Multidisciplinary management of a large pheochromocytoma presenting with cardiogenic shock: a case report.

BACKGROUND: Pheochromocytoma is well-known for sudden initial presentations, particularly in younger patients. Hemodynamic instability may cause serious complications and delay a patient's ability to undergo surgical resection. Larger tumors present a further challenge because of the risk of catecholamine release during manipulations. In the case we present, increases in systemic vascular resistance caused cardiogenic shock, and the size of the lesion prompted surgeons to veer off from their usual approach. CASE PRESENTATION: A 38-year-old female patient was admitted to our intensive care unit with hypertension and later cardiogenic shock. Profound systolic dysfunction (left ventricular ejection fraction of 0.12) was noted together with severely increased systemic vascular resistance, and gradually responded to vasodilator infusion. A left-sided 11-cm adrenal mass was found with computed tomography and confirmed a pheochromocytoma with a meta-iodo-benzyl-guanidine scintigraphy. Surgical treatment was carefully planned by the endocrinologist, anesthesiologist and surgeon, and was ultimately successful. After prolonged hemodynamic stabilization, open adrenalectomy and nephrectomy were deemed safer because of lesion size and the apparent invasion of the kidney. Surgery was successful and the patient was discharged home 5 days after surgery. She is free from disease at almost 2 years from the initial event. CONCLUSIONS: Large, invasive pheochromocytoma can be safely and effectively managed with open resection in experienced hands, provided all efforts are made to achieve hemodynamic stabilization and to minimize. Catecholamine release before and during surgery.

Factors predicting prolonged glucocorticoid therapy in patients with adrenal insufficiency after laparoscopic adrenalectomy.

Introduction and importance: Adrenal insufficiency (AI) is common after adrenalectomy for Primary Adrenal Cushing's syndrome (PACS), due to the inhibition of the Hypothalamic-Pituitary-Adrenal Axis (HPAA) by the functioning adrenal mass. The treatment of post-surgical AI is based mainly on glucocorticoid supplementation therapy. To date, however, there is no known predicting factor of the duration of supplementation therapy in patients treated with laparoscopic adrenalectomy for PACS. Case presentation: We report the case of a 22-year-old Caucasian female who presented with dyspnea, osteoporosis, vertebral collapses and fractures of the pelvis. The diagnosis of ACTH-independent Cushing's syndrome was provided. Abdominal MRI revealed a left adrenal mass suggestive for adrenal adenoma, highly suggestive for PACS. The patient underwent left laparoscopic adrenalectomy. After surgery, glucorticoid supplementation therapy was started. More than A-year steroid replacement therapy was necessary before the patient completely recovered the function of the HPAA. During this period the patient was strictly followed up in order to adjust pharmacologic treatment, thus allowing to investigate the possible causes of such a slow and hard recover of the contralateral adrenal gland function. Conclusion: AI is common after adrenalectomy for PACS due to HPAA suppression. The duration of steroid replacement therapy may be vary depending on patient's characteristics and may be uncommonly long, as in our case. We concluded the not only cortisol and ACTH level, but also radiological findings, such as the size of the mass, its functional activity as well as the hypotrophy or atrophy of the contralateral adrenal gland may be predictive of the duration of the steroid therapy. These factors, if correctly studied before surgery, may be of help in tailoring the postoperative management of the patients after adrenalectomy.

Naloxone decreases the inhibitory effect of somatostatin on GH release induced by cigarette smoking in man.

To establish whether somatostatin (SRIH) exerts its inhibitory effect on the nicotine-induced release of GH by interacting with an opioid pathway, normal volunteers were treated with naloxone during (2 no-filter) cigarettes smoking and with SRIH. Nicotine significantly increased serum GH levels about 3.5 fold. Naloxone alone did not change GH rise induced by cigarette smoking. The stimulatory effect of GH by nicotine was completely blocked by SRIH. In the presence of both SRIH and naloxone, GH levels rose 1.5 fold in response to nicotine. Since naloxone only partially reversed the inhibiting action of SRIH, only a partial involvement of opioid peptides in SRIH action might be supposed. Alternatively, SRIH and naloxone-sensitive opiates might produce this inhibiting effect on GH rise in response to cigarette smoking through independent pathways.

Association between primary hyperparathyroidism and thyroid disease. Role of preoperative PTH.

