Mass In Pancreatic Tail Research Articles

7743 A Case Study of Ectopic Adrenocorticotropic Hormone Secretion from Pancreatic Neuroendocrine Tumor

Abstract Disclosure: K.S. Wei: None. J. Huang: None. Introduction: Pancreatic neuroendocrine tumors (PanNETs) are uncommon cancers that account for less than 2% of pancreatic malignancies. Even rarer are ACTH-producing NETs which may only become symptomatic until after metastasis. This case study examines the distinct presentation and diagnostic complexities associated with ectopic adrenocorticotropic hormone (ACTH) secretion from a PanNET. Clinical Case:A 42 year-old male with hypertension and type 2 diabetes initially presented to the hospital with increased abdominal girth for one month, bilateral lower extremity edema for two weeks, and generalized abdominal pain. He had unremarkable surgical, family, and social histories. Patient was afebrile, normotensive, saturating well on room air, with exam notable for a distended abdomen and 2+ bilateral pitting edema. Admission labs were notable for potassium of 2.4 (ref: 3.5-5.1 mmol/L). MRI abdomen showed hepatomegaly with numerous heterogeneously enhancing masses and an ill-defined pancreatic tail mass with involvement of the spleen. Liver biopsy showed well-differentiated neuroendocrine tumor, grade 2. He was started on octreotide 20mg q4weeks and capecitabine/temozolomide q4weeks. Two months later, the patient was readmitted after presenting with back pain for two days, found to have acute compression fractures of T9-L1 without spinal PET activity. At follow-up visit with endocrinology, he endorsed easy bruising and muscle weakness and had hypertension (161/96 mmHg) with hypokalemia. Random cortisol was 43.7 (ref: 2.5-11.9 mcg/dL). ACTH was 124 (ref: 6-50 pg/mL) despite receiving chemotherapy. 24-hour urine cortisol was 3,049 (ref: 4-50 mcg/24 hrs). He was diagnosed with ectopic Cushing’s disease from ACTH-producing PanNET leading to osteoporosis and fracture of the vertebral bodies. For the ectopic Cushing’s disease, he was started on ketoconazole 200mg BID with plan to start osilodrostat once approved. For the fracture, he is scheduled for Zometa infusion and IR kyphoplasty. Conclusion: This case offers several important clinical insights. In patients with PanNET with Cushingoid exam findings, ACTH-producing PanNET should be high on the differential. Most ACTH NETs are aggressive often with liver metastases, whereby metastatic disease will have already occurred by the time the Cushingoid features develop. The initial severe hypokalemia and hypertension should have prompted investigation for mineralocorticoid excess from hypercortisolism, which may have led to an earlier diagnosis of ectopic ACTH Cushing’s disease, thereby decreasing the possibility of developing vertebral fractures, uncontrolled diabetes and hypertension. Ketoconazole or osilodrostat may be used to treat Cushing’s disease from ACTH NETs. Presentation: 6/1/2024

A case of extraosseous Ewing sarcoma diagnosed by EUS-FNA for a pancreatic tail mass

A case of extraosseous Ewing sarcoma diagnosed by EUS-FNA for a pancreatic tail mass

Visualization of Pancreatic Tail Cancer with Liver Metastasis through Abdominal Transsplenic Scan

Pancreatic cancer typically presents as a focal hypoechoic, hypovascular solid mass with irregular margins on ultrasound. Pancreatic tail disease can be difficult to detect on abdominal ultrasonography. A 75-year-old man visited our institution with upper abdominal pain. We successfully visualized a pancreatic tail mass on abdominal transsplenic scan and multiple liver masses via abdominal transverse scan. His diagnosis was confirmed as pancreatic tail cancer with liver metastasis following endoscopic ultrasound-guided fine-needle biopsy. Abdominal transsplenic scan proved valuable for diagnosing pancreatic tail disease because abdominal ultrasound has limited utility for evaluating pancreatic tail masses due to obscuration by bowel gas.

Gastrointestinal: Enlarged pancreatic tail mass during chemotherapy for pancreatic cancer.

Gastrointestinal: Enlarged pancreatic tail mass during chemotherapy for pancreatic cancer.

Unveiling lipid-rich ("clear cell") pancreatic neuroendocrine tumor: A small series of two cases with literature review.

