scholarly journals Pancreatic Neuroendocrine Tumor in the Setting of Dorsal Agenesis of the Pancreas

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Samih Nassif ◽  
Cecilia Ponchiardi ◽  
Teviah Sachs

Dorsal agenesis of the pancreas (DAP) is an uncommon embryological abnormality where there is absence of the distal pancreas. DAP is mostly asymptomatic, but common presenting symptoms include diabetes mellitus, abdominal pain, pancreatitis, enlarged pancreatic head, and, in a few cases, polysplenia. MRCP and ERCP are the gold standard imaging techniques to demonstrate the absence of the dorsal pancreatic duct. The literature on the association of pancreatic neoplasia and DAP is limited. We present the case of a pancreatic neuroendocrine tumor in a patient with dorsal agenesis of the pancreas, with a review of the related literature.

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Randhir Sagar Yadav ◽  
Ashik Pokharel ◽  
Shumneva Shrestha ◽  
Ashbita Pokharel ◽  
Deepshikha Gaire ◽  
...  

Mixed serous-neuroendocrine neoplasm constitutes pancreatic serous cystic neoplasms and pancreatic neuroendocrine tumor, two tumor components with different underlying pathologies. The differentiation of these tumors is important as the management and prognosis depend on the pancreatic neuroendocrine tumor component. We report a case of mixed serous-neuroendocrine neoplasm in a 47-year-old female who presented with epigastric pain abdomen for two years. Imaging studies, tumor markers, thorough systemic evaluation, surgical resection, histopathological examination, and timely follow-up constituted our management approach. A 4 cm × 4 cm mass in the distal pancreas with multiple cysts in the pancreatic parenchyma containing serous fluid on distal pancreatectomy and splenectomy was found. The histopathological examination revealed combined benign serous cystadenoma and neuroendocrine tumor. She did not have any recurrence or metastasis by four years of follow-up.


2016 ◽  
Vol 2 ◽  
pp. 176-176
Author(s):  
Nicola Passuello ◽  
Michele Valmasoni ◽  
Gioia Pozza ◽  
Elisa Sefora Pierobon ◽  
Alberto Ponzoni ◽  
...  

2017 ◽  
Vol 78 (11) ◽  
pp. 2525-2533
Author(s):  
Hiroaki TAKAHASHI ◽  
Yoshihiko OSAKA ◽  
Kazuhito UEMURA ◽  
Naotake HOMMA ◽  
Kazuaki SHIBUYA ◽  
...  

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S63-S63
Author(s):  
Rong Xia ◽  
Maryam Noori Koloori ◽  
Mohamed Alshal ◽  
Amir Dehghani ◽  
Qiang Xie ◽  
...  

Abstract Introduction Sclerosing variant of pancreatic neuroendocrine tumor (PanNET) is a rare variant of PanNET characterized by decreased tumor cellularity and prominent stromal fibrosis, and it is considered associated with serotonin expression. We herein present an unusual case of a sclerosing PanNET expressing glucagon. Methods The patient, a 75-year-old woman with refractory diabetes, scaly erythematous skin plaques in the eyelid, bilateral elbows and scalp, and episodes of nausea and poor appetite, presented to emergency department for acute abdominal pain. Abdominal computed tomography showed a 17-mm pancreatic head mass. Endoscopic ultrasound with fine-needle biopsy was performed for histopathological examination, followed by pancreaticoduodenectomy. Results Biopsy showed a background of predominantly blood and benign strips of pancreatic parenchyma and scanty fragments of fibrotic hyalinized stroma containing medium-sized atypical monotonous cells with round-to-ovoid nuclei, smooth borders, and inconspicuous nucleoli. These cells were difficult to characterize morphologically due to the scanty sampling. Immunohistochemical stains revealed the atypical cells were positive for AE1/AE2, synaptophysin, chromogranin, and glucagon and negative for insulin, serotonin, and pancreatic polypeptide. The lack of heterogeneous peptide cell composition supported a neoplastic proliferation of neuroendocrine cells. The pancreaticoduodenectomy specimen showed a 1.2-cm white firm mass in the pancreatic head. Histopathological study showed that, in a sclerotic background, the tumor cells grew mostly in trabecular patterns and occasionally in single cells, infiltrating into adjacent pancreatic parenchyma, and showed the same immunoprofile features as in the biopsy specimen. The proliferative index was <3%, supporting the diagnosis of low-grade sclerosing PanNET. The patient recovered well postsurgery. Moreover, the episodes of nausea and loss of appetite improved postsurgery. Patient did not show up for follow-up appointments in the dermatology clinic. Conclusion Sclerosing PanNET is challenging for diagnosis, especially on small biopsy specimens. Immunohistochemistry helps establish the diagnosis. Our case demonstrates a rare presentation of sclerosing PanNET expressing glucagon.


