Inferior vena cava obstruction and collateral circulation as unusual manifestations of hepatobiliary cystadenocarcinoma.

BACKGROUND: Hepatobiliary cystadenocarcinoma represents a rare epithelial malignant tumor derived from the intrahepatic bile duct. METHODS: A 71-year-old woman, who had undergone laparoscopic drainage of a cystic lesion of the right hepatic lobe, was misdiagnosed as having hepatic echinococcal disease, and received intracystic infusion of 95% ethanol four years ago. She was admitted to our hospital for further treatment. RESULTS: Physical examination revealed dilated superficial veins across the right abdominal wall. After mapping the direction of blood flow in these vessels, we assumed that this was a sign of inferior vena cava obstruction. Abdominal ultrasound, computed tomography, magnetic resonance imaging combined with magnetic resonance angiography showed a large cystic mass in the right upper quadrant and epigastrium, displacing the adjacent structures, adherent to the inferior vena cava, which was not patent, resulting in dilation of superficial epigastric veins. The patient underwent an exploratory laparotomy. Total excision of the huge mass measuring 16X15 cm was possible under selective vascular exclusion of the liver. Removal of the tumor resulted in immediate restoration of flow in the inferior vena cava. On the basis of the pathology and findings of immunohistochemical analysis, a hepatobiliary cystadenocarcinoma was diagnosed. CONCLUSIONS: In the present case, hepatobiliary cystadenocarcinoma was accompanied by dilated superficial venous collaterals due to inferior vena cava obstruction. Selective vascular exclusion of the liver allowed a safe oncological resection of the tumor.

Hepatobiliary cystadenomas and cystadenocarcinomas: a report of 33 cases.

BACKGROUND: Hepatobiliary cystadenomas and cystadenocarcinomas are rare and often misdiagnosed. AIMS: We report our experience with 33 cases over 20 years to discuss an algorithm for these diseases. METHODS: Patients presenting with a diagnosis of hepatobiliary cystadenomas and cystadenocarcinomas were retrospectively reviewed from January 1991 to October 2010. Clinical data were collected by examining hospital records and by follow-up questionnaire interviews. RESULTS: Thirty-three patients had pathologically diagnosed hepatobiliary cystadenomas (19/33, 17 females and two males) or cystadenocarcinomas (14/33, five females and nine males). Symptoms of cystadenomas at hospitalization were abdominal bloating or pain (9/19). Nine patients had an elevated level of carbohydrate antigen (CA) 19-9. The surgical procedures, i.e. cyst enucleation, segmentectomy, sectionectomy and hemihepatectomy, were performed with satisfactory outcomes. Symptoms of cystadenocarcinomas included abdominal bloating or pain (8/14) and fever (3/14). Seven patients had elevated CA19-9. The imaging characteristics of cystadenocarcinomas were similar to those of cystadenomas. The clinical outcomes for cystadenocarcinomas were mostly poor after either surgical or conservative treatment. CONCLUSIONS: Clinical symptoms are unreliable for these diagnoses and their differential diagnosis. Imaging evaluations and CA19-9 are of value for the recognition of cystadenoma and cystadenocarcinoma, but not for their differential diagnosis. Any recurrence of liver cyst after surgery or other treatments should lead one to suspect one of these diseases. Invasive examination and percutaneous fine-needle aspiration cytology are not recommended. Complete excision or careful enucleation should be the first treatment choice for a better prognosis.

Successful left hemihepatectomy and perioperative management of a patient with biliary cystadenocarcinoma, complicated with MELAS syndrome: report of a case.

Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, and Stroke-like syndrome (MELAS) is a rare, fetal disease caused by a mutation in mitochondrial DNA that leads to impaired oxidative metabolism in skeletal muscle, the central nervous system, and liver function. This report presents the case of a 50-year-old woman with biliary cystadenocarcinoma complicated by MELAS who underwent a successful left hemihepatectomy. In this case, the diagnostic key for the malignant tumor was an (18)F-fluorodeoxyglucose positron emission tomography study, which was useful even in a patient with MELAS, which causes abnormal glucose metabolism. The perioperative management of such patients includes special precautions to prevent lactic acidosis and deterioration of the reserved liver function after a hepatectomy, since the mitochondrial function in MELAS patients is abnormal. The patient in this report has remained free of liver dysfunctions and cancer recurrence for 2 years following the hepatectomy. This is the first report of a successful major hepatectomy for a patient with MELAS.