This study presents two cases of lipid-rich pancreatic neuroendocrine tumors (PanNETs), a rare variant posing significant diagnostic challenges in fine needle aspiration (FNA) cytology and small biopsies. The first case involves an elderly male with a pancreatic tumor, displaying distinct cytoplasmic vacuoles, while the second case is a middle-aged male present with a pancreatic tail mass exhibiting foamy cytoplasm and eccentric nuclei, infiltrating in the stroma. Both cases did not exhibit typical morphologic features of PanNET but demonstrated cytomorphologic features and infiltrative growth patterns that mimicked adenocarcinoma. Further work-up demonstrated that both tumors were immunoreactive for synaptophysin and chromogranin, and were interpreted as well-differentiated, PanNET, lipid-rich variant. The study highlights the overlapping morphological features between lipid-rich PanNETs and other pancreatic neoplasms and underscores the importance of comprehensive cytological and immunohistochemical analysis for accurately diagnosing this variant, particularly due to the risk of misinterpreting it as pancreatic adenocarcinoma. Recognizing lipid-rich PanNETs is crucial for appropriate clinical management, as their identification can significantly impact treatment decisions and patient outcomes.

O-05 A novel mutation of MEN1 gene in familial Multiple Endocrine Neoplasia Type 1 (MEN1): A report of two cases

Abstract Introduction Multiple Endocrine Neoplasia Type 1 (MEN1) is a rare (1-9/100000) autosomal dominant disease. It is characterized by primary hyperparathyroidism (90%), enteropancreatic tumor (60-70%), non-functional adrenal adenomas (30%) and pituitary tumors (10-20%). Herein, we present two cases in the same family with MEN1 syndrome caused by a novel mutation. Clinical Case 1: 49-year-old male presented to our clinic with fasting hypoglycemia. Fasting serum glucose (FSG) was 51 mg/dl and serum cortisol was 18 ug/dl. During the hypoglycemia in prolonged fasting test, the serum levels of glucose, insulin and C-peptide were 37 mg/dl, 17.3 mu/l, 7.75 ng/ml respectively (Table 1). Abdominal MRI revealed mass lesions in the pancreas (Figure 1). Ultrasonography of the neck and MIBI scintigraphy performed because of the high levels of calcium and parathyroid hormone (PTH), were consistent with parathyroid adenoma (Table 2). Pituitary MRI showed a 12 mm macroadenoma. He underwent parathyroidectomy and distal pancreatectomy simultaneously. The resected specimens revealed a well-differentiated neuroendocrine tumor (NET) of the pancreas and parathyroid adenoma. The patient had no new hypoglycemic episodes after surgery and serum calcium remained within the normal range (Table 2). Central hypothyroidism and hypogonadotropic hypogonadism which was resulted from the pressure of the adenoma was managed medically. Genetic testing revealed a heterozygous mutation in NM_001370251.1:c.949del(HET). First degree relatives of the patient were screened for MEN. Clinical Case 2: The son of case 1, an 18-year-old male, presented with fasting hypoglycemia. He had a BMI of 37 kg/m2 and underwent gastric botox 3 months prior to his presentation. FPG was 52 mg/dl and serum cortisol was 20 ug/dl. Prolonged fasting test confirmed the diagnosis of insulinoma (Table 1). Abdominal MRI showed a pancreatic tail mass 37×22 mm in size. Gallium 68 PET/CT DOTATATE revealed increased uptake in multiple lesions in the pancreas. Ultrasonography and MIBI scintigraphy showed a parathyroid adenoma. A nonfunctional microadenoma was detected in pituitary MRI. The patient underwent distal pancreatectomy. Following pancreatectomy, right superior and left inferior parathyroidectomies were performed. Since his serum calcium levels started to increase in 48 hours after the operation, the patient has had second operation for left superior parathyroidectomy. After the operation, the serum calcium and parathyroid hormone levels were 9.2 mg/dl, 58 pg/ml respectively. Specimens revealed well-differentiated NET and atypical parathyroid adenoma. The patient was found to have the same genetic mutation with his father and during the follow up the serum glucose and calcium levels remained within the normal range (Table 2). Conclusion In the genetic examination the heterozygous mutation NM_001370251.1:c.949del(HET) in exon 7/11 of the MEN1 gene found in our patients represents a novel mutation.Figure 1.MRI showing pancreatic masses Table 1.Laboratory findings Table 2.Prolonged fasting test

