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Abstract Background Diagnosis of multiple endocrine neoplasia type 1 (MEN1) is commonly based on clinical criteria, and confirmed by genetic testing. In patients without known MEN1-related germline mutations, the possibility of a casual association between two or more endocrine tumors cannot be excluded and subsequent management may be difficult to plan. We describe a very uncommon case of functioning glucagonoma associated with primary hyperparathyroidism (pHPT) in which genetic testing failed to detect germline mutations of MEN-1 and other known genes responsible for MEN1. Case presentation The patient, a 65-year old woman, had been suffering for more than 1 year from weakness, progressive weight loss, angular cheilitis, glossitis and, more recently, skin rashes on the perineum, perioral skin and groin folds. After multidisciplinary investigations, functioning glucagonoma and asymptomatic pHPT were diagnosed and, since family history was negative, sporadic MEN1 was suspected. However, genetic testing revealed neither MEN-1 nor other gene mutations responsible for rarer cases of MEN1 (CDKN1B/p27 and other cyclin-dependent kinase inhibitor genes CDKN1A/p15, CDKN2C/p18, CDKN2B/p21). The patient underwent distal splenopancreatectomy and at the 4-month follow-up she showed complete remission of symptoms. Six months later, a thyroid nodule, suspected to be a malignant neoplasia, and two hyperfunctioning parathyroid glands were detected respectively by ultrasound with fine needle aspiration cytology and 99mTc-sestamibi scan with SPECT acquisition. Total thyroidectomy was performed, whereas selective parathyroidectomy was preferred to a more extensive procedure because the diagnosis of MEN1 was not supported by genetic analysis and intraoperative intact parathyroid hormone had revealed “adenoma-like” kinetics after the second parathyroid resection. Thirty-nine and 25 months after respectively the first and the second operation, the patient is well and shows no signs or symptoms of recurrence. Conclusions Despite well-defined diagnostic criteria and guidelines, diagnosis of MEN1 can still be challenging. When diagnosis is doubtful, appropriate management may be difficult to establish.

Background: Casual detection of an adrenal mass, the so called incidentaloma (AI) requires an in-depth analysis of imaging phenotype together with hormonal investigation, in order to evaluate both its potential malignancy and the occurrence of a preclinical condition of hypercortisolism (Subclinical Cushing Syndrome, SCS). Aim of the present work is to evaluate surgical indications and results of surgery in patients harbouring an AI with inapparent hypercortisolism., Methods: The study has been carried on in a series of 26 Laparoscopic Adrenalectomies (LA) performed from January 2009 and January 2015. Indications to surgery included AI (11 cases), Cushing's syndrome (7 cases), suspected metastases (5 cases) and Conn's disease (3 cases). Six patients with AI had a SCS associated with variable forms of a metabolic syndrome: they were evaluated in detail analysing cortisol secretion and values of Arterial Hypertension, Diabetes Mellitus and BMI before and after surgery., Results: As far as SCS is concerned, LA was completed in 5 patients (one case converted). Pathology revealed 5 adenomas and one nodular hyperplasia. Four cases required oral cortisone administration at the discharge. At a mean follow- up of 33 months cortisol secretion returned to normal range in all patients; an improvement of metabolic condition was observed in 60, 25, and 50 per cent of hypertensive, diabetic and obese patients respectively., Conclusion: Indications to LA in case of AI and SCS is strongly supported by the presence of an associated metabolic syndrome. In spite of a limited number, our experience confirms the favourable results of surgery in such patients., Key Words: Adrenal incidentaloma, Laparoscopic adrenalectomies, Subclinical Cushing syndrome.

Introduction: Congenital duplication of the inferior vena cava is frequently associated with other vascular anomalies of its venous tributaries. Awareness of such occurrence facilitates laparoscopic surgery and avoids inadvertent vascular injuries., Case Report: An adrenal mass of increasing size was discovered in a 39 years old lady previously submitted to restorative proctocolectomy for Familial Polyposis Coli. Since the first preoperative work up, Computerized Tomography showed a duplicated inferior vena cava as well as other visceral and vascular anomalies. During laparoscopic adrenalectomy a double adrenal vein was discovered: the first one draining normally into the vena cava and the second one into the righ renal vein. Both of them were clipped and divided and surgical outcome was succesful., Discussion: The progress in cross-sectional imaging made easily recognisable congenital anomalies of the inferior vena cava in patients otherwise asymptomatic. Its occurrence has been evaluated through previous reports on venous anomalies during adrenal and renal surgery as well as through angiographic studies. Knowledge of these anomalies is very important for interventional radiologists, urologists and for general surgeons. However when facing adrenal surgery the operator should be aware that a double vein can be found in up to 10% of the cases and such occurrence is more predictable in case of pheochromocytoma and of large adrenal mass. Surgeons should rely both on preoperative dignostic imaging and careful dissection through laparoscopic magnified view to avoid harmful bleeding complications.

The aim of our study was to evaluate the quality of life (QoL) of a group of patients who underwent tension-free inguinal hernia repair in light of the possible medicolegal implications. We conducted a retrospective study on the QoL of patients who underwent inguinal hernioplasty. One hundred and fifty patients who answered the SF-36 questionnaire were included in the study. Twenty-six (17.3%) had chronic pain, 18 (12%), sensory deficits, and 106 (70.6%) did not complain of any symptoms. There was no a significant deterioration in QoL compared to the reference population. In the group with chronic pain, 10 patients (38.4%) reported being very limited in performing "physically demanding activities"; 18 (69.2%) reported a deficit of performance in activities of daily living; 8 (30.7%) complained of a reduction of time spent at work because of emotional disorders. In one case the pain was particularly severe and required surgical treatment. The answers obtained through the questionnaire show that tension-free hernioplasty does not degrade patients'QoL. However in the group of patients with chronic pain examined one year after surgery, QoL had deteriorated so much that it affected the employment sphere and the social and relational sphere. Although there is a relatively low incidence and frequency of problems relating to chronic pain following inguinal hernioplasty, chronic pain can sometimes have serious effects on QoL and socio-economic and legal implications.

