An Unusual and Interesting Presentation

50-year-old white man presented with hyperglycemia and a 6-month history of changes in bowel habits, bloating, epigastric pain that radiated to his back, an 11.3kg weight loss, and ultimately dark brown urine and jaundice. He underwent an abdominal computer-assisted tomographic scan and endoscopic retrograde cholangiopancreatography, which revealed a pancreatic mass. A stent was placed in the ampulla, and he was referred for surgical evaluation of the pancreatic mass. A radical pancreaticoduodenectomy (Whipple procedure) was performed. The common bile duct was 1 cm in diameter. Three peripancreatic lymph nodes were enlarged up to 4 cm in diameter. The lymph nodes were difficult to dissect because of fibrosis to adjacent structures. Within the head of the pancreas, a firm nodular mass was identified. The remaining pancreas had a rocklike consistency. The surgical specimen consisted of the distal portion of the stomach, proximal portion of duodenum, and proximal portion of pancreas with attached gallbladder and cystic duct. The cut surface of the enlarged peripancreatic lymph nodes showed a nodular area of effacement. The nodes were tan-pink and had confluent areas of hemorrhage and necrosis. There was a tumor within the head of the pancreas, 1.6 cm in diameter, that was located medial to the common bile duct. The cut surface had an appearance similar to that of the enlarged lymph node. A total of 17 lymph nodes were dissected, 3 of which were positive for tumor metastases. Microscopically, the tumor was strikingly cellular and divided into lobulations by thin fibrous strands. The tumor extended into adjacent pancreatic tissue in a scalloped nodular configuration. While there were areas displaying uniform solid nests of tumor cells, the regular ‘‘acinar’’ with back-to-back glandular arrangement was a predominant pattern throughout the tumor (Figure, A). The tumor cells had a granular eosinophilic cytoplasm. The nuclei were basal in orientation, hyperchromatic, and showed slight variability in size and shape. The mitotic count was low (,1/10 high-power fields). There was a positive periodic acid‐Schiff reaction following diastase digestion within apical cytoplasmic tips (Figure, B). Mucicarmine was sparsely positive. Staining for neuroendocrine markers, including chromogranin, synaptophysin, glucagon, somatostatin, gastrin, and vasoactive intestinal

Rationale:Tuberculosis remains a serious menace to the health of people. Isolated hepatic tuberculosis is rare and pancreatic tuberculosis is extremely rare. The preoperative diagnosis of pancreatic tuberculosis remains a great challenge.Patient concerns:A 58-year-old Asian woman was referred to our hospital for evaluation of low back pain for 4 years and abdominal pain for 1 month.Diagnoses:Computed tomography (CT) of the abdomen showed a hypodense mass in the pancreatic head and neck with abundant calcifications, a hypodense lesion in the liver without calcification, peripancreatic lymphadenopathy, calcifications in some lymph nodes. CT-guided fine needle aspiration biopsy of the hepatic lesion was carried out and the cytological examination revealed hepatic tuberculosis.Interventions:The patient was treated with antituberculous therapy for 1 year.Outcomes:Low back pain and abdominal pain disappeared 3 months after initial treatment and after 2 year of follow-up, the patient was asymptomatic.Lessons:Our data hint that calcifications in both pancreatic lesions and peripancreatic lymph nodes may suggest pancreatic tuberculosis rather than pancreatic malignancy.

Two additional cases of parotid neuroendocrine carcinoma: Is there a wall that cannot be overcome in the cellular morphology?

Insulinoma is a gastrointestinal tumor, usually benign, which derived from pancreatic beta cells and typically induced by endogenous hyperinsulinism with an incidence rate of 14 cases per million inhabitants each year. This case report describes the diagnostic challenges and dilemmas associated with finding optimal treatment for an insulinoma located in the head of the pancreas. A 41-year-old female was presented with recurrent, nagging headaches, loss of attention and episodes of anxiety accompanied by a feeling of heart palpitation. Based on the test results, reactive hypoglycemia was diagnosed and further symptomatic treatment was recommended. The patient was admitted again nine years after her initial hospitalization for symptoms of hypoglycemia. Abnormally high insulin and peptide C secretion was found. Computed tomography (CT) scan revealed a tumor with a size of 1.8x1.3x2.2 cm located within the uncinate process of the pancreas. Pancreatoduodenectomy was recommended, however, the patient refused surgery. She was given somatostatin analogues leading to a satisfactory clinical effect. Computed angiotomography was done which confirmed the presence of an abundantly vascularized tumor within the head of the pancreas. Embolization with histoacryl glue was performed. Due to the ineffectiveness of embolization, treatment with short-acting and then long-acting synthetic somatostatin analogues was reintroduced. A follow-up CT scan conducted six months after the procedure revealed regression of tumor size. Treatment of choice for insulinoma is surgery, but conservative treatment is recommended when surgery is impossible or contraindicated. Afferent vessel embolization is a safe treatment option. Chemotherapy may be an option for inoperable, malignant tumors.