Primary Carcinosarcoma of the Pancreas with Focal Cartilaginous Differentiation

Abstract Introduction/Objective Carcinosarcoma is a neoplasm reported in multiple sites with a biphasic pattern sharing both sarcomatous and epithelial morphology. Each component should constitute 30% of the neoplasm to qualify as carcinosarcoma, according to the World Health Organization (WHO) classification. This subtype of malignancy is most frequently found in the uterus but carcinosarcoma of the pancreas is extremely rare. It is a very aggressive tumor with a low survival rate. Methods/Case Report A 76-year-old Caucasian man with a history of hypertension was admitted to the emergency department with new-onset atrial fibrillation and ischemic stroke 5 months ago. Initial workup showed "moth-eaten" lesions in the skull base and clivus bone, suggesting malignancy. The further evaluation led to chest/abdomen and pelvis CT scans, revealing a subtle attenuation in the pancreatic tail. MRI of the abdomen showed a pancreatic tail mass concerning pancreatic cancer. CA19-9 and CEA were elevated (133 U/ mL, and 10 ng/ mL, respectively). Endoscopic ultrasound was performed and showed a 2 cm mass in the tail of the pancreas and fine needle aspiration (FNA) confirmed malignancy with a diagnosis of adenocarcinoma. The patient received neoadjuvant chemotherapy with gemcitabine and paclitaxel and underwent distal pancreatectomy, splenectomy, and left adrenalectomy. Histopathologic examination revealed a biphasic neoplasm with moderately differentiated adenocarcinoma and abruptly transitioning spindle cell malignancy with focal cartilaginous differentiation. Due to the presence of focal cartilaginous differentiation, no immunohistochemistry (IHC) was required and a final diagnosis of carcinosarcoma of the pancreas with multiple lymph node involvement (ypT2, pN2) was made. Results (if a Case Study enter NA) NA Conclusion Carcinosarcoma is a neoplasm of mixed epithelial and mesenchymal-like tumor cells on pathologic examination. IHC is often very helpful for correct diagnosis, especially Cytokeratin and CEA. In this case, due to the presence of high-grade spindle cell sarcoma and cartilaginous differentiation, no IHC is required. Surgical resection is the definitive treatment for operable patients and is recommended when possible. In this case, only the adenocarcinoma component was identified preoperatively on FNA, and standard pancreatic adenocarcinoma neoadjuvant chemotherapy was also utilized in treatment.

FRI603 Treatment Of Severe Refractory Hypoglycemia Due To Malignant Insulinoma With A Novel Anti-insulin Receptor Antibody

Abstract Disclosure: S. Osataphan: None. M. Vamvini: None. E.D. Rosen: Consulting Fee; Self; Novartis Pharmaceuticals. L. Pei: None. N. Erlikh: None. G. Singh: None. P. Dhorajiya: None. J. Parker: None. J.M. Dreyfuss: None. A. Rattani: Stock Owner; Self; Vertex Pharmaceuticals, Provention Bio, Bluebird Bio, Agenus, MacroGenics, Immunity Bio, Mirati Therapeutics, Actinium Pharmaceuticals, Bausch Health Companies, Compass Therapeutics, Magenta Therapeutics. M. Patti: Advisory Board Member; Self; Fractyl Laboratories. Consulting Fee; Self; AstraZeneca, Hanmi Pharmaceuticals, MBX. Grant Recipient; Self; Dexcom. Severe hypoglycemia caused by malignant insulinoma is often resistant to medical therapy targeting both tumor burden and insulin secretion. We report a patient who developed severe, treatment-resistant hypoglycemia after receiving 177lutetium-DOTATATE (Lu-177). Hypoglycemia was completely ameliorated after treatment with RZ358, a human monoclonal antibody that functions as a negative allosteric modulator of the insulin receptor, reducing insulin signaling. This 55 year old man presented with abdominal pain, fatigue, and weight loss; imaging showed a 1.8 cm pancreatic tail mass and numerous hepatic lesions. Liver biopsy demonstrated well-differentiated pancreatic neuroendocrine tumor, WHO Grade 2, and pathogenic MEN1 mutation. Following one year of octreotide therapy, both pancreatic and hepatic tumors increased in size, prompting Lu-177 therapy. Two days after the first dose, the patient became unresponsive, with capillary glucose 20 mg/dL. He developed recurrent neuroglycopenia on day 8, with venous glucose 41 mg/dL, insulin 45 µIU/mL, C-peptide 6.5 ng/mL and proinsulin 453 pmol/L, requiring intensive care unit admission for for intravenous glucose. High-dose diazoxide, everolimus, dexamethasone, glucagon, pasireotide, or enteral feeding did not produce a response. Despite multiple therapies, neuroglycopenia required frequent dextrose boluses and continuous intravenous glucose (up to 30 g/hr of 50% dextrose); up to 58% of sensor glucose was below 70 mg/dL and 19% below 54 mg/dL over 24 hours. CT imaging 1 month after Lu-177 showed significant reduction in liver metastases size. Given the severity of hypoglycemia despite tumor regression, we considered treatment with RZ358, a human monoclonal antibody that acts as a negative allosteric modulator of the insulin receptor, inducing insulin resistance. We obtained emergency use authorization from the FDA, approval from the local Institutional Review Board, and written informed consent. Following a 6 mg/kg dose of RZ358, there was transient worsening of hypoglycemia accompanied by an 8-fold increase in insulin, potentially due to reduced insulin clearance. After dose increase to 9 mg/kg weekly, glucose infusion was weaned. Metabolic stability was achieved after 6 doses, allowing a second dose of Lu-177 and eventual discharge. Diazoxide was discontinued, steroid doses were reduced, and RZ358 dosing was reduced to every 3-4 weeks. A third dose of Lu-177 was administered without complications. Despite elevated insulin (537 uIU/mL), C-peptide (10.1 ng/mL), and proinsulin (634.0 pmol/L), he remains free of level 3 hypoglycemia. No adverse effects have been observed. In summary, the anti-insulin receptor monoclonal antibody RZ358 effectively controlled hypoglycemia refractory to multiple other therapies, allowing restoration of normoglycemia and enabling additional successful cancer therapy. Presentation: Friday, June 16, 2023

