scholarly journals Orthotopic Liver Transplantation in a Case of Novel YAP1-TFE3 Hepatic Epithelioid Hemangioendothelioma [HEHE]

2021 ◽  
Vol 5 (2) ◽  
pp. 45-49
Author(s):  
Sugi SRV ◽  
Elankumaran Krishnan ◽  
Kulaseharan Venugopal Haribabu ◽  
Hari Balakrishnan ◽  
Shantha Ravishankar
Keyword(s):  
Liver Transplantation ◽  
Soft Tissues ◽  
Clinical Course ◽  
Fatal Outcome ◽  
Malignant Tumour ◽  
Epithelioid Hemangioendothelioma ◽  
Malignant Tumours ◽  
Vascular Neoplasm ◽  
Donor Liver Transplantation ◽  
Time Of Presentation

Epithelioid hemangioendothelioma is an extremely rare vascular neoplasm arising in soft-tissues and different visceral organs, with liver being the most commonly involved viscera. Hepatic epithelioid hemangioendothelioma (HEHE) is a malignant tumour with an indolent behaviour and unpredictable clinical course. It has a better prognosis among the malignant tumours of the liver, in spite of being a diffuse multifocal liver disease or metastatic at the time of presentation. HEHE is usually found to be noted in the fifth decade with slight female preponderance. No single treatment strategy has yet been established owing to its variable clinical course, ranging from an indolent tumour with prolonged survival to an aggressive, metastatic disease with a fatal outcome. Here, we present a case of a novel HEHE in a 25-year-old female who was treated successfully with orthotopic living donor liver transplantation and discuss the presentation, histopathology and management of this rare, fatal yet treatable malignant tumour.

scholarly journals Living-related liver transplant to treat epithelioid hemangioendothelioma – a case report

2005 ◽  
Vol 8 (4) ◽  
pp. 449-451
Author(s):  
Marcelo A. F. Ribeiro Jr ◽  
Christian Evangelista Garcia ◽  
Telma Eugênio dos Santos ◽  
Adavio de Oliveira e Silva ◽  
Regina Leitão ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Case Report ◽  
Liver Transplant ◽  
Soft Tissues ◽  
Liver Tumors ◽  
Epithelioid Hemangioendothelioma ◽  
Low Grade ◽  
Living Related Liver Transplant ◽  
Transplantation Technique ◽  
Living Related

Introduction: Epithelioid hemangioendothelioma of liver is a rare, low-grade neoplasm of vascular origin that has an unpredictable malignant potential. It preferentially arises in soft tissues and bones, and seldom in the liver. Biologically, it trends to be multi-focal and often unresectable. Objective: The outcome of a patient submitted to a living-related liver transplant to treat epithelioid hemangioendothelioma of the liver. Case report: The reported case involves a 39-year old female patient with an immense epithelioid hemangioendothelioma of the liver, involving segments III, IV, V, VII, and VIIl. The prognostic to any kind of treatment was considered unfavorable, and the decision of submitting her to a liver transplant was made. It was suggested a living-related liver transplant. The donor was the patient’s 36-year old brother, who donated his right liver lobe (segments V, VI, VII, VIII). The recipient´s original liver was large, presenting very solid consistence and no node evidence. The transplant itself was performed using the conventional living-related liver transplantation technique. The recovery was good, and she was discharged from hospital on the 15th postoperative day using micophenolate mofetil, tacrolimus, and corticoid. Conclusion: Liver transplantation for epithelioid hemangioendothelioma of the liver can be performed with acceptable survival rate. The living related liver transplant opens a great perspective to that group of patients presenting liver tumors and who are unfeasible to wait for an organ on a waiting list.

Clinical Course of Hepatic Artery Thrombosis After Living Donor Liver Transplantation Using the Right Lobe

2018 ◽  
Vol 24 (11) ◽  
pp. 1554-1560 ◽  
Author(s):  
Ho Joong Choi ◽  
Dong Goo Kim ◽  
Yumi Kim ◽  
Bong Jun Kwak ◽  
Jae Hyun Han ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Hepatic Artery ◽  
Living Donor ◽  
Living Donor Liver Transplantation ◽  
Clinical Course ◽  
Donor Liver ◽  
Donor Liver Transplantation ◽  
Artery Thrombosis ◽  
Right Lobe ◽  
The Right

scholarly journals Metastatic Hepatic Epitheloid Hemangioendothelioma in a Young Male: A Rare Presentation

