroidism / complications. Liver Neoplasms / complications. Thyroxine / therapeutic use
  • [MeSH-minor] Child, Preschool. Dose-Response Relationship, Drug. Drug Administration Schedule. Follow-Up Studies. Heart Failure / complications. Heart Failure / drug therapy. Humans. Male. Steroids / therapeutic use. Thyroid Function Tests. Treatment Outcome

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  • [Copyright] 2007 Wiley-Liss, Inc
  • (PMID = 16544297.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Steroids; Q51BO43MG4 / Thyroxine
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37. Albert A, Cruz O, Montaner A, Vela A, Badosa J, Castañón M, Morales L: [Congenital solid tumors. A thirteen-year review]. Cir Pediatr; 2004 Jul;17(3):133-6
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  • This is an interesting group of tumors because their type, relative incidence, natural history and response to treatment differ from those seen in older children.
  • There were 8 teratomas (3 sacrocoxigeal, 1 retroperitoneal, 1 in the CNS, 1 orbitary and two oronasal), two hepatic tumors (1 hepatoblastoma, 1 hemangioendothelioma, two CNS tumors, two giant nevus (one on a hamartoma), and one each Wilms tumor, infantile fibrosarcoma and myofibroblastic tumor.
  • Treatment was surgical resection alone in 17 cases (68%) and surgery + chemotherapy in 8 (32%) (5 neuroblastomas, one CNS tumor, one Wilms tumor and one presacral teratoma who developed a yolk sac tumor); 3 patients died (11%): one at surgery, one of tumoural airway obstruction at birth and one with craniopharyngioma.
  • Among the 14 tumors that were initially not malignant, two can be locally agressive, one was an immature teratoma, the giant nevus with hamartoma developed in situ melanoma, the other nevus had meningeal melanosis with hydrocephalus, and one mature presacral teratoma developed a yolk sac tumor.
  • CONCLUSIONS: Diagnosis of congenital tumors is performed earlier in recent years due to the wide use of prenatal ultrasound.
  • Complete surgical excision is the treatment of choice, most cases not need adjuvant chemotherapy.
  • We ought to pass this message on to our colleagues in prenatal diagnosis, so parents get reliable information.
  • [MeSH-major] Central Nervous System Neoplasms / congenital. Kidney Neoplasms / congenital. Liver Neoplasms / congenital. Neuroblastoma / congenital. Skin Neoplasms / congenital. Soft Tissue Neoplasms / congenital. Teratoma / congenital. Wilms Tumor / congenital
  • [MeSH-minor] Female. Follow-Up Studies. Humans. Infant, Newborn. Male. Neoplasm Recurrence, Local. Postoperative Complications. Pregnancy. Prenatal Diagnosis. Time Factors


38. 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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  • [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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  • (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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