Bilateral Gigantomastia, Multiple Synchronous Nodular Pseudoangiomatous Stromal Hyperplasia Involving Breast and Bilateral Axillary Accessory Breast Tissue, and Perianal Mammary-Type Hamartoma of Anogenital Mammary-Like Glands

Abstract

Mammary-type fibroepithelial lesions involving ectopic breast and anogenital region are rare and usually coexist with normal orthotopic breast. We present what we believe to be a unique case of synchronous bilateral gestational gigantomastia resembling fibrous mastopathy, synchronous rapidly growing pregnancy-associated nodular pseudoangiomatous stromal hyperplasia involving right breast and bilateral axillary ectopic breast tissue, and metachronous perianal mammary-type hamartoma involving anogenital mammary-like glands occurring in a 34-year-old patient with facioscapulohumeral muscular dystrophy. Also, we review the literature concerning these lesions.

Management of recurrent bilateral multifocal pseudoangiomatous stromal hyperplasia (PASH)

“…10 In support of this theory, there have been a few case reports of rapid bilateral breast enlargement associated with PASH during pregnancies, most likely in response to rising hormone levels. 11,12 The histologic features of PASH has also been shown to resemble those of intralobular stroma during the luteal phase of the menstrual cycle, when progesterone secretion is high. 13 Despite its hyperplastic nature, the condition is not associated with increased risk of breast malignancy.…”

Surgical correction of polymastia and polytelia with an uncommon presentation

“…The accessory mammary tissue has the same capacity to undergo benign and malignant alterations as the normal mammary tissue 11 ; however, the incidence of carcinoma in the accessory breast tissue is rare, corresponding to only 0.3% of the breast carcinomas 12 . There are multiple reports of benign alterations, such as fibroadenomas 4, [13][14][15] or hyperplasias 16,17 , but the incidence of these alterations in supernumerary breasts is uncertain. Polymastia may present in an aberrant form that is not accompanied by a nipple or areola and is commonly mistaken for lipoma, lymphadenopathy, or hidradenitis 11 .…”

<p>Progression of the role of CRYAB in signaling pathways and cancers</p>

“…Breast cancer (BC) is one of the most common cancers for females and the leading cause of cancer-related death in females globally, with a high incidence rate and mortality 70. BC may have evolved from a process of continuous progression of hyperplasia of mammary gland (HMG) 71,72. According to reports, the expression of CRYAB was significantly up-regulated in HMG,73 while it was also highly expressive in BC, such as basal-like, triple-negative breast cancer (TNBC), and mammary metaplastic carcinoma 74–76.…”