Epithelioid Hemangioendothelioma of the Liver Showing Spontaneous Complete Regression after the Cessation of Methotrexate Intake
A 71-year-old man with slight fever and dull abdominal pain was referred to our hospital. He had been receiving methotrexate (MTX) to treat his rheumatoid arthritis for more than 6 years but stopped taking MTX after admission due to the rapid aggravation of his liver function. Computed tomography (C...
| Main Authors: | , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Karger Publishers
2023-08-01
|
| Series: | Case Reports in Oncology |
| Subjects: | |
| Online Access: | https://beta.karger.com/Article/FullText/531133 |
| _version_ | 1827822808917344256 |
|---|---|
| author | Takahiro Shishimoto Shoji Oura Kenichiro Motozato Hiroto Tanaka Seigo Takamatsu Wataru Ono |
| author_facet | Takahiro Shishimoto Shoji Oura Kenichiro Motozato Hiroto Tanaka Seigo Takamatsu Wataru Ono |
| author_sort | Takahiro Shishimoto |
| collection | DOAJ |
| description | A 71-year-old man with slight fever and dull abdominal pain was referred to our hospital. He had been receiving methotrexate (MTX) to treat his rheumatoid arthritis for more than 6 years but stopped taking MTX after admission due to the rapid aggravation of his liver function. Computed tomography (CT) showed multiple liver lesions with late enhancement, highly suggesting them to be cholangiocarcinomas. Tumor marker levels were normal except for a slightly elevated PIVKA-II level, i.e., 45 mAU/mL (range 0–40 mAU/mL). We did a biopsy to the largest lesion and endoscopic biliary drainage to make a definitive diagnosis of the hepatic lesions and treat jaundice, respectively. Pathological study showed round, polygonal, and spindle-shaped epithelial atypical cells growing in a sarcomatoid fashion. Atypical cells were positive for CD31, CD34, vimentin, and TFE3, and some of them had intracellular vacuoles, leading to the diagnosis of epithelioid hemangioendothelioma (EHE) of the liver. The patient got well 4 weeks after the endoscopic biliary drainage. CTs showed marked regression of the EHE lesions 3 months after biliary drainage and complete regression in 12 months. The patient further developed Hodgkin lymphoma in the para-aortic lymph nodes 23 months after the biliary drainage and is now under chemotherapy for the malignant lymphoma. We, however, have not detected any EHE lesions in the liver or distant organs for at least 16 months after the confirmation of complete regression of the EHE lesions. Oncologists should note the spontaneous regression of the EHE and investigate the correlation between MTX cessation and EHE regression. |
| first_indexed | 2024-03-12T02:03:56Z |
| format | Article |
| id | doaj.art-186a35fa6f78450d886386428190bf14 |
| institution | Directory Open Access Journal |
| issn | 1662-6575 |
| language | English |
| last_indexed | 2024-03-12T02:03:56Z |
| publishDate | 2023-08-01 |
| publisher | Karger Publishers |
| record_format | Article |
| series | Case Reports in Oncology |
| spelling | doaj.art-186a35fa6f78450d886386428190bf142023-09-07T07:56:38ZengKarger PublishersCase Reports in Oncology1662-65752023-08-0116162863310.1159/000531133531133Epithelioid Hemangioendothelioma of the Liver Showing Spontaneous Complete Regression after the Cessation of Methotrexate IntakeTakahiro Shishimoto0Shoji Oura1Kenichiro Motozato2Hiroto Tanaka3Seigo Takamatsu4Wataru Ono5Department of Gastroenterology, Kishiwada Tokushukai Hospital, Kishiwada, JapanDepartment of Surgery, Kishiwada Tokushukai Hospital, Kishiwada, JapanDepartment of Gastroenterology, Kishiwada Tokushukai Hospital, Kishiwada, JapanDepartment of Gastroenterology, Kishiwada Tokushukai Hospital, Kishiwada, JapanDepartment of Gastroenterology, Kishiwada Tokushukai Hospital, Kishiwada, JapanDepartment of Gastroenterology, Kishiwada Tokushukai Hospital, Kishiwada, JapanA 71-year-old man with slight fever and