Tips to facilitate a preoperative diagnosis of adrenal ganglioneuroma. Report of a challenging experience and review of the literature.

AIM: Ganglioneuroma (GN) is the most uncommon and the most benign tumor among neuroblastic neoplasms, and in 29.7% of cases it finds in an adrenal gland. Usually asymptomatic, this tumor is detected incidentally in the majority of cases. It is generally challenging to obtain a precise diagnosis of adrenal ganglioneuroma (AGN) before surgery. Misdiagnosis rate of AGN on CT and MRI is 64.7% and clinicians and surgeons are often lacking in knowledge of this rare disease. For this reason, we pointed out the clinical, biochemical, radiologic and pathologic features of AGN in an our experience, with the aim to find out if there are some features able to facilitate a preoperative diagnosis. The present article also includes a review of the relevant literature in order to compare laparoscopic versus open adrenalectomy. CASE REPORT: Right AGN in a 42-year-old woman, in whom preoperative diagnosis was very difficult and only histopathological studies of the surgical specimen established the exact diagnosis. The patient underwent bilateral subcostal laparotomy for a large mass (sized measuring 14.5 x 11.6 x 6.5 cm.) and a right adrenalectomy was performed. Postoperative recovery was uneventful and the patient, at 12-months follow-up, is disease-free and in good health. DISCUSSION / CONCLUSIONS: Authors stress the importance of interdisciplinary collaboration between surgeons, radiologists and endocrinologists to optimize clinical management and surgical indications. Careful evaluation by endocrine examinations and multiple imaging procedures are necessary to provide a differential diagnosis. Surgeons should consider a diagnosis of AGN in case of: 1) an adrenal incidentaloma; 2) a nonfunctioning tumor with no elevated hormonal secretions, in which compressive symptoms may occur; 3) a homogeneous, encapsulated mass, with well-defined edges, without invasion of nearby structures (no vascular involvement), with presence of calcifications and nonenhanced attenuation of <40 HU on CT; 4) a homogeneous hypointense adrenal mass on T1-weighted MRI, a heterogeneous hyperintense mass on T2-weighted MRI and a poor delayed enhancement on dynamic MRI; a SUV level on PET less than 3.0. Nevertheless, diagnosis of AGN can be extremely challenging and can only be achieved by means of histology. Treatment is complete surgical resection without the need for chemotherapy or radiotherapy. Laparoscopic adrenalectomy is contraindicated in the presence of local infiltration or tumor greater than 12 cm. Even if AGN has an excellent prognosis and recurrences are rare after surgical resections, long-term follow-up is recommended. KEY WORDS: Adrenal gland, Adrenal ganglioneuroma, Laparoscopic adrenalectomy, Open adrenalectomy.

Assessment of guidelines to improve diagnosis and treatment of solid pseudopapillary tumor of the pancreas. A case report and literature review.

AIM: Solid pseudopapillary tumor (SPT) of the pancreas is a rare neoplasm, its preoperative diagnosis is difficult and therefore inappropriate therapy or postoperative complications are frequent. Reviewing the literature, the purpose of this article was to identify guidelines to improve diagnosis and treatment of SPT. CASE REPORT: Authors report a case of SPT of the pancreas in a 27-year-old woman in whom a mistaken radiologic diagnosis made surgical strategy difficult and caused postoperative complications. DISCUSSION/CONCLUSIONS: Clinicians and surgeons should: (1) consider the possible disease of SPT in young females, with pancreatic encapsulated lesion with well-defined borders and variable central areas of cystic degeneration, necrosis or hemorrhage showed on radiological examinations. (2) Intensity of the differentiation of the clinical symptoms, especially during the course of therapy of chronic gastritis and diabetes. (3) Use immunohistochemical stains of alpha-1-antitrypsin, alpha-1-antichymotrypsin, vimentin and neuron-specific enolase. (4) Keep this unusual but potentially curable tumor in mind, following patients who had suffered from acute pancreatitis or abdominal injury. Increasing experience with this tumor leads to a greater awareness of its clinical presentation and pathological features and a lower rate of misdiagnosis. (5) Finally, perform, where technically feasible, conservative surgical treatment, that is safe and effective.

[Surgical treatment of extraperitoneal rectal cancer]. / Trattamento chirurgico del carcinoma del retto extraperitoneale. Chirurgia "open" con conservazione sfinterica, TME nerve-sparing, fast-track, senza stomia di protezione.

The aim of the study was to evaluate the results of open surgery with sphincter preservation and nerve-sparing total mesorectal excision and a fast-track protocol, without a protective stoma in a consecutive series of patients with extraperitoneal rectal cancer. From 1998 to 2007, 89 patients with extraperitoneal rectal cancer were treated according to a prospective protocol. Eight-six patients were submitted to anterior resection with a low or ultra-low anastomosis and nerve-sparing total mesorectal excision. Fifty-four patients received neoadjuvant therapy. Twenty-eight patients were treated according to a fast-track postoperative protocol. Primary protective colostomies were performed in 6 cases (6.9%), while a secondary colostomy was necessary in 3 patients (3.4%). There was just one postoperative death (1.1%) and major morbidity occurred in 12.3%. Seven patients developed anastomotic dehiscence; 3 were successfully treated with a secondary colostomy and 4 were treated conservatively. 68.4% of the patients treated with the fast-track protocol could be discharged on postoperative day 4. 73% of patients were still surviving at a 5-year follow-up (48 patients). The incidence of local recurrences was 3.1%. Anterior resection in the form of open nerve-sparing total mesorectal excision with selective use of neoadjuvant therapy can be successfully performed without a protective stoma in more than 80% of patients. The fast-track protocol seems to increase the quality of the patient's postoperative condition and reduce the hospital stay.

Protective stoma in anterior resection of the rectum: when, how and why?

BACKGROUND: The use of protective stoma in anterior resection (AR) is controversial. Neoadjuvant therapy, TME and laparoscopy seem to increase the rate of anastomotic dehiscences (a.d.). PATIENTS AND METHODS: In a prospective study, 219 patients were submitted to elective open AR (109 patients), open AR+TME nerve-sparing (110 patients), 35 of which had intrasphinteric anastomosis. Fifty-five patients were treated by neoadjuvant therapy. Primary stoma was not performed. RESULTS: We had 15 (6.8%) a.d.: 5 (2.3%) major and 10 (4.4%) minor. In the five major a.d. an immediate colostomy was performed with one death. In the 10 minor the a.d. was cured conservatively. CONCLUSIONS: A protective stoma is necessary in less than 10% of the patients treated with AR, so avoiding further surgery, mortality, morbidity and higher medical costs in most patients.

[Colorectal cancer surgery. Analysis of risk factors in relation to incidence of morbidity and mortality]. / Chirurgia oncologica colo-rettale. Analisi dei fattori di rischio nell'incidenza di morbi-mortalità.

The aim of the study was to investigate risk factors in relation to the incidence of morbidity and mortality in surgery for colorectal cancer. Between 1986-2005, 328 patients underwent colorectal cancer surgery, 308 of whom (93.9%) in elective and 20 (6.1%) in emergency surgery. Radical resection was performed in 276 (84.2%) and palliative surgery in 52 (15.8%) patients. Bivariate statistical analysis was used for morbidity and mortality factors and multivariate analysis was performed in order to find independent variables (age, gender, ASA grade, elective or emergency surgery, tumour excision, cancer stage according to Dukes) associated with dependent variable interactions. Differences were considered statistically significant for p values < 0.05. The incidences of mortality and morbidity were 0.91% and 20.1%, respectively. In our study we observed a leakage incidence of 2.74% (9/328). In emergency surgery we found morbidity and mortality rates of 20% and 10%, respectively. Age and advanced cancer stage influenced results but were not found to be statistically significant. 18.3% of patients (60/328) were ASA I, 32% (105/328) ASA II, 39.6% (130/328) ASA III and 10.1% (33/328) ASA IV. Among the independent variables observed in the multivariate analysis, ASA grade was found to be the only positive predictive factor correlated with morbidity. Logistic regression showed an exponential increase in operative risk: odds ratio (OR) 2.9 in ASA I vs ASA II, OR 4.2 in ASA I vs ASA Ill, OR 10.3 in ASA I vs ASA IV (95% confidence interval). As regards the mortality rate, none of the independent variables were found to be statistically significant risk factors (p < 0.05).

[Extrauterine endometriosis: what interest for the general surgeon? Presentation of 3 clinical cases and review of the literature]. / Endometriosi extrauterina: quale interesse per il chirurgo generale? Presentazione di 3 casi clinici e revisione della letteratura.

Extragonadal endometriosis is rarely diagnosed preoperatively for the variety of its localizations. Presentations to general surgeons may be atypical and pose diagnostic difficulty, mimicking other acute diseases. We report three cases treated with surgical operation. Case 1: a 28-year-old woman admitted for bowel obstruction due to coecal endometriosis, with appendix mucocele, peritoneal pseudomyxoma and ovarian endometrioma. The patient underwent right colectomy and right adnexectomy in the emergency setting. Case 2: a 31-year-old woman with endometriosis of the distal extraperitoneal portion of the round ligament presenting as an irreducible inguinal hernia. An operation was performed: the round ligament and a polycystic structure encompassing it were completely excised. Case 3: a 41-year-old woman, with umbilical endometriosis diagnosed by her gynaecologist, was admitted to our department for excision. Surgical treatment of extragonadal endometriosis is adequate. However, postoperative follow-up is mandatory and hormonal suppressive therapy may be indicated by the gynaecologist.

[Severity of anemia and operative morbidity and mortality. Report on 3 clinical cases in Jehovah's Witnesses and review of the literature]. / Morbi-mortalità operatoria relativa alla severità dell'anemia acuta. Presentazione di 3 casi clinici complicati in pazienti Testimoni di Geova e revisione della letteratura.

It has been reported that patients who refuse blood transfusions, such as Jehovah's witnesses, can undergo major surgery. In a review of the literature, however, we critically examined the severity of anaemia in relation to operative mortality and morbidity rates. We report three cases of Jehovah's witnesses who underwent major surgery and presented complication during the postoperative period. Case 1: a 50-year-old man with oesophageal achalasia who underwent Heller's myotomy and Nissen's fundoplication. The postoperative period was complicated by massive haemorrhage and the patient was reoperated on postoperative day 1. After four years, he underwent total oesophagectomy because of severe chronic oesophagitis. On postoperative day 13 the patient suffered anteroseptal myocardial ischaemia, which was treated with medical therapy. Case 2: a 40-year-old man, admitted for ulcerative rectocolitis, who underwent total colectomy. On postoperative day 1 he presented massive haemorrhage and shock. He was reoperated and the postoperative period was complicated by myocardial ischaemia, renal failure and an enterocutaneous fistula. Case 3: a 65-year-old woman with ulcerative rectocolitis who underwent total colectomy and a temporary ileostomy. She suffered venous thrombosis of the lower limbs and pulmonary oedema. The patient died 14 months after surgery as a result of massive haemolysis by cryoagglutinins and cardiac arrest.