Giant biliary mucinous cystadenoma of the liver.

Biliary mucinous cystadenomas (BMC) of the liver are rare benign cystic tumors, however an estimated 20% undergo malignant transformation. They have recently been redefined as mucinous cystic neoplasms in the 2010 WHO classification. The preferred treatment is through radical resection, as there are high recurrence rates with other treatment modalities; however this is often not possible in patients with bilobar or giant cysts, and liver transplantation may be indicated. We present a patient with a giant biliary mucinous cystadenoma of the liver and discuss the management with reference to the literature. A 47 year-old woman presented with a 6-week history of moderate epigastric discomfort on a background of 12 months of symptom-free abdominal distension. A giant cystic bilobar tumor of the liver measuring 22 x 23 x 17 cm was diagnosed and characterised by ultrasound scan and magnetic resonance imaging. Serum bilirubin, alkaline phosphatase and gamma-glutamyl transpeptidase were elevated, though other laboratory data including tumor markers (CEA, aFP, CA19-9) were within normal limits. Total excision of the cyst was not possible due to its size and position, and the patient underwent cyst drainage, a sub-total cyst excision and omentoplasty. Histology confirmed a benign biliary mucinous cystadenoma with an ovarian stroma. Though the patient remained clinically well, routine post-operative computed tomography (CT) surveillance showed an 11 cm recurrent cyst at 6 months. A partial cyst resection with close follow-up, regular CA19-9 serology and ultrasound/CT imaging, may be a reasonable alternative for bilobar or giant cysts. However should any features pathognomonic of malignancy develop, then a liver transplantation is indicated.

Two synchronous somatostatinomas of the duodenum and pancreatic head in one patient.

Somatostatinomas are extremely rare neuroendocrine tumors of the gastrointestinal tract, first described in the pancreas in 1977 and in the duodenum in 1979. They may be functional and cause somatostatinoma or inhibitory syndrome, but more frequently are non-functioning pancreatic endocrine tumors that produce somatostatin alone. They are usually single, malignant, large lesions, frequently associated with metastases, and generally with poor prognosis. We present the unique case of a 57-year-old woman with two synchronous non-functioning somatostatinomas, one solid duodenal lesion and one cystic lesion within the head of the pancreas, that were successfully resected with a pylorus-preserving Whipple's procedure. No secondaries were found in the liver, or in any of the removed regional lymph nodes. The patient had an uneventful recovery, and remains well and symptom-free at 18 mo postoperatively. This is an extremely rare case of a patient with two synchronous somatostatinomas of the duodenum and the pancreas. The condition is discussed with reference to the literature.

Isolated segmental, sectoral and right hepatic bile duct injuries.

The treatment of isolated segmental, sectoral and right hepatic bile duct injuries is controversial. Nineteen patients were treated over a 26-year period. Group one was comprised of 4 patients in whom the injury was primarily repaired during the original surgery; 3 over a T-tube, 1 with a Roux-en-Y. These patients had an uneventful recovery. The second group consisted of 5 patients in whom the duct was ligated; 4 developed infection, 3 of which required drainage and biliary repair. Two patients had good long-term outcomes; the third developed a late anastomotic stricture requiring further surgery. The fourth patient developed a small bile leak and pain which resolved spontaneously. The fifth patient developed complications from which he died. The third group was comprised of 4 patients referred with biliary peritonitis; all underwent drainage and lavage, and developed biliary fistulae, 3 of which resolved spontaneously, 1 required Roux-en-Y repair, with favorable outcomes. The fourth group consisted of 6 patients with biliary fistulae. Two patients, both with an 8-wk history of a fistula, underwent Roux-en-Y repair. Two others also underwent a Roux-en-Y repair, as their fistulae showed no signs of closure. The remaining 2 patients had spontaneous closure of their biliary fistulae. A primary repair is a reasonable alternative to ligature of injured duct. Patients with ligated ducts may develop complications. Infected ducts require further surgery. Patients with biliary peritonitis must be treated with drainage and lavage. There is a 50% chance that a biliary fistula will close spontaneously. In cases where the biliary fistula does not close within 6 to 8 wk, a Roux-en-Y anastomosis should be considered.

Perigastric extraskeletal Ewing's sarcoma: a case report.

Ewing's sarcoma (ES) is a neoplasm of undifferentiated small round cells, which occurs in the bones and deep soft tissues of children and adolescents. We present a rare case of a 44-year-old woman with gastric ES presenting with epigastric pain and weight loss. Ultrasound and computed tomography scans indicated a solid/cystic mass in the pancreatic tail. At laparotomy, the tumor was found attached to the posterior surface of the stomach, completely free from the pancreas, with no lymphadenopathy or local metastases. The polynodal, partly pseudocystic, dark-red soft tumor was excised. Histopathology revealed an anaplastic small-round-cell tumor with strong membranous CD99 immunoexpression. Additionally, there was patchy immunostaining for S-100 protein, vimentin, protein gene product (PGP) 9.5 and neuron-specific enolase, and weak focal CD117 cytoplasmic immunoreactivity. The patient had no adjuvant chemotherapy; her postoperative recovery was uneventful, and she remains symptom-free, and without any sign of recurrence at 20 mo. To the best of our knowledge, this is only the third ever case of gastric ES.