Chylous ascites in gynecologic malignancies: cases report and literature review.

PURPOSE: Chylous ascites is an infrequent postoperative complication after retroperitoneal surgical procedure. Despite its infrequent occurrence, postoperative chylous ascites are associated with significant morbidity. Reports of chylous ascites or fistula after retroperitoneal lymph node dissection for gynecologic malignancies without radiation therapy are rare. A search in the English literature showed only 31 cases of chylous fistula for gynecologic malignancies. Treatment may be conservative with low-fat oral diet with medium-chain triglycerides associated or not to octreotide and total parenteral nutrition. In case of conservative measures failure, it can be managed by surgical intervention or peritoneo-venous shunt. METHODS: We report two cases of chylous fistula following systematic pelvic and retroperitoneal lymph node dissection for gynecological cancer without radiotherapy and review the literature. RESULTS: Both were successfully managed with the maintenance of the postoperative drain, total parenteral nutrition, octreotide and dietary intervention. CONCLUSIONS: Chylous ascites should be included in differential diagnosis of abdominal distention after surgical retroperitoneal approach or radiotherapy. Most of the patients may have their chylous ascites successfully treated with conservative management. However, the best policy is to prevent chylous complications by employing meticulous dissection techniques and careful control of the major lymphatics by suture ligation during the primary surgical intervention.

Primary biliary cystadenocarcinoma mimicking a complicated hydatid cyst.

Cystadenocarcinoma of the liver is a rare neoplasm that originates from hepatobiliary epithelium. Primary location of this tumor is generally intrahepatic and most cases are in the right hepatic lobe. Herein we present a case of intrahepatic cystadenocarcinoma in a 53-year-old man who had been followed up for 8 years as hydatic cyst disease of the liver. He was admitted to our hospital with jaundice and loss of appetite. Ultrasound and computed tomography showed a cystic lesion that looked like type II cyst hidatic. Thereafter magnetic resonance imaging revealed a cystic lesion associated with biliary tree. On diagnostic laparotomy peritoneal infiltrations were observed and pathologic examination revealed a biliary cystadenocarcinoma and peritonitis carcinomatosa was diagnosed. Unfortunately correct diagnosis was extremely late and no curative management was possible.

Pancreatic panniculitis and carcinoma of the pancreas.

Pancreatic panniculitis is a rare complication of carcinoma of the pancreas, most often accompanying the rare acinar cystadenocarcinoma. It presents with painful erythematous subcutaneous nodules typically located on the leg. We present a case of a 79-year-old man with neuroendocrine carcinoma of the pancreas and liver metastasis, who developed painful subcutaneous nodules on his shins. Laboratory values included a raised lipase level with normal amylase level and peripheral eosinophilia. The patient was treated with nonsteroidal anti-inflammatory drugs, dexamethasone and antibiotics, with resolution of the dermatological symptoms.

Case of biliary cystadenocarcinoma with intracystic bleeding.

Biliary cystadenocarcinomas are rare cystic tumors arising from the biliary epithelium. Symptoms vary and are sometimes absent. Most lesions are found incidentally during diagnostic radiologic procedures or surgical exploration for unrelated conditions. We describe an 80-year-old man with anemia due to intracystic bleeding. A computed tomographic scan showed a low-density hepatic lesion with thick, irregular walls and septa. Left lobectomy with cholecystectomy was performed. Histological examination revealed that the surgical specimen was a biliary cystadenocarcinoma. The concentrations of both carcinoembryonic antigen and CA19-9 were high in the cyst contents. The patient died of pneumonia 1 year 4 months after the operation, without the recurrence of cystadenocarcinoma.

[Comparative study of appendical mucocela and pseudomyxoma peritonei. About 25 cases]. / Etude comparative des mucoceles simples et des mucoceles compliques de pseudomyxome. A propos de 25 observations.