Introduction: Extra-nodal Non-Hodgkin’s lymphomas are mostly made of gastrointestinal sites1. Primary pancreatic lymphomas account for < 1% of all extra-nodal lymphomas and are extremely rare. Diagnosis is often missed or delayed due to the prevalence of chronic pancreatitis or pancreatic adenocarcinoma as thought to be the main causes of any pancreatic stricture or mass seen when diagnosing a patient. We present a case of pancreatic lymphoma presenting as acute recurrent pancreatitis. Case Description/Methods: A 51-year-old male with past medical history of rheumatoid arthritis and nicotine use presented with jaundice, abdominal pain, and fatigue and 30-pound weight loss. MRCP showed intra- and extra-hepatic biliary dilation, showing double duct sign. CT suggested acute pancreatitis. ERCP was done showing a single localized biliary malignant appearing stricture in the lower third of the main bile duct with upstream dilation. EUS showed a 19 mm by 19 mm mass in the head of pancreas and 8 mm dilation of the common bile duct. EUS fine needle biopsy showed chronic pancreatitis with dense fibrosis and benign peri-pancreatic lymph nodes. PET CT showed activity in the pancreas and retroperitoneal lymph nodes. Tumor markers (AFP, CEA, CA 19-9) were within normal limits. (Figure) Patient had no known risk factors for chronic pancreatitis therefore, the acute presentation and biopsy results in congruent with the clinical history led us to repeat EUS one month later. This showed a 20 mm by 15 mm irregular mass in the head of the pancreas with upstream pancreatic duct dilation. This time, pathology was sent for flow cytometry and came back positive for lymphoid infiltrate with a preponderance of B-cells surrounded by dense fibrosis and was positive for CD 19, 20, 22 and 10. By this point, malignancy other than adenocarcinoma was suspected and IR guided biopsy of retroperitoneal lymph nodes confirmed a diagnosis of diffuse large B cell lymphoma. Patient started chemotherapy with a 70% chance of being cured. ERCP one month later showed the stricture had resolved and the stent was removed. Discussion: Keeping a broad differential in mind for pancreatic masses is essential to prevent delayed diagnoses. With that in mind, although rare, pancreatic lymphoma should always be in the differential and physicians should consider sending for flow cytometry to avoid missing this diagnosis. When patients present with recurrent pancreatitis and no risk factors, lymphoma should be kept in the differential.Figure 1.: a) CT showing inflammatory reaction of the pancreas with bile duct dilation; b) Bile duct stricture on ERCP; c) EUS image showing common bile duct stent.

INTRODUCTION: Endoscopic Ultrasound Elastography (EUS-E) is increasingly used to augment EUS imaging and to direct EUS-guided tissue acquisition. The experience with EUS-E using new generation EUS processors is limited. We aimed to evaluate the diagnostic utility of EUS-E in a variety of gastrointestinal lesions in a diverse safety net patient population. METHODS: The EUS database was retrospectively queried for EUS-E procedures performed from 08/15/2019, when EUS-E first became available at our institution, to 06/15/2020. EUS-E was performed with a linear echoendoscope (GF-UCT180, Olympus USA) and Hitachi Aloka ProSound F75 processor (Olympus USA). The queried data included EUS-E strain ratios (SR), final pathologic diagnosis, EUS findings, and patient demographics. Where multiple SR obtained, the mean was reported. Previously reported SR cutoff of 18 was used to define high risk lesions. RESULTS: EUS-E was performed in 19 patients (mean age 55 y; 28–77; 9 men and 10 women); mean lesion size 3.3 cm ± 1.3 cm. Pancreatic masses, lymph node, thyroid mass, hepatic mass, gastric mass were among lesions analyzed. High risk lesions based on EUS-E were confirmed by cytology/tissue pathology to be five pancreatic adenocarcinomas, one pancreatic intraepithelial neoplasia, and one intraductal papillary mucinous neoplasia with mean SR 41.5 ± 22.8 (0.48–81.0). EUS-E with EUS-FNA/B as a diagnostic standard had the sensitivity, specificity, positive predictive value, negative predictive value and overall accuracy of 87.5%, 71.4%, 77.8%, 83.3%, and 80.0%, respectively (Table 1). One heterogeneous lesion with SR 0.48 was later determined to be pancreatic adenocarcinomaTable 1. Benign pathology was found in a peri-pancreatic mass, SR 3.8, a pancreatic head mass, SR 13.7, a pancreatic body mass, SR 0.75, a gastric subepithelial lesion, SR 0.71, and a hepatic lesion, SR 0.33. Two pancreatic lesions with benign pathology, SR 22.7 and 42.8, are now pending repeat EUS-FNB. Pathology was non-diagnostic on a thyroid mass with SR 9.5 and a pancreatic body mass with SR 1.4. Biopsy was not performed on two lesions, a peri-pancreatic lymph node with SR 25.3 and a peri-pancreatic splenule with SR 45.3. CONCLUSION: Our initial experience suggests that EUS-E could be a useful adjunct to EUS imaging and EUS-guided tissue acquisition. Lesions with benign pathology and high strain ratios should be considered for repeat sampling and closely followed.

