ΔNp73 status in peritoneal and ovarian dissemination of appendicular adenocarcinoids (goblet cells).

INTRODUCTION: Goblet cell carcinoma (GCC) is an appendicular neoplasia representing less than 5% of all appendicular tumors, found in 0.3-0.9% of the appendectomies, 35-58% of all appendicular neoplasms, and less than 14% of malign appendix tumors. The most frequent clinical presentation is abdominal pain associated with a picture of acute appendicitis. MATERIALS AND METHODS: We present 3 clinical cases of appendix GCC, 2 subjected to cytoreductory surgery plus intraperitoneal hyperthermic chemotherapy and a third, who is currently receiving neoadjuvant treatment with a good response to chemotherapy and who will be offered the same treatment as the first two patients. Given the unpredictable behavior of these tumors, the use of molecular markers could help us to predict their behavior and prognosis. In this context, the TP73 gene would make an interesting putative marker. ∆Np73 has been described as overexpressed in a great variety of tumor types including colon cancer and this up-regulation is associated with a poor prognosis. To evidence its role in this malignancy, we evaluate here the status of ∆Np73 in the primary tumor and normal counterpart tissues, in the metastatic implants and in healthy areas of the peritoneum from the appendicular GCC patients. In addition, we checked the expression levels of this p73 variant in the tumor and normal tissue of 26 patients with colon cancer. RESULTS: Remarkably, 2 patients showed significant ∆Np73 down-regulation in both the primary tumor and the implants. Case 1 presented a fourfold decrease of levels in the primary tumor and 20-fold decrease in the implants. Case 2 showed a seven- and fourfold down-regulation in the primary tumor and implants, respectively. However, Case 3 showed an up-regulation of 53- and threefold in the primary tumor and implants, respectively. CONCLUSION: Goblet cell carcinoma of the appendix is very rate. It tends to seed throughout the peritoneum, making aggressive surgical cytoreduction and chemotherapy viable treatment options. Investigation into the molecular basis of these tumors may improve the diagnosis, prognosis and therapeutic decisions regarding these patients. ∆Np73 seems a good candidate for further analysis in longer series.

Cutaneous bronchobiliary fistula treated with Tissucol sealant.

Bronchobiliary fistula is a rare and is an uncommon but severe complication of hydatid disease of the liver. Treatment has traditionally been surgical resection, but embolization and stent placement have been described. The invasive method seems to be a key component of patient treatment. We describe a case of a 58-year-old woman who, 25 years before, had undergone surgery for a hydatid cyst. A total cystectomy without previous puncture or parasite extraction was carried out. The lower aspect of the cyst was found to be completely perforated over the biliary duct. During the postoperative course, the patient had subphrenic right-sided pleural effusion and biliary fistula that subsided with medical treatment. Afterward, the patient came to the outpatient area of our hospital complaining of leakage of purulent exudate through the cutaneous opening, pain located on the right hypochondrium radiating to the right hemithorax, malaise, fever, chronic cough, and occasional vomiting of bile. Fistulography revealed an anfractuous cavity communicating with a residual cystic cavity on the right hepatic lobe. We observed communication with the intrahepatic canaliculi. Computed tomographic scan revealed a fistulous tract on the anterior liver border through the abdominal wall. There were no posttreatment complications. The patient is asymptomatic.

Acute appendicitis-like symptoms as initial presentation of ovarian vein thrombosis.

Postpartum ovarian vein thrombosis is a rare condition, with an incidence rate being 1/600 deliveries. It most often arises in the right ovarian vein. A 33-year-old patient who had had normal vaginal delivery presented with fever, pain in the right iliac fossa, and leukocytosis on the sixth day after delivery. An antibiotic course was instituted but 3 days later symptoms reappeared. Diagnosis of acute appendicitis was made. At surgery through a McBurney incision, a woody tumoration consistent with ovarian vein thrombosis was found. Anticoagulation therapy with heparin and antibiotics were instituted. Phlebography and color Doppler sonography confirmed the presence of thrombosis of both the common femoral iliac and inferior vena cava. Fribrolysis with urokinase was performed. The patient has remained stable and symptom-free over a 4-year follow-up. Ovarian vein thrombosis typically presents with symptoms suggestive of acute appendicitis, as was the case in our patient. Color Doppler sonography is the favored diagnostic procedure, with CT being a supplementary tool. Surgery is not necessary and treatment consists of anticoagulants and antibiotics. Even though postpartum ovarian vein thrombosis is rare, early recognition of the condition is of paramount importance to institute the adequate treatment and avoid potential serious sequelae.

Ruptured primary splenic angiosarcoma into the colon. Presentation as anal bleeding.

A 71-year-old woman presented with a six month history of constipation and abdominal discomfort, with anal bleeding during the last days. Ultrasonography and CT-scan of the abdomen showed a large heterogeneous mass that was located in the splenic region, but the nature and origin of the tumour could not be clearly established preoperatively. The clinical diagnosis was of abdominal tumour with colonic and splenic involvement, and a left hemicolectomy and splenectomy were performed. Pathologic examination revealed a primary angiosarcoma of the spleen with penetration and fistulization of the tumour into the large bowel. The patient received adjuvant radiation therapy, but she died of extensive metastastic disease from her primary angiosarcoma of the spleen nine months after surgery. In summary, splenic angiosarcoma is very difficult to diagnose preoperatively. This highly aggressive neoplasm has an overall poor prognosis, specially if it is associated with rupture and haemoperitoneum. As this case highlights, unusual forms of rupture may lead to atypical clinical presentations, increasing even more the difficulty in the diagnosis.

[Extra-adrenal retroperitoneal paraganglioma]. / Paraganglioma retroperitoneal extraadrenal.

OBJECTIVE: To report a case of paraganglioma localized in the organ of Zuckerkandl and to discuss its diagnosis, treatment and outcome. METHODS/RESULTS: A 37-year-old female presented with a periumbilical mass and pain that radiated to the lumbar region for the past three months. Abdominal US and CT disclosed a well vascularized mass lying adjacent to the aorta, 1 cm from its bifurcation. At laparotomy, an 8 x 10 cm tumor was discovered at the aortic bifurcation that displaced the left ureter and included the inferior mesenteric artery. Pathological analysis of the surgical specimen revealed a paraganglioma. CONCLUSION: Extra-adrenal paraganglioma should be taken into account in the differential diagnosis of retroperitoneal masses, particularly those adjoining the abdominal aorta.