Pseudomyxoma peritonei: a case of mucinous adenocarcinoma of the appendix presenting as inguinal hernia.

Pseudomyxoma peritonei is a rare neoplastic condition characterized by diffuse collections of gelatinous fluid associated with mucinous implants on the peritoneal surfaces and omentum. Typical presentations include suspected acute appendicitis, increasing abdominal girth, new onset hernia and in women, an ovarian mass. The exact pathological origin, classification, and ideal treatment have been the subject of debate in the literature. Although optimum treatment is debatable, most expert opinion favors extensive surgical debulking with or without adjuvant therapy. We present a case of a 51-year-old man who presented with an inguinal hernia that was, in fact, secondary to pseudomyxoma peritonei. It is best practice, we believe, that any mucoid fluid encountered during hernia repair should be recovered and, along with the hernial sac, be assessed histologically.

Pheochromocytoma associated with polycythemia: case report.

Secondary polycythemia has been noted in association with various neoplasms. An erythropoiesis stimulating factor (erythropoietin) has been demonstrated in the fluid or tissues obtained from most of these neoplasms and erythropoietin levels were found to be elevated in the serum and returned to normal after resection of these tumors. Recently, the potential of pheochromocytoma to produce a wide variety of hormones and neurotransmitters such as growth hormone, motilin, ACTH, atrial natriuretic factor (ANF) and others has been shown. Although elevated hematocrit has been observed in association with pheochromocytomas, the occurrence of absolute polycythemia in such cases is very rare. In this report, we describe a patient with a long history of hypertension and cardiac dysrhythmia as well as polycythemia which was secondary to pheochromocytoma. The patient's blood pressure normalized and the polycythemia regressed after resection of the tumor. Increased release of erythropoietin is the most favored explanation for this rare association. Pheochromocytoma should be included in the differential diagnosis of secondary polycythemia.

Testosterone-secreting adrenal adenoma that contained crystalloids of Reinke in an adult female patient.

We describe a testosterone-secreting adrenal adenoma that contained crystalloids characteristic of Leydig cells in a 55-year-old woman with virilization, in whom results of a steroid biochemical profile were normal except for a substantially increased serum testosterone level. After right adrenalectomy, the serum testosterone level returned to normal and has remained within the normal range 2 years postoperatively. Histologic examination confirmed the diagnosis of a testosterone-secreting adrenal adenoma with crystalloids of Reinke specific for Leydig cells. The exact pathogenesis of these testosterone-secreting adrenal adenomas is still unclear. Although crystalloids of Reinke have been identified in only three cases, their absence does not exclude a Leydig cell character in the rest of the reported cases. Our case provides additional support for the hypothesis that Leydig cells are present in the adrenal gland and may have an active role in the pathogenesis of testosterone-secreting adrenal tumors; thus, such cells should be sought in every case.

Idiopathic pulmonary hemosiderosis and alveolar hemorrhage syndrome: case report and review of the literature.

The presence of widespread hemorrhage from the microvasculature of the lung into the alveolar spaces defines what is called the "alveolar hemorrhage syndrome", which can occur in association with a wide variety of clinical disorders. The cardinal manifestations of this syndrome include: hemoptysis, unexplained anemia and diffuse alveolar infiltrates on chest roentgenograms. Since the pulmonary features are similar, the diagnosis usually depends on the clinical, laboratory and pathologic evaluations. Early diagnosis and treatment is crucial since the occurrence of pulmonary hemorrhage in this syndrome may represent a catastrophic event with fatal consequences. Idiopathic pulmonary hemosiderosis (IPH) has been identified as a cause of alveolar hemorrhage in a small number of cases, mainly by exclusion criteria. We report a case of a 70-year old man who presented with a 40-year history of intermittent hemoptysis and bilateral upper lobes alveolar infiltrates proved to be secondary to idiopathic pulmonary hemosiderosis. Although the lung apices are frequently spared in IPH, they were the site of the infiltrates in our case. To our knowledge, our patient had the longest survival time ever reported in the literature in adult IPH. A brief review of some of the disorders commonly associated with alveolar hemorrhage is also presented.

Erythromycin induced Torsades de Pointes: case report and review of the literature.

We report a case of an 82 year old woman with bilateral pneumonia who developed repeated episodes of polymorphic ventricular tachycardia with QT prolongation (Torsades de Pointes) after intravenous infusion of erythromycin. After discontinuation of erythromycin, the QT interval returned to normal and there was no recurrence of this arrhythmia. The association of intravenous erythromycin and this potentially fatal ventricular arrhythmia has been described in 15 similar cases reported in the literature. Both in vitro and in vivo studies have shown that erythromycin exerts electrophysiologic effects on the cardiac muscle similar to that of class IA antiarrhythmic drugs and that cross sensitivity may exist between this class of drugs and erythromycin. The definition, pathophysiology, etiology, clinical and electrocardiographic feature, and prevention and treatment of "Torsades de Pointes" are described in this article.

Carcinoma of the extrahepatic bile duct.

We present a case of a 70-year old American Indian who had a previous cholecystectomy for symptomatic cholelithiasis and who presented, one year later, with progressive painless jaundice and a dilated common bile duct. Work-up revealed an adenocarcinoma of the extrahepatic bile duct. In this article we discuss the common presentation, work-up, and treatment of carcinoma of the bile ducts.