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Journal of Gastroenterology and... Nov 2020
Topics: Aged; Biomarkers, Tumor; Biopsy, Fine-Needle; Diagnosis, Differential; Diffusion Tensor Imaging; Duodenal Neoplasms; Endosonography; Female; Humans; Tomography, X-Ray Computed
PubMed: 32390278
DOI: 10.1111/jgh.15075 -
Pancreas Sep 2022We report a father and his daughter who both had multiple somatostatinomas in the duodenal bulb without a known syndrome. The father, at age 68 years, was incidentally...
We report a father and his daughter who both had multiple somatostatinomas in the duodenal bulb without a known syndrome. The father, at age 68 years, was incidentally found to harbor 4 approximately 1.5-cm well-differentiated neuroendocrine tumors in the duodenal bulb. His preoperative somatostatin level was elevated. He underwent partial duodenectomy and regional lymph node dissection; one lymph node was positive for metastasis. One year postoperatively, a recurrence was found in the surgical bed; he was treated with octreotide for 2 years, which stabilized the recurrent tumor. Ten years postoperatively, the mucosa of his remaining duodenum was normal. His daughter, at age 53 years, was found to harbor multiple small neuroendocrine tumors in the duodenal bulb. Immunostaining of available specimens showed that the neuroendocrine tumors from the father and daughter both were strongly positive for somatostatin. Micronodules of somatostatin-expressing neuroendocrine cells were found in the parts of the specimens uninvolved with the tumors. Both patients exhibited no evidence of known syndromes associated with somatostatinoma. The daughter did not harbor mutations in 93 genes commonly found in genetic tumor syndromes. The 2 cases thus suggest a novel, autosomal dominant, genetic syndrome of familial duodenal somatostatinomatosis.
Topics: Male; Humans; Aged; Middle Aged; Duodenal Neoplasms; Neoplasm Recurrence, Local; Duodenum; Somatostatinoma; Neuroendocrine Tumors; Somatostatin; Pancreatic Neoplasms
PubMed: 36607953
DOI: 10.1097/MPA.0000000000002126 -
International Journal of Surgical... Sep 2018Simultaneous presence of pancreatic serous cystic neoplasms and neuroendocrine neoplasms is rare. We present a case with the incidental finding of a duodenal... (Review)
Review
Simultaneous presence of pancreatic serous cystic neoplasms and neuroendocrine neoplasms is rare. We present a case with the incidental finding of a duodenal neuroendocrine tumor (NET) with 2 lymph node metastases in a Whipple resection specimen performed to remove a pancreatic cystic neoplasm that postoperatively turned out to represent a serous cystic neoplasm (SCN). The patient was a 75-year-old female. She presented with loss of appetite and weight. Preoperative contrast-enhanced computed tomography scan of the abdomen showed a multicystic lesion in the head of pancreas. On histologic examination of the resection specimen, a pancreatic SCN was found, and in addition in 2 peripancreatic lymph nodes, metastases from a NET. Further examination of the resected specimen revealed a duodenal NET. Review of the literature revealed only one prior study with 4 cases of pancreatic SCN associated with a duodenal NET. In 1 of the 4 cases, the patient had von Hippel-Lindau disease. Our report emphasizes the importance of careful examination of pancreatic resection specimens including the peripancreatic lymph nodes also when dealing with SCNs, as coexisting and more malignant tumors may otherwise be missed.
Topics: Aged; Cystadenoma, Serous; Duodenal Neoplasms; Duodenum; Female; Humans; Incidental Findings; Lymphatic Metastasis; Neoplasms, Multiple Primary; Neuroendocrine Tumors; Pancreas; Pancreatic Neoplasms; Pancreaticoduodenectomy
PubMed: 29623746
DOI: 10.1177/1066896918766245 -
American Journal of Surgery May 2016Duodenal gastrointestinal tumors (GIST) present infrequently, and surgical resection with negative margins remains the mainstay of therapy; however, given the lack of... (Comparative Study)
Comparative Study
BACKGROUND
Duodenal gastrointestinal tumors (GIST) present infrequently, and surgical resection with negative margins remains the mainstay of therapy; however, given the lack of lymphatic and submucosal spread and anatomic location near the bile duct and pancreas, the optimal approach for resection is unknown. Options include local resection (LR), segmental resection, and pancreaticoduodenectomy (PD).
METHODS
All cases of gastrointestinal stromal tumors originating from the duodenum from 2000 to 2015 were identified from administrative databases. Clinical and pathologic information was abstracted from the medical record and compared between patients who received LR vs PD. The chi-square with Fisher's exact test was used to detect differences between groups.
