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.

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.

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.

Objectives: In the last decades, thyroid cancer incidence has increased all over the world. This may be due to heightened medical surveillance and more addressability to surgery, as many cancers are diagnosed while under 1 cm. The aim of our study was to obtain data about the prevalence and histological subtypes of primary thyroid carcinoma in patients undergoing thyroidectomy. Materials and methods: We retrospectively analysed the files of 953 patients who underwent thyroidectomy in our surgery department between January 2012- December 2017. Anthropometric, biologic and imagistic data, indication of thyroid surgery, surgical procedures and pathology results were recorded. Results: 222 (23 .29%) patients had a diagnosis of primary thyroid carcinoma. The primary indications for thyroid surgery included: 5 (2.3%) Graves’ disease and nodular goiter, 181 (81.5%) multinodular goiter, 23 (10.4%) uninodular goiter and 13 (5.9%) thyroid cancer. The mean age at diagnosis was 52.49±13.79, range 20-83 years, but 61 (27.47%) were diagnosed before the age of 45 years. The female to male ratio was 180:42=4.28. The surgical procedure was lobectomy in 3 and total thyroidectomy in the remaining 219 patients. 207 (93.24%) suffered from differentiated thyroid carcinoma [194 (87.38%) papillary, 13 (5.85%) follicular], 7 (3.15%) from medullary thyroid carcinoma, 4 (1.8%) from poorly differentiated and 4 (1.8%) from anaplastic thyroid carcinoma. Multifocality was present in 62 (27.9%) patients. Pathological tumor stage was: T1 in 99 (44.6%), T2 in 38 (17.1%), T3 in 76 (34.2%) and T4 in 9 (4.1%) patients. 78 (35.1%) patients had tumors of 1 cm or less in diameter. 79 (35.6%) patients associated histopathologic chronic autoimmune thyroiditis. Conclusions: One in five patients who underwent thyroidectomy had a thyroid cancer. This might be due to a real increased incidence of thyroid cancer or just a result of better selection of patients for surgery and diagnose of microcarcinomas that otherwise would have gone unnoticed. Papillary thyroid carcinomas constituted the vast majority of these neoplasms, this being usually associated with an iodide-sufficient area.

Objectives: Post-surgical hypoparathyroidism (PoSH) is a common long-term complication after thyroid surgery. The reported median incidence rates of temporary and permanent PoSH was 27% and 1% respectively. Methods and results: We retrospectively analyzed the files of 552 patients who underwent thyroidectomy in our surgery department between 2015 and 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 [153 women (89.5%), median(IQR) age 49(22) years] developed PoSH (88.37% transient). The median(IQR) duration of postoperative hypocalcemia was 60 (67.5) days. Compared to 39 age and sex matched patients without PoSH, patients with PoSH presented the same prevalence of thyroid pathology that recommended thyroidectomy: multinodular goiter 55.6% vs 46.2%, Graves’ disease 12.9% vs 17.9%, thyroid carcinoma 31.6% vs 35.9% (P=0.52). Preoperative biological parameters (calcemia, PTH, 25-hydroxyvitamine D, phosphatemia, alkaline phosphatase, creatinine, TSH, FT4, TPOAb) were the same in both groups; yet, median(IQR) magnesemia was significantly higher in PoSH group [2.04(0.17) vs. 1.89(0.28) mg/dl, P=0.005]. Preoperative ultrasound characteristics, surgical protocols and pathological data were similar in the two groups. The patients with thyroid cancer that developed PoSH compared to controls had a longer median duration of thyroid surgery [135(60) vs. 110(43) min, P=0.02]. In patients with PoSH, median postoperative calcemia 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 calcemia 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.

Introduction: Cushing disease is the most common cause of endogenous hypercortisolemia and most frequently is caused by microadenomas, but rarely, only in 10% to 20% of cases, the adenomas are large enough to produce mass effect.¹ Case report: We present the case of a 63 years old female patient, diagnosed in 2007 with a 22/18 mm non-secreting pituitary adenoma and panhypopituitarism. She undergone transsphenoidal surgery twice, followed by gamma knife irradiation, resulting in progressive tumoral regression and empty sella until 2014, when a follow-up CT scan revealed a 15/13/11 mm tumoral recurrence, with rapid tumoral growth in the next couple of months to 27/24/17 mm and invasion in the adjacent structures. The third transsphenoidal surgery and the second gamma knife irradiation followed, without much tumoral mass reduction. IHC revealed and biochemistry confirmed an ACTH secreting aggressive pituitary macroadenoma (ACTH=70.2 pg/ml, 8 am serum cortisol after 1 mg dexamethasone suppression test=13.8 mg/dl, Ki67=25%). Cabergoline therapy was tried without benefit on cortisol secretion (ACTH=211.4 pg/ml, 24-hours urinary free cortisol=1965 nmol/24h, 24 pm salivary cortisol=18.75 nmol/l) or tumor mass which continued progressing to 31/35/34 mm and determined complications such as right eye blindness, right eyelid ptosis and severe trigeminal neuralgia. Pasireotide therapy was initiated but discontinued due to worsening of diabetes mellitus control, even though there were beneficial effects on cortisol secretion. In 2018 temozolomide therapy was decided. The clinical, imagistic and biological follow-up showed improvement on ACTH and cortisol secretion (ACTH=148.8 pg/ml, 8 AM serum cortisol=6.34 ug/ml and 24- hours urinary free cortisol=286.58 nmol/24h), improvement of trigeminal neuralgia, excellent control of the secondary diabetes mellitus and high blood pressure with minimal therapy and stable tumor mass (38/30/29 mm). After five cycles of therapy, temozolomide had to be, at least temporary, discontinued because the patient developed venous catheter related sepsis. Conclusions: ACTH secreting aggressive pituitary adenomas are rare, but due to their aggressive behaviour and high recurrence rate after surgery and radiotherapy, must be early diagnosed, appropriately treated and followed-up. Temozolomide is an effective therapeutic agent for aggressive pituitary adenomas, that showed significant improvement on clinical and paraclinical parameters and should be used when other treatment options fail. Bibliography: 1. Gandhi, C. and Post, K. (2019). ACTH-Secreting Adenomas. [online] Ncbi.nlm.nih.gov. Available at: https://www.ncbi.nlm.nih.gov/books/NBK12462/ [Accessed 3 Feb. 2019].