SAT543 A Case of Non-iodine-Avid Papillary Thyroid Cancer With a Rare Distant Metastasis to the Pancreas

Abstract Disclosure: A.H. Alcain: None. M.C. Isidro: None. Papillary Thyroid Carcinoma is the most common type of differentiated thyroid cancer. Distant metastasis is rare occurring only in 5% of patients and typically involves the lungs and bones. Metastasis to the pancreas is extremely rare with few cases reported over the years. We present a case of a 65-year-old female, who presented with sudden onset of abdominal pain. A review of the patient’s medical history revealed a history of a non-iodine avid Papillary Thyroid Carcinoma in 2015. The patient underwent Radioactive Iodine (RAI) ablation therapy twice due to elevated Thyroglobulin (Tg) levels. Despite RAI, Tg levels were still elevated with no recurrence nor metastasis noted on scan. Chemotherapy was offered but the patient opted for Tomotherapy. The patient completed Tomotherapy in 2017 but afterward was lost to follow-up. A CT scan of the abdomen showed findings of a pancreatic tail and body mass (10 x 7 cm) with an invasion of the mid-descending colon with pulmonary nodules which are possibly metastatic. The patient underwent an endoscopic ultrasound-guided biopsy, and pathology showed findings of a pancreatic papillary neoplasm (CK and beta-catenin positive, synaptophysin negative). Chemotherapy was given (FOLFOX) and a PET scan was requested to monitor the response to treatment. However, PET-scan showed FDG-avid (SUV 32.1) mass in the pancreatic tail (7.2 x 7.8 x 13.3 cm), FDG-avid (SUV 17.61) nodules in the lungs, and an ill-defined heterogeneous mass in the left surgical site (2.2 x 2.6 x 5.3 cm). Given that patient had a thyroidectomy in 2015, recurrence was considered. The possibility of probable metastasis to the pancreas was entertained hence re-biopsy was done. Serum Tg levels were elevated (4,115 ng/ml NV: 1.59-50.0) and negative for Anti-Tg. Biopsy showed high-grade neoplasm with papillary features. IHC staining showed positive for CK7, Tg, and TTF-1, and negative for CK20 which was consistent with metastatic papillary thyroid carcinoma. The patient was given Lenvatinib and is at present tolerating the treatment well with evidence of decreasing Tg levels suggesting a response to treatment. This is a case of a rare site of distant metastasis of a non-iodine avid papillary thyroid carcinoma. Targeted therapy such as Tyrosine kinase inhibitors can improve the survival rate of patients with Radioiodine Refractory Thyroid cancers. Metastasis should be considered especially in patients with a history of prior malignancy. Presentation Date: Saturday, June 17, 2023