Cystic lymphangioma of the pancreas.

Lymphangioma of the pancreas is an extremely rare benign tumour of lymphatic origin, with fewer than 60 published cases. Histologically, it is polycystic, with the cysts separated by thin septa and lined with endothelial cells. Though congenital, it can affect all age groups, and occurs more frequently in females. Patients usually present with epigastric pain and an associated palpable mass. Complete excision is curative, even though, depending on the tumour location, surgery may be simple or involve extensive pancreatic resection and anastomoses. The authors present a 49-year-old woman in whom a polycystic septated mass, 35 mm multiply 35 mm in size, was discovered by ultrasonography (US) in the body of the pancreas during investigations for epigastric pain and nausea. At surgery, a well circumscribed polycystic tumor was completely excised, with preservation of the pancreatic duct. The postoperative recovery was uneventful. Histology confirmed a microcystic lymphangioma of the pancreas. Immunohistochemistry showed cystic endothelial cells reactivity to factor VIII-RA (++), CD31 (+++) and CD34 (-). Postoperatively, abdominal pain disappeared and the patient remained symptom free for 12 mo until now. Although extremely rare, lymphangioma of the pancreas should be taken into consideration as a differential diagnosis of a pancreatic cystic lesion, especially in women.

Portal cavernoma caused by a calcified hydatid cyst of the liver (case report).

A portal cavernoma is a network of porto-porto collateral dilated tortuous veins lying within the hepatoduodenal ligament, which develops as a sequel to portal vein obstruction. This can be the result of extra-hepatic portal vein obstruction from local extrinsic occlusion, or by a prothrombotic disorder, or both. A 56-year-old woman presented with right upper quadrant abdominal pain. Examination and investigations revealed the presence of gallstones, a cavernous portal vein, several calcified hydatid cysts within the liver, grade III haemorrhoids, but no oesophageal varicosities. She had no previous abdominal surgery, and had normal full laboratory workup, including inflammatory markers, clotting analyses, and thrombophilia screen. At open surgery it became apparent that the portal cavernoma had been caused by local pressure from a calcified hydatid cyst of the caudate lobe of the liver. To the best of our knowledge, this is the first report of portal cavernoma caused by a hydatid cyst of the liver.

Coronary artery revascularization prior to abdominal nonvascular surgery.

BACKGROUND: Does preoperative revascularization of the myocardium reduce cardiac risk in noncardiac surgery? The aim of this study was to evaluate the clinical effectiveness of preoperative cardioprotection by coronary artery revascularization in abdominal nonvascular surgery under general anesthesia. MATERIALS AND METHODS: The observational clinical study included 111 consecutive patients with angiographically verified coronary artery disease. Two stratification groups of patients were compared, those with coronary artery revascularization (34 patients, 30.6%) and those without coronary artery revascularization (77 patients, 64.9%), in relation to frequency of perioperative cardiac complications. The patients were followed up until the 30th postoperative day. During operation and in the following 72 postoperative hours, the patients were monitored by continuous ST-T segment recording. Twelve-lead electrocardiography was performed immediately after surgery and on postoperative days 1, 2, and 7 as well as 1 day before discharge. Serum troponin T levels were controlled at 6, 24, and 96 h postoperatively. RESULTS: The number of patients with major cardiac complications was 0 (0.0%, n=34) in the revascularized myocardium group and 10 (12.9%, n=77) in the nonrevascularized myocardium group (P<.05). Three patients in the nonrevascularized myocardium group died of acute myocardial infarction, congestive heart failure, and malignant arrhythmias, respectively, with severe coronary artery stenosis verified angiographically. CONCLUSIONS: Preoperative cardioprotection by coronary artery revascularization significantly reduces morbidity and mortality in patients who have undergone abdominal nonvascular surgery. Patients with severe coronary artery stenosis and indication for coronary artery revascularization independently of noncardiac surgery should first undergo cardiosurgical intervention prior to elective abdominal nonvascular surgery.

Metastatic melanoma to the common bile duct causing obstructive jaundice: a case report.

Metastatic melanoma to the common bile duct is very rare with only 18 cases reported so far. We report a 46 year old women who, 18 mo after excision of a skin melanoma, developed a painless progressive obstructive jaundice. At operation a melanoma within the distal third of the common bile duct was found. There were no other secondaries within the abdomen. The common bile duct, including the tumor, was resected and anastomosed with Roux-en-Y jejunal limb. The patient survived 31 mo without any sign of local recurrence and was submitted to three other operations for axillar and brain secondaries, from which she finally died. Radical resection of metastatic melanoma to the common bile duct may result in lifelong relief of obstructive jaundice. It is safe and relatively easy to perform. In other cases, a less aggressive approach, stenting or bypass procedures, should be adopted.