Extragonadal germ cell tumour with the "burned out" phenomenon mimicking a retroperitioneal tumour of neurogenic origin.

OBJECTIVE: To describe a case of retroperitoneal metastasis of a gonadal germ cell tumour with the "burned-out" phenomenon in a 35 year old patient with a suspected diagnosis of retroperitoneal tumour of neurogenic origin. METHODS: With the clinical and radiological suspicion of retroperitoneal tumour of neurogenic origin the tumour was removed, via the retroperitoneal space. RESULTS: Pathology showed classic seminoma with foci of atypical or anaplastic seminoma, confined to the tissue sample. After a genital examination showing no alterations, a scrotal ultrasound was requested. This revealed a badly delimited hypoechogenic mass with microcalcifications in the left testis and a heterogeneous echostructure in the right testis, with hypoechogenic areas and some microcalcification. Bilateral orchiectomy was performed, with a pathological study compatible with residual scar tissue in the left testicle and focal findings of germ cell neoplasia, with no intratubular seminoma in the right testis. CONCLUSIONS: The suspicion of an extragonadal germ cell tumour with the "burned-out" phenomenon modifies the therapeutic attitude, which should begin with orchiectomy, followed by systemic chemotherapy and the surgery kept in reserve for those cases where residual malignant tissue persists.

Tumor de células germinales extragonadal con fenómeno "burned-out" simulando tumor retroperitoneal de estirpe neurogénico / Extragonadal germ cell tumour with the "burned out" phenomenon mimicking aretroperitioneal tumour of neurogenic origin

OBJETIVO: Describir un caso de metástasis retroperitoneal de un tumor de células germinales gonadal con fenómeno "burned-out" en un paciente de 35 años con sospecha diagnóstica de tumor retroperitoneal de estirpe neurogénico. MÉTODOS: Con la sospecha clínico-radiológica de tumor retroperitoneal de estirpe neurogénico se realiza exéresis de la tumoración, mediante abordaje por vía retroperitoneal. RESULTADOS: La histología demostró seminoma clásico con focos de seminoma atípico o anaplásico, confinado a la pieza quirúrgica. Tras comprobar exploración genital sin alteraciones, se solicita Ecografía escrotal, que evidencia en teste izquierdo una masa mal delimitada hipoecogénica con microcalcificaciones y en teste derecho ecoestructura heterogénea, con áreas hipoecogéncias y alguna microcalcificacion. Se realiza orquiectomía bilateral, con resultado histológico de restos cicatriciales en teste izquierdo y hallazgos focales de neoplasia de células germinales, no seminoma intratubular en teste derecho. CONCLUSIONES: Sospechar un tumor de células germinales extragonadal con fenómeno "burned-out" modifica la actitud terapéutica, que debería comenzar por orquiectomía, siguiendo con la quimioterapia sistémica y reservando la cirugía para los casos de persistencia de tejido maligno residual (AU)

OBJECTIVE: To describe a case of retroperitoneal metastasis of a gonadal germ cell tumour with the "burned-out" phenomenon in a 35 year old patient with a suspected diagnosis of retroperitoneal tumour of neurogenic origin. METHODS: With the clinical and radiological suspicion of retroperitoneal tumour of neurogenic origin the tumour was removed, via the retroperitoneal space. RESULTS: Pathology showed classic seminoma with foci of atypical or anaplastic seminoma, confined to the tissue sample. After a genital examination showing no alterations, a scrotal ultrasound was requested. This revealed a badly delimited hypoechogenic mass with microcalcifications in the left testis and a heterogeneous echostructure in the right testis, with hypoechogenic areas and some microcalcification. Bilateral orchiectomy was performed, with a pathological study compatible with residual scar tissue in the left testicle and focal findings of germ cell neoplasia, with no intratubular seminoma in the right testis. CONCLUSIONS: The suspicion of an extragonadal germ cell tumour with the "burned-out" phenomenon modifies the therapeutic attitude, which should begin with orchiectomy, followed by systemic chemotherapy and the surgery kept in reserve for those cases where residual malignant tissue persists (AU)

Disturbed synthesis of insulinlike growth factor I and its binding proteins may influence renal function changes in liver cirrhosis.