dull abdominal pain was referred to our hospital. He had been receiving methotrexate (MTX) to treat his rheumatoid arthritis for more than 6 years but stopped taking MTX after admission due to the rapid aggravation of his liver function. Computed tomography (CT) showed multiple liver lesions with late enhancement, highly suggesting them to be cholangiocarcinomas. Tumor marker levels were normal except for a slightly elevated PIVKA-II level, i.e., 45 mAU/mL (range 0–40 mAU/mL). We did a biopsy to the largest lesion and endoscopic biliary drainage to make a definitive diagnosis of the hepatic lesions and treat jaundice, respectively. Pathological study showed round, polygonal, and spindle-shaped epithelial atypical cells growing in a sarcomatoid fashion. Atypical cells were positive for CD31, CD34, vimentin, and TFE3, and some of them had intracellular vacuoles, leading to the diagnosis of epithelioid hemangioendothelioma (EHE) of the liver. The patient got well 4 weeks after the endoscopic biliary drainage. CTs showed marked regression of the EHE lesions 3 months after biliary drainage and complete regression in 12 months. The patient further developed Hodgkin lymphoma in the para-aortic lymph nodes 23 months after the biliary drainage and is now under chemotherapy for the malignant lymphoma. We, however, have not detected any EHE lesions in the liver or distant organs for at least 16 months after the confirmation of complete regression of the EHE lesions. Oncologists should note the spontaneous regression of the EHE and investigate the correlation between MTX cessation and EHE regression.https://beta.karger.com/Article/FullText/531133epithelioid hemangioendotheliomalivermethotrexatespontaneous regression |
| spellingShingle | Takahiro Shishimoto Shoji Oura Kenichiro Motozato Hiroto Tanaka Seigo Takamatsu Wataru Ono Epithelioid Hemangioendothelioma of the Liver Showing Spontaneous Complete Regression after the Cessation of Methotrexate Intake Case Reports in Oncology epithelioid hemangioendothelioma liver methotrexate spontaneous regression |
| title | Epithelioid Hemangioendothelioma of the Liver Showing Spontaneous Complete Regression after the Cessation of Methotrexate Intake |
| title_full | Epithelioid Hemangioendothelioma of the Liver Showing Spontaneous Complete Regression after the Cessation of Methotrexate Intake |
| title_fullStr | Epithelioid Hemangioendothelioma of the Liver Showing Spontaneous Complete Regression after the Cessation of Methotrexate Intake |
| title_full_unstemmed | Epithelioid Hemangioendothelioma of the Liver Showing Spontaneous Complete Regression after the Cessation of Methotrexate Intake |
| title_short | Epithelioid Hemangioendothelioma of the Liver Showing Spontaneous Complete Regression after the Cessation of Methotrexate Intake |
| title_sort | epithelioid hemangioendothelioma of the liver showing spontaneous complete regression after the cessation of methotrexate intake |
| topic | epithelioid hemangioendothelioma liver methotrexate spontaneous regression |
| url | https://beta.karger.com/Article/FullText/531133 |
| work_keys_str_mv | AT takahiroshishimoto epithelioidhemangioendotheliomaofthelivershowingspontaneouscompleteregressionafterthecessationofmethotrexateintake AT shojioura epithelioidhemangioendotheliomaofthelivershowingspontaneouscompleteregressionafterthecessationofmethotrexateintake AT kenichiromotozato epithelioidhemangioendotheliomaofthelivershowingspontaneouscompleteregressionafterthecessationofmethotrexateintake AT hirototanaka epithelioidhemangioendotheliomaofthelivershowingspontaneouscompleteregressionafterthecessationofmethotrexateintake AT seigotakamatsu epithelioidhemangioendotheliomaofthelivershowingspontaneouscompleteregressionafterthecessationofmethotrexateintake AT wataruono epithelioidhemangioendotheliomaofthelivershowingspontaneouscompleteregressionafterthecessationofmethotrexateintake |