Adenoma malignum (minimal deviation adenocarcinoma) of the cervix responsive to hormonal treatment

Adenoma malignum (AM) is a rare variant of cervical adenocarcinoma with an unfavorable prognosis despite radiation therapy, surgery, or chemotherapy either alone or in combination. Hitherto, however, the effectiveness of hormonal therapy for this condition has not been evaluated. We report on a patient with cervical AM treated with progesterone before surgery. The progesterone therapy resulted in a complete clinical response and partial surgical response. Later on the treatment was changed to tamoxifen because of side effects of the progesterone treatment. The patient is still without evidence of disease 42 months after the start of the hormonal therapy. The progesterone receptor analysis on the biopsy was clearly positive. This is, to our knowledge, the first case of an AM responsive to hormonal treatment. Furthermore, this is the first case of an AM with bone metastases at the time of primary diagnosis.     

Humoral and cellular effects of the K+-channel activator cromakalim in man

Summary The effect of cromakalim, a K⁺-channel activator, on the plasma renin-angiotensin-aldosterone system, catecholamines and -atrial natriuretic peptide, and on the intraerythrocyte concentration and transmembrane fluxes of Na⁺ and K⁺ has been investigated in 18 normal male subjects, in a double-blind parallel study. After a run-in period on placebo for 1 week, the subjects were treated either with placebo (n=6) or cromakalim (n=12) for 1 week.Plasma renin activity was significantly increased during cromakalim. No effect of cromakalim on plasma angiotensin II, aldosterone, adrenaline, noradrenaline and -atrial natriuretic peptide was demonstrated. The intra-erythrocyte K⁺ concentration was decreased during cromakalim administration and Ca²⁺-dependent K⁺-channels in red blood cells were increased.     

CA 125 concentrations in ovarian 'chocolate' cyst fluid can differentiate an endometriotic cyst from a cystic corpus luteum.

In a prospective study, the concentrations of CA 125, 17 beta-oestradiol and progesterone were assayed in 52 consecutive ovarian cysts, laparoscopically suspected to be endometriomas. Cysts with dark brown 'chocolate' fluid (n = 42) were excised by CO2-laser endoscopy. Cysts with clear fluid were diagnosed by pathology as follicular cysts (n = 5) or pseudoperitoneal cysts (n = 5). Fluids (n = 53) aspirated during echo-guided puncture for in-vitro fertilization (IVF) were assayed simultaneously. Of the 42 women undergoing a cystectomy, the clinical diagnosis of an endometrioma was confirmed by pathology in only 68%, the other cases being corpora lutea (27%) or follicular cysts (5%). Cyst fluids from corpora lutea had lower CA 125 concentrations (< 1000 IU/ml) together with high 17 beta-oestradiol concentrations (> 2000 pg/ml) and/or high progesterone concentrations (> 100 ng/ml). Endometriotic cysts had either very high CA 125 concentrations (> 10,000 IU/ml) as occurred in 78% or lower CA 125 concentrations (< 1000 IU/ml) together with low 17 beta-oestradiol and/or progesterone concentrations. 'Chocolate' fluid-containing cysts aspirated during IVF had similar concentration profiles of CA 125, 17 beta-oestradiol and progesterone and the diagnoses derived from these concentrations were not contradicted in 19/27 women undergoing a laparoscopy within 4 months. In eight women, however, with high CA 125 concentrations in their cyst fluid, no endometriotic cysts were found at laparoscopy. Only 68% of cysts containing 'chocolate' material were endometriotic cysts and CA 125 could be useful in making this diagnosis. This method is recommended when dark brown fluid is aspirated in IVF.     

Short limbed dwarfism,genital hypoplasia,sparse hair,and vertebral anomalies: a variant of Ellis-van Creveld syndrome?

A male newborn with acromesomelic short limbed dwarfism, genital hypoplasia, and vertebral anomalies is reported. As the child had an important number of clinical and radiological symptoms seen in patients with Ellis-van Creveld syndrome, we raise the question of whether he may represent a variant example of this syndrome despite the absence of cardinal symptoms such as postaxial polydactyly and ectodermal changes (nail hypoplasia).     

