European Journal of Plastic Surgery (2020) 43:91–96

https://doi.org/10.1007/s00238-019-01552-8

CASE REPORT

Rapidly-growing pseudoangiomatous stroma hyperplasia (PASH)

causing bilateral gigantomastia in a 15-year-old patient
Maarten Vijverberg 1 & Henriette Kroese-Deutman 1 & Carla Meeuwis 2 & Robert Kornegoor 3

Received: 23 February 2019 / Accepted: 9 July 2019 / Published online: 14 August 2019
# The Author(s) 2019

Abstract
A 15-year-old female patient presented to our clinic with rapidly-growing breasts, causing her back and neck pain, mastalgia, and
unwanted attention at school. Clinical evaluation showed bilateral enlarged breasts with multiple palpable tumors. Her height and
body weight were 1.76 m and 54 kg, respectively. Both ultrasound and magnetic resonance imaging (MRI) were conducted.
Histological biopsies showed pseudoangiomatous stroma hyperplasia (PASH). Bilateral diffuse multiple tumors leading to
massive bilateral breast enlargement are rare, especially in pediatric patients. To the best of our knowledge, all previous reported
cases were patients of 29 years old or older. We present the evaluation and treatment in a rare case of multiple PASH causing
bilateral gigantomastia in an adolescent patient.
Level of evidence: Level V, therapeutic study.

Keywords Gigantomastia . PASH . Breast reduction . Bilateral breast hypertrophy.

Introduction When gigantomastia occurs in younger patients, multi-

disciplinary approach is important in guiding the patient
Gigantomastia is a rare condition defined by diffuse extreme through the diagnostic and treatment process. Surgeons,
bilateral breast growth. Complaints like neck or back pain and radiologists, pathologists, and endocrinologist all play
unwanted social attention could be both physically and psy- their part. This provides a more holistic approach to the
chosocially disabling. It is mostly associated with excessive patient.
body weight. Other causes are pregnancy or puberty, which is Pseudoangiomatous stromal hyperplasia (PASH) is a
thought to be caused by hypersensitivity of breast tissue to benign proliferation breast stroma cells, characterized by
circulating hormones or an excess of circulating hormones anastomosing slit-like spaces in fibrous tissue which are
[1]. Different classifications of gigantomastia have been pro- present within as well as between breast lobules. Often,
posed. We have used the classification by Dafydd et al., pro- spindle stromal cells are present as margins of these
posing breast tissue that contributes 3% or more to the pa- spaces, simulating endothelial cells [3, 4]. The diagnosis
tient’s body weight [2]. of PASH relies on the histological examination of
biopsied material or excised lesion. It can present in a
Level of evidence: Level V, therapeutic study. various range of severity, from microscopic finding to
palpable mass. Ultimately, this could lead to enlargement
* Maarten Vijverberg of the breasts.
[email protected] Although PASH is not associated with (pre)malignancy,
surgery may be indicated to relieve symptoms associated with
1
Department of Plastic and Reconstructive Surgery, Rijnstate gigantomastia and prevent further growth. Treatment options
Hospital, Wagnerlaan 55, 6815 AD Arnhem, the Netherlands include breast reduction surgery or mastectomy with implant
2
Department of Radiology, Rijnstate Hospital, Wagnerlaan 55, 6815 reconstruction. Breast reduction surgery is considered the
AD Arnhem, the Netherlands treatment of choice but also mastectomy with reconstruction
3
Department of Pathology, Rijnstate Hospital, Wagnerlaan 55, 6815 is an option due to the chance on recurrence hypertrophy after
AD Arnhem, the Netherlands breast reduction surgery.
92 Eur J Plast Surg (2020) 43:91–96

We describe the multidisciplinary approach in a 15-year- FSH, and hCG/ beta-hCG), and ACTH. Also, tumor markers
old girl who presented with rapidly-growing tumors causing A1-fetoprotein and CA-125 were tested. No abnormalities
bilateral gigantomastia and her treatment. were found.