2021 ◽  
Vol 8 (2) ◽  
pp. 58-62
Author(s):  
Tanweerul Huda ◽  
Mohammad Masoom Parwez ◽  
Bharati Pandya
Keyword(s):  
Liver Transplantation ◽  
Incisional Hernia ◽  
Soft Tissues ◽  
Young Male ◽  
Chemotherapeutic Agents ◽  
Epithelioid Hemangioendothelioma ◽  
Low Grade ◽  
Rare Presentation ◽  
Large Defect ◽  
Multiple Biopsies

Hepatic EHE (epithelioid hemangioendothelioma) is an uncommon entity of vascular origin and a low-grade malignant tumor. Primary hepatic EHE is rare. These tumors can be multifocal at presentation like in the soft tissues, bones, brain, liver, and small intestine. First described by Weiss and Enzinger in 1982 as a malignant vascular neoplasm with indolent behavior. We report the case of a 23-year-old male, known case of chronic liver disease, who presented with incisional hernia following exploratory laparotomy 8 months back, performed for intestinal obstruction. Contrast-enhanced computed tomography abdomen revealed an incisional hernia with a large defect along with multiple lesions in the liver (suspected metastases) and peritoneal deposits and a few discrete lung nodules. Some areas of interloop collections were also noted. Biopsies were taken from the liver which revealed benign cirrhotic lesion. Relevant to the findings, multiple biopsies were performed and fluid was sent for evaluation. The liver biopsy came out positive for borderline vascular malignancy (epithelioid hemangioendothelioma). This was confirmed with the immunohistochemistry report. Epithelioid hemangioendothelioma occurs mostly in soft tissues of extremity and lungs. The involvement of the liver may be seen as metastasis or rarely as a primary tumor. The incidence of primary malignant hepatic hemangioendothelioma is about 0.1/100,000; the mean age at the time of diagnosis is 41.7 years, and male:female ratio is 2:3. Liver transplantation, hepatectomy, chemoembolization, radiotherapy, and chemotherapeutic agents are reported treatment regimens. Malignant EHE of liver presents as multiple hepatic nodules. Being locally aggressive, it can invade the peritoneum, gut, and lungs. Orthoptic liver transplantation appears to be the only remedy because of the multifocal nature of the disease. Partial hepatectomy is possible for localized tumors.

Staging Cancer: TNM Literature 1963—1982

1983 ◽  
Vol 22 (04) ◽  
pp. 218-226
Author(s):  
G. Wagner ◽  
G. Posner ◽  
T. Wolf
Keyword(s):  
Soft Tissues ◽  
Tnm Classification ◽  
Primary Tumour ◽  
Malignant Tumour ◽  
Field Studies ◽  
Malignant Tumours ◽  
Specific Therapy ◽  
Tnm System ◽  
Ann Arbor ◽  
The Central Nervous System

The prognosis of a malignant tumour largely depends on the stage of the disease at the beginning of a specific therapy; statistics of therapy are comparable only if they show the distribution of the cases to the different stages of the disease. Aiming to improve the comparability of the data and findings acquired from the cancer patient, the UICC has, for more than 30 years, been endeavouring to develop uniform rules for acquiring the stage of the disease and the degree of spread with malignant tumours. For this purpose, UICC has developed the TNM system which makes it possible to state accurately and in a reproduceable form the stage of the primary tumour (T) and the regional lymphatic nodes (N) as well as the possible existence of metastases (M). The third edition of the TNM rules appeared in English in 1978, in the following years in numerous other languages. Today the TNM system represents the only basis for the documentation of the cancer stage which has been introduced worldwide. However, it is by no means »complete«; for some forms of cancer proposals for TNM classification are still outstanding (e.g., tumours of the pancreas, the central nervous system, the bones and the soft tissues). Moreover, malignant lymphomas cannot be classified according to the principles of TNM. Here the so-called »Ann Arbor Classification« from the year 1971 is recommended.The UICC has called upon its national committees to check the classification proposals available so far as to their usefulness and practicability. Corresponding field studies are under way in several countries. This bibliography covers a section of the TNM literature of the last 20 years. From a total number of about 2,000 publications, applying the TNM system, we selected 300 which discuss the system critically and, in most instances, make suggestions for its improvement. The first part of the bibliography contains an alphabetically arranged list of the titles according to authors’ names; the second part differentiates the tumors dealt with by their site.We thank Mrs. L. Blumenthal, Mrs. I. Schäfer and Mrs. U. Wolber for their cooperation in setting up this bibliography.

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