AIM: to study the clinicopathological features of the different types of appendical mucocele and to compare them with those of pseudomyxoma peritonei. METHODS: 25 cases of appendical mucocele were operated in the Cap-Bon region in Tunisia during a period of 13 years from 1994 to 2006. RESULTS: 9 retentionnal cysts, 13 mucinous cystadenomas, one serrated adenoma, one hyperplasia of the mucosa and one cystadenocarcinoma were diagnosed. Five cystadenomas as well as the only case of cystadenocarcinoma were associated with pseudomyxoma peritonei. Pseudomyxoma peritonei occurred 20 years later than in simple appendiceal mucocèle and complicated 5 cases of cystadenoma with low grade dysplasia and 1 case of cystadenocarcinoma. CONCLUSION: Preoperative diagnosis of appendical mucocele and pseudomyxoma peritonei should be made on scannographic features in order to assess the adequate surgical management.

Obstructive jaundice due to hepatobiliary cystadenoma or cystadenocarcinoma.

Hepatobiliary cystadenomas (HBC) and cystadenocarcinomas are rare cystic lesions. Most patients with these lesions are asymptomatic, but presentation with obstructive jaundice may occur. The first patient presented with intermittent colicky pain and recurrent obstructive jaundice. Imaging studies revealed a polypoid lesion in the left hepatic duct. The second patient had recurrent jaundice and cholangitis. Endoscopic retrograde cholangiopancreatography (ERCP) showed a cystic lesion at the confluence of the hepatic duct. In the third patient with intermittent jaundice and cholangitis, cholangioscopy revealed a papillomatous structure protruding into the left bile duct system. In the fourth patient with obstructive jaundice, CT-scan showed slight dilatation of the intrahepatic bile ducts and dilatation of the common bile duct of 3 cm. ERCP showed filling of a cystic lesion. All patients underwent partial liver resection, revealing HBC in the specimen. In the fifth patient presenting with obstructive jaundice, ultrasound examination showed a hyperechogenic cystic lesion centrally in the liver. The resection specimen revealed a hepatobiliary cystadenocarcinoma. HBC and cystadenocarcinoma may give rise to obstructive jaundice. Evaluation with cross-sectional imaging techniques is useful. ERCP is a useful tool to differentiate extraductal from intraductal obstruction.

[Bivalvular non bacterial thrombotic endocarditis associated with cancer and transesophageal echocardiography]. / Endocarditis trombótica no bacteriana bivalvular asociada a cáncer y ecocardiograma transesofágico.

Non bacterial thrombotic endocarditis is the most frequent cause of ischemic stroke in cancer patients. Up to 9% of autopsies of cancer patients show non infectious valvular masses. However, bivalvular involvement is not frequently occurring in 9% of non bacterial thrombotic endocarditis. We report a patient with ovarian cancer who presented aphasia. The MRI was compatible with cerebral ischemia. The transthoracic echocardiogram was normal and a transesophageal echocardiogram showed vegetations in aortic and mitral valves. We emphasize the importance of suspecting non bacterial thrombotic endocarditis in patients with cancer and systemic embolism and the low frequency of bivalvular involvement.

Metastatic lymph node impostor in pancreatic cystadenocarcinoma.

CONTEXT: Lymph node involvement in pancreatic cancer is a predictor of poor patient long-term survival. The detection of multiple metastatic peri-pancreatic nodes by EUS-FNA may dissuade the surgeon from undertaking a curative pancreatic resection. CASE REPORT: We report an interesting case of a man with chronic lymphocytic leukemia, who presented with the diagnostic problem of a pancreatic solid-cystic lesion and multiple malignant-looking peri-pancreatic lymphadenopathy on EUS. EUS-FNA yielded chronic lymphocytic leukaemia involvement in the peri-pancreatic lymph nodes and a markedly elevated CEA in the cystic fluid. The absence of adenocarcinoma involvement of the lymph nodes prompted surgery on the pancreatic lesion with a curative intent. Pancreatic mucinous cystadenocarcinoma was diagnosed and a sub-total pancreatectomy was performed with clear resection margins. All 30 resected peri-pancreatic lymph nodes showed chronic lymphocytic leukemia involvement only. CONCLUSIONS: This case illustrates that abnormal lymphadenopathy adjacent to a primary pancreatic lesion may not necessarily be due to the latter. Systemic lymphoproliferative disease, as in this case, can masquerade as metastatic adenocarcinoma lymph nodes on EUS. EUS-FNA is useful in diagnosing lymphoproliferative disease.