SAT158 Hypoglycemia After Bariatric Surgery Caused By Insulinoma

Abstract Disclosure: A. Yaseen: None. H. Lam: None. Introduction: Post bariatric surgery hypoglycemia occurs at least 6 to 12 months after bariatric surgery, presents as postprandial neuroglycopenia with documented blood glucose <54 mg/dl occurring 1 to 3 hours after meals, resolving with carbohydrate intake, and normal fasting glucose. Fasting hypoglycemia or hypoglycemia > 4 hours after caloric intake is not typical of post-bariatric hypoglycemia and should raise concern for other etiologies, specifically insulinoma. Clinical Case: A 62 year old female who had Roux-en-Y gastric bypass (RYGB) surgery 18 years ago presented with confusion and a blood glucose level of 35 mg/dl.She had been having episodic hypoglycemia fasting and nonfasting for 4 years prior to presentation. Initial workup revealed serum insulin 10 uIU/ml, C-peptide 2.3 ng/ml, and serum glucose of 19 mg/dl. A dextrose 20% in water intravenous infusion was started in addition to octreotide injections, acarbose, and diazoxide. Prednisone was added later and acarbose discontinued. Magnetic resonance imaging of the abdomen and magnetic resonance cholangiopancreatography showed a 1.8 x 1.3 cm enhancing lesion within the pancreatic tail. Selective arteriography and intra-arterial calcium stimulation with hepatic venous sampling revealed significant elevation of insulin after calcium gluconate injection into the accessory pancreatic tail artery arising from the superior mesenteric artery which supplies the hypervascular pancreatic tail mass. Patient had an exploratory laparotomy and splenic preserving distal pancreatectomy. Surgical pathology revealed a 2.1 cm grade 1, well-differentiated neuroendocrine tumor that was limited to the pancreas. Hypoglycemia resolved after surgery and she was discharged on glargine insulin and metformin. 1 month after surgery, C-peptide was 0.3 ng/ml, serum glucose was 101 mg/dl and HbA1c was 6.5%. Discussion and Conclusion: The incidence of hypoglycemia after RYGB is uncertain but has been reported to be 0.1 to 0.4% of cases. Only 9 cases of insulinoma in patients with a history of gastric surgery have been reported, 7 of 9 (78%) being female with an average age of 56 years old. Symptoms included fasting and postprandial hypoglycemia occurring 6 months to 20 years after bariatric surgery. CT, MRI, and endoscopic ultrasound have been found to have excellent sensitivity for preoperative localization of insulinoma. Selective arteriography and intra-arterial calcium stimulation with hepatic venous sampling is recommended when non-invasive imaging modalities fail to localize the tumor. Surgical resection remains the mainstay for cure. Dietary modification and pharmacological therapy with diazoxide or somatostatin analogues are used in poor surgical candidates. Insulinoma after RYGB is rare, but needs to be considered in patients with hypoglycemia occurring in the fasting state, as this is atypical for post-bariatric hypoglycemia Presentation: Saturday, June 17, 2023

Epidermal Cyst in an Intrapancreatic Accessory Spleen.

A 59-year-old asymptomatic woman with no disease history presented to our institution for investigation of a pancreatic tail mass. Hematological parameters were within normal ranges, including serum carcinoembryonic antigen levels and carboxyhydrate antigen 19-9. Computed tomography revealed a strongly enhanced solid peripheral mass with an intracystic component in the pancreatic tail (Figure A, arrow). Magnetic resonance imaging revealed a cystic lesion on the T2 image surrounded by a solid component with density and intensity levels similar to those in the spleen parenchyma (Figure B, arrow). Endoscopic ultrasonography showed a solid hyperechoic 20-mm mass and an intracystic hypoechoic component showing stronger enhancement by Sonazoid (MSD Inc) than the pancreatic parenchyma (Figure C, arrow). Laparoscopic distal pancreatectomy was used for diagnosis. Pathological examination revealed a white and red sinus of the ectopic spleen in the pancreatic tail, with a unilocular cyst lined with stratified squamous epithelium, indicating an epidermal cyst in the intrapancreatic accessory spleen (Figure D, arrows). Most epidermal cysts in the intrapancreatic accessory spleen cases are found incidentally, typically as single monolobular cystic lesions in the pancreatic tail with a thickened cystic wall or solid components identical in density to the spleen on imaging examinations, making a definite preoperative diagnosis extremely difficult, resulting in unnecessary surgical intervention.

S3084 Metastatic Pancreatic Tail Adenocarcinoma Presenting With Multiple Hepatosplenic Abscesses: A Case Report

Introduction: Pancreatic cancer is the third leading cause of cancer-related deaths in the United States. It is often detected in advanced stages and may present in unusual ways such as pyogenic liver abscesses (PLA) or splenic abscesses which may be a diagnostic and therapeutic challenge. Case Description/Methods: A 78-year-old dialysis-dependent man with a history of end stage renal disease, hypertension and cerebrovascular accident was evaluated for diffuse abdominal pain and altered mental status for one day. The examination was pertinent for generalized weakness and altered sensorium. Laboratory tests revealed transaminitis and leukocytosis. Computed tomography (CT) of the abdomen and pelvis showed multiple hypoattenuating hepatic foci with hepatosplenomegaly. A set of blood cultures grew Clostridium inoculum. A pyogenic liver abscess was diagnosed, drained percutaneously and the culture grew pansensitive Escherichia coli. A follow up CT of the abdomen and pelvis revealed a large splenic abscess. Exploratory laparotomy, splenectomy, liver biopsy and peritoneal lavage were performed. Immunostaining of the sample was positive for CK7, CK20 and CDX2. Pancreaticobiliary adenocarcinoma metastatic to the spleen and liver was confirmed by pathology. CA 19-9 and CA 125 were elevated. A repeat CT of the abdomen with pancreatic protocol showed a pancreatic tail mass. Vancomycin resistant Enterococcus faecium grew on peritoneal fluid culture. Daptomycin and ceftriaxone were started. The patient improved clinically but he was deemed unsuitable for further invasive procedures or chemotherapy. (Figure) Discussion: Uncommon presentations of pancreatic adenocarcinoma include gastrointestinal (GI) bleeding from metastasis and acute abdomen from splenic infarct. PLA is associated with GI malignancies including pancreatic cancer. Some experts recommend cancer evaluation for GI malignancies in patients with PLA. Mucosal barrier breach by cancer cells, translocation of bacteria into the circulation and inoculation at metastatic sites are possible explanations for developing liver and splenic abscesses. Dialysis is also a risk factor for hepatosplenic abscess. In an elderly patient with hepatosplenic abscess and elevated tumor markers, a thorough evaluation for GI malignancies including pancreatic cancer should be done.Figure 1.: Image 1 shows cancer in the spleen. Images 2-3 show positive immunostains for CK7 in image 2, CK20 in image 3. Image 4 shows the cancer in the liver biopsy (no normal liver is present). Image 5 shows non-enhancing hypoattenuated foci in the right and left lobes of the liver most consistent with multifocal liver abscesses. Image 6 shows splenomegaly with multiple hypoattenuated foci and a mass within the tail of the pancreas.

