Pseudoangiomatous Stromal Hyperplasia Presenting as Gigantomastia - A Case Report and Review of Literature

Abstract

Gigantomastia is a rare breast disease with massive bilateral enlargement of breast causing physical and psychosocial disability for the patient. Diffuse pseudoangiomatous stromal hyperplasia causing gigantomastia is a very rare presentation. We present a case of 45-year-old female with bilateral gigantomastia and cyclical mastalgia, who underwent bilateral mastectomy, postoperatively diagnosed with pseudoangiomatous hyperplasia. Gigantomastia is a rare condition characterised by excessive breast tissue growth resulting in massive enlargement of both breast. An arbitrary definition for gigantomastia is breast size more than 1.5 kg or when the breast tissue comprises 3 % or more of patients total body weight. Gigantomastia is accompanied by skin ulceration, postural problems, back pain, traction nerve injury and social stigmata. It is physically and psychosocially disabling for the patient. The aetiopathogenesis of gigantomastia is poorly understood, but generally classified as idiopathic, drug induced (penicillamine, cyclosporine) and due to hormonal imbalance (gestational and pubertal).1 Pseudoangiomatous stromal hyperplasia (PASH) is a benign mesenchymal proliferative disease of the breast which is usually an incidental finding accompanying other breast pathology like fibroadenoma, fibrocystic disease, breast cancer, gynaecomastia etc. It was first described by Vuitch, Erlandson and rosen (1986). Ibrahim et al. reported the occurrence of incidental microscopic PASH in breast specimens as around 23 %.2 Around 200 cases of PASH presenting as a tumoral palpable mass is there in literature. Another very rare and under recognised presentation is as diffuse PASH with bilateral or unilateral massive breast enlargement. Here we report a case of diffuse PASH with gigantomastia and a review of literature.