Congenital muscular dystrophy associated with lethal arthrogryposis multiplex congenita

Summary Two unrelated patients with severe arthrogryposis multiplex congenita (AMC) who died perinatally, are presented. In both, postmortem examination revealed an intact nervous system and striking dystrophic muscle changes, consistent with congenital muscular dystrophy (CMD). Few similar cases have been reported before, but since the condition is not well known, it seems probable that in the past many have been labeled as mere multiple malformations. The possibility of an underlying muscular disorder, either primary myopathic or neurogenic should be considered in any patient with early lethal AMC. Our findings confirm that the fetal akinesia-arthrogryposis sequence is a nonspecific clinical syndrome resulting from various causes of muscular inactivity in utero. The main objective of this report is to provide reasonable guidelines on how to approach the problem of classification. We favor a pathogenetic approach, depending upon careful sampling of the central nervous system and skeletal muscles at autopsy.     

In-vitro adhesion of endometrium to autologous peritoneal membranes: effect of the cycle phase and the stage of endometriosis

BACKGROUND: Endometrium can adhere to autologous peritoneum. This study was undertaken to determine the effect of the menstrual cycle phase and the presence and stage of endometriosis on in-vitro adhesion of endometrium onto autologous peritoneum. METHODS: This was performed in an academic medical research centre. Sixty-seven subfertile women with a visually normal pelvis (n = 18) and with biopsy-proven endometriosis (n = 49) were included. Endometrial and peritoneal biopsies were obtained at laparoscopy during menstrual, follicular and luteal phase. Endometrium was cultured in vitro with autologous peritoneum, followed by fixation, paraffin embedding, serial sectioning, hematoxylin-eosin and immunohistochemical staining. Endometrial-peritoneal adhesion was evaluated using light microscopy. RESULTS: Endometrial-peritoneal adhesion was observed in approximately 80% of the adhesion assays and was not affected by the phase of the cycle, or by the presence and stage of endometriosis. The continuity of the mesothelial layer was disrupted at the attachment sites. Epithelialization was observed along the edges to integrate the endometrial implant. After adhesion, histological changes were observed within and below the implant. CONCLUSIONS: Endometrium obtained during menstrual, follicular or luteal phase appears to have a similar potential to implant in vitro on autologous peritoneum, and this adhesion process is not affected by the stage of endometriosis.     

5q- anomaly in a patient with disseminated teratoma

A 5q- anomaly associated with other chromosome anomalies was found in the infiltrated bone marrow of a patient with a highly malignant teratoma originally located in the mediastinum. There was no evidence of a second malignancy, and it is likely that the 5q- anomaly was, indeed, associated with the malignant teratoma cells.     

Membrane filtration test for rapid presumptive differentiation of four Candida species

A rapid enzymatic two-step test for the presumptive differentiation of four Candida species commonly occurring in various clinical samples is described. The technique involves membrane filtration of a liquid sample, followed by preincubation of the membrane filter on Sabouraud glucose agar supplemented with ticarcillin-clavulanic acid to yield microcolonies. In a separate assay step, parts of the filter are placed on absorbent pads impregnated with fluorogenic 4-methylumbelliferyl (4-MU) enzyme substrates (4-MU-N-acetyl-beta-D-galactosaminide, 4-MU-phosphate, 4-MU-pyrophosphate, and 4-MU-beta-D-galactoside) in combination with 0.1% digitonin acting as a membrane permeabilizer. The membrane filter in contact with the assay medium is incubated to allow cleavage of the enzyme substrate, resulting in fluorescent microcolonies under long-wavelength UV light. This approach, tested on 301 clinical samples, is able to presumptively differentiate C. albicans, C. glabrata, C. krusei, and C. tropicalis and to distinguish them from other Candida spp. in about 9 to 11 h. Overall agreement with the conventional methods of 94.4% (one Candida species present in the sample) to 83.8% (multiple Candida spp. present) was obtained. The false-negative rates with reference to identification by traditional methods were 1.3% (single species) and 3.8% (multiple species).