2021 ◽  
Vol 26 (1) ◽  
pp. 1-9
Author(s):  
Jun-Ho Choi

Pancreatic neuroendocrine tumors (PNETs) are rare tumors with malignant potential, but their incidence has appreciably increased over the last few decades. Diagnosis of PNETs is often difficult with conventional imaging methods. Cross-sectional imaging localizes less than 10% of PNETs less than 1 cm in diameter. Endoscopic ultrasound (EUS) has been shown to be superior to other imaging techniques in the preoperative localization and diagnosis of PNETs. The finding of a hyperenhanced lesion on contrastenhanced EUS was highly predictive of PNETs different from adenocarcinoma. Preoperative assessment of tumor differentiation and Ki-67 is an important prognostic factor for grading. Thus, EUS-guided fine needle biopsy may play a key role in the work-up of nonfunctioning PNETs, and attempts to measure Ki-67 on cytologic and histologic samples have been made. According to the European Neuroendocrine Tumor Society guidelines, there is no clear evidence of a survival benefit of surgery for nonfunctioning G1 PNETs <2 cm in diameter. The benefits of curative surgery must be weighed against operative morbidity and mortality. Although surgical resection remains the mainstay for PNETs, EUS-guided ablation offers a relatively safe and effective treatment option in patients deemed unfit for, or who do not want to undergo, surgery. EUS-guided ablation provides an attractive therapeutic nonsurgical option for patients; however, further studies that better elucidate the long-term outcomes with standardization of technique will help define its role in the treatment of PNETs. The role of EUS in the diagnosis and treatment of PNETs is expected to make much progress in the future.


2019 ◽  
Vol 69 (12) ◽  
pp. 3500-3502
Author(s):  
Ana Maria Trofin ◽  
Mihai Zabara ◽  
Ramona Cadar ◽  
Madalina Palaghia ◽  
Delia Rusu-Andriesi ◽  
...  

Pancreatic cancer is one of the most aggressive malignant diseases due high rate of recurrence and the lack effective medical therapy. Surgery remains the only option for curable treatment but unfortunately, less than 20% of patients are eligibles at the time of diagnosis therefore identifying the risk factors represent a big step for cancer research. Pancreatic cancer is frequently associated with diabetes or glucose intolerance. There are two hypotheses at the base of this observation: either the diabetes cause pancreatic cancer or is a concequences of the cancer. In these theses we studied the patients diagnosticated with pancreatic cancer and with diabetes mellitus type 2. A total of 256 pancreatic cancer cases were identified and 71 patients had diabetes mellitus and 21 patients had glucose intolerance. Mean age 62.2 years, 81% cases were male and in 71% cancer originated form the pancreatic head. In 51.4% cases the diagnosis was in stage IV of the disease. Patients with pancreatic cancer and diabetes mellitus had reduced survival compared with those without diabetes but the difference was not statistically significant. Diabetes mellitus is associated with a decreased survival among patients with pancreatic cancer and reveal a link between chronic glucose intolerance and pancreatic cancer survival. The complex relationship between pancreatic cancer and diabetes requires more clinical research in order to developed new therapeutical posibilities.


