Neuroendocrine tumours (NET) are rare. They usually arise from the gastrointestinal or bronchopulmonary systems. Most are discovered incidentally and the small bowel tumours pose special difficulty in detection and treatment. Primary mesenteric involvement is very rare. Here we report such a case with a liver metastasis. This was preoperatively diagnosed and treated by enucleation of the mesenteric tumour together with right hepatectomy in a single sitting.
Figures - uploaded by Lileswar Kaman
Author content
All figure content in this area was uploaded by Lileswar Kaman
Content may be subject to copyright.
Discover the world's research
- 20+ million members
- 135+ million publications
- 700k+ research projects
Join for free
Tropical Doctor
2020, Vol. 50(1) 65–68
!The Author(s) 2019
Article reuse guidelines:
sagepub.com/journals-permissions
DOI: 10.1177/0049475519887657
journals.sagepub.com/home/tdo
Case Report
Primary mesenteric neuroendocrine
tumour with liver metastasis: a common
presentation of an uncommon tumour
Ambuj Agarwal
1
, Lileswar Kaman
2
, Ashish Gupta
3
,
Krishna Ramavath
4
and Kim Vaiphei
5
Abstract
Neuroendocrine tumours (NET) are rare. They usually arise from the gastrointestinal or bronchopulmonary systems.
Most are discovered incidentally and the small bowel tumours pose special difficulty in detection and treatment. Primary
mesenteric involvement is very rare. Here we report such a case with a liver metastasis. This was preoperatively
diagnosed and treated by enucleation of the mesenteric tumour together with right hepatectomy in a single sitting.
Keywords
Neuroendocrine tumour, mesenteric neuroendocrine tumour, liver metastasis, chromogranin
Background
The neuroendocrine tumour (NET) arises from neuroen-
docrine or the Kulchitsky cells.
1
These are rare tumours
with a reported incidence of approximately 3.56 per
100,000 people.
1
The majority (55%) arise from the
gastrointestinal tract. They primarily involve the
midgut, followed less commonly by the appendix and
colon.
2
The median age of diagnosis of midgut NET is
64 years.
3
The mesentery is usually involved secondary
to direct infiltration from the involved bowel or by meta-
static involvement.
4,5
Primary mesenteric NET has been
rarely reported
6–8
and liver metastasis is still rarer.
Case report
A 67-year-old woman presented with right hypochondrial
and epigastric pain for the previous six months. This was
associated with episodic bilious vomiting 30 min after
taking a meal. There was no history of jaundice, mass,
constipation, loss of weight, diarrhoea or facial flushing.
She was known to be treated for hypertension.
On examination, her vital signs were normal.
Abdominal examination revealed a liver palpable 4 cm
below the right costal margin with a firm consistency
and nodular surface. Liver span measured 20 cm.
Her biochemical investigations were normal. A con-
trast-enhanced computed tomography (CT) scan of the
abdomen demonstrated a heterogeneously enhancing
mass lesion 3.8 2.8 cm in size in the root of the mesen-
tery near the duodenojejunal flexure closely abutting the
proximal jejunal loop (Figure 1a, black arrow). The liver
was enlarged (20.4 cm) with a well-defined hypervascular
lesion with central necrosis, measuring 11.7 8.6 cm, in
segments VII and VIII. In the venous phase, the lesion
was isodense with the parenchyma, without calcification
(Figure 1b, black arrow).
The tumour markers CEA (1.08 ng/mL), CA-19–9
(12 IU) and AFP (2.32 ng/mL) were within normal
limits. Fine needle aspiration cytology of both masses
was suggestive of a neuroendocrine neoplasm.
