1/358. Isolated polycystic liver disease not linked to polycystic kidney disease 1 and 2.Autosomal dominant polycystic liver disease occurs commonly in association with autosomal dominant polycystic kidney disease, types 1 and 2. It may also exist as a separate entity, genetically distinct from autosomal dominant polycystic kidney disease types 1 and 2, as has been recently established to exist in a Belgian family. We report here a large Argentinian family of Spanish-Belgian ancestry with autosomal dominant polycystic liver disease, where proximal and distal markers for both polycystic kidney disease 1 and 2 failed to demonstrate genetic linkage. The data support the notion that polycystic liver disease and autosomal dominant polycystic kidney disease may have separate chromosomal loci.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
2/358. Fibropolycystic disease of the hepatobiliary system and kidneys.This complicated case of fibropolycystic disease of the hepatobiliary system and kidneys was ably and incisively analyzed by Professor Sheila Sherlock. Her clinical acumen was revealed by her ability to differentiate congenital hepatic fibrosis, Caroli's disease, and adult polycystic disease of the liver and kidney. Interesting histologic features of this case included hepatic fibrosis with intact limiting plates anc central veins and the presence of bile plugs in the ducts, but the absence of bile statsis in the parenchyma. A percutaneous transhepatic cholangiogram demonstrated the dilated intrahepatic and extrahepatic ducts. Washing out the "gunk" from the biliary tract by T-tube drainage has great limitations in this type of case. Therefore, Dr. Adson suggested irrigation of the biliary ductal system using tubed placed transhepatically, plus a wide choledojejunostomy. Dr. Sherlock questioned this surgical approach. The use of chenodeoxycholic acid for this "gunk" was suggested. In spite of the dilated ducts and pathologic changes in the liver, the patient was not jandiced and did not have stones in her biliary tract. The genetics of this patient's problems was discussed.- - - - - - - - - - ranking = 0.75keywords = kidney (Clic here for more details about this article) |
3/358. Portal hypertension due to extensive hepatic cysts in autosomal dominant polycystic kidney disease.liver cysts are a well-recognized feature of autosomal dominant polycystic kidney disease (ADPKD) and occur in 77% of patients more than 60 years old. Serious sequelae, however, are rare, the two most common complications being pain and cyst infections. Portal hypertension has been reported in ADPKD due to the rare presence of congenital hepatic fibrosis. We report a case of ADPKD in a patient who had portal hypertension due to distortion of portal vein and venules by extensive hepatic cysts.- - - - - - - - - - ranking = 0.625keywords = kidney (Clic here for more details about this article) |
4/358. Neonatal detection and evaluation of infantile polycystic disease by gray scale echography.Infantile polycystic disease (IPCD) is an uncommon pathologic entity involving the kidneys and liver. Gray scale echography can detect this pathologic process within the kidneys, despite the presence of renal failure. In addition, the sonic study may detect associated hepatic abnormalities even though isotopic liver scan is normal. These capabilities make the ultrasonic examination uniquely suited for evaluating patients with IPCD.- - - - - - - - - - ranking = 0.25keywords = kidney (Clic here for more details about this article) |
5/358. Evaluation and management of benign, non-congenital tongue masses in children.Lingual tumors are rare, primarily benign, lesions in the pediatric population. Congenital lesions, such as hemangiomas, lymphatic malformations, dermoids, hamartomas and thyroglossal ducts cysts, are seen more commonly. Primary, non-congenital lingual neoplasms are less common in children. We present three patients with benign lingual neoplasms. Evaluation, management, pathology and follow-up are discussed.- - - - - - - - - - ranking = 0.019731418903178keywords = neoplasm (Clic here for more details about this article) |
6/358. Papillary cystadenoma: a rare tumor of the minor salivary glands.Papillary cystadenoma of the minor salivary glands is a rare benign neoplasm that clinically resembles mucous cysts. Characteristic histological features are diagnostic. However, salivary gland histology is particularly difficult to interpret. Primarily, as further clinical and histological differential diagnoses have to take into account the well-differentiated cystic mucoepidermoid carcinoma and the papillary cystic type of acinic cell carcinoma, both malignant neoplasms of the salivary glands. We report on a 39 year old female with a bluish cystic lesion at the buccal mucosa, which occurred 14 years after the excision of a similar appearing, histologically proven mucous retention cyst at the same location. The histology of this tumor, however, revealed a papillary cystadenoma. Although rare, benign and malignant salivary gland neoplasms occur in minor salivary glands, and are clinically indistinguishable from mucous retention cysts. The dermatologist should be familiar with these differential diagnoses, since different therapeutic consequences result from an early diagnosis obtained by excision and histological examination of oral cystic tumors.- - - - - - - - - - ranking = 0.029597128354767keywords = neoplasm (Clic here for more details about this article) |