ASVIDE ◽  
2016 ◽  
Vol 3 ◽  
pp. 487-487
Author(s):  
Nicola Passuello ◽  
Michele Valmasoni ◽  
Gioia Pozza ◽  
Elisa Sefora Pierobon ◽  
Alberto Ponzoni ◽  
...  

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A558-A559
Author(s):  
Banafsheh Motazedi ◽  
Laura E Cowen ◽  
Jacqueline Jonklaas

Abstract Introduction: Ectopic adrenocorticotrophic hormone (ACTH) producing Pancreatic Neuroendocrine Tumor (p-NET) are extremely rare with incidence of 1.2% and confer a poor prognosis (5-year survival &lt;20%). Case Report: 31-year old female with diabetes mellitus type 2 (DM-2) and hypertension (HTN) was found to be severely hypokalemic on routine labs and was sent to the Emergency Department for potassium replacement. Vitals; BP:139/87 mmHg, HR:108 bpm, RR:18, T:37°C and saturating 98% on ambient air. Examination revealed cushingoid features; hyperpigmented face, bilateral upper extremity pigmented papules, proximal muscle weakness, acanthosis nigricans, violaceous abdominal striae, truncal obesity, ecchymoses, bilateral lower extremity edema, memory impairment and anxiety. Initial labs; BG: 289 mg/dL, HbA1c: 9.5%, K: 2 mmol/L (N 3.5-5.2), WBC: 12.5 k/μL (N 4-10.8), Cortisol &gt;150 mcg/dL (N 5-23), TSH: 0.325 μIU/mL (N 0.45-4.5), and FT4: 0.69 ng/dL (N 0.82-1.77). Diagnosis of Cushing’s syndrome was made based on elevated cortisol level and confirmed with elevated 24-hour urinary free cortisol: 13,294 μg (N&lt;45), midnight salivary cortisol &gt;15 ug/dL (N 0.09-1.5) and 1 mg dexamethasone suppression test (Cortisol &gt;150 mcg/dL, ACTH: 621 pg/ml). Additional labs showed Chromogranin A: 717 ng/mL (N 0-160). She was started on prophylactic Enoxaparin & Trimethoprim/Sulfamethoxazole and treated with Spironolactone, Basal/Bolus insulin, and Ketoconazole for hypercortisolemia. Pituitary MRI was negative for pituitary adenoma. Abdominal/Pelvic CT scan demonstrated a solid 5 cm pancreatic head mass with innumerable haptic metastases consistent with metastatic p-NET which was confirmed on PET-CT Ga68 Dotatate scan. Liver biopsy was positive for a well differentiated p-NET with ki-67 &lt;5% and negative ACTH staining. Hospital course was complicated by fluctuating cortisol levels with intermittent psychotic manifestations despite up-titration of Ketoconazole, addition of Octreotide as well as anti-psychotics. She later developed transaminitis on maximum dose of Ketoconazole and thus underwent laparoscopic total adrenalectomy for treatment of her hypercortisolemia. Post-operatively, she was started on Hydrocortisone & Fludrocortisone and Spironolactone was discontinued. She was transitioned to monthly Lanreotide and treated with Capecitabine and Temozolomide with adequate therapeutic response and subsequent down trending of her Chromogranin A: 188 ng/mL and ACTH: 228 pg/ml levels. Currently, she is doing well with significant improvement of her DM-2, HTN, psychiatric symptoms and overall clinical status. Conclusions: Symptoms of ectopic Cushing’s syndrome may be delayed, however given the rare and aggressive nature of ACTH producing p-NET, timely diagnosis, prophylaxis and individualized treatment approach is crucial in achieving favorable prognosis.


Sign in / Sign up

Export Citation Format

Share Document