Treatment
During surgery, minimal ascites was found; there was
a3 2 cm hard mass in the root of the small bowel
mesentery near the first jejunal branch of the superior
mesenteric artery with no other palpable growth along
the rest of mesentery and small bowel (Figure 2a, black
arrow). The liver was enlarged with a 20 8 8cm
1
Senior resident, Department of General Surgery, Post Graduate
Institute of Medical Education and Research, Chandigarh, India
2
Professor, Department of General Surgery, Post Graduate Institute of
Medical Education and Research, Chandigarh, India
3
Assistant Professor, Department of General Surgery, Post Graduate
Institute of Medical Education and Research, Chandigarh, India
4
Senior resident, Department of General Surgery, Post Graduate
Institute of Medical Education and Research, Chandigarh, India
5
Professor, Department of Histopathology, Post Graduate Institute of
Medical Education and Research, Chandigarh, India
Corresponding author:
Lileswar Kaman, Professor, Department of General Surgery, PGIMER,
Chandigarh, 160012 India.
Email: kamanlil@yahoo.com
Agarwal et al. 65
Figure 2. (a) Intraoperative photo showing a 3 2 cm hard mass in the root of the small bowel mesentery with the first jejunal
branch of the superior mesenteric artery passing through the mass. (b) Liver enlarged with hard mass in the right lobe. (c) Lesion
encapsulated with few cystic areas. Excised jejunal mesenteric neuroendocrine neoplasm. (d) Resected specimen of the right liver lobe.
Figure 1. (a) CT scan showing a well-defined hypervascular lesion in the jejunal mesentery at the duodenojejunal flexure level, with
overlying jejunum. (b) CT showing a well-defined hypervascular lesion with central necrosis in the right lobe of the liver.
66 Tropical Doctor 50(1)
hard mass present in the right lobe, the left lobe remain-
ing clear (Figure 2b). Resected specimens (Figure 2c
and d) were sent for histopathological examination:
the jejunal mesenteric mass measured 4 3.5 3.5 cm,
was well encapsulated, positive for synaptophysin and
chromogranin, and with a Ki67 index < 1%, hence con-
firming a neuroendocrine tumour grade 1 (Figure 3a
and c); the liver moiety likewise demonstrated similar
features (Figure 3b and d).
Recovery was well sustained after six months.
Discussion
Most mesenteric NETs are incidental findings. They may
present with small bowel obstruction, abdominal pain or
a mass.
7
Sweating, flushing and palpitations have been
reported by patients with classical carcinoid syndrome,
6
but such symptoms may be absent in the case of non-
secreting tumours or where substances are metabolised
in the liver. Thus, most mesenteric NETs are metastatic
at the time of presentation.
6
These patients usually have
normal biochemical profiles; however, serum chromo-
granin is usually raised, the sensitivity and specificity
of this test being in the range of 70–100%.
9,10
Surgical
extirpation, including draining enlarged lymph nodes, is
the mainstay of the treatment.
2
Declaration of conflicting interests
The author(s) declared no potential conflicts of interest with
respect to the research, authorship, and/or publication of
this article.
Funding
The author(s) received no financial support for the research,
authorship, and/or publication of this article.
ORCID iD
Lileswar Kaman https://orcid.org/0000-0003-1837-2614
Figure 3. (a) Histopathology of the mesenteric tumour, with a thick fibrous capsule; cut surface is firm, grey-white with areas of
haemorrhagic necrosis. (b) Two slices of resected liver specimen showing large, nodular, well-defined haemorrhagic tumour, reaching
the liver capsule. (c) Tumour photomicrograph showing sheets of medium-sized plasmacytoid tumour cells with nesting pattern. (d)
Immunohistochemistry showing strong diffuse cytoplasmic staining for chromogranin.
Agarwal et al. 67
References
1. Dasari A, Shen C, Halperin D, et al. Trends in the inci-
dence, prevalence, and survival outcomes in patients with
neuroendocrine tumors in the United States. JAMA
Oncol 2017; 3: 1335–1342.
2. Boudreaux JP, Klimstra DS, Hassan MM, et al. The
NANETS consensus guideline for the diagnosis and man-
agement of neuroendocrine tumors: well-differentiated
neuroendocrine tumors of the jejunum, ileum, appendix,
and cecum. Pancreas 2010; 39: 753–766.