RESULTS
Fifteen patients met the inclusion criteria, of which 7 had an LR and 8 had a PD. The second portion of the duodenum was the most common origin of GIST in the PD group, whereas the third portion was most common in the LR group. Patients who underwent LR tended to be younger, but there was no difference in tumor size, mitotic rate, margin positivity, readmission rate, or recurrence. PD was associated with more complications, higher blood loss, and longer length of stay.
CONCLUSIONS
Local resection is a reasonable option for resection of duodenal GIST and should be routinely considered if technically feasible.
Topics: Adult; Aged; Colectomy; Databases, Factual; Disease-Free Survival; Duodenal Neoplasms; Duodenoscopy; Duodenum; Endosonography; Female; Gastrointestinal Stromal Tumors; Humans; Male; Middle Aged; Multimodal Imaging; Neoplasm Invasiveness; Neoplasm Staging; Pancreaticoduodenectomy; Prognosis; Retrospective Studies; Risk Assessment; Survival Analysis; Tomography, X-Ray Computed
PubMed: 27033254
DOI: 10.1016/j.amjsurg.2016.02.006 -
Four Neuroendocrine Tumor Types and Neuroendocrine Carcinoma of the Duodenum: Analysis of 203 Cases.Neuroendocrinology 2017Several types of neuroendocrine neoplasms (NENs) have been described in the duodenal tract, from low-grade tumors (NETs) to high-grade neuroendocrine carcinomas (NECs)....
BACKGROUND
Several types of neuroendocrine neoplasms (NENs) have been described in the duodenal tract, from low-grade tumors (NETs) to high-grade neuroendocrine carcinomas (NECs). A comprehensive analysis of histology, hormonal profile and prognostic parameters of a sufficiently large duodenal NEN series to cover all main kinds of neoplasms is however lacking.
METHODS
We collected a retrospective series of 203 duodenal wall and ampullary region NENs, from six specialized endocrine pathology centers. All were characterized histopathologically and histochemically, and 190 were followed for a median of 9 years.
RESULTS
Twenty-seven poorly differentiated NECs, mostly from the ampullary region, were identified and shown to lead to patient demise in a median of 10 months. Among 176 NETs, four subtypes were characterized, including 20 gastrinomas, 37 ampullary-type somatostatin-producing NETs (ASTs), 12 gangliocytic paragangliomas (GPs) and 106 nonfunctioning NETs (nfNETs). ASTs and GPs were mostly localized in the ampullary/periampullary region, while gastrinomas and nfNETs were mainly from the proximal duodenum. ASTs and gastrinomas showed high rates of local infiltration (especially lymphoinvasion and deep duodenal wall/pancreatic tissue invasion) and lymph node metastasis, while nfNETs had significantly lower and more size-dependent local invasive potential. Disease-specific survival differed significantly between NETs and NECs, though not among NET subtypes. NET cases with distant metastases (n = 23) were significantly associated with larger size, higher proliferative grade, lymphovascular invasion, deep invasion and local lymph node metastasis.
CONCLUSION
Our careful analysis of a large series of duodenal NENs identified five histologically and prognostically different histotypes of potential clinical relevance.
Topics: Aged; Carcinoma, Neuroendocrine; Duodenal Neoplasms; Female; Humans; Male; Middle Aged; Neoplasm Grading; Neuroendocrine Tumors; Retrospective Studies; Survival Analysis
PubMed: 26910321
DOI: 10.1159/000444803 -
Journal of Surgical Oncology Dec 2019While sub-2 cm pancreatic neuroendocrine tumors (NETs) are often observed, small bowel-NETs undergo resection and lymphadenectomy regardless of size. Aim was to define...
BACKGROUND
While sub-2 cm pancreatic neuroendocrine tumors (NETs) are often observed, small bowel-NETs undergo resection and lymphadenectomy regardless of size. Aim was to define the natural history of duodenal (D-NETs), determine the role of resection, and define the factors associated with overall survival (OS) after resection.
METHODS
National Cancer Database (2004-2014) was queried for the patients with nonmetastatic/nonfunctional D-NETs. Local resection (LR): local excision/polypectomy/excisional biopsy. Anatomic resection (AR): radical surgery. Tumor size was divided into less than 1 cm, 1 to 2 cm, and ≥2 cm. Propensity score weighting was used to create balanced resection and no-resection cohorts. The primary endpoint was OS.
RESULTS
Among 5502 patient, the median age was 65 years. The median follow-up was 49 months. The median tumor size was 0.8 cm. Resection was performed in 72% (n = 3954; LR: 61%, AR: 39%). Lymph node (LN) resection was performed in 26% (43% had metastasis). A total of 74% had negative margins. Resection and no-resection cohorts were propensity score weighted for age/sex/race/Charlson-Deyo score/tumor grade (all independently associated with OS on multivariable analysis). Resection was associated with improved median OS compared to no resection in all sizes (<1 cm: median not reached vs 194 months; 1-2 cm: median not reached vs 56 months; >2 cm: median not reached vs 90 months; all P < .01). Subset analysis of each resection size cohort demonstrated that neither type of resection, LN retrieval, LN positivity, or margin status was associated with OS (all P > .05).
