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Background: Mature cystic teratomas of the ovary (MCTO) are a type of germ cell tumor that may contain well-differentiated tissues developed from three germ cell layers and constitute about 20% of ovarian germ cell tumors. They are rare ovarian tumors with an annual incidence variable from 1.2 to 14.2 cases per 100,000 that occur mainly in women of reproductive age. They are frequently benign with a slow growth rate, even if they can undergo a malignant transformation in about 1-2% of cases., Case Presentation: Here, we present the case of an elderly woman referred to gynecological first aid for acute abdominal pain and showing a giant and unusual MCTO at rapid growth with malignant transformation in squamous cell carcinoma (FIGO stage Ia). The patient underwent pelvic mass removal trough emergency longitudinal midline laparotomic incision with intraoperative frozen pathologic examination. A complete surgical staging during the first surgery was performed. After about 9 years of follow-up, she died of non-oncological reasons without recurrence., Conclusions: Present case highlights that CMTO with malignant transformation should always be suspected in elderly women in presence of pelvic mass at rapid growth, even if in absence of other clinical and ultrasonographic signs of malignancy. An intraoperative frozen pathologic examination may drive the best treatment., (© 2022. The Author(s).)

Background: The burden of pregnancy complications associated with well defined, already established systemic rheumatic diseases preexisting pregnancy such as rheumatoid arthritis, systemic lupus erythematosus or scleroderma is well known. Systemic rheumatic diseases are characterized by a long natural history with few symptoms, an undifferentiated picture or a remitting course making difficult a timely diagnosis. It has been suggested that screening measures for these diseases could be useful but the impact of unrecognized systemic rheumatic disorders on pregnancy outcome is unknown. The objective of the study was to evaluate the impact of previously unrecognized systemic autoimmune rheumatic on the incidence of preeclampsia and fetal growth restriction (FGR)., Methods: A longitudinal cohort-study with enrolment during the first trimester of pregnancy of women attending routine antenatal care using a two-step approach with a self-reported questionnaire, autoantibody detection and clinical evaluation of antibody-positive subjects. The incidence of FGR and preeclampsia in subjects with newly diagnosed rheumatic diseases was compared to that of selected negative controls adjusting for potential confounders by logistic regression analysis., Results: The prevalence of previously unrecognized systemic rheumatic diseases was 0.4 % for rheumatoid arthritis (19/5232), 0.25 % (13/5232) for systemic lupus erythematosus, 0.31 % (16/5232) for Sjögren's syndrome, 0.3 % for primary antiphospholipid syndrome (14/5232) and 0.11 % (6/5232) for other miscellaneous diseases. Undifferentiated connective tissue disease was diagnosed in an additional 131 subjects (2.5 %). The incidence of either FGR or preeclampsia was 6.1 % (36/594) among controls and 25.3 % (50/198) in subjects with unrecognized rheumatic diseases (excess incidence = 3.9 % (95 % CI = 2.6-9.6) or 34 % (95 % CI = 22-44) of all cases of FGR/preeclampsia). The incidence of small for gestational age infant (SGA) was higher among subjects with unrecognized rheumatic diseases (41/198 as compared to 46/594; adjOdds Ratio = 3.1, 95 % CI =1.96-4.95) than in controls. The excess incidence associated with unrecognized rheumatic diseases was 2.7 % (95 % CI = 1.5-4) or 25 % (95 % CI = 12.8-34.8) of all SGA cases., Conclusions: Unrecognized autoimmune systemic rheumatic disorders are associated with a significant proportion of preeclampsia and fetal growth failure, suggesting that their role in the etiology of adverse pregnancy outcome is probably undervalued.

