Erdheim–Chester disease is a rare disease with systemic non-Langerhans cell histiocytosis, the diagnosis of which with conventional imaging modalities is challenging. We describe a case of a 73-year-old woman who was referred with a progressive history of bilateral proptosis. The magnetic resonance imaging (MRI) orbit demonstrated bilateral orbital masses with optic nerve encasement. A subsequent 18F-FDG PET/CT scan showed multi-organ disease with involvement of the orbits, pericardium, aorta, pararenal fascia, and appendicular bones. Metabolically active, easily accessible areas were selected for CT-guided biopsy. The biopsy showed sheets of foamy histiocytes with the expression of CD 68 and CD 163 consistent with a diagnosis of Erdheim–Chester disease. The FDG PET/CT played a pivotal role in establishing the diagnosis with the assessment of disease extent and further guided in the targeted biopsy.
survival estimates and Cox proportional hazards model adjusted for covariates were used for analyses.
ResultsThe risks of recurrence of EOC increased steadily with increasing time from the start of primary treatment from 13.6% in 6-months to 71.0% after 12-months. In the final multivariate analyses, recurrence within 6 months of treatment was a significant independent predictor of poor OS in EOC patients (HR=7.23, 95%CI: 3.87-13.51, P<0.01). Conclusions Our study suggests that recurrence within 6months is an important prognostic factor that predicts poor OS in EOC. Early tumour recurrence may be a useful surrogate of overall survival and thus this information should be considered in the design of future tailored randomized controlled trials. Future strategies to improve OS in EOC patients should focus on identifying effective measures to prevent early tumour recurrence.
Splenogonadal fusion (SGF) is a rare anomaly, and to the best of our knowledge, none have mimicked a strangulated inguinal hernia. A 6-year-old boy presented with a painful left inguinal swelling mimicking a strangulated hernia but turned out to be a SGF, which was managed successfully with an inguinal procedure.
We present an unusual case of iatrogenic small bowel perforation in a woman with endometrial cancer. A 57-year-old postmenopausal woman with past history of total colostomy with loop ileostomy for ulcerative colitis, was referred to our department for evaluation and further management of suspected endometrial cancer following dilatation and curettage (D&C) elsewhere. The histopathology showed intestinal element which was attributed to metaplasia; however, no malignancy was identified in the biopsy specimen. Imaging carried out elsewhere after D&C showed thickened endometrium with suspicious small bowel infiltration. The patient was completely asymptomatic with no signs of bowel injury or peritonitis. After a complete evaluation, a class 1 extrafascial hysterectomy and bilateral salpingo-oophorectomy with segmental ileal resection and anastomosis and frozen section were performed. Intraoperatively, small bowel loop was found adherent to the posterior uterine wall extending up to the cervix with no obvious sign of any uterine or bowel perforation. The final histopathology revealed endometrioid adenocarcinoma grade 1, stage IA, with tumor located at the fundus along with cervical isthmic perforation on the posterolateral wall with full thickness ileum perforation. Uterine perforation at D&C is a known complication, but a silent bowel injury presenting with intestinal tissue in endometrial biopsy is very unusual. A high degree of suspicion is required to detect such complications at D&C especially in postmenopausal women with past history of abdominal surgery.
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