CONCLUSION
Patients with nonmetastatic and nonfunctional D-NETS should be considered for resection regardless of tumor size. Given the lack of prognostic value, the resection type and extent of LN retrieval should be tailored to each patient's clinical picture and safety profile.
Topics: Aged; Duodenal Neoplasms; Female; Follow-Up Studies; Humans; Lymph Node Excision; Male; Margins of Excision; Middle Aged; Multivariate Analysis; Neuroendocrine Tumors; Prognosis; Registries
PubMed: 31621090
DOI: 10.1002/jso.25731 -
American Journal of Kidney Diseases :... Feb 2019Membranous nephropathy (MN) associated with malignancies is a well-known entity. However, its association with benign neoplasm is not broadly recognized. A 69-year-old... (Review)
Review
Membranous nephropathy (MN) associated with malignancies is a well-known entity. However, its association with benign neoplasm is not broadly recognized. A 69-year-old man with recurrent nephrotic syndrome presented with pedal edema and proteinuria of 5 months' duration. Laboratory results showed hypoalbuminemia and hyperlipidemia. Proteinuria was estimated to be protein excretion of 3.5g/d. Studies were negative for viral hepatitis, syphilis, human immunodeficiency virus, autoimmune diseases, and paraproteinemia. Kidney biopsy disclosed MN with negative phospholipase A receptor (PLAR) staining, favoring a secondary form of MN. Computed tomography detected a 7.6-cm duodenal schwannoma. Elective surgical resection was performed. Pathologic study showed that THSD7A (thrombospondin type 1 domain-containing 7A) was positive in both glomeruli and schwannoma. Commonly, secondary MN is related to underlying conditions, including lupus, hepatitis, and neoplasm, and can be medication induced. The risk for developing a concomitant neoplasm among patients with PLAR-negative MN is up to 12 times higher than in the general population. Most of these neoplasms are malignancies, and the presence of autoantibodies directed at similar tissue targets is hypothesized as the potential mechanism. In our case, THSD7A may be the autoantibody that has linked the schwannoma and the development of MN. Although benign tumors rarely produce renal manifestations, effective treatment may lead to resolution of nephrotic syndrome.
Topics: Aged; Biopsy, Needle; Duodenal Neoplasms; Follow-Up Studies; Glomerulonephritis, Membranous; Humans; Immunohistochemistry; Male; Nephrotic Syndrome; Neurilemmoma; Rare Diseases; Treatment Outcome
PubMed: 30454884
DOI: 10.1053/j.ajkd.2018.09.003 -
Medical Science Monitor : International... Jun 2020BACKGROUND This study was designed to predict prognosis of patients with primary duodenal neuroendocrine neoplasms (D-NENs) by developing nomograms. MATERIAL AND METHODS...
BACKGROUND This study was designed to predict prognosis of patients with primary duodenal neuroendocrine neoplasms (D-NENs) by developing nomograms. MATERIAL AND METHODS Patients diagnosed with D-NENs between 1988 and 2015 were queried from the SEER database and a total of 965 appropriate cases were randomly separated into the training and validation sets. Kaplan-Meier analysis was used to generated survival curves, and the difference among the groups was assessed by the log-rank test. Independent prognostic indicators were acquired by Cox regression analysis, and were used to develop predictive overall survival (OS) and cancer-specific survival (CSS) nomograms. Harrell's concordance index (C-index), area under the curve (AUC), calibration curves, and decision curve analysis (DCA) were used to assess the efficacy of nomograms. Tumor stage was regarded as a benchmark in predicting prognostic compared with the nomograms built in this study. RESULTS The C-index was 0.739 (0.690-0.788) and 0.859 (0.802-0.916) for OS and CSS nomograms, respectively. Calibration curves exhibited obvious consistency between the nomograms and the actual observations. In addition, C-index, AUC, and DCA were better than tumor stage in the evaluative performance of nomograms. CONCLUSIONS The nomograms were able to predict the 1-, 5-, and 10-year OS and CSS for D-NENs patients. The good performance of these nomograms suggest that they can be used for evaluating the prognosis of patients with D-NENs and can facilitate individualized treatment in clinical practice.