Microcystic serous cystadenoma mimicking pancreatic neuroendocrine neoplasm: report of a resected case with preoperative diagnostic difficulty and review of the literature

BackgroundMicrocystic pancreatic serous cystadenoma (SCA) can be managed without surgery in selected patients. However, the preoperative diagnosis of microcystic SCA remains challenging, and it is potentially misdiagnosed as other pancreatic cystic neoplasms or solid tumors, especially small microcystic SCA.Case presentationThis was a case of a 27-year-old male patient with microcystic SCA causing difficulty in the differential diagnosis from pancreatic neuroendocrine neoplasm (pNEN). A pancreatic tail mass was incidentally discovered on abdominal ultrasound (US). A contrast-enhanced computed tomography (CT) scan revealed a solid tumor measuring 13 mm with early enhancement in the arterial phase at the pancreatic tail. The tumor showed low intensity on T1-weighted magnetic resonance image, high intensity on T2-weighted image, and a slightly hyperechoic mass on endoscopic US (EUS). EUS-fine needle aspiration (EUS-FNA) did not lead to a definitive diagnosis. The tumor was clinically diagnosed as a pNEN, and a laparoscopic spleen-preserving distal pancreatectomy using the Warshaw technique was performed. The final histopathological diagnosis was microcystic SCA.ConclusionSmall microcystic SCA is difficult to distinguish from a hypervascular pancreatic tumor such as pNEN on imaging studies, and it is necessary to focus on the tumor echogenicity of EUS to differentiate microcystic SCA from pNEN preoperatively.

The great masquerader of pancreatic tuberculosis

BackgroundPancreatic tuberculosis is a rare clinical entity. It represents a diagnostic challenge as the clinical presentation may mimic pancreatic malignancy.Case presentationA 66-year-old gentleman presented with an incidental finding of a pancreatic tail mass on routine ultrasonography of the abdomen whilst working up on chronic kidney disease. He denied abdominal pain, fever, night sweats, constitutional symptoms or tuberculosis contact. On examination, there was no palpable mass per abdomen. The tumour marker of Ca 19-9 was normal. Ultrasonography revealed a lobulated heterogeneous hypovascular and hypoechoic mass at the tail of the pancreas. Contrast-enhanced computed tomography (CT) of the thorax and abdomen revealed a thickened right pleura, right pleural effusion with right lung collapsed consolidation and multiple mediastinal lymph nodes. There is an ill-defined hypodense mass seen in the tail and body of the pancreas measuring 3 × 7 × 3 cm with the presences of calcification within. The constellation of CT findings suggests a pancreatic malignancy with metastasis to the lungs. Endoscopic ultrasonography (EUS) assessment showed an irregular hypoechoic mass measuring 3.0 × 2.7 cm at the tail of the pancreas. Multiple rounded hypoechoic lesions were also seen scattered within the body of the pancreas with multiple enlarged para-aortic lymph nodes. A fine-needle aspiration biopsy of the lesion was consistent with granulomatous inflammation. The diagnosis of disseminated tuberculosis was made. The patient was subsequently started on antitubercular medication and recovered well.ConclusionA high index of suspicion is needed to diagnose pancreatic tuberculosis, especially in patients whose radiological imaging shows a pancreatic mass with necrotic peripancreatic lymphadenopathy in endemic countries. EUS-guided fine-needle aspiration is the diagnostic modality of choice, and vigorous attempts should be made at obtaining a preoperative histological or bacteriological diagnosis to avoid unnecessary surgery.

