- Email: [email protected]
A Rare Cause of a Subepithelial Periampullary Duodenal Lesion
Accepted Manuscript A Rare Cause of a Subepithelial Periampullary Duodenal Lesion Majid Alsahafi, David Owen, Fergal Donnellan
PII: DOI: Reference:
S1542-3565(17)30059-9 10.1016/j.cgh.2017.01.010 YJCGH 55074
To appear in: Clinical Gastroenterology and Hepatology Accepted Date: 17 January 2017 Please cite this article as: Alsahafi M, Owen D, Donnellan F, A Rare Cause of a Subepithelial Periampullary Duodenal Lesion, Clinical Gastroenterology and Hepatology (2017), doi: 10.1016/ j.cgh.2017.01.010. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
A Rare Cause of a Subepithelial Periampullary Duodenal Lesion
Majid Alsahafi 1, David Owen 2 and Fergal Donnellan 1 Division of Gastroenterology, 2 Department of Pathology, Vancouver General Hospital, University of British Columbia, Vancouver, Canada
RI PT
1
SC
Corresponding Author:
Fergal Donnellan MD, Division of Gastroenterology, Vancouver General Hospital, University
M AN U
of British Columbia, 5153 - 2775 Laurel Street, Vancouver V5Z 1M9, Canada. [email protected] Tel: +1604-875-5244
TE D
Fax: +1 604-875-5161
No conflict of interest for all authors. Funding: none
EP
Contributions:
Dr.Majid Alsahafi reviewed the literature and wrote the manuscript, Dr.Fergal Donnellan,
AC C
edited and approved the manuscript, Dr.David Owen edited the manuscript and provided the pathology slides.
ACCEPTED MANUSCRIPT
A 71 year-old man presented with vague upper abdominal pain, loose stools and anorexia. His past medical history was significant for coronary artery disease, hypertension, dyslipidemia, gout and
RI PT
depression. The abdominal examination was unremarkable. He underwent a colonoscopy which was unremarkable except for a small adenomatous polyp, and an esophagogastroduodenoscopy which revealed a subepithelial lesion at the ampulla (Figure A). Computed tomography scan of the abdomen showed a 1.9 cm non-calcified soft tissue mass in the periampullary region. Subsequently, endoscopic
SC
ultrasound revealed a 1.7 cm subepithelial periampullary mass with no associated pancreatic or bile duct involvement (Figure B). No regional lymphadenopathy was noted. The EUS guided biopsy revealed
M AN U
uniform cells with a pseudo-glandular architecture and eosinophilic cytoplasm. Somatostatin stain was diffusely positive (Figure C). Somatostatin receptor scintigraphy scan was normal. The patient underwent a Whipple procedure. Pathological examination revealed a 2 cm somatostatinoma with 3 out of 28 peripancreatic lymph nodes positive for metastatic disease. Postoperatively, the patient had a pancreatic leakage treated conservatively with total parenteral nutrition and antibiotics. No adjuvant chemotherapy
TE D
was given.
Somatostatinomas are rare neuroendocrine tumors that account for less than 1% of all 1
gastrointestinal stromal tumors. Approximately 56% of somatostatinomas originate in the pancreas and
EP
are usually functional. Extra-pancreatic somatostatinomas are commonly located in the duodenum but 2
occasionally in the biliary tract or the rest of small bowel. Duodenal somatostatinomas are usually located in the periampullary region and frequently associated with Von Recklinghausen disease.
1,2
In
AC C
contrast to pancreatic somatostatinomas, duodenal somatostatinomas are rarely associated with the somatostatin syndrome which is characterized by the triad of hyperglycemia, steatorrhea and cholelethiasis.
1,3
Patients with periampullary somatostatinomas often present with obstructive jaundice,
gastrointestinal bleeding or non-specific symptoms such as abdominal pain. Occasionally the tumor can be detected after an attack of pancreatitis or incidentally discovered.
2
The diagnosis is usually suspected by the characteristic features on the biopsy and then confirmed by the positive staining for somatostatin. The presence of psammoma bodies is a characteristic
ACCEPTED MANUSCRIPT
feature of duodenal somatostatinomas and rarely seen in tumors located in the pancreas. tomography imaging is essential to rule out metastatic disease.
2,3
Computed
Endoscopic ultrasound has several
advantages including small tumors localization, accurate staging, and preoperative diagnosis through 1
diagnostic sampling. The tumor commonly metastasizes to the liver and regional lymph nodes.
1,2
Because of the high malignant potential, surgical resection is the treatment of choice in the absence of
1
M AN U
SC
periampullary tumors less than 1 cm in size.
RI PT
metastatic disease. Endoscopic resection is considered to be a potential option for localized non-
References:
1. Hoffmann KM, Furukawa M, Jensen RT. Duodenal neuroendocrine tumors: Classification, functional syndromes, diagnosis and medical treatment. Best Pract Res Clin Gastroenterol. 2005;19:675-97.
TE D
2. Nesi G, Marcucci T, Rubio CA et al. Somatostatinoma:clinico-pathological features of three cases and literature reviewed. J Gastroenterol Hepatol. 2008;23:521-6.
