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.

Recurrent extreme bilateral gigantomastia caused by pseudoangiomatous stromal hyperplasia (PASH) syndrome: a case report / Parisi, Paola; Vinci, Federica; Di Meglio, Filippo; Marruzzo, Giovanni; De Luca, Alessandro; Ascione, Andrea; Vittori, Luigi; Ribuffo, Diego. - In: PLASTIC AND RECONSTRUCTIVE SURGERY – GLOBAL OPEN. - ISSN 2169-7574. - 11:1(2023). [10.1097/GOX.0000000000004571]

Recurrent extreme bilateral gigantomastia caused by pseudoangiomatous stromal hyperplasia (PASH) syndrome: a case report

Parisi, Paola
Primo
;
Vinci, Federica
Secondo
;
Di Meglio, Filippo;Marruzzo, Giovanni;De Luca, Alessandro;Ascione, Andrea;Vittori, Luigi
Penultimo
;
Ribuffo, Diego
Ultimo
2023

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.
2023
PASH; gigantomastia; breast; pseudoangiomatous; stromal; hyperplasia
01 Pubblicazione su rivista::01i Case report
Recurrent extreme bilateral gigantomastia caused by pseudoangiomatous stromal hyperplasia (PASH) syndrome: a case report / Parisi, Paola; Vinci, Federica; Di Meglio, Filippo; Marruzzo, Giovanni; De Luca, Alessandro; Ascione, Andrea; Vittori, Luigi; Ribuffo, Diego. - In: PLASTIC AND RECONSTRUCTIVE SURGERY – GLOBAL OPEN. - ISSN 2169-7574. - 11:1(2023). [10.1097/GOX.0000000000004571]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1668118
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