3. Surveillance, Epidemiology, and End Results (SEER)
Program. SEER*Stat Database: SEER 17 Regs Nov
2006 sub (1973Y2004). Bethesda, MD: National Cancer
Institute, DCCPS, Surveillance Research Program,
Cancer Statistic Branch, 2006. Available at:
www.seer.cancer.gov.
4. Woodside KJ, Townsend CM Jr and Evers BM.
Current management of gastrointestinal carcinoid
tumors. J Gastrointest Surg 2004; 8: 742–756.
5. Barnardo DE, Stavrou M, Bourne R, et al. Primary car-
cinoid tumor of the mesentery. Hum Pathol 1984; 15:
796–798.
6. Karahan OI, Kahriman G, Yikilmaz A, et al.
Gastrointestinal carcinoid tumors in rare locations: ima-
ging findings. Clin Imaging 2006; 30: 278–282.
7. Park IS, Kye BH, Kim HS, et al. Primary mesenteric
carcinoid tumor. J Korean Surg Soc 2013; 84: 114–117.
8. Yamanuha J, Ballinger R, Coon D, et al. Carcinoid
tumor presenting as a primary mesenteric mass: a case
report and review of the literature. Hawaii Med J 2009;
68: 137–139.
9. Bernini GP, Moretti A, Ferdeghini M, et al. A new
human chromogranin 'A' immunoradiometric assay for
the diagnosis of neuroendocrine tumours. Br J Cancer
2001; 84: 636–642.
10. Bajetta E, Ferrari L, Martinetti A, et al. Chromogranin
A, neuron specific enolase, carcinoembryonic antigen,
and hydroxyindole acetic acid evaluation in patients
with neuroendocrine tumors. Cancer 1999; 86: 858–865.
Tropical Doctor
2020, Vol. 50(1) 68–70
!The Author(s) 2019
Article reuse guidelines:
sagepub.com/journals-permissions
DOI: 10.1177/0049475519870980
journals.sagepub.com/home/tdo
Case Report
Brunner's gland hamartoma presenting
as gastric outlet obstruction: unusual
presentation and review of literature
Amit Gupta
1
, Depak Rajput
2
, Utkarsh Kumar
3
,
Sweety Gupta
4
, Ashok Singh
5
and Sumit Sanyal
6
Abstract
Brunner's gland hamartoma (or Brunneroma) is an uncommon tumour with an incidence of < 0.01%, accounting for
approximately 5–10% of benign duodenal tumours. Usually asymptomatic, it may manifest occasionally with duodenal
obstruction or upper gastrointestinal haemorrhage and rarely with biliary fistulation, cholestatic jaundice and intussus-
ception. It may be associated with uraemia and chronic pancreatitis. The diagnosis is usually confirmed by imaging studies
and upper gastrointestinal endoscopy. Surgical excision or endoscopic resection is preferred for symptomatic large
hamartomas. Here we report a case of 45-year-old man presenting with features of pancreatitis and gastric outlet
obstruction due to a large Brunner's gland hamartoma, on his endoscopic, radiologic, surgical and pathologic findings.
Keywords
Digestive, physiology, surgery, treatment
1
Additional Professor, Department of Surgery AIIMS Rishikesh, Rishikesh,
India
2
Assistant Professor, Department of Surgery AIIMS Rishikesh, Rishikesh,
India
3
Senior Resident, Department of Surgery AIIMS Rishikesh, Rishikesh,
India
4
Assistant Professor, Department of Radiation Oncology AIIMS
Rishikesh, Rishikesh, India
5
Assistant Professor, Department of Pathology AIIMS Rishikesh,
Rishikesh, India
6
Additional Professor, Department of GI Surgery AIIMS Rishikesh,
Rishikesh, India
Corresponding author:
Amit Gupta, Additional Professor, Incharge HPB Unit 5th Floor Medical
College Block, Department of Surgery, AIIMS Rishikesh, 249203 India.