Topics: Adolescent; Adult; Black or African American; Age Factors; Aged; Aged, 80 and over; Carcinoid Tumor; Carcinoma, Neuroendocrine; Digestive System Surgical Procedures; Duodenal Neoplasms; Ethnicity; Female; Gastrinoma; Humans; Kaplan-Meier Estimate; Male; Marital Status; Middle Aged; Neoplasm Grading; Neoplasm Staging; Neuroendocrine Tumors; Nomograms; Prognosis; Proportional Hazards Models; SEER Program; Sex Factors; White People; Young Adult
PubMed: 32564052
DOI: 10.12659/MSM.922613 -
Digestive Diseases and Sciences Sep 2014Small case series have alluded to an association between sporadic duodenal adenomas and colorectal neoplasia. The strength of the association remains uncertain. This...
BACKGROUND AND AIM
Small case series have alluded to an association between sporadic duodenal adenomas and colorectal neoplasia. The strength of the association remains uncertain. This case-control study was designed to test this association in a large national pathology database.
METHODS
This study, performed at Miraca Life Sciences, a specialized pathology laboratory that receives gastrointestinal biopsy specimens from outpatient centers throughout the US, included all subjects who underwent a bidirectional endoscopy with biopsy results from both procedures between January 2008 and December 2011. The association between duodenal and colonic neoplasms was investigated using odds ratios (OR) and their 95 % confidence intervals (CIs) derived from univariate and multivariate analyses.
RESULTS
There were 203,277 patients who underwent bidirectional procedures within the study period (mean age 58 years, 58 % females). Duodenal adenomas were present in 537 patients (median age 65 years, 51 % females; OR for male sex 1.34, 95 % CI 1.13-1.59). Hyperplastic colon polyps were present in 30,205 and colon adenomas in 85,801 patients. Hyperplastic polyps were more common in patients with duodenal adenomas (1.45, 1.07-1.95). Patients with duodenal adenomas also had a significantly greater prevalence of all types of colonic adenomas (2.65, 2.16-3.25), particularly of advanced adenomas (4.30, 3.24-5.70) and colorectal cancer (3.13, 1.38-7.12). Duodenal adenomas were associated with an equally increased risk for left and right colon adenomas.
CONCLUSIONS
Patients with duodenal adenomas harbor an increased risk for any type of colonic neoplasm. This association may provide new insights into the general mechanisms underlying mucosal proliferation in the gastrointestinal tract.
Topics: Adenoma; Adult; Age Factors; Aged; Case-Control Studies; Colonic Neoplasms; Colonic Polyps; Duodenal Neoplasms; Endoscopy, Gastrointestinal; Female; Humans; Male; Middle Aged; Neoplasms, Multiple Primary; Prevalence; Risk Factors; United States
PubMed: 24705697
DOI: 10.1007/s10620-014-3131-5 -
Gastrointestinal Endoscopy Feb 2019The greatest known risk factor for duodenal cancer in familial adenomatous polyposis (FAP) is Spigelman stage (SS) IV duodenal polyposis. Endoscopic surveillance is...
BACKGROUND AND AIMS
The greatest known risk factor for duodenal cancer in familial adenomatous polyposis (FAP) is Spigelman stage (SS) IV duodenal polyposis. Endoscopic surveillance is recommended in FAP patients with SS 0 to IV, and prophylactic duodenectomy should be considered in SS IV. Cancer occurs in patients without SS IV polyposis. We assessed the relationship of SS and other factors with duodenal cancer in FAP.
METHODS
We performed a case-control study on 18 FAP patients with duodenal cancer and 85 randomly selected FAP control subjects with similar age characteristics. Demographic, clinical, and endoscopic features were compared using univariate and logistic regression analyses to assess factors associated with duodenal cancer.
RESULTS
Fifty-three percent of cases had no SS IV history. SS components positively associated with cancer included duodenal polyp size (77% vs 47%, P = .015), and high-grade dysplasia (HGD; 29% vs 6%, P = .003) but not polyp number or histology. In the papilla, the frequency of tubulovillous or villous histology (80% vs 22%, P < .001) and HGD (30% vs 4%, P = .010) was greater in cases than control subjects.
CONCLUSIONS
SS IV polyposis was absent in half of FAP patients with duodenal cancer. Only 2 of 4 SS components (large duodenal polyp size and HGD) were positively associated with duodenal cancer. Advanced pathology of the papilla appears to be an important feature. Revision of SS to emphasize these findings should be considered to better estimate cancer risk.
Topics: Adenocarcinoma; Adenomatous Polyposis Coli; Adult; Aged; Case-Control Studies; Duodenal Neoplasms; Endoscopy, Digestive System; Female; Humans; Logistic Models; Male; Middle Aged; Neoplasm Staging; Neoplasms, Multiple Primary; Tumor Burden
PubMed: 30081000
DOI: 10.1016/j.gie.2018.07.033