A Case of Autoimmune Cholangitis Misdiagnosed for Cholangiocarcinoma: How to Avoid Unnecessary Surgical Intervention?

INTRODUCTION: Autoimmune cholangitis or immunoglobulin G4-associated cholangitis (IAC) has been recently regarded as a new clinical and histopathological entity and is a part of a complex autoimmune disorder--IgG4-related systemic disease (ISD). ISD is an autoimmune disease with multi-organic involvement, characterized with IgG4-positive plasmocytic infiltration of various tissues and organs with a consequent sclerosis, which responds well to steroid therapy. Most commonly affected organs are the pancreas (autoimmune pancreatitis, [AIP]) and the common bile duct (IAC). IAC and cholangiocarcinoma (CCA) share many clinical, laboratory and imaging findings. CASE OUTLINE: We present a case of a 60-year-old male with a biliary stricture of a common bile duct, which was clinically considered as a bile duct carcinoma and treated surgically. Definite histopathological findings and immunohistochemistry revealed profound chronic inflammation, showing lymphoplasmacytic IgG-positive infiltration of a resected part of a common bile duct, highly suggestive for the diagnosis of IAC. In addition, postoperative IgG4 serum levels were also increased. CONCLUSION: It is of primary clinical importance to make a difference between IAC and CCA, in order to avoid unnecessary surgical intervention. Therefore, IAC should be considered in differential diagnosis in similar cases.

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.

Validation and feasibility of ex vivo sentinel lymph node "mapping" by methylene blue in colorectal cancer.

BACKGROUND/AIMS: There are currently divided opinions about the usefulness of sentinel lymph node mapping in colorectal carcinoma. This technique can potentially be useful in determining the volume of resection, reducing the number of analyzed lymph nodes limited to sentinel lymph nodes, and re-staging when metastases are detected in the sentinel lymph node. The aim of this study was to examine the feasibility of postoperative sentinel lymphatic node detection (hereinafter referred to as ex vivo sentinel lymph node mapping) in patients with colorectal carcinoma. METHODOLOGY: The clinical study included a total of 58 patients. Thirteen patients were intraoperatively excluded. Ex vivo sentinel lymph node mapping by methylene blue was used in this study to detect the lymphatic micrometastases. Lymph node preparations were also stained with hematoxylin eosin, followed by immunohistochemical staining of serial sections. RESULTS: Ex vivo sentinel lymph node technique was performed in 45 patients, successfully in 41/45 (91.1%). 22.9 lymph nodes (range: 11 to 43) and 1.7 sentinel lymph node (range: 0 to 4) were resected and stained. Sentinel lymph node staining was negative in 15/45 patients (33, 3% false negative results). CONCLUSIONS: Limited histopathology analysis by ex vivo sentinel lymph node mapping can not replace a complete histological analysis of all resected lymph nodes.

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.

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.

Double-barreled wet colostomy: urinary and fecal diversion.

PURPOSE: Double-barreled wet colostomy represents simultaneous urinary and fecal surgical diversion performed most commonly after pelvic exenteration as a palliative procedure or after actinic damage. We report the structural and functional results of double-barreled wet colostomy with special attention to surgical technique, morbidity and functional results compared to those described in the available literature. MATERIALS AND METHODS: We retrospectively followed 38 patients who underwent double-barreled wet colostomy at our institution from April 2003 to November 2007. The parameters were patient age and gender, the indication for double-barreled wet colostomy, postoperative morbidity and mortality, length of hospital stay and functional assessment by excreting excretory urography. RESULTS: A total of 38 double-barreled wet colostomies were performed at our institution, including 24 following total pelvic exenteration, 14 without resection, 9 in inoperable tumor cases and 5 in actinic damage cases. The postoperative morbidity rate was 15.7% with no treatment related mortality. Two patients had late postoperative complications, including stenosis of the ureterocolonic anastomosis and conduit necrosis, respectively. CONCLUSIONS: In our experience double-barreled wet colostomy has an acceptable morbidity and mortality rate, is performed without technical difficulties and does not require prolonged operative time. Double-barreled wet colostomy represents the procedure of choice in patients who require concurrent urinary and fecal diversion.

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.

Mucinous cystadenoma of the appendix associated with adenocarcinoma of the sigmoid colon and hepatocellular carcinoma of the liver: report of a case.

Mucinous cystadenoma of the appendix is a rare condition and represents one of the three entities with the common name mucocele of the appendix. It is characterized by a cystic dilatation of the lumen with stasis of mucus inside it. Histopathologically mucocele is divided into three groups: focal or diffuse mucosal hyperplasia, mucinous cystadenoma and mucinous cystadenocarcinoma. This condition is often associated with other neoplasia, especially adenocarcinoma of the colon and ovaries. We here describe a 57 year old male patient who presented with abdominal discomfort, constipation, fresh blood in stool and frequent urination. He had a big cystadenoma of the appendix associated with adenocarcinoma of the colon and hepatocellular carcinoma of the liver. The patient underwent right haemicolectomy, sigmoid colon resection and segmental resection of the liver. Now 3 years later he has no evidence of disease relapse. According to this, we stress the need of accurate preoperative diagnosis and intraoperative exploration of the whole abdomen in these patients.