PubMed · 8558680
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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/
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Of the six GATA transcription factors, GATA-4 and GATA-6 are expressed in the mouse and human adrenal with distinct developmental profiles. GATA-4 is confined to the fetal cortex, i.e. to the less differentiated proliferating cells, while GATA-6 is expressed both in the fetal and adult adrenal. In vitro, GATA-4 regulates inhibin-alpha and steroidogenic factor-1 implicated in normal adrenal function. GATA-6 probably has roles in the development and differentiation of adrenocortical cells, and in the regulation of steroidogenesis. GATA-4 expression is dramatically upregulated and GATA-6 downregulated in gonadotropin dependent mouse adrenocortical tumors. This is accompanied by the appearance of luteinizing hormone receptor (LHR). In vitro, GATA-4 transactivates LHR promoter, and gonadotropins upregulate GATA-4 levels. Human adrenal tumors occasionally express GATA-4, whereas GATA-6 levels are usually lower than normal.
A contemporaneous presentation of a second breast cancer in a mother and an extremity rhabdomyosarcoma (RMS) in her daughter led to the diagnosis of the Li Fraumeni syndrome (LFS). Although the association between LFS and RMS in young patients is well recognised 1 there are no guidelines as to how this knowledge should influence the optimal management of these patients. After reviewing the literature about the natural history of the LFS 2, the incidence of second malignancy (SMN) in RMS survivors 3-6 and the management of extremity RMS 7-9, we are concerned that contemporary RMS treatment, combining non-mutilating surgery with chemoradiotherapy, may be associated with an excessive SMN risk in LFS patients with advanced RMS. We question whether treatment should be individualised and, where possible and acceptable to the family, measures such as amputation should be the considered to attain local control for LFS patients with RMS as this will avoid the need for local radiotherapy without compromising long-term function and quality of life 10.
BACKGROUND: Today, the posterior retroperitoneoscopic technique has become a standard procedure in adrenal surgery. The procedure allows direct access to the adrenal glands, but it seems to be difficult because of the uncommon anatomic view. This study compares the learning period of the new procedure of "posterior retroperitoneoscopic adrenalectomy" in the primary invention phase and the secondary introductory phase in a different hospital 10 years later. MATERIALS AND METHODS: The analysis included 100 posterior retroperitoneoscopic adrenalectomies (PRA) and involved 50 procedures in each center. Group A consisted of 44 patients (14 males, 30 females; age: 48.7 +/- 14.5 years) undergoing surgery between 07/1994 and 8/1996 (24 right, 26 left; 8 Cushing adenomas, 14 Conn adenomas, 11 pheochromocytomas, 7 nonfunctioning adrenocortical adenomas, 10 ACTH-dependent adrenal hyperplasias). Group B consisted of 50 patients (12 males, 38 females; mean age 59.3 +/- 10.7 years) operated between 01/2004 and 01/2006 (28 right, 22 left tumors; 5 Cushing adenomas, 12 Conn adenomas, 4 pheochromocytomas, 29 nonfunctioning adrenocortical adenomas). All PRAs were performed with the patient in the prone position with 3-4 trocars placed caudally in the region of the 11th and 12th ribs. In group A, the surgical team developed the technique of PRA themselves. Before their first PRA, the surgical team of group B was introduced to the technique by the group A surgeons and afterwards were supervised continuously. RESULTS: No serious intraoperative or postoperative complication occurred in either group. Group A experienced 7 conversions to open surgery, whereas group B had one conversion and one early reoperation due to bleeding (P = 0.03; chi(2)-test). The mean operative time was 117 +/- 41 minutes versus 83 +/- 35 minutes (group A and B respectively; P < 0.001; t-test). Estimated blood loss was similar in the two groups (47.2 +/- 46.2 ml versus 54 +/- 16.3 ml, group A versus B, respectively; P = 0.36; t-test). CONCLUSIONS: The study demonstrates the feasibility, safety, and reproducibility of the new surgical method of PRA both when it is employed in the early phase of invention, as well as when performed by surgeon-learners. After comprehensive training, the operative time and conversion rate are dramatically reduced, allowing for a short learning period.