Metastatic retroperitoneal tumor from a non-functional neuroendocrine neoplasia of the left ethmoid-nose-orbitary region: Case report and short review of literature.

INTRODUCTION: NeuroEndocrine Neoplasms (NENs) are rare and can originate from any epithelial organ. We describe a very rare case of retroperitoneal metastasis from a non-functioning neuroendocrine tumor of the ethmoid region and focus on the various issues related to NENs, from their nosological classification up to the most recent acquisitions in the diagnostic and therapeutic field. PRESENTATION OF CASE: A woman presented with a mass in the right retroperitoneal area that infiltrated liver and kidney. The anamnestic data showed a previous undifferentiated small cell tumor of the left ethmoid-nasal-orbital region. The mass was removed surgically and the definitive histological examination revealed a non-functioning undifferentiated malignant small cell neoplasm. DISCUSSION: NENs represent a chapter of oncology whose systematization remains difficult. The lack of hormonal syndrome in Biologically Inactive Neuroendocrine Tumors (BINTs) may delay the diagnosis. Clinical manifestations relate to the size and location of the neoplasm. Small cell NeuroEndocrine Carcinoma (NEC) of the ethmoid-nose-orbital region is an extremely rare occurrence and generally the most frequent metastatic localization is represented by the larynx. CONCLUSION: This case highlights the contradictions typical of NENs and only new scientific acquisitions in the histopathological field can help us in the future. Surgery remains the only possibility of treatment and diagnosis of large masses of inactive NENs. This is the only case in the world of metastasis from NEC of the nose-orbital region in the retroperitoneum.

Sclerosing angiomatoid nodular transformation (SANT) of spleen mimicking a splenic abscess: Case report and review of the literature.

INTRODUCTION: SANT of the spleen is a benign lesion that does not recur after splenectomy with unknown etiology. PRESENTATION OF THE CASE: We report a case of sclerosing angiomatoid nodular transformation (SANT) of the spleen mimicking a splenic abscess in a patient with imaging studies that failed to differentiate this lesion from other splenic lesions. DISCUSSION: SANT of the spleen is a rare condition, with only less than 100 cases reported in the literature. Clinically, SANT is only a kind of described pathological diagnostic conception. As in our case, these splenic lesions are often incidental findings on imaging studies performed for other reasons. CONCLUSION: The patient was treated with splenectomy, which has proved to be both diagnostic and therapeutic. Although histology can lead to the diagnosis of vascular tumor, immunohistochemistry is the only way to confirm the diagnosis.

Adenocarcinoma below stapled ileoanal anastomosis after restorative proctocolectomy for ulcerative colitis.

A case of adenocarcinoma arising in a 39-year-old patient after restorative proctocolectomy is reported. The patient underwent an ileal pouch-anal anastomosis with double-stapled technique for severe ulcerative colitis 18 years earlier, without evidence of associated neoplasm or dysplasia in operative specimen. After endoscopic diagnosis of adenocarcinoma, the patient was submitted to excision of the pouch and permanent ileostomy, followed by combined radiotherapy and chemotherapy. Pathology showed an AJCC stage III moderately differentiated mucinous adenocarcinoma. The patient died 24 months after the operation, due to cancer progression. There are 50 reported cases in the indexed medical literature of carcinoma arisen after ileal pouch-anal anastomosis for ulcerative colitis. Twenty-five out of these arose after mucosectomy and hand-sewn anastomosis, and 25 after stapling technique. Furthermore, in 48% of the patients, dysplasia or cancer was already present at the time of the colectomy. The increase of reported cases suggests a routine long-term endoscopic surveillance in patients with long-standing ileal pouches, especially in presence of dysplasia or cancer in the proctocolectomy specimen.

Biliodigestive anastomosis with circular mechanical device after pancreatoduodenectomy: our experience.