A Pancreatic Tumor With Homogeneous Hyper-Enhancement Under Contrast-Enhanced Endosonography Showing an Unexpected Pathology Finding

Question: A 35-year-old man without past systemic disease presented with epigastralgia for 1 week and weight loss of 10 kg during the past few months. Abdominal sonography found a suspicious lesion over pancreatic body-tail, and contrast-enhanced computerized tomography (CE-CT) showed one 3.0-cm well-circumscribed mass in pancreatic tail, with hypo-density in the precontrast phase and progressive peripheral enhancement in the arterial phase (Figure A). Endosonography was performed, which showed a 30.6-mm x 21.9-mm isoechoic tumor at pancreatic tail, and homogeneous hyper-enhancement of the lesion was noted 12 seconds after sonazoid 0.5-mL injection, without evident washout during the procedure (the observation time lasted up to 210 seconds; Figure B).

A Pancreatic Tail Mass in a Young Male

A Pancreatic Tail Mass in a Young Male

A Rare Presentation of Multiple Endocrine Neoplasia Type 1

Background: Multiple endocrine neoplasia type 1 (MEN1) is a rare, autosomal dominant inherited syndrome caused by mutations in the MEN1 tumor suppressor gene with a reported incidence of 2 in 100,000.Clinical Case: A 26-year-old Caucasian female was seen for surveillance screening given positive familial mutation in the MEN1 gene. She had a significant family history of pathogenic MEN1 in her son, brother, father and paternal aunt. On presentation, she denied any history of headache, nipple discharge, kidney stones, fractures, heart burn, abdominal pain, diarrhea, hypoglycemia, flushing, lightheadedness. She was on hormonal IUD for contraception and had not had menstrual cycles for the last 6 months. Vital signs and physical examination were unremarkable. Her initial evaluation included a normal calcium of 9.8mg/dL (RR:8.6-10.6mg/dL), PTH of 70pg/ml (RR:12-88pg/ml), 25OH-vitamin-D of 11ng/ml (RR:20-80ng/ml). She had normal gastrin, chromogranin A, glucagon, and vasoactive intestinal peptide levels. Prolactin was 17.6ng/ml (RR:3.3-26.7 ng/ml). MRI pituitary showed a 6mm lesion representing a Rathke’s cleft cyst, not compressing surrounding structures.Two months following the initial encounter, she presented with abdominal pain, nausea, vomiting. She was found to have an obstructing renal stone requiring stent placement. Calcium was 10.1mg/dl. She was also noted to have an incidental pancreatic tail mass of 4.2cm on CT abdomen. She underwent laparoscopic robotic distal spleno-pancreatectomy. Surgical pathology showed a well differentiated 3.5cm neuroendocrine tumor with negative margins, which stained positively for insulin. She had not reported symptoms of weight gain or any episodes suspicious for sympathoadrenal activation or neuroglycopenia, and was again confirmed after surgery.Patient was re-admitted to the hospital for right-sided flank pain and was found to have a new 6mm obstructing calculus with moderate hydronephrosis of the right kidney. Corrected calcium level was found to be mildly elevated at 11.1mg/dL. Repeat PTH was stable at 81pg/ml, and urinary calcium was elevated at 447.3mg/24h (RR:50-250mg/24h). Subsequently, she underwent 3.5 gland parathyroidectomy and thymectomy. On POD20, PTH was 29pg/ml and calcium was 8.6mg/dl.Conclusion: This case highlights a rare presentation of MEN1 with an asymptomatic insulinoma and nephrolithiasis, despite having normal calcium and PTH levels initially. Primary hyperparathyroidism is the most common presentation of MEN1, seen in up to 90% of patients. The second most common finding in MEN1 patients, reported in 70% of the cases, are entero-pancreatic tumors, the most common being gastrinoma. Insulinoma, which is less common and is seen in only 10-30% patients, needs documentation of Whipple’s triad. Interestingly, our patient never had any history of hypoglycemia.

