Propranolol as an alternative treatment option for pediatric lymphatic malformation.

2013

Ozeki M, Kanda K, Kawamoto N, Ohnishi H, Fujino A, Hirayama M, Kato Z, Azuma E, Fukao T, Kondo N.

Source

Department of Pediatrics, Gifu University Graduate School of Medicine, Gifu University.

Abstract

Lymphatic malformation (LM), which was previously termed lymphangioma, is a rare congenital malformation of the lymphatic system and its treatment is still challenging. Propranolol (beta blocker) has been recently developed as a first-line treatment of infantile hemangioma. Our study aimed to assess the effect of propranolol on pediatric LM and the relationship between its effectiveness and vascular endothelial growth factor (VEGF) family members (VEGF-A, C and D). Six Japanese patients with LM (age range: 10 months-19 years old; 2 macrocystic, 2 microcystic and 2 combined type) were enrolled. Oral propranolol was administered at 2 mg/kg/day. The efficacy of propranolol for LM was evaluated by the rate of volume change as calculated from MRI imaging and by symptomatic improvement. In all patients, there were no significant side effects. Patients 3 and 5 were classified as objective responders with tumor volume reduction of 30.6% and 22.9%, respectively, at 24 weeks. Patient 1 showed 8% tumor volume reduction and patient 6 showed symptomatic improvement, hence, both were classified as minimal responders. The other two patients were classified as non-responders. Plasma VEGF-A, C, and D levels were significantly higher in the LM group than in the controls (all P < 0.01 by Mann-Whitney test). VEGF-A and D levels at 24 weeks were significantly lower than those at pre-treatment (P = 0.031, 0.047 by Wilcoxon matched pairs test). Though further trials with this treatment must be carried out, we propose that propranolol may be an alternative therapy option for intractable LM.

PubMed

Hyaline vascular type of unicentric Castleman's disease with proliferation of glomerular endothelial cells.

Dec 2012

Wakabayashi K, Asanuma K, Takeda Y, Arakawa A, Osawa I, Horikoshi S, Yao T, Tomino Y.

Abstract

Castleman's disease (CD) is a lymphoproliferative disorder of uncertain etiology. It can be classified histopathologically as hyaline vascular (HV) or plasma cell (PC) type; and clinically as unicentric or multicentric. Multicentric CD mostly manifests as the PC type and is often associated with a variety of systemic complications, although renal complications are uncommon. We present here a case of HV type, unicentric CD with a variety of systemic and renal complications, including the proliferation of glomerular endothelial cells. Various cytokines are thought to be involved in the etiology of this disease, especially interleukin-6 (IL-6) and vascular endothelial growth factor (VEGF), which are the main cytokines inducing systemic complications. It has been reported that most PC types and/or multicentric types of CD show increasing levels of serum IL-6 and VEGF, as well as systemic symptoms. Although the current case showed high serum IL-6 and VEGF levels as well as systemic symptoms, the pathological and clinical type were of the HV type and unicentric type, respectively. After immunosuppressive therapy, the serum levels of IL-6 and VEGF returned to the normal range, and systemic complications, including renal involvement, also improved. We report the possibility of pathogenesis via the serum and the pathology of this rare case.

PubMed