The authors describe the technique to perform a mechanical biliodigestive anastomosis after pancreatoduodenectomy that could be able to reduce the time of surgical intervention and the major systemic postoperative complication especially in elderly patients. Pancreaticoduodenectomy (PD) is considered to be the most effective procedure for the treatment of tumors of the pancreatic head and periampullary tumors. Postoperative morbidity remains high, reaching 40-50% in some series. Leakage and stricture of hepaticojejunal anastomosis have a special significance and occur in 2.5-5% of cases. The usefulness of mechanical staplers is well established in gastric and colorectal surgery, but their use in creating biliodigestive anastomosis is still a very controversial application. In the last 2 years (Nov 2008-Nov 2010), seven patients who underwent PD and presented at the time of operation, a main bile duct (MBD) greater of 20 mm, received a mechanical biliodigestive anastomosis with surgical stapler. All the patients were postoperatively controlled with an ultrasonography at 3 and 6 months, and a cholangio MRI at 1 year from operation. The follow up of the seven patients ranged from 3 to 18 months. Six patients are still alive and free of oncologic disease. One patient presented a single episode of cholangitis with a significant reduction of caliber of the biliodigestive anastomosis. The remaining five patients showed a good caliber of hepaticojejunal anastomosis. The authors suggest this procedure as a safe, feasible, more rapid and easier technique than traditional suture for bilioenteric anastomosis, when a suitable caliber of MBD allows to perform it.

[Role of anastomotic configuration in ileocolic resection for Crohn's disease]. / Ruolo della configurazione anastomotica nella resezione ileocolica per malattia di Crohn.

Crohn's disease is characterised by a high incidence of perianastomotic recurrence after ileocolic resection. The influence of the anastomotic configuration on the incidence of reoperation was evaluated in patients undergoing resection for Crohn's disease. In our Institution, from 1993 to 2007, 308 consecutive patients affected by ileocolic Crohn's disease were submitted to 343 ileocolic resections or right colectomies. In 292 cases (85.1%), an antiperistaltic side-to-side (or functional end-to-end) anastomosis was performed, with an 80 mm linear stapler in 190 cases, a 100 mm linear stapler in 79, and a hand-sewn anastomosis in 23. The other hand-sewn anastomotic configurations were: 30 (8.8%) side-to-side isoperistaltic, 15 (4.3%) end-to-side and 6 (1.8%) end-to-end. The overall morbidity was 7.3%, with two postoperative deaths (0.6%) with no significant differences between groups. There were 38 overall recurrences (11%). In the side-to-side antiperistaltic group, the rate of recurrence was 8.2%, significantly lower than the recurrence rates observed in the other anastomoses (26.9%, p = 0.002), especially side-to-side isoperistaltic anastomosis (33.3%, p = 0.001). Early recurrences (< 1-year) were 2.6%, without significant differences between groups. There was a higher trend for end-to-end anastomosis (16.6%). In the side-to-side antiperistaltic group the morbidity was higher in the large mechanical anastomoses (100 mm length), but the recurrence rate was lower in this group as compared to the 80 mm anastomoses (1.2% vs. 12.1%, p = 0.006). Our non-randomised study suggests a better trend for the wide side-to-side antiperistaltic technique in terms of recurrence rate. These observations need further investigation with randomised controlled trials to compare the different anastomotic procedures.

[Perforated jejunal diverticulum: a rare cause of acute abdomen]. / Il diverticolo digiunale perforato: una rara causa di addome acuto.

Jejunal diverticulum is an uncommon, acquired condition, and the majority of patients are usually asymptomatic. Jejunal diverticula become clinically relevant when complications, such as diverticulitis, intestinal bleeding, obstruction, or perforation occur. A rare case of acute abdomen due to a perforated jejunal diverticulum is presented. The diagnosis was initially suggested by CT and confirmed intraoperatively when a 74-year-old male patient underwent a segmental jejunal resection with primary anastomosis. A review of the literature indicates the rarity of this condition, and therefore the aetiology, pathogenesis, diagnosis, and management are briefly discussed. An early diagnosis, based on ultrasonography and CT, and consequently prompt resection of the jejunum affected are the keys to a successful outcome. Because a longer duration of symptoms before operation correlates with a worse prognosis, the possibility of a clinical diagnosis of perforated jejunal diverticulum should be entertained as part of any evaluation of acute abdomen, especially in the elderly.