Treatment of malignant Leydig cell tumor

Article Abstract:

Much of the mass of the testis consists of the seminiferous tubules, from which the sperm cells begin their long journey. Within the seminiferous tubules are the small clusters of Leydig cells, also called the interstitial cells. These cells are responsible for manufacturing the male sex hormone testosterone. Tumors arising from the Leydig cells are quite uncommon; only about 250 cases have been reported. In general, these Leydig cell tumors are benign, but a small fraction, roughly 7 to 10 percent, are cancerous. When the disease is in fact malignant, prognosis is poor. About 20 percent of the patients with malignant Leydig cell tumor already have metastatic disease spread to distant sites of the body at the time of initial diagnosis. Another 40 percent will develop metastatic disease within two years. The average survival is around two years. The authors add to the existing case histories another patient, a 68-year-old man who had developed Leydig cell tumor in 1978. At that time, the patient's right testicle was removed. Eight years later, the patient complained of a sense of abdominal fullness with severe pain on the day of admission. An abdominal aneurysm was suspected, but a tumor mass was discovered upon surgery. Microscopic examination showed a poorly differentiated cancer, but the diagnosis became certain when comparison was made with slides of the patient's tumor from eight years prior. The undifferentiated mass was a metastatic Leydig cell tumor. The cancer was surgically removed; chemotherapeutic treatment was not given because patient's symptoms had resolved and because no effective treatment for this cancer is known. Symptoms returned the following year, however, and the patient was found to have multiple metastases in the abdomen, chest, and near the spine. The patient was treated with doxorubicin and mitotane to no avail. This case illustrates that malignant Leydig cell tumor may recur even following a long period after removal of the primary tumor. Furthermore, in this case the cancer was unresponsive even to high doses of doxorubicin. In the rare instances of malignant Leydig cell tumor, the disease appears to be untreatable and fatal. (Consumer Summary produced by Reliance Medical Information, Inc.)

Testis, Testicular cancer

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Endocardial fibroelastosis in infants with hydrops fetalis

Article Abstract:

Hydrops fetalis is the abnormal accumulation of large amounts of clear, watery fluid in the body tissues of the developing fetus. It can occur in fetuses with heart defects or malformations, congestive heart failure, or in those with Rh positive blood having mothers with Rh negative blood. In the latter case, it is called hemolytic disease of the newborn, or erythroblastosis fetalis, and it causes anemia by destroying red blood cells. Endocardial fibroelastosis is another condition that can occur in newborn infants with heart defects or malformations. It causes the membranes that form the inner lining of the heart to become very thick. It is not known whether endocardial fibroelastosis is a specific disease, or if it occurs as a result of heart malformations or heart failure. If endocardial fibroelastosis is a result of heart malformations, it would be expected to develop in infants with hydrops fetalis, because hydrops fetalis occurs in infants with heart malformations. To investigate this further, the medical records from 1,589 fetal autopsies were reviewed. Thirty-four cases of hydrops fetalis were identified. Endocardial fibroelastosis occurred in 10 of the 34 infants with hydrops fetalis. Congestive heart failure was believed to cause hydrops fetalis in 8 of the 10 infants with endocardial fibroelastosis. Also, endocardial fibroelastosis was not observed in any of the infants without congestive heart failure. It is concluded that endocardial fibroelastosis is likely to occur as a result of heart malfunction or malformation. (Consumer Summary produced by Reliance Medical Information, Inc.)

Abnormalities, Heart, Hydrops fetalis

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Mucopolysaccharidosis I presenting with endocardial fibroelastosis of infancy

Article Abstract:

Endocardial fibroelastosis (ESE) is a heart condition characterized by a thick, noncompliant left ventricle, the chamber that pumps blood into the circulation. ESE can develop after a viral infection, because of a heart defect, a compromised blood supply to the endocardium, or poor lymph drainage of the heart. Two cases describe infants with ESE as the initial symptom of mucopolysaccharidosis I (Hurler syndrome), a genetic condition that causes an abnormal accumulation of mucopolysaccharides in the tissues. Congestive heart failure developed in one patient who died at nine moths of age. Postmortem tissue analysis indicated a mucopolysaccharide storage disorder affecting the muscle cells of the heart. The second infant was born with mild anomalies and abnormal head circumference, and developed recurrent upper respiratory tract infections. She was also developmentally delayed. The infant was found dead in her crib at eight months of age after having a mild upper respiratory infection. Autopsy findings confirmed a diagnosis of mucopolysaccharidosis I. A second infant born to the same family appeared developmentally normal, but physically resembled her deceased sister. Further evaluation revealed that she had Hurler syndrome with similar thickening of the left ventricular wall. These cases suggest that infants with ESE should be evaluated for mucopolysaccharide storage disorders. (Consumer Summary produced by Reliance Medical Information, Inc.)

Mucopolysaccharidosis, Mucopolysaccharidoses

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