ABSTRACT
Glucagonomas are neuroendocrine tumors (NETs) that arise from the alpha cells of the pancreatic islets. They are typically slow-growing tumors associated with abnormal glucagon secretion, resulting in one or more non-specific clinical features, such as necrolytic migratory erythema (NME), diabetes, diarrhea, deep vein thrombosis, weight loss, and depression. Here, we report the case of a 44-year-old male with a history of diabetes mellitus, presenting with a pruritic and painful disseminated cutaneous eruption of erythematous plaques, with scales and peripheral pustules, misdiagnosed as disseminated pustular psoriasis and treated for 2 years with oral retinoid and glucocorticoids. During this period, the patient complained of weight loss of 32 kg and diarrhea and developed deep vein thrombosis. These symptoms, together with an inadequate response to therapy of the skin lesions, led to the reassessment of the initial diagnosis. Laboratory tests confirmed elevated plasma glucagon levels (>1000 pg/mL) and computed tomography (CT) scans revealed a 35/44 mm tumor in the pancreatic tail. Due to considerable disease complications and the COVID-19 pandemic, the surgical removal of the tumor was delayed for nearly 2 years. During this time, somatostatin analogue therapy efficiently controlled the glucagonoma syndrome and likely prevented tumor progression. As in other functional pancreatic NETs, the early clinical recognition of hormonal hypersecretion syndrome and the multidisciplinary approach are the keys for best patient management.
ABSTRACT
Somatostatinomas are rare neuroendocrine tumors (NET) that arise in the gastrointestinal (GI) tract. Because of their insidious growth, they are usually asymptomatic until late stages, presenting as malignant disease. We report the case of a 50-year-old woman who presented with epigastric abdominal pain, diarrhea and significant weight loss in the last two years. On clinical examination the patient met the criteria for neurofibromatosis type 1 (NF1). Abdominal CT and MRI revealed an infiltrative duodenal mass, with pancreatic invasion, locoregional enlarged lymph nodes and disseminated hepatic nodules. Microscopy and immunohistochemistry uncovered a neuroendocrine tumor, staining positive for chromogranin A (CgA), synaptophysin and somatostatin, with a Ki67 = 1%. Somatostatin receptors (SSTRs) type 2 were negative and SSTRs type 5 were positive in less than 50% of tumoral cells. Our patient was classified as a T3N1M1 stage IV metastatic duodenal grade 1 somatostatinoma and treatment with somatostatin analogues and chemotherapy with capecitabine and temozolomide was started, with so far abdominal imaging follow-up showing stable disease. When a patient is diagnosed with a rare NET, such as a somatostatinoma, it is of utmost importance to determine if it is a sporadic tumor or just a feature of a genetic disorder.
ABSTRACT
Background: Post-surgical hypoparathyroidism (PoSH) is a common long-term complication after thyroid surgery. The reported median (range) incidence rates of temporary and permanent PoSH was 27% (19 - 38%) and 1% (0 - 3%) respectively. Material and Methods: We retrospectively analyzed the files of 552 patients who underwent thyroidectomy in our surgery department between 2015- 2017 with the aim to assess the prevalence of PoSH and to identify patient and disease related factors associated with postoperative hypocalcemia. Results: 171 (30.97%) patients developed PoSH, 88.37% transient, 11.63% permanent. The median (IQR) duration of postoperative hypocalcemia was 60 (67.5) days. Preoperative biological parameters were similar in PoSH and the control group, except median (IQR) serum magnesium level that was significantly higher in PoSH group [2.04 (0.17) vs. 1.89 (0.28) mg/dl, p=0.005]. In the subgroup of patients with thyroid carcinoma the surgery duration was longer in PoSH patients compared to the control group [135 (60) vs. 110 (43) minutes, p=0.020]. In patients with PoSH, median post-operative serum calcium was significantly higher in patients with reported difficult surgery [8.2 (0.2) vs. 7.9 (0.6) mg/dl, p=0.043] and the mean serum calcium decrease was higher in patients with cervical neck dissection and lymphadenectomy (1.94 +-0.59 vs. 1.68 +-0.56 mg/dl, p=0.033). Conclusions: Our data show a high prevalence of PoSH that is likely to increase given the rising number of thyroid surgeries being performed. Further research is needed in order to better define this condition, to establish appropriate treatment and preventive measures.