Solid Pseudopapillary Tumor (SPT) of the pancreas is a rare epithelial tumor of low malignant potential. Its natural history differs from the more common pancreatic adenocarcinoma in that it has a female predilection, is more indolent, and carries a better prognosis. We are reporting a case of SPT of the pancreas diagnosed in a young female patient. An 18-year-old female presented with abdominal pain. The patient was otherwise healthy. Physical exam was unremarkable. Hematologic and metabolic parameters, including tumor markers, were within normal limits. MRI of the abdomen showed a 2.5 cm x 1.5 cm cystic lesion in the tail of the pancreas. Endoscopic ultrasound (EUS) of the lesion revealed a complex cystic mass with a thick wall and irregular internal solid components, measuring 2 cm x 1.5 cm. Fine needle aspiration was performed and 1.5 ml of serosanguinous fluid was obtained. The cyst was completely drained. Cytology revealed atypical papillary clusters with peri-capillary architecture. The patient underwent a laparoscopic distal pancreatectomy with splenectomy, as the tumor was invading the spleen. Pathology of the resected mass confirmed the diagnosis of SPT of the pancreas. Eight peripancreatic lymph nodes were resected and were all free of malignancy. Since the disease was localized and the excision was complete, the patient was not given adjuvant treatment. SPT is an uncommon pancreatic neoplasm, representing about 1-3% of exocrine pancreatic neoplasms. It usually occurs in young females. It has low-grade malignant potential and a favorable prognosis. A patient might present with abdominal pain, nausea, vomiting, a palpable abdominal mass, and occasionally jaundice due to obstruction of the bile duct. Most tumors are diagnosed through ultrasound or CT, but MRI also defines the hypervascular, well-encapsulated, round tumors with mixed cystic and solid components. EUS and FNA provide pre-operative diagnosis of SPT. Treatment involves complete excision of the tumor, which results in complete cure in majority of the cases. Overall 5-year survival following primary resection approaches 95%. In conclusion, a cystic pancreatic mass in a young female patient should raise suspicion for a solid pseudopapillary pancreatic tumor. The characteristic imaging features can help to differentiate SPT from other pancreatic tumors. Early detection is important, since timely resection usually results in long-term survival.2915_A Figure 1. T2-weighted MR of the abdomen, axial view, showing a solid pseudopapillary tumor in the tail of the pancreas.2915_B Figure 2. Serosanguinous aspirate obtained during EUS-FNA.2915_C Figure 3. H&E stain showing solid pseudopapillary tumor (T) and normal pancreas tissue (P)