Objective: To evaluate the impact of preclinical systemic autoimmune rheumatic disorders on pregnancy outcome., Methods: In this longitudinal cohort study, patients were enrolled during the first trimester of pregnancy if they reported having had connective tissue disorder symptoms, were found to be positive for circulating autoantibodies, and on clinical evaluation were judged to have a preclinical or incomplete rheumatic disorder. The incidence of fetal growth restriction (FGR), preeclampsia, and adverse pregnancy outcomes in patients with preclinical rheumatic disorders was compared with that in selected controls, after adjustment for confounders by penalized logistic regression. Odds ratios (ORs) and 95% confidence intervals (95% CIs) were calculated., Results: Of 5,232 women screened, 150 (2.9%) were initially diagnosed as having a suspected rheumatic disorder. After a mean ± SD postpartum follow-up of 16.7 ± 5.5 months, 64 of these women (42.7%) had no clinically apparent rheumatic disease and 86 (57.3%) had persistent symptoms and positive autoantibody results, including 10 (6.7%) who developed a definitive rheumatic disease. The incidences of preeclampsia/FGR and of small for gestational age (SGA) infants were 5.1% (23 of 450) and 9.3% (42 of 450), respectively, among controls, 12.5% (8 of 640) (OR 2.7 [95% CI 1.1-6.4]) and 18.8% (12 of 64) (OR 2.2 [95% CI 1.1-4.5]), respectively, among women with no clinically apparent disease, and 16.3% (14 of 86) (OR 3.8 [95% CI 1.9-7.7]) and 18.6% (16 of 86) (OR 2.3 [95% CI 1.2-4.3]), respectively, among those with persisting symptoms at follow-up. Mean ± SD umbilical artery Doppler pulsatility indices were higher among women with no clinically apparent disease (0.95 ± 0.2) and those with persisting symptoms (0.96 ± 0.21) than in controls (0.89 ± 0.12) (P = 0.01 and P < 0.001, respectively)., Conclusion: In our study population, preclinical rheumatic disorders were associated with an increased risk of FGR/preeclampsia and SGA. The impact of these findings and their utility in screening for FGR/preeclampsia need to be confirmed in population studies., (© 2016, American College of Rheumatology.)

Background: In dialysis-related amyloidosis, beta2-microglobulin accumulates as amyloid fibrils preferentially around bones and tendons provoking osteoarthritis. In addition to the pathologic role played by the amyloid fibrils, it can be speculated that a pathogenic role is also played by the high concentrations of soluble beta2-microglobulin because it is toxic for certain cell lines like HL60 and mitogen for other cells such as the osteoclasts. The discovery that beta2-microglobulin can influence the biology of certain cells may lead to the assumption that it might affect neuronal cells that are quite sensitive to amyloidogenic proteins in the oligomeric state. Such a concern might be supported by clinical evidence that haemodialysis is associated with the risk of a cognitive impairment., Methods: The cytotoxicity of beta2-microglobulin on the SH-SY5Y neuroblastoma cells was assayed by the MTT test. The beta2-microglobulin concentration was determined in the cerebrospinal fluid of four different patients by means of immunonephelometry and western blot., Results: Oligomeric beta2-microglobulin is cytotoxic for the SH-SY5Y cells at a concentration that can be easily reached in the plasma of patients on haemodialysis. However, the beta2-microglobulin concentration, measured in the cerebrospinal fluid of a haemodialysis patient, appears to be independent of its plasma concentration and is maintained under the lower limit of cytotoxicity we have determined in the cell culture., Conclusions: Although beta2-microglobulin is potentially neurotoxic, it is unlikely that this protein plays a role in the pathophysiology of cognitive impairment observed in haemodialysis patients due to the protective effect of the blood brain barrier that maintains a low concentration of beta2-microglobulin in the cerebrospinal fluid.

In this article, we describe a case of ganglioneuroma observed in a 27-year-old woman. The tumor was found in an unusual location, the parapharyngeal space. The lesion was asymptomatic and was surgically excised after a fine-needle aspiration biopsy that was considered inadequate. The lesion measured 4 x 3 cm and was capsulated. Two distinct cells groups were identified: ganglion cells and Schwann cells placed in a loose myxoid stroma. Immunohistochemical stains (performed with the following monoclonal antibodies: high-molecular-weight cytokeratins, vimentin, neuron-specific enolase, neurofilaments, and S100) confirmed the diagnosis of ganglioneuroma. The pathologic findings and a review of recent literature are discussed.