Intramural florid cystic endosalpingiosis of the uterus: a case report and review of the literature.

OBJECTIVE: We report a case of intramural florid cystic endosalpingiosis in the lower uterine segment of the uterus. CASE REPORT: A 43-year-old female presented with vaginal bleeding. Abdominal computed tomography suggested a leiomyoma with cystic degeneration. A total hysterectomy revealed a 4.0 cm × 3.8 cm cystic mass in the lower uterine segment. The cystic space microscopically was lined with a single layer or stratified layer of ciliated columnar cells that resembled tubal epithelium without cytologic atypia. The glandular spaces were surrounded by normal myometrium with no evidence of periglandular endometrial stroma, which was consistent with the diagnosis of florid cystic endosalpingiosis. CONCLUSION: Florid cystic endosalpingiosis involving the uterus is a rare and clinically unexpected finding; however, it should be considered in the differential diagnosis of a uterine mass.

Biliary cystadenocarcinoma: an unusual cause for recurrent hemobilia.

Hemobilia is an uncommon presentation of biliary tract or pancreatic disease. The investigation and management of this clinical problem is challenging. We report on a case of biliary tract hemorrhage from an otherwise asymptomatic right lobe biliary cystadenocarcinoma and review the literature on this unusual presentation. Hemobilia from primary or secondary liver tumors is not frequently reported in the literature. Hemobilia in this setting is usually observed in association with an obvious liver mass or abnormal liver function tests. This is a report of a case of hemobilia as the primary presentation of a small right lobe cystadenocarcinoma. Literature on the incidence and treatment of hemobilia associated with liver tumors has been reviewed. Hemobilia is investigated and definitively treated with angiography. In our case, initial imaging was equivocal and the lesion was only demonstrated after rebleeding, requiring a second angiogram. Surgical resection of the mass was required for definitive control of bleeding. This case illustrates the difficulties of investigating and treating hemobilia caused by primary or secondary liver tumors. Cystadenocarcinoma of the liver is not a common tumor, and biliary tract hemorrhage as the primary presentation of this tumor in the absence of a significant mass or abnormal liver function tests has not been previously described.

Pancreatic cystadenocarcinoma associated with strongyloides.

A 60-year-old woman presented with a mass in the left upper abdominal quadrant. Noninvasive studies showed a cystic structure arising from the pancreas. Pathologic studies of the tumor revealed pancreatic cystadenocarcinoma; the fluid contained Strongyloides larvae. There was no evidence of disseminated Strongyloides or immunosuppression. Results of stool examinations were also negative for Strongyloides. Were these larvae the etiologic agent for the carcinoma? Other parasitic infestations have been associated with carcinoma. This is the first reported association between Strongyloides and cystadenocarcinoma.

Pseudomyxoma peritoneii: treatment with the argon beam coagulator.

The argon beam coagulator facilitates tumor debulking of peritoneal carcinomatosis. A patient with pseudomyxoma peritoneii originating from the left ovary with multiple peritoneal and omental implants was treated with the argon beam coagulator, enabling ablation of all gross disease.

Fatal recurrent reexpansion pulmonary edema.

A case is reported of fatal recurrent unilateral reexpansion pulmonary edema following thoracentesis in a woman with stage IV ovarian adenocarcinoma. Reexpansion pulmonary edema is a rare complication resulting from reexpansion of a collapsed lung after treatment of pleural effusion, pneumothorax, or atelectasis. The etiology is unknown, and there are no guaranteed measures of prevention. The clinical presentation varies from asymptomatic to rapidly progressive fatal pulmonary edema. The diagnosis is established by chest x-ray. Depending on the severity of the clinical symptoms, treatment varies from monitoring with serial chest x-rays to mechanical ventilation. Reexpansion pulmonary edema is fatal in 20% of cases. Gynecologists should be aware of this rare entity because it may require immediate diagnosis and aggressive treatment.

Endometrioid-type cystadenocarcinoma arising in the mesosalpinx.

A case is presented of what is believed to be the first reported instance of endometrioid-type cystadenocarcinoma discovered in a paraovarian cyst. The literature on paraovarian carcinomas is reviewed.