[Cystic dystrophy of the duodenal wall in ectopic pancreas].

INTRODUCTION: Cystic dystrophy of the duodenal wall is a rare complication of the ectopic pancreas that is characterized by cyst/s formation within thickened duodenal wall. CASE OUTLINE: A 61-year-old male with recurrent abdominal pain, weight loss (about 25 kg) who had been moderate alcohol abuser and heavy smoker was presented. On ultrasonography, very thickened duodenal wall (2.5 cm), an enlarged head of the pancreas with cyst of 3 cm in diameter as well as dilated pancreatic duct (< 6 mm) were seen. Barium meal showed stenosis of the first and second part of the duodenum. CT and endoscopic ultrasound confirmed the ultrasonographic finding.The patient underwent surgery. The pathologic finding was established only on first two portions of the duodenum and limited part of the head of the pancreas along duodenum while the rest of the pancreas was normal. Due to poor general condition, gastrojejunostomy was performed. Although some improvement was evident, the patient did not become asymptomatic, and, therefore, four months later a cephalic duodenopancreatectomy was carried out which made him fully asymptomatic. A year later, the patient was symptom-free and in good health. Histologic examination showed a cystic dystrophy of the duodenal wall in the ectopic pancreas. CONCLUSION: Unless there are strong contraindications, cephalic duodenopancreatectomy is best treatment of the disease.

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.

[Solid solitary hamartoma of the spleen].

INTRODUCTION: Hamartoma of the spleen is a rare, sometimes asymptomatic similar to hemangioma benign tumor of the spleen, which, owing to the new diagnostic imaging methods, is discovered with increasing frequency. It appears as solitary or multiple tumorous lesions. CASE OUTLINE: We present a 48-year-old woman in whom, during the investigation for Helicobacter pylori gastric infection and rectal bleeding, with ultrasonography, a mass 6.5x6.5 cm in diameter was discovered by chance within the spleen. Splenectomy was performed due to suspected lymphoma of the spleen. On histology, tumor showed to be of mixed cellular structure, with areas without white pulp, at places with marked dilatation of sinusoids and capillaries to the formation of "blood lakes" between which broad hypercellular Billroth's zones were present. Extramedullary hematopoiesis was found focally. The cells that covered vascular spaces were CD34+ and CD31+ and CD8- and CD21-. CONCLUSION: Hamartoma has to be taken into consideration always when well circumscribed hypervascular tumor within the spleen is found, particularly in children. Although the diagnosis of hamartoma may be suspected preoperatively, the exact diagnosis is established based on histological and immunohystochemistry examinations. Treatment is most often splenectomy and rarely a partial splenectomy is possible, which is recommended particularly in children.

[Mucinous cystadenomas of the liver].

INTRODUCTION: Biliary cystadenomas of the liver are rare benign tumors prone to malignant alteration so that a total excision is recommended. OBJECTIVE: The aim of the paper is to present our experience in treatment and to evaluate whether a simple ablation represents the appropriate treatment. METHODS: Over a 10-year period 25 patients (24 women) of the average age of 58 years suffering from cystadenomas of the liver, 18 in the right, 4 in the left and 3 in both lobes of the liver were operated. Twenty-three patients had a single lesion, while two patients had 3 and 6 lesions, respectively. Pain was present in 20, occasional vomiting in 4, discomfort in 2 and a sense of fullness in 2 patients.Three patients were jaundiced, 1 due to cystadenoma of the liver, 2 due to concomitant tumors of the head of the pancreas, while 5 patients had concomitant diseases. RESULTS: A total ablation was performed in 22 patients, left lateral bisegmentectomy in 1 and partial excision in 2 patients. Six additional procedures were performed. Five cystadenomas of the liver had,,ovarian like" stroma, all in women. A focal malignant alteration was found in 2 patients aged 66 and 79 years, respectively. Recurrence was registered in 1 female patient in whom a partial excision had been done. Two patients with concomitant malignancy and 1 patient who developed malignant histiocytosis six months after surgery, died after 1, 2 and 3 years, respectively. CONCLUSION: Biliary cystadenomas of the liver may be misdiagnosed as simple liver cysts, so that ,,frozen section" histology is highly recommended. In most cases the tumor may be successfully treated by ablation up to the healthy liver tissue. Major liver resections are rarely necessary.