AC C
EP
3. Green BT, Rockey DC. Duodenal somatostatinoma presenting with complete somatostatinoma syndrome. J Clin Gastroenterol. 2001;33:415-7.
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
PII: DOI: Reference:
S1542-3565(17)30059-9 10.1016/j.cgh.2017.01.010 YJCGH 55074
To appear in: Clinical Gastroenterology and Hepatology Accepted Date: 17 January 2017 Please cite this article as: Alsahafi M, Owen D, Donnellan F, A Rare Cause of a Subepithelial Periampullary Duodenal Lesion, Clinical Gastroenterology and Hepatology (2017), doi: 10.1016/ j.cgh.2017.01.010. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
A Rare Cause of a Subepithelial Periampullary Duodenal Lesion
Majid Alsahafi 1, David Owen 2 and Fergal Donnellan 1 Division of Gastroenterology, 2 Department of Pathology, Vancouver General Hospital, University of British Columbia, Vancouver, Canada
RI PT
1
SC
Corresponding Author:
Fergal Donnellan MD, Division of Gastroenterology, Vancouver General Hospital, University
M AN U
of British Columbia, 5153 - 2775 Laurel Street, Vancouver V5Z 1M9, Canada. [email protected] Tel: +1604-875-5244
TE D
Fax: +1 604-875-5161
No conflict of interest for all authors. Funding: none
EP
Contributions:
Dr.Majid Alsahafi reviewed the literature and wrote the manuscript, Dr.Fergal Donnellan,
AC C
edited and approved the manuscript, Dr.David Owen edited the manuscript and provided the pathology slides.
ACCEPTED MANUSCRIPT
A 71 year-old man presented with vague upper abdominal pain, loose stools and anorexia. His past medical history was significant for coronary artery disease, hypertension, dyslipidemia, gout and
RI PT
depression. The abdominal examination was unremarkable. He underwent a colonoscopy which was unremarkable except for a small adenomatous polyp, and an esophagogastroduodenoscopy which revealed a subepithelial lesion at the ampulla (Figure A). Computed tomography scan of the abdomen showed a 1.9 cm non-calcified soft tissue mass in the periampullary region. Subsequently, endoscopic
SC
ultrasound revealed a 1.7 cm subepithelial periampullary mass with no associated pancreatic or bile duct involvement (Figure B). No regional lymphadenopathy was noted. The EUS guided biopsy revealed
M AN U
uniform cells with a pseudo-glandular architecture and eosinophilic cytoplasm. Somatostatin stain was diffusely positive (Figure C). Somatostatin receptor scintigraphy scan was normal. The patient underwent a Whipple procedure. Pathological examination revealed a 2 cm somatostatinoma with 3 out of 28 peripancreatic lymph nodes positive for metastatic disease. Postoperatively, the patient had a pancreatic leakage treated conservatively with total parenteral nutrition and antibiotics. No adjuvant chemotherapy
TE D
was given.
Somatostatinomas are rare neuroendocrine tumors that account for less than 1% of all 1
gastrointestinal stromal tumors. Approximately 56% of somatostatinomas originate in the pancreas and
EP
are usually functional. Extra-pancreatic somatostatinomas are commonly located in the duodenum but 2
occasionally in the biliary tract or the rest of small bowel. Duodenal somatostatinomas are usually located in the periampullary region and frequently associated with Von Recklinghausen disease.
1,2
In
AC C
contrast to pancreatic somatostatinomas, duodenal somatostatinomas are rarely associated with the somatostatin syndrome which is characterized by the triad of hyperglycemia, steatorrhea and cholelethiasis.
1,3
Patients with periampullary somatostatinomas often present with obstructive jaundice,
gastrointestinal bleeding or non-specific symptoms such as abdominal pain. Occasionally the tumor can be detected after an attack of pancreatitis or incidentally discovered.
2
The diagnosis is usually suspected by the characteristic features on the biopsy and then confirmed by the positive staining for somatostatin. The presence of psammoma bodies is a characteristic
ACCEPTED MANUSCRIPT
feature of duodenal somatostatinomas and rarely seen in tumors located in the pancreas. tomography imaging is essential to rule out metastatic disease.
2,3
Computed
Endoscopic ultrasound has several
advantages including small tumors localization, accurate staging, and preoperative diagnosis through 1
diagnostic sampling. The tumor commonly metastasizes to the liver and regional lymph nodes.
1,2
Because of the high malignant potential, surgical resection is the treatment of choice in the absence of
1
M AN U
SC
periampullary tumors less than 1 cm in size.
RI PT
metastatic disease. Endoscopic resection is considered to be a potential option for localized non-
References:
1. Hoffmann KM, Furukawa M, Jensen RT. Duodenal neuroendocrine tumors: Classification, functional syndromes, diagnosis and medical treatment. Best Pract Res Clin Gastroenterol. 2005;19:675-97.
TE D
2. Nesi G, Marcucci T, Rubio CA et al. Somatostatinoma:clinico-pathological features of three cases and literature reviewed. J Gastroenterol Hepatol. 2008;23:521-6.
AC C
EP
3. Green BT, Rockey DC. Duodenal somatostatinoma presenting with complete somatostatinoma syndrome. J Clin Gastroenterol. 2001;33:415-7.
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT