Recurrent Extreme Bilateral Gigantomastia Caused by Pseudoangiomatous Stromal Hyperplasia (PASH) Syndrome: A Case Report.

Plastic and reconstructive surgery. Global open 2023 Vol.11(1) p. e4571

Parisi P, Vinci F, Di Meglio F, Marruzzo G, De Luca A, Ascione A, Vittori L, Ribuffo D

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Abstract

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.

추출된 의학 개체 (NER)

유형영어 표현한국어 / 풀이UMLS CUI출처등장
해부 breast 유방 dict 7
시술 breast reduction 유방성형술 dict 1
해부 breast tissue scispacy 1
약물 Tamoxifen C0039286
tamoxifen
scispacy 1
질환 PASH → Pseudoangiomatous Stromal Hyperplasia C2931031
Pseudoangiomatous stromal hyperplasia
scispacy 1
질환 Pseudoangiomatous stromal hyperplasia C2931031
Pseudoangiomatous stromal hyperplasia
scispacy 1
질환 sarcoma C1261473
Sarcoma
scispacy 1
질환 breast PASH syndrome scispacy 1
질환 gigantomastia C0020565
Hypertrophy of Breast
scispacy 1
질환 palpable C0522499
Palpable
scispacy 1
질환 low-grade sarcoma angiomatous scispacy 1
질환 breast specimens' histological scispacy 1
질환 breast PASH scispacy 1
기타 patient scispacy 1

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