Recurrent Extreme Bilateral Gigantomastia Caused by Pseudoangiomatous Stromal Hyperplasia (PASH) Syndrome: A Case Report. Issue 1 (24th January 2023)

• Record Type: Journal Article
• Authors: Parisi, Paola; Vinci, Federica; Di Meglio, Filippo; Marruzzo, Giovanni; De Luca, Alessandro; Ascione, Andrea; Vittori, Luigi; Ribuffo, Diego
• Is Part Of: Plastic and reconstructive surgery. Volume 11:Issue 1(2023)
• Page Start: e4571
• Publication Date: 2023-01-24
• Subjects: Surgery, Plastic -- Periodicals; Surgery, Plastic -- Periodicals; Reconstructive Surgical Procedures -- Periodicals; 617.95205
• DOI: 10.1097/GOX.0000000000004571
• Languages: English
• ISSNs: 2169-7574

Abstract

Abstract : Summary: Pseudoangiomatous stromal hyperplasia (PASH) of the breast is a rare and benign medical condition in which the breast tissue is affected by an abnormal myofibroblastic proliferation, which mimics a low-grade sarcoma angiomatous proliferation. PASH usually presents itself either as a palpable mass or as an incidental diagnosis during breast specimens' histological examination. A few cases have been reported in the literature of a diffuse form of breast PASH syndrome in which the clinical presentation is a bilateral form of gigantomastia without palpable masses. In such cases, the optimal surgical management is still debated due to a significant risk of relapse after breast reduction. Mastectomy seems to be the endpoint of this condition in relapsing cases. Recent studies report a good outcome with a Tamoxifen regimen when surgery cannot be performed, supporting a hormonal component for the etiology of the condition. This study reports on an extremely rare case of bilateral, rapid, and severe PASH in a young patient, presenting as a truly disabling gigantomastia that forced the patient to use a wheelchair due to the excessive breast weights (25 kg the right breast and 21 kg the left). We describe her complicated medical history, her diagnosis, and our course of treatment.