Malignant stromal tumor of the stomach with giant cystic liver metastases prior to treatment with imatinib mesylate.

INTRODUCTION: Gastrointestinal stromal tumors (GISTs) are rare and account for 0.1%-3% of all gastrointestinal neoplasms. GISTs are most commonly located in the stomach (60%) and 20%-25% are malignant, with metastases involving the peritoneum or the liver. Cystic liver metastases are extremely rare. Only two previous cases of patients with cystic liver metastases, prior to treatment with imatinib mesylate, have been described so far. CASE REPORT: We reported a 52-year-old woman presented with a history of abdominal fullness and discomfort. Clinical examination revealed two palpable masses, first in the right upper abdomen and second left to the umbilicus. Examinations revealed 4 cystic metastases in the liver, 3 in the right lobe (including a huge one measuring 20.5 x 16 cm), and 1 in the left lobe, together with a primary tumor on the greater curvature of the stomach. Gastric tumor was removed with a Billroth II gastrectomy. Partial excision of the largest liver metastasis was performed for histopathology. Immunohistochemistry confirmed the diagnosis of a GIST in both tissue samples. After an uneventful recovery the patient was commenced on imatinib mesylate therapy. The patient remainsed symptom-free at 24 months follow-up. CONCLUSION: This was the third reported case of gastric GIST with giant cystic liver metastases present prior to treatment with imatinib mesylate. Although extremely rare, GISTs may present with cystic liver metastases prior to treatment with imatinib mesylate, and should be considered in the differential diagnoses of patients with liver cysts of uncertain aetiology.

[Pendular stromal tumour of the stomach with dominant PDGFRA immunoexpression: case report and short literature review].

INTRODUCTION: Gastrointestinal stromal tumours are most frequent mesenchimal tumours of the gastrointestinal tract that originate from Cajal's interstitial cells that are most frequently CD-117 positive. Stromal tumours of the stomach are the most frequent mesenchimal tumours of the gastrointestinal tract. Such tumours are usually sessile, but rarely pendular when they can be easily removed with a limited local excision of the stomach wall around the pedicle. Major stomach resections are rarely necessary. CASE OUTLINE: In a 54-year-old woman with abdominal pain and fever of unknown aetiology, a large spherical mobile and almost painless mass was found within the upper right abdomen. US and CT showed a mainly cystic, partly solid tumour, of 15.5x12.5 cm in diameters. Laboratory data including tumour markers were within normal limits. At operation a mobile and free tumour of the stomach attached to the anterior wall with a 2.5 cm pedicle was found and easily excised. Abdominal mucosa was normal. There was no liver metastasis or peritoneal dissemination. Hystology and imunohistochemistry showed a rare sclerosing sincitial subtype of stromal tumour with imunophenotype heterogenicity with a dominant PDGFRA and rare CD-117 immunoexpression. The postoperative recovery was uneventful. The patient was symptom-free with no sign of recurrence after a year and a half. CONCLUSION: A rare subtype of histological highly malignant stromal tumour of the stomach, macroscopically of pendular type, that was easily excised, was presented which so far showed a favourable evolution with no signs of recurrence.

[Intraabdominal abscesses of unknown etiology].

INTRODUCTION: Intraabdominal abscesses are in 98-99% cases the result of secondary and only in 1-2% of primary peritonitis. They are easy and successfully diagnosed. Abdominal abscesses of unknown cause are extremely rare. CASE OUTLINE: The authors present a 68-year-old man, without significant data in past history, who suddenly developed epigastric pain, nausea, vomiting and leukocytosis which was treated with antibiotics resulting in the alleviation of complaints and reduction of white blood cells count. After five days ultrasonography showed incapsulated collection of dense fluid in the epigastrium confirmed by CT scan two days later. Upper endoscopy excluded ulcer and/or perforation of the stomach and duodenum. Under local anesthesia, through the upper part of the left rectal muscle, puncture followed by incision was done, and about 50 ml of dense pus was removed. Finger exploration of the cavity showed no foreign body within the cavity. Using drainage, the recovery was quick and uneventful. By preoperative and postoperative abdominal investigations no cause of the abscess was found. Two and a half years after surgery the patient remained symptom-free with normal clinical, laboratory and ultrasonographic findings. CONCLUSION: The authors presented an intraabdominal abscess of unknown cause that was successfully treated with antibiotics, percutaneous puncture and drainage under local anaesthesia. In spite of all diagnostic methods the cause of the abscess could not be found. Thus, such a possibility, although being rare, should be taken into account.