Insulinlike growth factor-1 (IGF-1) is an anabolic hormone synthesized by the liver upon stimulation by growth hormone (GH). IGF-1 exerts important effects on renal hemodynamics and renal sodium handling. The bioactivity of this hormone is influenced by its binding proteins (BP) of which IGF-BP3 favors retention in the capillary lumen while IGF-BP1 facilitates the transport to the target tissues. IGF-BP1 modulates the actions of IGF-1 on target cells including renal tubules. Although a number of reports have dealt with disturbances of the IGF-1/IGF-BP system in cirrhosis, no studies have yet addressed the relationship between alterations in this system and renal function changes in cirrhosis. In the present study we have included 20 patients with cirrhosis and 10 healthy subjects (control group). As compared with the controls, patients showed lower circulating levels of IGF-1 and IGF-BP3, higher IGF-BP1 levels, and a tendency to higher insulinemia and GH values. The index IGF-1 x IGF-BP1/IGF-BP3 (IGF-1-IGF-BP index, reflecting the accessibility of circulating IGF-1 to target cells) was higher in patients with ascites. IGF-1 directly correlated with renal blood flow (P < 0.05), with IGF-BP3 (P < 0.001) and inversely with the Pugh's score (P < 0.02). A negative correlation was found between IGF-1-IGF-BP index and fractional sodium excretion (P < 0.01) and between IGF-BP1 and urinary sodium excretion (P < 0.02). Our findings support the hypothesis that the disturbance of the IGF-1/IGF-BP axis may be related to the degree of renal vasodilation and renal sodium retention in cirrhotic patients.

Hemoperitoneum as a first manifestation of hepatocellular carcinoma in western patients with liver cirrhosis: effectiveness of emergency treatment with transcatheter arterial embolization.

Hemoperitoneum is a well-known form of hepatocellular carcinoma presentation and represents a frequent complication in countries with a high incidence of hepatocellular carcinoma, but it is rarely seen in Western countries. Our aim was to report the results and describe the arteriographic and CT-scan characteristics in a series of seven consecutive patients. They were admitted to our hospital because of hemoperitoneum due to ruptured tumor as a first manifestation of hepatocellular carcinoma, and the rupture was effectively controlled by transcatheter arterial embolization. From April 1989 to April 1998, 440 consecutive patients were admitted to our liver unit with the diagnosis of hepatocellular carcinoma and liver cirrhosis. Fourteen patients (3%) presented with acute hemoperitoneum due to tumor rupture as a first manifestation of hepatocellular carcinoma. We here report our experience in the group of patients treated by transcatheter arterial embolization. Mean age was 67.1+/-5 years (range, 61-73). All patients presented with sudden abdominal pain, abdominal distension, and four patients had symptoms of acute anemia. In all cases the ruptured tumor was subcapsular. The procedure was effective in the control of bleeding in all cases, without significant impairment in liver function or treatment-related deaths. In six of the seven patients, a self-limited postembolization syndrome was observed. Mean survival time was 273+/-488.7 days (range: 15-1290). Three patients survived more than six months but at the time of evaluation, only one patient was alive. In conclusion, the present results confirm that transcatheter arterial embolization is an effective and well-tolerated treatment in the management of hemoperitoneum due to ruptured hepatocellular carcinoma in patients with liver cirrhosis.

Outcome and hepatic hemodynamics in liver transplant patients with portal vein arterialization.

UNLABELLED: Few cases of successful portal vein arterialization in orthotopic and auxiliary liver transplantation have been reported. AIM: To evaluate the effect of portal vein arterialization on hepatic hemodynamics and long-term clinical outcome in three patients undergoing liver transplantation. METHODS: Two patients with extensive splanchnic venous thrombosis received an orthotopic liver transplant and one with fulminant hepatic failure received an auxiliary heterotopic graft. Portal vein arterialization was performed in all cases. RESULTS: One patient died 4 months after transplant and two are still alive. Auxiliary liver graft was removed 3 months post-transplant when complete native liver regeneration was achieved. Immediate post-transplant liver function was excellent in all cases. Only one patient developed encephalopathy and variceal bleeding owing to prehepatic portal hypertension secondary to arterioportal fistula 14 months after transplant. He was successfully treated by embolization of the hepatic artery. Hepatic hemodynamic measurements demonstrated a normal pressure gradient between wedged and free hepatic venous pressures in all cases. Liver biopsy showed acceptable graft architecture in two cases and microsteatosis in one. CONCLUSIONS: Liver transplantation with portal vein arterialization is an acceptable salvage alternative when insufficient portal venous flow to the graft is present. The double arterial supply does not imply changes in hepatic hemodynamics, at least in the early months post-transplant.

