Esophageal leiomyosarcoma is classified as polypoid in
Esophageal leiomyosarcoma is classified as polypoid in 60% of cases and infiltrative in 40%. Yamashita et al reviewed seven cases of malignant fibrous histiocytoma in the esophagus; all the cases involved pedunculated or polypoid tumors. Those reports suggested that esophageal sarcomas usually present as a polypoid mass. Esophagography is a useful tool for evaluating the morphology of esophageal tumors. Endoscopic examination is useful for assessing the characteristics of the tumor and biopsy can be helpful in differentiating sarcomas from carcinoma. However, a superficial biopsy may yield a false-negative result, due to the esophageal mucosa overlying the tumor. In our case, we could not completely evaluate the morphology of the tumor by endoscopic examination or esophagography on account of the huge size of tumor, so we were unaware that it was a polypoid lesion until surgical resection.
Unlike carcinoma, esophageal sarcoma has a favorable prognosis following radical resection. DeMeester et al reported five cases of polypoid esophageal sarcoma. In their study, in spite of the large size (mean size 7.1 cm) of the tumors, they remained superficial within the esophageal wall, and nodal or distant organ CM-272 was absent in four of the five patients. Rocco et al reviewed 17 cases of esophageal leiomyosarcomas and curative resection was carried out in 11 patients. The results showed that curative resection offered a significant survival benefit to the patients and the authors suggested that esophagectomy should be the standard management for most patients with esophageal leiomyosarcoma. We reviewed previous reports of undifferentiated pleomorphic sarcoma, and the eight cases, including ours, are summarized in Table 1. In our case, although the cell type was uncommon (high-grade undifferentiated pleomorphic sarcoma), the clinical presentation was similar to that in previous reports. Although the tumor was huge, it remained superficial within the esophageal wall. However, in our case, the pre-operative images and endoscopic findings failed to confirm the degree of invasion of tumor completely; they showed only that the tumor was just at the opening of the esophagus. The total esophagectomy and total laryngectomy were performed to ensure adequate radical resection. After surgical resection, the patient remained disease-free at the most recent follow-up (1 year after surgery). The characteristics of intraluminal polypoid growth appear to allow endoscopic resection in certain selected patients. In some early cancer lesions which are small in size and with an obvious stalk, endoscopic polypoidectomy may be feasible.
Numerous modes of treatment, including surgical resection, radiation, and chemotherapy have been used either alone or in combination in limited cases. Chemotherapy does not appear to show any benefit in the treatment of esophageal sarcomas. In our case, the initial planned treatment was neo-adjuvant therapy for down-staging. But the neo-adjuvant chemotherapy with the MAID regimen only resulted in a partial response with an initial slight reduction in tumor size. Lokesh et al reported a case of spindle cell sarcoma of the esophagus in a 55-year-old woman managed with radical radiotherapy alone. However, the evidence for successful radiotherapy in esophageal sarcomas is limited and its role in treatment strategies for such lesions has not been well established.
Introduction Peribiliary cysts are cystic dilatations of the peribiliary glands, which are normal components of both the intra- and extrahepatic bile ducts. They are found mainly in patients with severe liver disease, such as alcoholic liver dysfunction, portal hypertension, and liver cirrhosis. There are most often multiple lesions in patients presenting with obstructive jaundice. We present here a patient with obstructive jaundice that was caused by a solitary peribiliary cyst compressing the extrabiliary tract.
Case report A series of image studies were then performed. Abdominal computed tomography (CT; Fig. 1) and sonography showed dilatation of the intrahepatic bile ducts and common hepatic duct, but not of the common bile duct. A 2 cm × 2 cm × 2.5 cm hypoechoic lesion was found in the hilar region. Magnetic resonance cholangiopancreatography (MRCP; Figs. 2 and 3) also showed a hypointense lesion in the hilar area with mild extension to both intrahepatic ducts, and a Klatskin tumor, Bismuth type IV, was strongly suspected. Endoscopic retrograde cholangiopancreatography (ERCP; Fig. 4) showed stricture of the extrahepatic duct at the level of the cystic duct with enhancement of the gallbladder and upstream dilatation of the bile ducts. Percutaneous transhepatic cholangiography (PTC; Fig. 5) also showed a stricture at the common hepatic duct with dilatations of the intrahepatic ducts.