Lymphoepithelial cyst mimicking pancreatic cancer: a case report and literature review

BackgroundPancreatic lymphoepithelial cyst (LEC) is a rare nonmalignant cyst consisting of a benign collection of keratinizing squamous epithelial cells with lymphoid tissue. Diagnosing LEC preoperatively is considered difficult because of its non-specific clinical features; therefore, LEC is generally treated the same as a malignant tumor.Case presentationOur case was a 65-year-old man who underwent pancreatoduodenectomy 3 years previously for carcinoma arising from the ampulla of Vater. A pancreatic mass in the remnant pancreatic tail was detected through follow-up abdominal contrast-enhanced computed tomography (CT). This revealed two adjacent ring-enhanced masses that had been in tight contact with the left diaphragm and were enlarged. The tumors had high signal intensity in diffusion-weighted images of magnetic resonance imaging, and fluorodeoxyglucose-positron emission tomography (FDG-PET) showed abnormal uptake (standardized uptake value maximum: 17.4). Therefore, we conducted a partial resection of the remnant pancreas with concomitant resection of the left diaphragm. Microscopically, one of the tumors revealed rare fragments of apparently benign squamous epithelium on a background of keratinous debris, cyst contents, and scattered lymphocytes, and the adjacent mass revealed infiltrated neutrophils. The histopathological diagnosis was an LEC with chronic abscess. The patient recovered uneventfully and was discharged on postoperative day 10.ConclusionsWe reported a rare case of LEC with chronic abscess that was positively visualized on FDG-PET. When a pancreatic malignancy cannot be excluded, surgical resection is considered inevitable.

Breast cancer in multiple endocrine neoplasia type 1 (MEN1).

SummaryA 38-year-old female was identified as carrying a heterozygous pathogenic MEN1 variant (c.1304delG) through predictive genetic testing, following a diagnosis of familial hyperparathyroidism. Routine screening for parathyroid and pituitary disease was negative. However, cross-sectional imaging by CT revealed a 41 mm pancreatic tail mass. Biopsy via endoscopic ultrasound confirmed the lesion to be a well-differentiated (grade 1) pancreatic neuroendocrine tumour (pNET) with MIB1<1%. Biochemically, hyperinsulinaemic hypoglycaemia was confirmed following an overnight fast, which was subsequently managed by diet alone prior to definitive surgery. Pre-operative work-up with octreotide SPECT CT demonstrated avid tracer uptake in the pancreatic lesion and, unexpectedly, a focal area of uptake in the left breast. Further investigation, and subsequent mastectomy, confirmed ductal carcinoma in situ pT2 (23 mm) grade 1, N0 (ER positive; HER2 negative). Following mastectomy, our patient underwent a successful distal pancreatectomy to resect the pNET. Loss of heterozygosity (LOH) at the MEN1 locus was found in both the breast tumour and pNET, thereby in keeping with a 'two-hit' hypothesis of oncogenesis, a suggestive but non-definitive clue for causation. To obtain further support for a causative relationship between MEN1 and breast cancer, we undertook a detailed review of the published literature which overall supports the notion that breast cancer is a MEN1-related malignancy that presents at a younger age and histologically, is typically of ductal subtype. Currently, clinical guidance regarding breast cancer surveillance in MEN1 does not exist and further research is required to establish a clinical and cost-effective surveillance strategy).Learning pointsWe describe a case of pNET and breast cancer diagnosed at a young age of 38 years in a patient who is heterozygous for a pathogenic MEN1 variant. Loss of the wild-type allele was seen in both breast tissue and pNET specimen.Breast cancer may be an under-recognised MEN1-associated malignancy that presents at a younger age than in the general population with a relative risk of 2–3.Further research is required to determine the cost-effectiveness of breast cancer surveillance approach at a younger age in MEN1 patients relative to the general population .

Predictive Values of Preoperative Markers for Resectable Pancreatic Body and Tail Cancer Determined by MDCT to Detect Occult Metastases.

To evaluate the clinical value of preoperative markers in predicting occult metastases in resectable pancreatic body and tail cancer judged by a recent multidetector computed tomography (MDCT) scan of the abdomen. The data from a retrospective collected database from 2010 to 2019 with 699 patients who had MDCT scan predicted resectable mass in pancreatic body and tail and were pathological confirmed as adenocarcinoma after surgery. Receiver operating characteristic (ROC) curve was plotted for serum CA19-9, CA125, CEA and tumor size measured by MDCT. The optimal cut-off point-related sensitivity and specificity were calculated, respectively. Occult metastases were found in 73 (73/699, 10.4%) pancreatic body and tail cancer patients underwent exploration. The area under curve (AUC) for CA19-9, CA125, CEA and tumor size were 0.624, 0.733, 0.561 and 0.697, respectively. The optimal cut-off for CA19-9, CA125 and tumor size is 226 U/ml, 22.1 U/ml and 3.3cm, respectively. The sensitivity and specificity of CA19-9 for predicting occult metastases were 67.1% and 60.4%, 72.6% and 64.7% for CA125, 80.8% and 51.4% for tumor size. CA125 is superior to CA19-9 and tumor size for predicting occulting metastases in MDCT scan suggested resectable pancreatic body and tail cancer. The high level of CA125 (≥ 22.1 U/ml) is regarded as high risk for occulting metastases, and laparoscopy should be applied for these patients.