Email: dramit2411@gmail.com
68 Tropical Doctor 50(1)
ResearchGate has not been able to resolve any citations for this publication.
Primary mesenteric carcinoid tumor is very rare, although secondary mesenteric involvement is common, reported as 40% to 80%. And distant metastasis rate reported as 80% to 90%, when the size is larger than 2 cm. We present a case of very rare primary mesenteric carcinoid tumor showing benign character though large size. The patient visited St. Vincent's Hospital, The Catholic University of Korea with increasing palpable abdominal mass. At laparotomy, a well encapsulated mass arising from the mesentery near the ligament of Treitz was found without any adjacent organ invasion or distant metastasis. The mass was measured as 8.2 × 7.3 cm and histopathologically benign character. At 11 months of follow up, the patient was recurrence free.
Importance: The incidence and prevalence of neuroendocrine tumors (NETs) are thought to be rising, but updated epidemiologic data are lacking. Objective: To explore the evolving epidemiology and investigate the effect of therapeutic advances on survival of patients with NETs. Design, setting, and participants: A retrospective, population-based study using nationally representative data from the Surveillance, Epidemiology, and End Results (SEER) program was conducted to evaluate 64 971 patients with NETs from 1973 to 2012. Associated population data were used to determine annual age-adjusted incidence, limited-duration prevalence, and 5-year overall survival (OS) rates. Trends in survival from 2000 to 2012 were evaluated for the entire cohort as well as specific subgroups, including distant-stage gastrointestinal NETs and pancreatic NETs. Analyses were conducted between December 2015, and February 2017. Main outcomes and measures: Neuroendocrine tumor incidence, prevalence, and OS rates. Results: Of the 64 971 cases of NETs, 34 233 (52.7%) were women. The age-adjusted incidence rate increased 6.4-fold from 1973 (1.09 per 100 000) to 2012 (6.98 per 100 000). This increase occurred across all sites, stages, and grades. In the SEER 18 registry grouping (2000-2012), the highest incidence rates were 1.49 per 100 000 in the lung, 3.56 per 100 000 in gastroenteropancreatic sites, and 0.84 per 100 000 in NETs with an unknown primary site. The estimated 20-year limited-duration prevalence of NETs in the United States on January 1, 2014, was 171 321. On multivariable analyses, the median 5-year OS rate varied significantly by stage, grade, age at diagnosis, primary site, and time period of diagnosis. The OS rate for all NETs improved from the 2000-2004 period to the 2009-2012 period (hazard ratio [HR], 0.79; 95% CI, 0.73-0.85). Even larger increases in OS between these periods were noted in distant-stage gastrointestinal NETs (HR, 0.71; 95% CI, 0.62-0.81) and distant-stage pancreatic NETs (HR, 0.56; 95% CI, 0.44-0.70). Conclusions and relevance: The incidence and prevalence of NETs are steadily rising, possibly owing to detection of early-stage disease and stage migration. Survival for all NETs has improved over time, especially for distant-stage gastrointestinal NETs and pancreatic NETs in particular, reflecting improvement in therapies. These data will help to prioritize future research directions.