INTRODUCTION: Pancreatic cancers are very aggressive cancers with high metastasizing potential. Pancreatic neuroendocrine tumors (PNETs) are rare cancers that account for 1-2% of pancreatic tumors. We present an unusual case of metastatic PNET occurring in a 32-year-old man. CASE DESCRIPTION/METHODS: A 32-year-old male without past medical history presented with worsening diffuse abdominal pain and nausea for 3 weeks. He also reported clay-colored stool, intermittent diarrhea, and stabbing abdominal pain. Labs showed total bilirubin 3.1 (0.3-1.2), direct bilirubin 2.2 (0-0.3), alk phos 173 (50-136), AST 124 (15-40), ALT 200 (4-47), and lipase 707 (59-393). Hepatomegaly was noted on the physical exam. Abdominal US demonstrated hepatomegaly with multiple isoechoic hepatic masses. CT A and P showed pancreatic body neoplasm with diffuse hepatic metastasis. Ca-19 and CEA tumor markers were within normal limits. Endoscopic US revealed an irregular pancreatic body mass measuring 30 mm × 25 mm. FNA showed high-grade neuroendocrine carcinoma with small cell features. FNA malignant cells were positive for CD56, Claudin4, pankeratin, with high proliferative activity (Ki-67 proliferation index is 60%). The patient was diagnosed with metastatic Stage 4 pancreatic cancer. The patient was discharged and later admitted to a different hospital with intractable abdominal pain and worsening jaundice. MRI of the pancreas showed 3.6 cm × 2.9 cm primary pancreatic neoplasm and innumerable hepatic metastasis replacing x >50% of the liver parenchyma. US-guided FNA of hepatic metastasis revealed small cell carcinoma with the Ki67 proliferation index greater than 90%. The tumor was positive for pancytokeratin (AE1/AE3), epithelial membrane antigen, synaptophysin, and CD56. Few tumor cells were positive for GI markers CDX2 and SATB2. Unfortunately, the patient was not a candidate for any intervention, and he was discharged to continue palliative chemotherapy at home. DISCUSSION: PNETs are malignant tumors with an insidious presentation. PNETs have a varied clinical manifestation, including abdominal pain, nausea, jaundice, and anorexia. Unfortunately, as demonstrated in this patient, pancreatic cancers often present with non-specific early symptoms and are identified at advanced stages with multiple metastases. Treating metastatic pancreatic cancer, especially in a young man, is challenging as the mainstay of treatment remains palliative.Figure 1.: PNET with diffuse hepatic metastasis on CT abdomen and pelvis.Figure 2.: PNET with diffuse hepatic metastasis on CT abdomen and pelvis.

Tu1659 Forward Viewing EUS Versus Standard Oblique Viewing EUS in the Performance of EUS-Guided Fine Needle Aspiration of Solid Lesions: a Prospective Randomized Controlled Study

Myeloid sarcoma as a pancreatic cancer mimic: Refining the clinical differential of pancreatic masses

Abdominal computed tomography revealed a 19×13mm delayed enhancing mass and dilation of the distal pancreatic duct in the head of the pancreas. Magnetic resonance cholangiopancreatography showed pancreatic duct stenosis in the tail of the pancreas. Endoscopic retrograde pancreatography revealed an abrupt interruption of the main pancreatic duct at the tail of the pancreas. We could not assess the distal side of the pancreatic stenosis due to the large extent of obstruction. The pancreatic head mass was diagnosed as adenocarcinoma using endoscopic ultrasound-fine needle aspiration biopsy. However, we could not determine whether the pancreatic duct stenosis in the tail of the pancreas was malignant. Nevertheless, we performed a total pancreatectomy with splenectomy. Histological examination showed poorly differentiated adenocarcinoma in the pancreatic head mass but the pancreatic duct stenosis in the tail of the pancreas was diagnosed as pancreatic granuloma caused by Cryptococcus. Fungal infections may reportedly promote the development of pancreatic cancer, as further suggested by this case of cryptococcal infection.

Objective: Objective of this study is to highlight the importance of considering clinical and radiological findings in parotid tumors before operating tumors with high suspicion of malignancy though reported as benign on histopathology. Material and Methods: 20 years old female patient underwent radical parotidectomy for epithelial-myoepithelial carcinoma of the left parotid gland which was diagnosed on FNAC. Contrast CT and contrast MRI also showed a tumor involving left parotid gland with destruction of ramus of mandible and loss of tissue planes medially & laterally. Facial nerve was infiltrated by tumor, thus sacrificed. Post operatively, patient had left facial palsy and was discharged after 7 days without any other sequelae. Postoperative histopathology showed pleomorphic adenoma with positive resection margins medially, superiorly & posteriorly. No further treatment was given in view of the benign nature of the tumor. Results: Patient was kept on monthly follow up and was disease free at one year follow up. Conclusion: Aggressive surgical treatment should be planned for malignant parotid tumors to give a better tumor free survival. Misdiagnosis can occur on FNAC as it can hit only one part of the tumor thus missing a malignant tumor or can interpret pleomorphic adenoma as epithelial-myoepithelial carcinoma.