7/358. Prostatic cancer with huge cystic degeneration.This report details a case of prostatic cancer associated with cystic degeneration. A cystic mass may occur in the pelvis for various reasons. A congenital cyst is associated with an abnormality in the paramesonephric (mullerian) duct or mesonephric (wolffian) duct. An acquired cyst can be classified into three main types, retention, malignant and parasitic, however a cyst accompanying prostatic cancer is rare. In this particular case the size of the cyst under imaging was approximately 15 cm in diameter; this makes it one of the largest among those previously reported.- - - - - - - - - - ranking = 0.047792491845501keywords = cancer (Clic here for more details about this article) |
8/358. Multicystic autoimmune thyroiditis-like disease associated with hiv infection. A case report.BACKGROUND: Human immunodeficiency virus (hiv) infection and resulting acquired immunodeficiency syndrome (AIDS) may involve virtually every organ system, including the endocrine glands. Thyroid dysfunction most commonly reflects advanced disease and generally resembles euthyroid sick syndrome. Rarely do opportunistic infections, hemorrhage, neoplasms and drugs account for alterations in thyroid tissue. Multiple lymphoepithelial cysts of parotid gland and thymus have been identified, but similar findings in thyroid gland have not been reported. CASE: A 41-year-old, hiv-seropositive woman, asymptomatic for seven years, developed a squamous cell carcinoma of the cervix with local-regional extension. At the same time, bilateral complex thyroid cysts and high titers of antimicrosomal antibodies (1/6,400) were detected. Ultrasound-guided fine needle aspiration biopsy of the thyroid showed a heterogeneous lymphocytic population with a reactive appearance and occasional groups of epithelial cells with an immature squamous pattern, along with cytologic features of autoimmune thyroiditis. Immunocytochemistry was positive for CD20, CD3 and CD5. Immunoglobulin heavy chain gene rearrangement by polymerase chain reaction from cytologic material showed a polyclonal lymphoid population. External radiotherapy resulted in a significant reduction in the pelvic lesion. Four months after diagnosis, abdominal ultrasound displayed multiple hepatic metastasis, the patient's condition rapidly deteriorated, and she died about a month later. CONCLUSION: This case had unique features and probably represented an AIDS-related lesion and distinct entity.- - - - - - - - - - ranking = 0.009865709451589keywords = neoplasm (Clic here for more details about this article) |
9/358. Endometrial carcinoma presenting as hematometra mimicking a large pelvic cyst.Large pelvic cysts are commonly seen in gynecologic practice; their heterogeneous origin is reflected in their pleomorphic clinical features. We report the case of a 64-year-old multiparous postmenopausal woman with an unusual manifestation of endometrial adenocarcinoma that presented as hematometra mimicking a large pelvic cyst. In this case, hematometra was well demonstrated by transabdominal sonography, but transvaginal sonography allowed better visualization of the endometrial lining and suggested the correct diagnosis of endometrial cancer. Abnormal vaginal bleeding or hematometra in postmenopausal women should lead to assessment of the endometrial mucosa. Transvaginal sonography can be used to visualize neoplastic lesions in the endometrium when hematometra is detected through transabdominal sonography.- - - - - - - - - - ranking = 0.0079654153075834keywords = cancer (Clic here for more details about this article) |
10/358. Spontaneous rupture of a nonparasitic liver cyst complicated by intracystic hemorrhage.a case of spontaneous rupture of simple liver cyst complicated by intracystic hemorrhage is described. This rare condition was detected in a 61-year-old man who underwent left trisegmentectomy of liver under a suspected diagnosis of cystadenocarcinoma because of elevated serum levels of carbohydrate antigen (CA) 19-9 and DUPAN 2, and the presence of an intracystic structure. The resected specimen showed a benign liver cyst with intracystic hematoma and high levels of CA19-9 and DUPAN 2 in the cystic fluid. It is suggested that cyst rupture may increase serum levels of tumor markers whose levels are high in the cystic fluid, and that repeated observations of an intracystic structure may be the most reliable method to distinguish intracystic hemorrhage from cystic neoplasm.- - - - - - - - - - ranking = 0.009865709451589keywords = neoplasm (Clic here for more details about this article) |
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