PubMed · 8558680
The source did not provide an abstract. Follow the original record for more information.
Explore connections, maps & timelines
M Stumvoll, A Fritsche, M Wehrmann, F Dammann, H D Becker, M Eggstein. 1996. A functioning adrenocortical hemangioma.. https://pubmed.ncbi.nlm.nih.gov/8558680/
Cite the original work for its findings. Save a collection to share your selection of sources.
KEEP EXPLORING
UNCOMMON MALIGNANCY: Adrenocortical carcinoma is a very rare malignancy with poor prognosis. Median survival ranges from 12 to 25 months. Most clinicians recommend aggressive surgical management of either local or recurrent and metastatic disease. ANTICORTISOL AGENTS: Mitotane, the most tested agent against inoperable and metastatic adrenocortical carcinoma, procures overall response rates of 20 to 25%, but recent data do not support its use in the adjuvant setting. CHEMOTHERAPY: The efficacy of cytotoxic chemotherapy is low but new agents or associations (with platinum salts), new concepts (dose-intensification, MDR (the chemoresistance gene) reversing agents) may be useful and five some hope in this difficult disease. RADIOTHERAPY: The role of radiation therapy must be developed.
One kb of the 5'-flanking region of the human ACTH receptor gene was isolated and partially characterized. Transient transfections with hGH reporter confirmed the promoter activity in Y1 cells. Putative elements for transcription factors involved in regulation were noted in the sequence. The promoter activity of some of the constructs is responsive to a treatment by forskolin, due to the presence of several CRE-like sequences.
Twenty of 67 children registered on the International Registry of Childhood Adrenocortical Tumors between May 1988 and December 1994 had small adrenocortical tumors (defined for this study as measuring < or = 200 cm3 and/or weighing < or = 100 g). We reviewed the records of these 20 patients to characterize the clinical and pathologic findings and outcomes of children with small adrenocortical tumors. Median patient age was 2 years (range, 4 months to 5 years). There was only one boy. All had clinical signs of virilization, and seven had signs or symptoms of Cushing syndrome. A median 5.5 months (range, 1-40 months) had elapsed between the first signs of endocrine dysfunction and diagnosis. All tumors were surgically resected. Tumor volume was 3.3-195 cm3 (median, -8.7 cm3), and weight was 3.7-100 g (median, 36 gm Tumor samples were histologically reviewed in 18 cases. Eight were adenomas, and 10 were carcinomas (6 low grade and 4 high grade). Pathology records described tumor with diagnostic features of adrenocortical carcinoma in two patients. One patient received mitotane for 8 months after surgery. Only one patient had recurrent disease, which was detected 6 months after diagnosis and proved rapidly fatal. Another has been lost to follow-up. The remaining 18 patients are alive with no evidence of disease at a median 2.3 years (range, 6 months to 6.1 years) after diagnosis. Our data suggest that children with small adrenocortical tumors have an excellent prognosis with surgery as the sole therapy, regardless of tumor histiotype.