A patient with a juxtaglomerular cell tumor with histological vascular invasion.

BACKGROUND: A 51-year-old woman was referred to the Hypertension Clinic of L'Hôtel-Dieu de Québec Hospital, University of Québec Hospital Centre, with hypertension. Her hypertension had been evolving for approximately 30 years and was refractory to maximum doses of four antihypertensive agents. Routine blood testing revealed mild hypokalemia. INVESTIGATIONS: Physical examination, urine and blood analyses including measurement of renin and aldosterone levels, echocardiography, fundoscopy, abdominal-pelvis CT scan and histopathology studies. DIAGNOSIS: Juxtaglomerular cell tumor with vascular invasion. MANAGEMENT: Radical nephrectomy, and follow-up visits to monitor blood pressure and renin levels.

Circulating endothelial progenitor cells and vascular anomalies.

Recent findings regarding pathways of stem/progenitor cell involvement in adult blood vessel growth (postnatal vasculogenesis) suggest new theories for the pathogenesis of vascular anomalies. The somatic growth of vascular malformations and the mysterious pattern of proliferation and involution in infantile hemangioma can no longer be purely understood through the paradigm of angiogenesis. Molecular signals for postnatal vasculogenesis are being discovered in numerous animal models of cancer and ischemia, yet little research has addressed the importance of vasculogenesis in the growth of vascular anomalies. In this review, we discuss early studies that have investigated stem/progenitor cell involvement in the pathophysiology of infantile hemangioma and other congenital vascular anomalies.

Postmenopausal intravenous leiomyomatosis with high levels of estradiol and estrogen receptor.

BACKGROUND: Intravenous leiomyomatosis is a rare variant of leiomyoma. CASE: The patient was a 49-year-old gravida 3, para 3 woman with menopause at age 46. She presented with a history of syncope. Vaginal examination revealed an enlarged and elastic-soft mass of the uterus. A pelvic ultrasound, computed tomography scan, and magnetic resonance imaging showed a heterogeneous, irregularly shaped 8- to 10-cm tumor. In addition, the inferior vena cava was almost completely occluded. Cardiac ultrasound demonstrated a mobile mass in the right atrium. The serum estradiol was 208 pg/mL (normal 0-59). Intravenous leiomyomatosis with cardiac extension was diagnosed preoperatively. A resection of the intracardiac and intracaval mass and a subtotal hysterectomy with bilateral salpingo-oophorectomy were performed. The uterine tumor weighed 600 g, and the cordlike intravascular tumor extending from the internal iliac vein into the right ventricle was 40 cm long and weighed 60 g. Pathologic examination confirmed intravenous leiomyomatosis with no evidence of atypia. The level of estrogen receptor in the tissue was 140 fmol/mg protein. The postoperative course was uneventful, and she has been in good health for 17 months after the operation. CONCLUSION: We report a case of intravenous leiomyomatosis extending into the right ventricle treated with a one-stage operation. It is possible that a high concentration of serum estradiol and high level of tissue estrogen receptor are related to the intravenous leiomyomatosis.

Angiolymphoid hyperplasia with eosinophilia involving skeletal muscle.

A vascular tumour involving the trapezius muscle is described. In addition to proliferating blood vessels with perithelial cuffing, there was a distinctive stroma of lymphoid aggregates, plasmacytoid cells, some mast cells and large numbers of eosinophils, many with a perivascular distribution. This solely intramuscular lesion was considered to be an example of angiolymphoid hyperplasia with eosinophilia. The aetiology and pathogenesis are discussed.