[Polycythaemia in a patient with uterus myomatosus]. / Polycytemie bij een patiënte met een uterusmyoom.

BACKGROUND: Myomatous erythrocytosis syndrome (MES) is characterised by a combination of polycythaemia, uterus myomatosus and the normalisation of erythrocyte count after hysterectomy. CASE DESCRIPTION: A 58-year-old postmenopausal woman was referred to the gynaecologist with symptoms of vaginal blood loss, increased abdominal circumference and pollakiuria. Physical examination indicated her uterus was enlarged to the size of a 24-week gestation. Endometrial malignancy was excluded and ultrasound showed a myoma. In consultation with the patient a hysterectomy was planned. Pre-operative blood tests showed increased haemoglobin levels (14.2 mmol/l). No indications of polycythaemia vera or secondary polycythaemia were found after which the diagnosis of MES was made. Haemoglobin levels normalised after hysterectomy without any further intervention. CONCLUSION: MES is common, although relatively unknown. Its pathophysiology is most likely based on ectopic production of erythropoietin by leiomyoma tissue. The combination of polycythaemia and uterus myomatosus should alert clinicians to this syndrome, especially as polycythaemia normalises after hysterectomy.

IgG4-related disease: a disease we probably often overlook.

IgG4-related disease (IgG4-RD) is an increasingly recognised entity characterised by tumefied lesions that can affect multiple organs. Awareness of IgG4-RD is important, as it has been shown to mimic other diseases and may result in irreversible organ damage if not treated. If suspected, immunostaining for IgG4-positive plasma cells is essential for diagnosis and revision of old biopsies may be necessary.

A rare cause of chylothorax and lymph edema.

Chylothorax is a form of pleural effusion rarely caused by metastasis of solid tumors. Because chylothorax causes complaints by local compression of the lung, as well as weight loss resulting from loss of triglycerides, it needs thorough investigation. We present the case of gastric carcinoma presenting with a chylothorax and unilateral lymph edema. Although rare, the differential diagnosis of chylothorax should include gastric cancer even in the absence of upper abdominal complaints.

[Diffuse panbronchiolitis in an Asian woman with severely obstructed pulmonary disease]. / Diffuse panbronchiolitis bij een Aziatische vrouw met een ernstig obstructief gestoorde longfunctie.

A 41-year-old woman was referred for severely obstructed pulmonary disease 4 years after she stopped smoking. Treatment for chronic obstructive pulmonary disease (COPD) had no effect and lung function worsened in the following years. The clinical, radiological and pathological characteristics led us to the diagnosis of diffuse panbronchiolitis. She was treated with clarithromycin. After 1 year, major improvement was seen in clinical, radiological and spirometric features. Diffuse panbronchiolitis is a rare obstructive disorder that is usually seen in people of Japanese descent. Patients respond well to treatment with low-dose macrolide treatment.

Combined E-cadherin, alpha-catenin, and beta-catenin expression is a favorable prognostic factor in endometrial carcinoma.

Cell adhesion molecules, such as epithelial cadherin (E-cadherin), might be involved in the processes of tumor invasion and differentiation. The aim of this study was to investigate the expression of E-cadherin, alpha-catenin, and beta-catenin in endometrial carcinoma and to determine the prognostic value of these factors. We have investigated the expression of E-cadherin, alpha-catenin, and beta-catenin by immunohistochemistry in 225 endometrial carcinomas. The correlation between the E-cadherin and the catenins and their correlation with several histologic and clinical parameters were analyzed. Negative E-cadherin, alpha-catenin, and beta-catenin expression was observed in 44%, 47%, and 33% of endometrial carcinomas, respectively, and was correlated with histologic FIGO grade 3 (P < 0.001). Negative E-cadherin expression was more often observed in nonendometrioid endometrial carcinomas (NEECs) than in endometrioid carcinomas (75% versus 43%; P= 0.04). Combined positive E-cadherin, alpha-catenin, and beta-catenin expression was an independent positive prognostic factor for survival in patients with grade 1-2 carcinomas (P= 0.02). Negative E-cadherin expression was found to be associated with histologic grade 3 and with NEEC. Combined positive E-cadherin, alpha-catenin, and beta-catenin expression was a significant prognostic factor.

The use of Gore-Tex membrane for adhesion prevention in tethered spinal cord surgery: technical case reports.

OBJECTIVE: The incidence of retethering caused by postoperative adhesions at the repair site after initial tethered spinal cord surgery is not uncommon. To assess the effectiveness of a Gore-Tex membrane in preventing these adhesions, only clinical radiological and experimental animal evaluation has been reported. In this report, we describe two cases in which Gore-Tex membrane was implanted at the initial untethering surgery and in which we were able to confirm the real effectiveness of the Gore-Tex membrane during a second operation. METHODS: In the first patient, Gore-Tex membrane was used for dural repair in the untethering surgery of the split spinal cord malformation. Because of the suspicion of a thickened filum terminale, repeated surgery was indicated 10 months after the initial procedure. In the second patient, Gore-Tex membrane was implanted during the initial untethering surgery for a lipomyeloschisis and a dermal sinus. Because of a persistent fistula of the dermal sinus, a second operation was necessary 1 year after the first operation. RESULTS: During the repeated surgery, a thorough inspection of the implanted Gore-Tex membrane revealed no adhesions between the Gore-Tex membrane and the intradural content in both cases. CONCLUSION: We support the use of Gore-Tex membrane in the prevention of postoperative dural adhesions in the repair of spinal dysraphism.