Design: To evaluate the efficacy of the main tools in the diagnostic localization of hyperfunctioning parathyroids (HP) in primary hyperparathyroidism (pHPT) with concomitant thyroid diseases., Methods: Forty-three patients with pHPT associated with nodular goiter (NG, n=32) and/or autoimmune thyroid diseases (AITDs, n=11) for a total of 63 neck lesions were considered. Sixteen patients displaying HP (16 lesions), unequivocally localized by sestaMIBI scintigraphy (MIBI) and neck ultrasound (US) (group I), were compared with 27 patients (47 neck lesions) displaying equivocal parathyroid localization (group II). In all cases, neck US, MIBI scan, cytology, and parathyroid hormone assay in fine-needle aspiration washout fluid (FNA-PTH) were performed. All patients finally underwent surgery., Results: According to histological examination, high FNA-PTH values (>103 pg/ml) correctly identified all HP in both groups of patients (100% of sensitivity and specificity). Both MIBI and US correctly identified all HP only in group I patients; in contrast, four patterns of mismatch between these techniques were observed in group II patients, leading to low diagnostic performances of neck US (71.4% sensitivity and 78.9% specificity) and of MIBI scan (35.7% sensitivity and 42.1% specificity). The latter was due to both false-negative (mainly in AITD) and false-positive (mainly in NG) scan images., Conclusions: Coexistent thyroid diseases are responsible for mismatch between MIBI and US images resulting in equivocal HP localization. In these cases, FNA-PTH resulted in the most accurate tool to identify HP. However, although safe, it should be advised only to patients with uncertain HP localization.

"Forgotten" goiter is an extremely rare disease which is defined as a mediastinal thyroid mass found after total thyroidectomy. The authors report their experience of 7 cases of residual mediastinal goiter after total thyroidectomy for substernal goiter. Six patients were female and one male with a median age of 56 years (range: 25-70 years). Four patients were asymptomatic, 2 patients showed signs of mediastinal compression, and one patient had persistent hyperthyroidism. A sternal splitting incision associated with a cervical incision was required in 3 patients while an exclusively cervical incision was sufficient in 4 patients. Histology did not show neoplasia. Postoperative outcome was uncomplicated in 6 patients and in one patient a transient recurrent laryngeal nerve paralysis occurred. In the majority of cases forgotten goiter is the consequence of the incomplete removal of a plunging goiter, although sometimes it may be attributed to a concomitant, unrecognized mediastinal goiter which is not connected to the thyroid. The residual goiter has the same clinical presentation as an ordinary intrathoracic goiter. Our experience confirms that surgical treatment of forgotten goiter is associated with only minimal morbidity although a sternal split is sometimes required. Forgotten goiter is a rare pathology which can be prevented if particular attention is paid to preoperative imaging and intraoperative management during the first operation. However surgical treatment for forgotten goiter, when performed in specialized centers, is associated with low morbidity.

Background: Exclusive abdominal nodal involvement due to Hodgkin or non Hodgkin lymphoma (HL,NHL) is a rare condition, but its occurrence requires a good enough specimen for diagnosis and therapy. Aim of this work is to evaluate the results of laparoscopic lymph node biopsy (LLNB) in a series of patients where a primitive or relapsing lymphoma was suspected., Methods: This study has been carried on the patients submitted to LLNB from 2007 to 2011. Cases records were retrospectively reviewed for age, sex, diagnostic work up, previous biopsy, conversion to laparotomy, pathologic diagnosis and perioperative complications., Results: Eleven cases were evaluated including 4 males and 7 females; mean age was 56.5 years. All patients underwent Ultrasound (US) and CT scan. A previous US or CT guided biopsy was performed in 5 cases, but in all of them failed to obtain a diagnosis. LLNB was successfully performed in 8 cases and required a conversion in three. Causes of conversion were obesity in one case and previous surgery in two. Results of pathology were NHL 5 cases, HL 2, follicular hyperplasia 2, sarcoidosis and tuberculosis 1 case respectively. No mortality nor morbidity occurred. Mean hospital stay was of 2.7 days., Conclusion: LLNB proved to be a safe procedure with no perioperative complications. Laparoscopy however should not be undertaken if superficial lymphadenopathy is present. A thorough CT evaluation of abdominal nodal involvement by the surgical team is mandatory prior to laparoscopy in order to plan the surgical approach and the route to reach the node.

Endometriosis involving the rectus abdominis muscle is very rare; until now, only 19 such cases have been reported in the medical literature since it was first described in 1984 by Amato and Levitt; almost all were associated with previous abdominal surgery such as cesarean section or other operations. We report two additional cases of this very rare condition presenting with an abdominal mass which was surgically excised with an accompanying margin of normal tissue. Both patients are well and without recurrence. Endometriosis pain has generally been described as cyclical and this condition usually develops in an old surgical scar. Endometriosis has no pathognomonic imaging findings on CT, MRI or sonography, as its appearance depends on the phase of the menstrual cycle, the proportion of stromal and glandular elements, the amount of bleeding and the degree of surrounding inflammatory and fibrotic response. Surgery is the treatment of choice including 5-10 mm of surrounding healthy tissue as surgical margin, to prevent recurrence. Our experience is in agreement with the data of the literature. We suggest that endometriosis must be included in the differential diagnosis of a symptomatic mass in the abdominal wall in women with and without a surgical history.