BACKGROUND: The association between patients with surgically treatable thyroid disease and patients affected by PHPT is not just accidental. MATERIALS: We report 591 patients who underwent total thyroidectomy in our center. Data, collected during the preoperative period according to our protocol for candidates to total thyroidectomy, included: type of thyroid disease, sex, age, type of surgical procedure, preoperative PTH and plasmatic calcium level. Calcium plasmatic level has been monitored at 24 hours after surgery on day 6 and monthly for 6 months. RESULTS: On 591 cases, PTH above the normal range were present in 19.1% (113 patients), all asymptomatic for PHPT 30 were males (26.6%) and 83 females (73.4%), with a mean age of 62.97 +/- 12.51 years and 57.38 +/- 15.09 years ( p = 0.19). The mean preoperative PTH and calcium plasmatic level were 104.4 +/- 21.96 pg/ml and 119.7 +/- 37.93 pg/ml (p = 0.39) and 9.21 +/- 0.59 mg/dL e 9.37 +/- 0.87 mg/dl (p = 0.45) respectively. Intraoperative exploration proved a pathological parathyroid gland in 12 on 113 cases. In 9 of the 12 patients with parathyroid adenoma, hypocalcaemia developed. It resolved in 7 days for 4 patients and within 30 days for the others. No hypocalcaemia has been recorded at a 6 months follow up for the 97 considered (4 were lost at follow up). CONCLUSION: Preoperative PTH measurement for all patients undergoing total thyroidectomy may offer a concrete tool to screen and identify the above-described category of patients, with no additional cost for further radiological investigations, because this class of patients will be submitted to bilateral cervical exploration associated with a total thyroidectomy.

Is there a correlation between thyroiditis and thyroid cancer?

BACKGROUND: In the past few decades, the incidence of thyroid cancer has increased significantly all over the world. In the same period, there also seems to have been an increase in the incidence of Hashimoto's Thyroiditis-the most common inflammatory autoimmune thyroid disease. Several studies have linked thyroiditis to thyroid cancer. METHODS: In our study, we examined 2304 cases of thyroid surgery collected over a 12-year period starting from 2004. In 2090 cases (90.7%) out of our sample, it has been possible to compare the presence, or lack thereof, of thyroiditis by means of a histological diagnosis post-surgery; 214 (9.3%) cases were excluded from our study due to insufficient data. We then divided the different histological classifications into two groups. Group A included all the benign histological classifications and Group B included all the malignant histological classifications. In each group, we then assessed the presence, or lack thereof, of thyroiditis in order to evaluate if thyroiditis can be linked to a higher incidence of thyroid cancer. RESULTS: Data analysis showed a higher incidence of thyroiditis in Group B, 36.4% (malignant pathology report), than in Group A, 32.4% (benign pathology report), but no statistically significant difference emerged between those two groups (P > 0.05). CONCLUSIONS: Our conclusion was that a correlation between thyroiditis and a higher incidence of thyroid cancer is still undefined.

A challenging case of laparoscopic synchronous bilateral adrenalectomy for Cushing's disease. Case report.

INTRODUCTION: Laparoscopic adrenalectomy is a treatment option in patients with Cushing's syndrome. Preoperative comorbities as well as surgical and anesthesiological difficulties can make the procedure challenging. PRESENTATION OF THE CASE: We present the case of a 53-year-old obese man diagnosed with Cushing's syndrome, also suffering from other endocrine pathologies, neurofibromatosis type 2, cardiomiopathy with severe hypertrophy and diastolic dysfunction, deep vein thrombosis (DVT) and obstructive sleep apnea syndrome (OSAS).After multidisciplinary team discussion of the case, the patient underwent laparoscopic synchronous bilateral adrenalectomy. The laparoscopic approach was a part of a balanced enhanced recovery program which resulted in uneventful discharge in 4 days. CONCLUSION: Laparoscopic synchronous bilateral adrenalectomy is feasible and effective and should be considered also in patients with wide preoperative comorbidities and challenging intraoperative management, as long as the patient is meticulously studied preoperatively. An approach including a multidisciplinary team discussion is recommended.

The Schmidt Syndrome.

Addison's disease (AD) is a rare endocrine condition related to adrenal insufficiency. Autoimmune adrenalitis is commonly associated with autoimmune diseases. Autoimmune Addison's Disease (AAD) describes Autoimmune Polyendocrine Syndrome (APS) in 60% of patients with an important immunitary pathogenesis imprinting. We describes a case of Autoimmune Polyendocrine Syndrome charachterize by adrenal insufficiency and thyroid disease (Schmidt Syndrome). In this case report, Addison's disease had a slow onset in absence of the typical weight loss. In our considerations this is due to the concomitant hypothyroidism that masked some typical signs and also limited acute presentation.