Intrahepatic arterioportal shunt: helical CT findings.

The purpose of this study was to characterize the appearance of intrahepatic arterioportal shunts (APS) on two-phase helical CT, with emphasis on the importance of the hepatic arterial-dominant phase (HAP) to demonstrate perfusion disorders. We review eight cases of APS diagnosed by helical CT in our institution from January 1996 to March 1997 and describe the CT findings that established diagnosis. Five of them were confirmed by angiography. In seven (87. 5 %) cases of APS we found early enhancement of the peripheral portal branches during the HAP of helical CT, whereas the superior mesenteric and splenic veins remained unenhanced. In five (62.5 %) cases of APS, transient, peripheral, triangular parenchymal enhancement was depicted during the HAP of helical CT; in four of these cases there was associated early enhancement of the portal branches. Helical CT can show perfusion alterations that might remain undiagnosed with conventional CT. An understanding of the hemodynamic changes that occur in APS can help in the interpretation of focal transient hepatic parenchymal enhancement and to differentiate APS from hypervascular tumors. We believe that the helical CT findings described herein are characteristic enough to suggest the diagnosis of APS.

Circulating adrenomedullin in cirrhosis: relationship to hyperdynamic circulation.

BACKGROUND/AIMS: Peripheral arterial vasodilation may be the key factor in the sodium and water retention of cirrhosis. The mechanism responsible for this vasodilation remains to be fully elucidated. Adrenomedullin is a novel peptide, highly expressed in cardiovascular tissues, with potent and long-lasting vasodilating activity. METHODS: The possible implication of adrenomedullin in the hemodynamic changes of cirrhosis has been investigated. We measured the plasma concentration of adrenomedullin in 20 cirrhotic patients and 11 healthy subjects. In addition, systemic, portal and renal hemodynamics, hormonal factors and renal function parameters were evaluated in the same patients. RESULTS: Circulating adrenomedullin was significantly higher in the group of patients with cirrhosis (72.1; 46-100 vs 21.6; 11-34 fmol/dl, respectively; p<0.02) and was directly correlated with the Pugh score (r: 0.6; p: 0.01), inversely correlated with the creatinine clearance (r: -0.6; p<0.01) and tended to inversely correlate with systemic vascular resistance index (r: -0.46; p: 0.07). There were no portal-peripheral differences in adrenomedullin levels. Transjugular intrahepatic portosystemic shunt insertion did not induce changes in the peripheral concentration of adrenomedullin, but baseline values of this hormone predicted the degree of hyperdynamic circulation after TIPS. CONCLUSIONS: Circulating adrenomedullin is increased in cirrhosis. These levels increase with the severity of the disease, especially in patients with hepatorenal syndrome. This peptide may contribute to vasodilation of cirrhosis.

[Thyroid clinical dysfunction in patients chronically treated with amiodarone]. / Disfunción tiroidea clínica en pacientes tratados crónicamente con amiodarona.

This is a prospective study of 132 patients, without previous thyroid dysfunction, chronically treated with amiodarone for cardiac arrhythmias, to determine the incidence of thyroid dysfunction. Age was 62 +/- 11 years (mean +/- SD); 54 were female and 78 male. The arrhythmia was supraventricular in 66%, ventricular in 26.5%, and both in 7.5%. Amiodarone dose was 2,390 +/- 65 mg/week, and follow-up 20 +/- 9 months (minimum 9 months). Thyroid status was evaluated at the onset and at regular intervals during follow-up by means of clinical indexes defined by Crooks et al and Billewicz et al. During follow-up 4 patients developed diagnostic indexes (two hyperthyroid and two hypothyroid) and seven more developed suggestive symptoms without reaching a diagnostic index. Biochemical serum determinations of thyroid function proved dysfunction in the four with diagnostic indexes, and were normal in the other seven. The prevalence of new thyroid dysfunction in patients chronically treated with amiodarone in our population is 3%, with equal incidence of hyper and hypofunction. This is the expected incidence for an area with adequate dietary iodine intake. The use of clinical indexes of thyroid dysfunction appear as a useful and economical means of following thyroid function in these patients, saving a large number of biochemical tests.