Hepatic leiomyosarcoma: ultrastructural study and review of the differential diagnosis.

A primary leiomyosarcoma of the liver in a 12 year old female is reported. It is the eleventh such tumor to be recorded, the first to be documented in an adolescent and the first to be studied electron microscopically. By light microscopy the well differentiated portions of the tumor were characteristic of leiomyosarcoma. Ultrastructurally a minority of tumor cells contained myofilaments along with cytoplasmic dense bodies and marginal dense plaques, the most distinctive subcellular markers of smooth muscle cells. A brief review of the differential diagnosis is provided.

Pediatric kidney transplantation for cystinosis.

In an experience of more than 700 kidney transplants, three recipients are known to have had congenital cystinosis. A cadaver kidney transplant in a 10 yr old child with nephropathic cystinosis functioned well for 7 1/2 yr before it was rejected and the graft was free of recurrent cystinosis after that interval. This prolongation of life was associated with marked accumulation of cystine in the patient's thyroid gland, presenting as a mass in the neck. If additional longevity is achieved in this patient, abnormal intracellular cystine accumulation may be expected in other host tissues. Retransplantation was carried out with the father as donor, accepting the possibility of increased cystine levels in the retransplant. This patient's age of 18 yr identifies him as one of the longest known survivors of infantile nephropathic cystinosis.

Cervical esophagostomy in dogs: endoscopic, radiographic, and histopathologic evaluation of esophagitis induced by feeding tubes.

Prolonged tube feeding is frequently necessary in patients with head and neck cancer, facial trauma, or central nervous system disease. The advantages of cervical esophagostomy over nasogastric and gastrostomy tube feeding are established. One frequently overlooked complication of tube feeding is distal esophagitis and subsequent stricture formation. This complication has been widely reported in the medical literature although the pathophysiologic mechanism has in part been speculative. We have completed a prospective, randomized, controlled animal study in an attempt to delineate the mechanism of feeding tube esophagitis and to devise a method of avoiding it. Twelve dogs underwent cervical esophagostomy and were studied by endoscopy, contrast fluoroscopy, gross and microscopic pathology. The esophageal effects of placement of polyethylene and silicone rubber esophagostomy tubes in the customary position, through the cardioesophageal junction into the stomach, were compared with placement of the distal end of the tube only as far as the midesophagus. Midesophageal placement of silicone rubber tubes greatly reduced esophageal injury inherent is esophagostomy tube feeding. A new esophagostomy feeding tube is presented with advantages over previously used tubes.

Yolk sac carcinoma (endodermal sinus tumor): ultrastructure and histogenesis of gonadal and extragonadal tumors in comparison with normal human yolk sac.

Human yolk sac carcinomas have been studied only twice with the electron microscope, and have never been compared at this level with normal human yolk sac. In the present study, the ultrastructural features of three primary ovarian yolk sac carcinomas, omental metastases from one of these, and a primary retroperitoneal yolk sac carcinoma in a male are reported, as are the ultrastructural findings in human yolk sac from normal 7- and 12-week gestations. The most prominent feature of the tumors is the presence of voluminous basement membrane material (the nature of which is confirmed by indirect enzyme-labeled antibody technique in one case) in both intra-and extracellular location, corresponding to the PAS-positive hyaline globules seen in these tumors by light microscopy. The tumor cells are also demonstrated to produce this material in tussue culture. Although basement membrane has not been described previously in normal human yolk sac at 8 and 10 weeks' gestation, it was present in the 7-week specimen which we studied, suggesting that its production may be a feature of only very young sac. Other ultrastructural findings are also similar in human yolk sac carcinoma, normal human yolk sac, and rodent yolk sac and yolk sac carcinomas. Thus, these studies confirm the suggested germ cell-derived yolk sac origin of the human tumor.

Association of carcinoma with congenital cystic conditions of the liver and bile ducts.

Reported cases of carcinoma arising in association with congenital cystic conditions of the liver and bile ducts are tabulated with respect to the specific type of congenital abnormality. The occurrence of carcinoma in solitary nonparasitic cysts of the liver or as a complication of polycystic liver disease is distinctly rare. Carcinoma will arise with a frequency of approximately 1% in congenital hepatic fibrosis, 4% in choledochal cyst and 7% in congenital cystic dilatation of the intrahepatic bile ducts. Those congenital conditions exposing the epithelium to the bile directly are at greater risk for the development of malignancy. Carcinoma in this clinical setting occurs several decades earlier than otherwise expected.

Epithelioid sarcoma: case report with ultrastructural review, histogenetic discussion, and chemotherapeutic data.

A case of abdominal wall epithelioid sarcoma, studied by light and electron microscopy over a 3-year period, is presented. Ultrastructurally, there appear to be two types of tumor cells, light and dark, which differ by virtue of a heavier concentration of microfibrils and dilated rough endoplasmic reticulum in the dark cells. Both tumor cell types contain prominent Golgi systems, abundant free ribosomes, and numerous pinocytotic vesicles. The ultrastructural characteristics of the tumor cells resembel those of epithelioid cells of experimental human granulomas, as well as those of normal human synovium. A multifaceted relationship between histiocytes and synovial cells is demonstrated and it is concluded that the tumor is probably derived from mesenchymal reserve cells capable of differentiating a long histiocytic or synovial lines. Preliminary chemotherapeutic data are reviewed.

Squamous cell carcinoma originating in an hepatic cyst. Case report with a review of the hepatic cyst-carcinoma association.

A case of squamous cell carcinoma originating in an hepatic cyst is presented. A review of the literature reveals five other cases of carcinoma arising within congenital intrahepatic cysts; three adenocarcinomas and two squamous cell carcinomas. Neoplastic transformation may also be seen in association with Meyenburg complexes, considered by some to be the structures from which congenital cysts arise.