Primary Hepatic Gastrinoma Causing Zollinger-Ellison Syndrome: A Rare and Challenging Diagnosis
The majority of gastrinomas causing Zollinger-Ellison syndrome (ZES) are located in the duodenum or the pancreas. Primary hepatic gastrinomas (PHG) are extremely rare and difficult to diagnose because the liver is the commonest site of metastatic disease and gastrinomas can be very small. Furthermor...
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2012-02-01
|
| Series: | Cancers |
| Subjects: | |
| Online Access: | http://www.mdpi.com/2072-6694/4/1/130/ |
| _version_ | 1797765953415544832 |
|---|---|
| author | Elijah Dixon Janice L. Pasieka Hassan Al-Bisher Adrian Harvey |
| author_facet | Elijah Dixon Janice L. Pasieka Hassan Al-Bisher Adrian Harvey |
| author_sort | Elijah Dixon |
| collection | DOAJ |
| description | The majority of gastrinomas causing Zollinger-Ellison syndrome (ZES) are located in the duodenum or the pancreas. Primary hepatic gastrinomas (PHG) are extremely rare and difficult to diagnose because the liver is the commonest site of metastatic disease and gastrinomas can be very small. Furthermore, gastrinomas are typically slow-growing thus a missed, occult primary tumour may not become evident for many years. The diagnosis of PHG is therefore dependent on a careful search for a primary and long-term biochemical follow-up following curative hepatic resection. We report a case of a 7 cm PHG in a 48 year old man with ZES. Preoperatively, both a basal and stimulated gastrin levels were elevated. Surgical exploration including intraoperative ultrasound and duodenotomy, failed to reveal a primary. Patient underwent a right hepatectomy. Yearly, gastrin and secretin stimulation tests remain normal 6 years following surgery. He remains symptom free off all medication. An additional 26 cases of PHG were found. Including this case, 21 had at least 1 year follow-up, however only eight had greater than 5 years (median 24 months). Post-op gastrin levels were reported in 25, however provocative testing was done in only 10. Persistence and recurrence occurred in one and four, respectively. PHG causing ZES is extremely rare. Although the current literature claims to include 26 additional cases of PHG, without a thorough search for the primary and long-term follow-up data including provocative testing, this diagnosis remains a challenge. |
| first_indexed | 2024-03-12T20:17:34Z |
| format | Article |
| id | doaj.art-6b5efa0e24d9425ab074d3123a749d63 |
| institution | Directory Open Access Journal |
| issn | 2072-6694 |
| language | English |
| last_indexed | 2024-03-12T20:17:34Z |
| publishDate | 2012-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cancers |
| spelling | doaj.art-6b5efa0e24d9425ab074d3123a749d632023-08-02T01:11:05ZengMDPI AGCancers2072-66942012-02-014113014010.3390/cancers4010130Primary Hepatic Gastrinoma Causing Zollinger-Ellison Syndrome: A Rare and Challenging DiagnosisElijah DixonJanice L. PasiekaHassan Al-BisherAdrian HarveyThe majority of gastrinomas causing Zollinger-Ellison syndrome (ZES) are located in the duodenum or the pancreas. Primary hepatic gastrinomas (PHG) are extremely rare and difficult to diagnose because the liver is the commonest site of metastatic disease and gastrinomas can be very small. Furthermore, gastrinomas are typically slow-growing thus a missed, occult primary tumour may not become evident for many years. The diagnosis of PHG is therefore dependent on a careful search for a primary and long-term biochemical follow-up following curative hepatic resection. We report a case of a 7 cm PHG in a 48 year old man with ZES. Preoperatively, both a basal and stimulated gastrin levels were elevated. Surgical exploration including intraoperative ultrasound and duodenotomy, failed to reveal a primary. Patient underwent a right hepatectomy. Yearly, gastrin and secretin stimulation tests remain normal 6 years following surgery. He remains symptom free off all medication. An additional 26 cases of PHG were found. Including this case, 21 had at least 1 year follow-up, however only eight had greater than 5 years (median 24 months). Post-op gastrin levels were reported in 25, however provocative testing was done in only 10. Persistence and recurrence occurred in one and four, respectively. PHG causing ZES is extremely rare. Although the current literature claims to include 26 additional cases of PHG, without a thorough search for the primary and long-term follow-up data including provocative testing, this diagnosis remains a challenge.http://www.mdpi.com/2072-6694/4/1/130/gastrinomaprimary hepatic gastrinomaZollinger-Ellison syndrome |
| spellingShingle | Elijah Dixon Janice L. Pasieka Hassan Al-Bisher Adrian Harvey Primary Hepatic Gastrinoma Causing Zollinger-Ellison Syndrome: A Rare and Challenging Diagnosis Cancers gastrinoma primary hepatic gastrinoma Zollinger-Ellison syndrome |
| title | Primary Hepatic Gastrinoma Causing Zollinger-Ellison Syndrome: A Rare and Challenging Diagnosis |
| title_full | Primary Hepatic Gastrinoma Causing Zollinger-Ellison Syndrome: A Rare and Challenging Diagnosis |
| title_fullStr | Primary Hepatic Gastrinoma Causing Zollinger-Ellison Syndrome: A Rare and Challenging Diagnosis |
| title_full_unstemmed | Primary Hepatic Gastrinoma Causing Zollinger-Ellison Syndrome: A Rare and Challenging Diagnosis |
| title_short | Primary Hepatic Gastrinoma Causing Zollinger-Ellison Syndrome: A Rare and Challenging Diagnosis |
| title_sort | primary hepatic gastrinoma causing zollinger ellison syndrome a rare and challenging diagnosis |
| topic | gastrinoma primary hepatic gastrinoma Zollinger-Ellison syndrome |
| url | http://www.mdpi.com/2072-6694/4/1/130/ |
| work_keys_str_mv | AT elijahdixon primaryhepaticgastrinomacausingzollingerellisonsyndromearareandchallengingdiagnosis AT janicelpasieka primaryhepaticgastrinomacausingzollingerellisonsyndromearareandchallengingdiagnosis AT hassanalbisher primaryhepaticgastrinomacausingzollingerellisonsyndromearareandchallengingdiagnosis AT adrianharvey primaryhepaticgastrinomacausingzollingerellisonsyndromearareandchallengingdiagnosis |