- Emilio Bajetta M.D
- Leonardo Ferrari M.Sc
-
![Antonia Martinetti]()
- Emilio Bombardieri M.D
BACKGROUND Chromogranin A (CgA), neuron specific enolase (NSE), carcinoembryonic antigen (CEA), and urinary 5-hydroxyindole-3-acetic acid (5-HIAA) are the markers currently used in the diagnosis, prognosis, and follow-up of patients with neuroendocrine tumors (NETs). The authors examined the role of such biomarkers in a large series of patients with NETs.METHODS One hundred and twenty-seven patients entered the study. Multiple blood and 24-hour urine specimens were assayed for biomarker quantitation.RESULTSThe accuracy of each marker was assessed in patients with (n = 106) and without (n = 21) disease. CgA proved to be the best marker (specificity of 85.7% and sensitivity of 67.9%). Patients with disease had significantly higher CgA and NSE levels compared with disease free patients (P = 0.00003 and P = 0.00240, respectively). NSE and 5-HIAA determination showed a very high specificity (100%) but a rather low sensitivity (32.9% and 35.1%, respectively). CEA was found to have little diagnostic value (sensitivity of 15.4%). CgA was the most sensitive marker for detecting patients with disseminated disease and 5-HIAA displayed the highest sensitivity in identifying syndromic patients. Tumor marker modifications were studied during follow-up. In particular, rises in CgA were associated with progressive disease in 83.3% of cases and stable CgA was associated with stable disease in 53.8% of cases. The relation between CgA modifications and liver lesions during follow-up also was studied; increases in CgA levels were associated with local progression in 100% of cases and stable marker levels were found in 68.7% of the patients with unchanged lesions.CONCLUSIONS The results of the current study demonstrate that CgA has the highest accuracy and is the most reliable biomarker reflecting the clinical evolution of NETs. Cancer 1999;86:858–65. © 1999 American Cancer Society.
-
![J. Philip Boudreaux]()
- David S Klimstra
- Manal Hassan
- James C Yao
Well-differentiated neuroendocrine tumors (NETs) of the jejunum, ileum, and appendix are also collectively known as midgut carcinoids. Similar to NETs in general, the diagnosed incidence of the midgut NETs is on the rise. Their presenting symptoms vary depending on stage and primary site. Local-regional NETs often present with vague and nonspecific symptoms. Classic carcinoid syndrome is more likely to appear in patients with advanced disease. Local-regional NETs of the small bowel should be resected whenever possible. With the exception of small well-differentiated NETs of the appendix, NETs of the midgut have substantial risk of relapse after resection and need to be followed for at least 7 years.Metastatic/advanced NETs of the midgut are incurable. Optimal management requires a multidisciplinary approach. Somatostatin analogs are effective in the management of carcinoid syndrome. Octreotide long-acting release has also recently been shown to delay disease progression. Liver-directed therapy and surgical debulking can improve quality of life in selected patients. Pivotal phase 3 studies with bevacizumab targeting vascular endothelial growth factor and everolimus targeting mTOR (mammalian target of rapamycin) are ongoing and may lead to improved outcome. Further studies of novel approaches such as peptide receptor radiotherapy are also warranted.
- Justin Yamanuha
- Ray Ballinger
- David Coon
- James Navin
We report a case of a primary carcinoid tumor arising in the mesentery of the small bowel of a 52-year-old man. The mesenteric mass in this patient was first identified as an incidental finding on CT imaging for bilateral nephrolithiasis. As a result of the abnormal findings, follow-up evaluation identified a partially calcified lobulated mesenteric lymph node. Diagnostic laparoscopy with lymph node sampling showed no evidence of hepatic or other metastatic disease. Pathologic analysis and immunohistochemistry of the mesenteric tissue revealed a malignant neuroendocrine tumor. Since all radiographic studies and subsequent workup revealed no evidence of other primary masses, this was determined to be a primary carcinoid tumor arising in the mesentery of the small bowel. Primary mesenteric carcinoid tumors are very rare because of their location of origin.
- David E. Barnardo
- MD BS MRCPath Margaret Stavrou
- Roger Bourne
- Wladimir V. Bogomoletz
A primary carcinoid tumor of the mesentery was associated with intestinal obstruction in a 74-year-old man. Increased levels of plasma pancreatic polypeptide were subsequently demonstrated. An autopsy performed four years later showed carcinoid metastatic dissemination and confirmed the primary mesenteric origin of the carcinoid tumor initially resected at surgery.