Imaging
Case
At first, both mammography and hormonal tests were per-
A 15-year-old girl was redirected to the department of surgery formed. The breast lesions could not be accurately assessed
due to rapidly-growing breasts, which grew from zero volume on the mammography. Subsequently, magnetic resonance im-
to cup G in a short time. She had no relevant history of any aging (MRI) and ultrasound of the breasts were conducted.
disease, medication use, or previous surgery. Her family his- MRI showed multiple well-bounded masses in both breasts
tory of breast cancer and gigantomastia was negative. She had with varying intensity (Fig. 1). There were no signs of malig-
her first menstruation 1 month ago. There was no pregnancy. nancy as the MRI was classified as BI-RADS category 2 by
On physical examination, there was a disproportional breast the radiologist, indicating a definitive benign finding.
size in comparison with the rest of her body and multiple solid
tumors were palpable in both breasts. There were no patho- Histopathology
logic enlarged lymph nodes palpable in both axilla. An endo-
crinologist was consulted for further screening. A blood test Next, three histological biopsies were conducted based on the
was performed, including cortisol, thyroid (TSH, FT4, and previous conducted magnetic resonance imaging (Fig. 2).
T3), estrogen, prolactin, progesterone, gonadotropins (LH, Lesion 1, located medial and superior in the left breast,

Fig. 1 MRI showing lesions in

both breasts in transverse (1, 2, 3)
and sagittal plane (4), and T1- (1,
3) and T2 (2, 4)-weighted images

1 2

3 4
Eur J Plast Surg (2020) 43:91–96 93

advantages and disadvantages, she opted for a breast reduction

surgery.
1 1 Pre-operatively, another ultrasound of both breasts was
3 3 2 2 conducted to confirm the locations of the lesions. In the left
breast, well-bounded masses were seen retro-areolar and cau-
dal, which had a similar aspect as a lipoma (Fig. 4). In the
lateral side of the breast, a few similar but smaller masses were
Fig. 2 Numbers indicating the locations of the lesions in both breasts of seen. In the right breast, a similar distribution was seen, but
which histopathology was obtained by biopsy with slightly bigger masses. During surgery, the radiologist
was present to provide ultrasound guidance in order to remove
the lesions in toto.
showed fibrosis and ductal hyperplasia. Lesion 2, located cen- The breast reduction was done according to the Wise
tral and superior in the left breast and with internal inhomo- pattern with a medial-cranial vascularization of the nipple-
geneity on MRI, showed ductal hyperplasia with areola complex. The procedure was started with the left
pseudoangiomatous stroma hyperplasia (PASH). Lesion 3, lo- breast. After the preparation from the inframammary fold
cated central and superior in the right breast and with internal (IMF) until the pectoralis major’s fascia, the lesions were
septae on MRI, also showed ductal hyperplasia with PASH. directly identified using ultrasound and palpation and sub-
sequently separated from the glandular tissue. Four lesions
were removed from the left breast. Two lesions (I and II)
Treatment were located central in the superior part of the breast. One
(III) of the lesion was located medially in the inferior part of
Surgical resection was not performed at this time. To monitor the breast, which was fixed on the fascia of the m. pectoralis
the growth of the lesions, a MRI every 6 months was done. No and therefore difficult to release. Laterally (IV), a big lesion
significant changes compared to the previous MRIs were seen. was removed causing less filling of the inferior part of the
Two years later, the patient was directed to the department of breast. A total of 381 g was removed from the left breast
plastic surgery asking for a breast reduction. She was 17-year- (Fig. 5). After closing the left breast, the same procedure
old and had multiple complaints, including neck and back and approach was done at the right side. In the right breast,
pain, mastalgia, and unwanted attention at school. She three lesions were also detected by ultrasound and removed.
stopped playing volleyball due to these complaints. During The lesions were located centrocaudal (V), laterocaudal
clinical examination (Fig. 3), both breasts were enlarged and (VI), and centrocranial (VII).
multiple tumors were palpable in both breasts. Her skin was A total of 324 g was removed from the smaller right
extremely thin and there was no subcutaneous fat layer. The breast. The surgery went without complications and the
left breast was larger than the right one. The patient had a very patient was dismissed from the hospital after 2 days.
slender posture with a body weight of 54 kg. She was 175-cm Given the age of the patient, a mastectomy was not desir-
tall, resulting in a body mass index of 17.6. She has breast cup able. We did not remove more tissue than the tumors itself.
H, with an extent of 60 cm. Her breasts have not grown com- Therefore, the patients’ breasts were still relatively large
pared with the year before. At this point, we estimated that the postoperatively (breast cup E, with an extent of 60 cm)
patient’s breasts were over 1.5 kg (3% of her body weight), (Fig. 3). The skin was extremely loose and thin that exci-
indicating gigantomastia. After being fully informed with the sion of more breast tissue would have led to less projection