[Biliobronchial fistula secondary to percutaneous dilatation of the benign biliary stricture].

INTRODUCTION: Biliobronchial fistula is rare. Very rarely it may be congenital, more frequently it is acquired as a complication of the hydatide cyst of the liver, pyogenic abscess, serious trauma and resection of the liver as well as recurrent cholangitis due to benign bile duct stricture or cholangiolithiasis. The main causes of the biliobronchial fistula are billiary obstruction and infectious lesion (abscess) in the liver. CASE OUTLINE: We present a 56-year-old man with benign stricture of the hepaticojejunostomy performed after operative common bile duct injury, who developed biliobronchial fistula following repeated percutaneous drainage of the liver abscess and percutaneous dilatation of the strictured anastomosis. Over the years the patient developed atrophy/hypertrophy complex, portal hypertension, grade II esophageal varicosities, ascites and splenomegaly. Although biliobronchial fistula was solved by a successful surgical reconstruction (new wide hepaticojejunostomy), the operation had a limited value as it was performed late after permanent lesions of the liver and intrahepatic bile ducts had already developed. CONCLUSION: Surgical reconstruction of strictured biliodigestive anastomosis should be considered on time as a possibly better solution than percutaneous dilatation. According to the authors' knowledge, a similar case of biliobronchial fistula as a complication of percutaneous dilatation of the benign biliary stricture has not been reported before in the literature.

[Well differentiated endocrine carcinomas of the pancreas].

INTRODUCTION: For the difference from poorly differentiated, well differentiated endocrine carcinomas of the pancreas are the tumours in whom with aggressive surgery and chemotherapy fair results can be achieved. OBJECTIVE: The aim of the study was to point out the importance of such treatment. METHODS: Over a 6-year period eight patients (seven female and one male) of average age 51 years (ranging from 23 to 71 years) were operated on for well differentiated endocrine carcinoma: six of the head and two of the tail of the pancreas. There were two functional and six nonfunctional tumours. Pain in the upper part of the abdomen in seven, mild loss in weight in two, strong heartburn in two, obstructive jaundice in three, diarrhoea in one, sudden massive bleeding from gastric varicosities due to prehepatic portal hypertension caused by pancreatic head tumour in one, and bruise in one patient were registered preoperatively. US and CT in all, angiography in one, octreoscan in two and PET scan in one patient were performed. Whipple's procedure was performed in six and distal pancreatectomy in two patients, as well as systemic lymphadenectomy in all and excision of liver secondary tumours in two patients. In the patient with massive gastric bleeding a total gastrectomy was performed first, followed by Whipple's procedure a month later. RESULTS: R0 resection was achieved in all patients. Lymph nodes metastases were found in six patients. Six patients were given chemotherapy. One patient died 3 years after surgery, seven are still alive, on average 2.5 years. A local recurrence after distal pancreatectomy that occurred 5 years after surgery was successfully reresected and the patient is on peptide-receptor radiotherapy. In other six patients there were no local recurence or distant metastases. CONCLUSION: With aggressive surgery and chemotherapy fair results can be achieved in well differentiated endocrine carcinomas of the pancreas.

[Giant haemorrhagic (pseudo)cyst of the pancreas of unknown aetiology].

INTRODUCTION: Cystic lesions of the pancreas are frequent. Most frequently their aetiology can be easily established. It is very rare that the nature and aetiology of these lesions cannot be reliably established. CASE OUTLINE: A 40-year-old male without trauma, alcohol abuse, acute or chronic pancreatitis in illness history was successfully operated on for a haemorrhagic (pseudo)cyst of the back wall of the pancreas tail containing 4.5 litres of haemorrhagic content. The cyst did not contain either epithelium or tumour stroma, and the pancreas did not show any changes of acute or chronic pancreatitis. Hystology did not show signs of angiectatic pseudocyst. CONCLUSION: In spite of all efforts, the authors could not establish the real nature of the (pseudo)cyst of the pancreas or find a similar case described in the literature. Nevertheless, the authors believe that it was probably an angiectatic cyst.

[Pancreatic tail pseudocyst of type II treated with resection of the tail of the pancreas and splenectomy].

INTRODUCTION: Pancreatic pseudocysts of type II are postnecrotic cysts that appear during an acute-on-chronic pancreatitis. In case that surgical treatment is necessary, as a rule it is performed using internal drainage operations. Pancreatic resections are rarely indicated. CASE OUTLINE: The authors present a 34 year-old man with a long-lasting history of moderate alcohol consumption in whom an episode of drinking caused an acute-on-chronic pancreatitis so that a 7 cm in diameter cyst was developed inside the tail of the pancreas causing left subcostal pain, mild pleural effusion and pain in the left shoulder. At operation almost entirely inside the tail of the pancreas a cyst of type II unsuitable for internal drainage operation was found so that a spared resection of the tail of the pancreas and splenectomy were carried out. The postoperative recovery was prolonged due to recurrent left pleural effusion requiring punctions, mild suppurative secretion from the splenic fossa and transient postsplenectomy thrombocytosis. Six months after surgery the patient is in good condition and with normal findings. CONCLUSION: Although rare, pancreatic cysts of type II may be unsuitable for internal drainage operations so that resection of the effected part of the pancreas could be a much better solution than external drainage.

[Choledochoduodenal fistula of ulcer etiology].

INTRODUCTION: Choledochoduodenal fistulas are very rare and in most cases are caused by a long-lasting and poorly treated chronic duodenal ulcer. They may be asymptomatic or followed by symptoms of ulcer disease, by attacks of cholangitis or bleeding or vomiting in cases of ductoduodenal stenosis. The diagnosis is simple and safe, however treatment is still controversial. If surgery is the choice of treatment, local findings should be taken into consideration. As a rule, intervention involving closure of fistula is not recommended. CASE OUTLINE: The authors present a 60-year-old woman with a long history of ulcer disease who developed attacks of cholangitis over the last three years. Ultrasonography and CT showed masive pneumobilia due to a choledochoduodenal fistula. As there was no duodenal stenosis or bleeding, at operation the common bile duct was transected and end-to-side choledochojejunostomy was performed using a Roux-en Y jejunal limb. From the common bile duct, multiple foreign bodies of herbal origin causing biliary obstruction and cholangitis were removed. After uneventful recovery the patient stayed symptom free for four years now. CONCLUSION: The performed operation was a simple and good surgical solution which resulted in complication-free and rapid recovery with a long-term good outcome.

[Glucagonoma without glucagonoma syndrome].

INTRODUCTION: Glucagonomas are rare, frequently malignant tumours, arising from the Langerhans' islets of the pancreas. They usually secrete large amounts of glucagon that can cause a characteristic "glucagonoma syndrome", which includes necrolytic migratory erythema, glucose intolerance or diabetes, weight loss and sometimes, normochromic normocytic anaemia, stomatitis or cheilitis, diarrhoea or other digestive symptoms, thoromboembolism, hepatosplenomegaly, depression or other psychiatric and paraneoplastic symptoms. In certain cases, some or all glucagonoma symptoms may appear late, or even may be completely absent. CASE OUTLINE: The authors present a 43-year-old woman in whom an investigation for abdominal pain revealed a tumour of the body of the pancreas. During operation, the tumour of the body of the pancreas extending to the mesentery measuring 85 x 55 x 55 mm was excised. Histology and immunohistochemistry showed malignant glucagonoma, with co-expression of somatostatin in about 5% and pancreatic polypeptide in a few tumour cells. The recovery was uneventful. The patient stayed symptom-free with no signs of local recurrence or distant diseases 15 years after surgery. CONCLUSION: Glucagonoma syndrome may be absent in glucagonoma tumour patients so that in unclear pancreatic tumours the clinician should frequently request the serum hormone level (including glucagon) measurement by radioimmunoassay and the pathologist should perform immunohistochemistry investigation. Those two would probably result in discovery of more glucagonomas and other neuroendocrine tumours without characteristic clinical syndromes.

[Cholangiocellular carcinoma of the common hepatic duct causing obstructive jaundice in a patient with metastatic colonic carcinoma within liver].

INTRODUCTION: Colorectal carcinoma, one of the most frequent carcinomas, produces liver metastasis very frequently. Surprisingly, those secondaries rarely cause obstructive jaundice. If it appears, it is usually caused by compression or infiltration of the major bile ducts close to the hepatic hilus, less frequently with bile duct obstruction by gelatinous mucus produced by the timour, much rarer by the tumour growth within the, otherwise intact, common bile duct and very rarely by metastasis into the biliary tree. CASE OUTLINE: We present a 67-year-old man who had been submitted to left colectomy for sygmoid colon carcinoma four years earlier, now, admitted with an obstructive jaundice, along with a number of liver and lung secondaries. Obstructive jaundice was caused by the vegetative tumour of the proximal part of the common hepatic duct which was resected and anastomosed with a Roux-en-Y jejunal limb. The postoperative recovery was uneventful. The patient died 7 months later without jaundice due to liver and lung secondaries. Histological findings showed cholangiocellular carcinoma of the common hepatic duct, while the histological findings of the liver tumour specimen confirmed metastatic colonic carcinoma. CONCLUSION: In case of obstructive jaundice in patients with metastatic colonic carcinoma within liver, other aethiological factors of biliary obstruction can not be excluded and have to be taken into differential diagnosis.

[Symptomatic calcified splenic artery aneurysm: case report].

INTRODUCTION: Although the third most common aneurysm within the abdomen, after aneurysms of the aorta and iliac arteries, splenic artery aneurysms are rare, but not exceptionally. Owing to new imaging techniques, they have been discovered with increasing frequency. CASE OUTLINE: Authors present a 47-year-old woman, multipara, who presented with left upper abdominal pain in whom X-ray showed a calcified ring in the area of distal pancreas. Selective angiography confirmed a splenic artery aneurysm of its proximal part. During an open surgery the aneurysm was excised (aneurismectomy) without immediate, early or late complications. The patient became symptom-free. CONCLUSION: In patients, particularly women, the multiparas who present with epigastric or left upper abdominal pain of unknown aetiology, splenic artery aneurysm has to be taken into account. Further diagnostic procedures such as plain X-ray and selective angiography in suspected cases should be performed. Surgery or other treatment modalities are to be seriously considered in all patients, particularly in those with increased risk of rupture.

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.

[Actinomycosis of the caecum simulating carcinoma in a patient with a long-term intrauterine device].

INTRODUCTION: Actinomycosis of the caecum is a rare, but most frequently an abdominally localized disease. It often simulates inflammatory malignancy, rarely a periappendicular abscess or Chron's disease and is only exceptionally the cause of intestinal obstruction or bleeding. CASE OUTLINE: The authors present a 35-year-old woman with an intrauterine device which remained inserted for over three years, causing the development of pain, fever, vaginal secretion and bleeding that continued even after the device was removed. Ultrasonography showed a tumorous mass of irregular form located close to the uterus, which after a few months developed into a colliquation filled with pus requiring incision. Bacteriological examination failed to show actinomycosis. Due to the tumorous lesions involving the terminal ileum, appendix, caecum, ascending colon and omentum, a right hemicolectomy was performed. Based on histolopathological findings of the resected sample the diagnosis of actinomycosis was made. Therefore, after surgery the patient began treatment with antibiotics resulting in full recovery. CONCLUSION: Although rare, actinomycosis of the caecum should be taken into consideration in the differential diagnosis of tumorous lesions of the caecoascending part of the colon, particularly if the tumour is associated with inflammation.