An unusual presentation of primary sclerosing cholangitis.

This case report describes the unusual presentation of a patient who had findings which were initially suggestive of a type IV choledochal cyst. Her liver biopsy demonstrated biliary cirrhosis. She was treated with endoscopic retrograde cholangiopancreatography and biliary stent exchanges over one year. Her cholangiogram one year later demonstrated resolution of the biliary cystic dilation which led to her initial diagnosis, with beading and stricturing of the hepatic ducts consistent with primary sclerosing cholangitis. Liver-associated enzymes and physical findings also improved. A liver biopsy one year later demonstrated a marked improvement in hepatic fibrosis with no evidence of cirrhosis.

Acute hepatic sequestration in sickle cell disease.

Sickle cell anemia is a disease that affects one out of every 600 African Americans. It is often debilitating and can cause many physical restrictions to individuals with the disease. The disease has many complications which can be vexing for patients and their physicians. The hepatic complications attributed to vascular occlusion encompass a variety of clinical syndromes of which the relationship among clinical presentation, biochemical findings, and histologic features remains unclear. The conditions range from the self-limiting hepatic right upper quadrant syndrome (hepatic crisis) to the potentially lethal intrahepatic cholestasis and acute hepatic sequestration syndromes. Few cases have been documented, and there have not been many sizable studies on acute hepatic sequestration in sickle cell disease. This case is useful for clinicians who are not familiar with the intrahepatic vaso-occlusive syndromes in sickle cell disease. It provides insight into the presentation, diagnosis, and management of these syndromes.

An unusual presentation of metastatic esophageal adenocarcinoma presenting as thigh pain.

The association between esophageal adenocarcinoma and distant skeletal muscle metastasis is extremely rare. Only three cases have been previously reported in the literature. All reported involvement of the gastroesophageal junction. We describe a 58 year-old Caucasian man who presented with worsening right hip pain for nine months. Computerized axial tomography (CT) scan demonstrated a 10 cm by 8 cm by 12 cm intra-pelvic mass involving the right iliacus muscle with central destruction of the right mid-ileum. CT-guided biopsy of the right hip mass demonstrated poorly differentiated carcinoma. CT scan of the chest revealed a 5 cm by 4 cm circumferential mass involving the distal esophagus. Endoscopic biopsy showed poorly differentiated adenocarcinoma. Immunohistochemical analysis of each specimen correlated for pancytokeratin. Final diagnosis was primary esophageal adenocarcinoma with distant metastasis to the right ileum and iliacus muscle. We review distinctions between esophageal adenocarcinoma and adenocarcinoma of the gastroesophageal junction. A brief discussion of diagnostic modalities and treatment options are provided.