-
![Giampaolo Bernini]()
- Angelica Moretti
- M Ferdeghini
- Antonio Salvetti
We investigated whether plasma chromogranin A (CgA), measured by a new immunoradiometric assay, may be a sensitive and specific marker of phaeochromocytoma and of other neuroendocrine tumours. This study involved 121 patients of whom 20 with phaeochromocytoma, 28 with other neuroendocrine tumours (19 gastroenteropancreatic tumors, 3 medullary thyroid and 6 small cell lung carcinomas), 25 with solid nonfunctioning adrenocortical tumours and 48 with essential hypertension. In addition, 130 normal subjects were taken as controls. Plasma catecholamines were measured by using high-performance liquid chromatography, and CgA by a two-site sandwich immunoradiometric assay involving monoclonal antibodies raised against the unprocessed central domain (145-245) of human CgA. Plasma CgA in controls (49.0 ± 3.1 ng ml -1, mean ± SE) and in essential hypertensives (50.8 ± 3.5 ng ml -1) was lower (P < 0.0001) than in adrenocortical tumours (91.8 ± 13.2 ng ml -1), in phaeochromocytomas (254 ±49 ng ml -1) and in patients with other neuroendocrine tumours (469 ±84 ng ml -1). Plasma CgA and catecholamines identified 13 and 18 out of 20 phaeochromocytomas with sensitivity of 65% and 90%, respectively. Combined measurement of both markers improved sensitivity up to 100%. In the other neuroendocrine tumours, CgA was abnormal in 23/28 cases (sensitivity 82%) and in 6 it was the only circulating marker of disease. In gastroenteropancreatic tumours, CgA measurement identified all cases (sensitivity 100%). Specificity of CgA in patients with essential hypertension was 98%. In conclusion, CgA determination showed high sensitivity in identifying gastroenteropancreatic tumours and, in association with catecholamines, in detecting patients with phaeochromocytoma. CgA sometimes appeared to be the only circulating marker of disease. Since the specificity of CgA proved to be excellent, this assay may be useful for diagnosis both of functioning and non-functioning neuroendocrine tumours.
- Kenneth J Woodside
-
![Courtney Townsend]()
- B. Mark Evers
Gastrointestinal carcinoid tumors are rare neuroendocrine tumors arising from the embryologic primitive gut. Depending on the location in the gastrointestinal tract, these tumors may secrete a variety of hormonally active substances. However, many of these tumors are found incidentally, or the diagnosis is made postoperatively. Also, there is a significant incidence of multicentric carcinoid tumors and synchronous noncarcinoid malignancies in these patients. Treatment is usually based on the size of the tumor. Surgical resection remains the cornerstone of therapy. For advanced metastatic disease, somatostatin analog therapy and surgical debulking provide the best symptomatic relief and may improve survival. Recent studies have demonstrated a benefit from radiolabeled somatostatin analogs for carcinoid tumor localization. In contrast, radiolabeled somatostatin analogs have shown little therapeutic benefit. Future directions include somatostatin receptor profiling of carcinoid tumors, with somatostatin analog therapy targeting the specific receptors.
In this article, we present radiological findings in three patients with mesenteric, gastric, and rectal carcinoid tumors. More than 90% of the carcinoid tumors arise in the gastrointestinal system (GIS). They comprise 2% of all GIS tumors and are usually located in the small intestine and the appendix. On the other hand, gastric, rectal, and mesenteric carcinoids are rare. Better knowledge of radiological features of carcinoid tumors in rare locations will prevent diagnostic delays and development of carcinoid syndrome.
Bethesda, MD: National Cancer Institute, DCCPS, Surveillance Research Program, Cancer Statistic Branch
- Epidemiology Surveillance
- End Results
Surveillance, Epidemiology, and End Results (SEER) Program. SEER*Stat Database: SEER 17 Regs Nov 2006 sub (1973Y2004). Bethesda, MD: National Cancer Institute, DCCPS, Surveillance Research Program, Cancer Statistic Branch, 2006. Available at: www.seer.cancer.gov.
Source: https://www.researchgate.net/publication/337327647_Primary_mesenteric_neuroendocrine_tumour_with_liver_metastasis_a_common_presentation_of_an_uncommon_tumour
Posted by: joshuajoshuazechiele0271677.blogspot.com
