SUN-903 Insulinoma - a Tricker Diagnosis When Some Pieces Are Missing

Insulinoma is a rare pancreatic neuroendocrine tumour that secretes insulin, causing hypoglycemia. Because of the nonspecific symptoms, the diagnosis could constitute a challenge. Early detection is important to prevent serious consequences. A 31-year old woman was admitted for prolonged fasting test. She had no relevant past medical or surgical history till eight months before, when she had an episode of generalized tonic-clonic seizure with loss of consciousness. At this time, she was taken to emergency, with identification of a hypoglycaemia of 33 mg/dL. Unfortunately this was undervalued and she was discharged with an appointment on a neurologist. After evaluation, she did an EEG, which was normal, and blood tests that identified a fasting glycemia of 50 mg/dL. By recommendation of her general practitioner, she began to monitor her glycemia during the day, identifying multiple glycemia <50mg/dL – in fasting and post-prandial period. After the first generalized seizure, she had multiple seizures, always associated with hypoglycaemia. During the night she had to wake up every two hours to eat, in order to prevent hypoglycaemia. Moreover, in the last 6 months, she augmented 12 Kg. She also described two episodes of behavioural changes with confusion and speech alteration. She wasn’t under any medication that could be associated with hypoglycemias. Previous records showed she had a fasting glycemia of 50 mg/dL two years ago. When she was admitted to our department, besides she had eat one hour before, she had glycemia <55 mg/dL. Blood tests showed glucose level=22 mg/dL, insulin=39 μU/mL (normal range 2.6-24.9 μU/mL), C-Pep=0.90 ng/mL (normal range 1.1-4-4 ng/mL). Plasma B-hydroxybutyrate was negative. After Glucagon EV, glucose level increase to 53 mg/dL (>25 mg/dL). We also evaluated cortisol and growth hormone that were normal. Abdominal computed tomography scan with contrast demonstrated a well-defined hypervascular lesion involving pancreas tail. Abdominal MRI was also performed showing a hypervascular lesion involving pancreas tail with 11x21mm. Laparoscopic surgery to enucleate the lesion was made. Pathological evaluation revealed a well-differentiated neuroendocrine tumour (positive staining for synaptophysin, cromogranin and insulin) measuring 0.3 cm. The diagnosis of pancreatic insulinoma was confirmed. After surgery, the glucose level increased to the normal range. The patient is currently in 6 months follow-up with a good evolution. The diagnosis of insulinoma requires high suspicion. In this case, the patient didn’t have the typical insidious neurogenic symptoms. There is a need to value neuroglycopenic symptoms associated with hypoglycemia, otherwise serious consequences can occur.

A suspected insulinoma with the unexpected histopathological finding of a nesidioblastosis – A case report and literature review

Introduction: Nesidioblastosis is a hyperplasia of the beta cells that causes persistent hyperinsulinemic hypoglycemia, unfortunately, it is difficult to diagnose through imaging studies. Case presentation: A 68-year-old woman with palpitations, diaphoresis, dizziness and alterations in the level of consciousness that improve with food intake. Laboratories: glucose 35.0 mg/dl, insulin 12.5 mUI/ml, proinsulin 14.1 pmol/L and peptide C 2.55 ng/ml. Octreoscan reveals an abnormal epigastric uptake area and the tomography shows a focal hypervascular lesion in pancreatic body of 12×11 mm. A distal pancreatectomy was performed without palpating the tumor in transoperative period, and a diffuse nesiodioblastosis was reported in the histopathological study. The patient persists with hypoglycemia and an additional pancreatic resection was performed, resecting 90% of the organ. Although an insulinoma was not located in the piece, an area surrounded by connective tissue was observed where islet hyperplasia was more accentuated. Discussion: The pathophysiology of nesidioblastosis is unknown, its main differential diagnosis is insulinoma and this should be suspected when the tumor can not be identified. Most agree that a pancreatectomy that involves 60-80% of the total organ can control glucose levels with low risk of diabetes and pancreatic insufficiency. Conclusion: The nesidioblastosis should be suspected when a hyperinsulinemic hypoglycemia is difficult to control and when a tumor can not be identified. The extension of the pancreatectomy should be individualized and if an insulinoma is not localized and a nesidioblastosis is suspected, an intraoperative histopathological examination with frozen section evaluation for the margins could determine the extent of pancreatectomy.

Positive molecular breast imaging: challenges and problem solving with contrast-enhanced spectral mammography

Molecular breast imaging (MBI) is a relatively new technique with high sensitivity for breast cancer detection. However, because it only provides limited anatomical information, cross-correlation of MBI findings with conventional breast imaging modalities such as full field digital mammography can be challenging. We report a case of a positive MBI study in a supplemental screening setting, where cross-correlation of MBI, ultrasound, mammogram and biopsy findings was difficult. Contrast-enhanced spectral mammography (CESM) demonstrated a hypervascular lesion at the biopsy clip, helping to prove imaging/histopathological concordance. This case highlights the challenges of incorporating MBI into conventional imaging workup, as well as the use of CESM in problem solving.

Intrapancreatic accessory spleen: an enigmatic entity

Solitary hypervascular lesion in the distal body/tail of pancreas in a patient with non-specific abdominal symptoms is a diagnostic challenge. Neuroendocrine neoplasm (NEN) and metastasis from renal cell carcinoma are the most common differentials and intrapancreatic accessory spleen (IPAS) is the rarest of its differential diagnosis. We present, here, a case of a 56-year-old man with a space-occupying lesion in body/tail of pancreas that was preoperatively diagnosed as a NEN based on elevated chromogranin levels and hyperenhancing lesion on contrast-enhanced CT scan. He underwent a spleen-preserving distal pancreatectomy. The final histopathology revealed an IPAS.

A case report of hepatic perivascular epithelioid cell tumours (pecomas) and literature review

Perivascular epithelioid cell tumours (PEComas) belongs to the family of mesenchymal neoplasms that can occur in many organs, but rarely found in liver. Preoperative diagnosis could be challenging due to unspecific and variable radiologic patterns, which can be clinically misdiagnosed with hepatocellular carcinoma and other benign tumors. This report aims to announce a case of hepatic PEComa with extramedullary hematopoiesis. A 44- year- old woman accidentally presented a nodular mass in the left hepatic segment on ultrasonography with normal liver function result. Abdominal enhanced CT Scan showed hypervascular lesion. Morphology exibits classical characteristics of PEComa with mature fat tissue predominance, thick-wall vessel; somewhat amount of extramedullary hematopoiesis was also recognized. Tumor cells are diffusely immunoreactive with HMB45. PEComa is a rare disease in liver, pathologists need to recognize three components of this tumor to avoid misdiagnosing with malignant conditions.