Fig. 3 Photo of both breasts

preoperatively (left) and 1 year
postoperatively (right)
94 Eur J Plast Surg (2020) 43:91–96

All seven lesions were sent for pathological evaluation.

Histological examination showed glandular tissue conform
age. Various amounts of PASH was seen and focal gyneco-
mastia like ductal hyperplasia was found. This ductal hyper-
plasia in the breast is a proliferation of ductal epithelium with
the histological features of (florid) gynecomastia. It should be
regarded as a histological variant of benign ductal hyperplasia
with typical three-layered ductal epithelium. No in situ carci-
noma or malignancy was found.

Follow-up

In the first week of follow-up, there was a minor wound de-

hiscence which did not lead to any further problems. Although
the breasts were still large, she was pleased with the result and
observed less functional problems 4 months after surgery. One
year after the surgery, she is happy with the size of her breasts
and is not opting for further breast reduction surgery yet. The
skin was retracted and the scars have healed well over time.

Discussion

We described our diagnostic workup and surgical treatment in

a patient in which gigantomastia occurred at 15 years of age
due to bilateral multiple benign (PASH) tumors. Tumor-
forming PASH is uncommon and usually presents as solid
Fig. 4 Ultrasound, made pre-operatively, showing the aspects of two
lesions with their measurements well-circumcised mass. Bilateral diffuse multiple tumors lead-
ing to massive bilateral breast enlargement are rare. Other
of the breasts and thus a wrinkled skin. Also, in this case, a reports have been published presenting patients with bilateral
revision surgery is feasible in the future with acceptable gigantomastia due to PASH [5–9], all reporting bilateral breast
cosmetic outcome, if required. enlargement, with palpable masses in some cases. However,

Fig. 5 Pre-operative photos

showing the bulging of the tumors
in the left breast (upper) and the
lipomatous aspect of one of the
encapsulated tumors during exci-
sion (lower)
Eur J Plast Surg (2020) 43:91–96 95

all these patients were between 29 and 46 years old which is in Funding There was no external funding for the study.
contrast to our adolescent patient. The exact etiology of PASH
is unclear. The general accepted hypothesis is that stromal Compliance with ethical standards
hyperplasia is the result of an atypical response to hormonal
Conflict of interest Maarten Vijverberg, Henriette Kroese-Deutman,
stimuli, with progesterone as main stimulus [4].
Carla Meeuwis, and Robert Kornegoor declare they have no conflict of
It is important to make histological distinction with a low- interest.
grade angiosarcoma, since this requires invasive treatment,
including wide excision and chemotherapy. Additionally, the Ethical standards For this retrospective study, formal consent from a
differential diagnosis consists of mammary hamartoma, local ethics committee is not required.
fibroadenoma, and myofibroblastoma. However, PASH is de-
Patient consent Patient provided consent prior to her inclusion in the
scribed as a benign entity; malignancy arising from a PASH
study and for the use of her images.
nodule has been previously described [10]. PASH can co-exist
with malignant processes within the breast. Open Access This article is distributed under the terms of the Creative
There are no specific features to identify PASH in radio- Commons Attribution 4.0 International License (http://
creativecommons.org/licenses/by/4.0/), which permits unrestricted use,
logical appearance; however, common features of PASH have distribution, and reproduction in any medium, provided you give appro-
been described in the literature. On mammography, the most priate credit to the original author(s) and the source, provide a link to the
common appearance is a solid, non-calcified mass or localized Creative Commons license, and indicate if changes were made.
increased stroma. On ultrasound, a well-defined and
hypoechoic mass was most common [11]. In our case, both
mammography and ultrasound were conducted. The ultra-
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