INTRODUCTION: Metastatic lesions of the pancreas are uncommon, with reported frequencies of intrapancreatic metastases ranging from 4.5% to 11%, with isolated metastases around 2% of cases. Metastases from malignant melanoma is quite rare, with fewer then 200 cases reported in literature. CASE DESCRIPTION/METHODS: 79-year-old female who presented to ED in June 2018 with fever, dysuria, and dyspnea. Past medical history significant for prednisone dependent sarcoidosis, atrial fibrillation, and basal cell carcinoma. Labs showed elevated WBC and UA concern for UTI. She was admitted for treatment. CT scan was done due to dyspnea showing LLL consolidation. Incidentally, a new 2.6 × 1.8 cm mass within the pancreas neck was seen. At discharge she was referred to our facility for EUS and FNA. EUS showed a pancreatic mass abutting the superior mesenteric vein without invasion. Cytological analysis obtained by EUS-FNA (Figure 1) showed malignant melanoma with abundant melanin pigments (Figure 2). Positive for Sox-10, Melan A and HMB-45. Referral to medical oncology was made for further work-up. PET scan was preformed due to lack of previous melanoma history, showing no evidence of disease outside the pancreatic area (Figure 3). With no primary lesion identified further referrals to Dermatology and Ophthalmology, however these evaluations were negative for overt cutaneous or ocular melanoma. Colonoscopy was performed and showed no lesions. Due to the isolated lesion, deemed an appropriate candidate for resection. September 2018, partial pancreatectomy and regional lymph node resection completed. Pathology of the 4.5 cm pancreatic neck mass, in addition to celiac and portal lymph node, consistent with malignant melanoma. October 2018 started immunotherapy of Opdivo and Ipilumimab. Repeat PET scan showed nodule along right flank, superior to iliac crest, FDG avid, suspicious for melanoma and thought to be the sight of primary lesion. Tolerated immunotherapy well, but in December 2018, was admitted due to respiratory distress secondary to aspiration pneumonia, was transitioned to comfort cares due to worsening clinical status, passing away. DISCUSSION: This case is a unique presentation of an isolated metastatic pancreatic melanoma, found incidentally in an otherwise asymptomatic patient. With the widespread use of imaging, clinicians can expect an increasing number of “incidentalomas” being found, stressing the role of EUS-FNA in gaining a precise diagnosis which is critical in the overall prognosis and management of patients.

This study describes an autopsy case of pancreatic/peripancreatic myeloid sarcoma in a 70-year-old man, initially presenting with obstructive jaundice. Pathologically, diffuse infiltration of round cells containing atypical nuclei with marked cleavage was observed mainly in the pancreas head, peripancreatic lymph nodes, spleen, bilateral lung, and bone marrow. Immunohistochemically, the tumor cells were negative for CD20, CD79a, CD3, CD5, c-kit, CD34, and TdT and positive for myeloperoxidase, CD33, CD68, and CD163. Flow cytometry of the peripheral blood showed underexpression of CD11c and aberrant expression of CD56 in the monocyte subset. The peripheral blood smear showed an increase in monocytes and atypia in neutrophils and monocytes, as well as enlarged platelets and polychromatic erythroblasts. Hence, it was suggested that the myeloid sarcoma was derived from the acute transformation of chronic myelomonocytic leukemia. Myeloid sarcoma is an extramedullary-mass-forming hematologic malignancy that is difficult to diagnose, especially when the initial presentation is a pancreatic mass. However, early diagnosis is important for appropriate therapy. Although bone marrow examination could not be performed because of the patients' severe condition, the pathological specimen obtained with autopsy helped subtype the patient's leukemia. The immunohistochemical features of this case merit attention.

To determine the morphological pattern of parotid tumours. Case series. ENT Department of Karachi Medical and Dental College and Abbasi Shaheed Hospital, Karachi, from 1990 to 2004. During this study, 204 patients with parotid tumours were registered. The patients of all ages and both gender were included in this study. All patients were evaluated by history, clinical examination, F.N.A.C. and ultrasound, C.T/MRI was done in selected cases. All patients were surgically managed and their tumour specimen was sent for histopathology. Classification of individual tumour was based on 1991 World Health Organization Classification. Discrete data was presented in percentage and proportions. Out of 204 cases, 152 (74.5%) were benign and 52 (25.5%) were malignant. Of these, 117 (57.35%) patients were females and 87 (42.65%) males. Benign tumours were more common in females whereas malignant tumours were common in males. The mean age of patients was 34 years and 42 years for benign and malignant tumours respectively. Pleomorphic adenoma was most common benign tumor (83.5%), followed by Warthins tumour. The most common malignant tumour was mucoepidermoid carcinoma (60%), followed by adenoid cystic carcinoma. Superficial lobe of parotid gland was the commonest site, 120 benign and all 52 malignant tumours arising from it while 32 benign tumours originated from deep lobe. Parotid swelling for years was main feature of benign tumours, whereas malignant tumours presented with pain, fixation to skin or underlying structure, cervical lymphadenopathy and facial palsy. Pleomorphic adenoma was the most common benign tumour and mucoepidermoid carcinoma was most common malignant tumour. The morphological patterns and distribution followed the known pattern.