A rare case of thyroid haemangiosarcoma.

The incidence of haemangiosarcoma in the literature is variable especially in the Alpine region and in Austria, ranging from 2 to 10% of all thyroid neoplastic lesions. This thyroid disease is characterised by positive endothelial markers (CD 31, CD 34 and FVIII), and co-positive markers for cytokeratins, epithelial membrane antigen and a loss of thyroglobulin can sometimes be found. Immunochemistry does not help the physician to classify the neoplasia as a variant of anaplastic carcinoma or sarcoma of endothelial origin. We present a case of epithelioid haemangiosarcoma in an elderly woman from outside the Alpine region with a contralateral papillary cancer treated by total thyroidectomy. The prognosis is poor and case reports are rare.

The surgical approach to nodule Thyr 3-4 after the 2.2014 NCCN and 2015 ATA guidelines.

BACKGROUND: The incidence of palpables thyroid nodules in general population is 5% and the prevalence of non -palpable nodules is higher (35-60%) in the endemic goiter area. In the last years the new guidelines and new classification related to thyroid nodule have changed the indication to treat it. MATERIAL AND METHOD: We analyzed the patients treated from January 2013 to June 2016 for Thyr 3 and Thyr 4 thyroid nodule sec. Bethesda system. We have divided in I and II period related to the 2.2014 and 2015 ATA guidelines and we have evaluated the indication to treat, the type of surgical procedure, the incidence of thyroid carcinoma and the adverse events. RESULTS: We selected from 909 cases, 252 cases surgically treated with preoperative diagnosis of Thyr 3(80 cases) and Thyr 4(172 cases); carcinoma was found in 21/80 (26.2%) and in 62/172 (26.05%). The period was divided from January 2013 to December 2014 and from January 2015 to june 2016 (first and second period). In II period we found carcinoma in 8/40 Thyr3 and in 26/88 Thyr 4. The incidence of lobectomy in II period was higher than I period (p < 0.0001) sec.guidelines indications. No difference in adverse events. The number of cancer is lower in patients treated with lobectomy than those who underwent total thyroidectomy (12,5%vs 21,8% in Thyr 3; 15,3% vs 32% in Thyr 4). CONCLUSIONS: The indications to treat related to Thyr 3 and Thyr4 are changed in the two periods. The number of cancer is lower in patients treated with lobectomy. The new guidelines have changed the surgical approach to thyroid nodule.

[Hypocalcemia after thyroidectomy: analysis on 804 treated patients]. / L'ipocalcemia post-tiroidectomia Analisi prospettica su 803 pazienti operati.

BACKGROUND: Hypocalcemia is a possible sequela of thyroidectomy; the causes are not fully understood. METHODS: We analyzed 804 patients (594 total thyroidectomy, 209 emithyroidectomy) treated in our Institute from January 1995 to December 2000. Serum calcium, ionized calcium, parathyroid hormone (PTH), fosforemia were screened pre- and postoperatively. RESULTS: Hypocalcemia, defined by a serum calcium less than 7.5 mg/dL, occurred in 126 patients (21.2%). In 90.6% of these patients the serum calcium was normal at seven days after thyroidectomy. In two patients we have registered at 180 days after thyroidectomy a permanent hypoparathyroidism. CONCLUSIONS: Several factors are important in the incidence of postthyroidectomy hypocalcemia but the inadvertent excision of parathyroid gland, ischemia and injury are the major causes of lowering of serum calcium concentration.

The nocturnal serum thyrotropin surge is inhibited in patients with adrenal incidentaloma.

