Tuberous Breast: Revised Classification and a New Hypothesis for its Development

Abstract

The tuberous breast classification proposed by Grolleau does not account for a minor form of the deformity characterized by isolated nipple-areola protrusion with a normal breast base. We have observed this minor form in six patients with normal breast shape and in ten patients with hypertrophic breasts. We propose an anatomical and physiological hypothesis for the development of the mammary gland. The breast is subjected to hormonal influences as early as the prepubertal period. These influences result in thrusting forces with both horizontal (estrogen) and vertical (progesterone) vectors, unfortunately not always balanced and harmonious. Close observation of the anomaly in our patients substantiated the basic anatomical defect, namely, the structural congenital dermal weakness of the nipple-areola complex (NAC) already described in all forms of tuberous breast deformity. This weakness explains the morphologic anomaly and confirms that all types of tuberous breast deformity constitute a spectrum of a single entity. It indicates also that the classification of tuberous breasts should include, in addition to the three types (types I-III) already described, a fourth type (type 0) to describe isolated simple areola protrusion, either permanent or intermittent, that is associated with a normal mammary base. The revised classification of tuberous breasts and the proposed hypothesis of breast development allow better assessment of all possible variants of breast morphologic anomalies. In the six cases of isolated herniated NAC, the deformity was corrected through a perinipple approach (not circumareolar), with adequate stable correction of the deformity and minimal scarring.

Disorders of Sexual Development in Adult Women

“…Progestins are avoided initially in order to avoid the development of tubular breasts. 46 In adult women, the risk of endometrial cancer is higher than the general population, and therefore evaluation should include pelvic ultrasound, endometrial biopsy, or both to evaluate irregular bleeding. 47 …”

Tuberous breast and predisposition to breast deformity in consanguineous

“…The observation of consanguinity among patients affected by tuberous breast let us suppose the possible role of genetic transmission of this deformity. To date, no etiologic theory for the development of tuberous breast has been proved . We believe that focusing on the possible role of a genetic predisposition could help understanding a potential etiologic factor of this deformity.…”

“…To date, no etiologic theory for the development of tuberous breast has been proved. 18 We believe that focusing on the possible role of a genetic predisposition could help understanding a potential etiologic factor of this deformity.…”

Retro-Areola Distally Based Flap in the Management of the Full Expression of Tuberous Breast: A Simple Strategy to Resolve a Weak Point of the Deformity

“…Clinically it may appear from a moderate hypoplasia of the inferior medial quadrant to major hypoplasia involving the breast entirely, often associated with varying degrees of ptosis and breast asymmetry. In its full expression, the malformation is characterized by a small volume of the gland, a cylindrical rather than a conic shape, shorter orthogonal axes, and a higher inframammary fold constricted by a fibrous ring due to a thickening of Camper's superficial fascia, which is absent below the areola [1,2]. The existence of this denser ring has been histologically demonstrated and does not allow the normal development of the lower mammary pole during puberty.…”