BACKGROUND: Alterations in hypothalamic-pituitary function have been described in patients with incidentally discovered adrenal adenomas and have been attributed to their subtle hypercortisolemic status. METHODS: To establish whether the central control of the hypothalamic-pituitary-thyroid axis is altered in these endocrine conditions, the nocturnal (10:30 PM-2:00 AM) serum thyroid-stimulating hormone (TSH) surge (measured by dividing the difference between nighttime and morning TSH values by the morning TSH value and then multiplying by 100), the TSH response to thyrotropin-releasing hormone (200 microg as an intravenous bolus) and serum free thyroid hormone levels were evaluated in patients with adrenal incidentaloma (experimental group) and in normal controls (control group). Urinary free cortisol concentrations were also measured. RESULTS: The nocturnal TSH surge was observed in the normal controls, whereas it was inhibited in the patients of the experimental group. Serum free triiodothyronine levels were similar in the two groups, whereas the TSH response to thyrotropin-releasing hormone was significantly lower in the experimental than in the control group. Urinary free cortisol levels were significantly higher in the experimental group. CONCLUSION: These data indicate that even conditions of slight glucocorticoid excess may exert inhibitory effects on TSH secretion, which suggests the presence of a slight central hypothyroidism in patients with adrenal incidentaloma.

Are we changing our inclusion criteria for the minimally invasive videoassisted thyroidectomy?

AIM: The first cases treated with Minimally Invasive videoassisted thyroidectomy (MIVAT) were characterized by inclusion and exclusion criteria that are changing with the experience. MATERIALS AND METHODS: We have analyzed the patients treated from july 2005 to december 2010 with MIVAT All these cases were treated in accord with Miccoli's technique with a minicervicotomy of 1.5-2 cm above the sternal notch. We have divided the cases on the surgical period highlighting changing in the inclusion criteria and the adverse events (0-211 cases; 212-300 cases). All the cases treated were followed up at days 7 (ambulatory visit) and days 30-12 months (ambulatory visit or telephone contatct). The patients classified in the II° period were chracterized by the exclusion of the clinical thyroiditis. We have related these cases with the cases treated with conventional thyroidectomy(CT). RESULTS: No differences in postoperative pain,nerve palsy and hypocalcemia in MIVAT group and CT group. We have registered a postoperative pain at 24 hours lower in MIVAT group. The percentage of transitory nerve palsy in the MIVAT group in the first period was 2,84 % versus 1,12 % in the second period. CONCLUSION: MIVAT technique is safe and reproducible, with an excellent cosmetic results. In our experience MIVAT remains the better surgical options for the patients that meet the inclusion criteria.These cases are 15% of patients treated with thyroidectomy in our Endocrine surgery Unit.

Oxytocin does not modify GH, ACTH, cortisol and prolactin responses to Ghrelin in normal men.

BACKGROUND: In order to test the possible effect of Oxytocin (OT) on Ghrelin-stimulated GH, PRL, ACTH and cortisol, ten healthy normal men were studied. TESTS: Ghrelin (0.2 µg/kg body weight (BW)) as an iv bolus; Ghrelin plus OT (2 IU as bolus plus 0.07 IU/min administered for 90 min). RESULTS: The administration of OT did not change GH, PRL, ACTH and cortisol release induced by Ghrelin. CONCLUSIONS: The data suggests that in humans OT did not modulate the GH, PRL, ACTH and cortisol response to Ghrelin.

Increased arginine-vasopressin response to hypertonic stimulation and upright posture in idiopathic hyperprolactinemia.

To evaluate the possible influence of idiopathic hyperprolactinemia on the arginine-vasopressin (AVP) response to osmotic and pressure-volumetric stimuli, 14 idiopathic hyperprolactinemic women and 13 normoprolactinemic women were studied during a hypertonic saline infusion test (0.51M NaCl infusion for 2h) and an orthostatic test (standing upright and maintaining an orthostatic position for 20min). In both experimental conditions, the AVP response was significantly higher in women with idiopathic hyperprolactinemia than in normal normoprolactinemic women. These results indicate that in women hyperprolactinemia influences the AVP response to hyperosmotic and hypovolemic stimuli.

Glucoreceptors located in the brain mediate NPY release induced by hypoglycemia in normal men.

The NPY secretory pattern after an insulin tolerance test (ITT) (0.15 IU/kg body weight) was evaluated in 8 normal men. They were infused with normal saline (control test), glucose or fructose. Insulin-induced hypoglycemia produced a significant increment in serum NPY in the control test. The infusion of fructose was unable to change the NPY secretory pattern during insulin-induced hypoglycemia. In contrast, the NPY increase during ITT was completely abolished when the concomitant infusion of glucose prevented insulin-induced hypoglycemia. These results exclude a direct role of hyperinsulinemia in the mechanism underlying the stimulation of NPY secretion during ITT. Furthermore, since glucose but not fructose crosses the blood-brain-barrier (BBB), the NPY increase during ITT appears to be generated by low glucose concentrations at the level of glucosensitive areas located inside the brain.