Unilateral gestational macromastia − an unusual presentation of a rare disorder
- Abdul Hamid Zargara,
- Bashir Ahmad Lawaya,
- Shariq Rashid Masoodia,
- Nisar Ahmad Chowdrib,
- Mir Iftikhar Bashira,
- Arshad Iqbal Wania
- aSher-i-Kashmir Institute of Medical Sciences, Srinagar, Kashmir, India Department of Endocrinology, bDepartment of General Surgery
- Professor Abdul Hamid Zargar, Post Box No 1098, GPO Srinagar, 190001 Kashmir, India
- Accepted 27 July 1998
Macromastia (mammary gigantism) is an uncommon clinical entity. Macromastia occurring during pregnancy (gestational macromastia) is rare. A case of unilateral gestational macromastia is reported which required reduction mammoplasty. We documented hyperprolactinaemia in the patient. This case report is particularly interesting because, to our knowledge, no such case has previously been reported.
Macromastia (gigantomastia), an uncommon clinical entity, has been referred to as massive enlargement of breasts in non-obese women in whom weight bearing itself is uncomfortable and stretching of overlying skin causes ulceration.1 This disorder is most commonly classified into three types: pubertal macromastia, macromastia during pregnancy (gestational macromastia), and macromastia in adult women in whom no initiating cause is identified.2 In addition, this disorder may be associated with penicillamine therapy.3 Gestational macromastia is exceedingly rare.4 We reported a case of this disorder in 1995.5 In the present report we describe a case of unilateral gestational macromastia. A Medline search, covering the last 30 years did not reveal any other report of unilateral gestational macromastia.
A 30-year-old, gravida 5, para 4, non-obese, woman, 6 months pregnant, presented to the endocrinology clinic at Sher-i-Kashmir Institute of Medical Sciences, Srinagar, Kashmir, India with a 2-month history of progressive enlargement of the right breast. Initially the breast was painless but later on became quite painful. She had no history of discharge from the nipple. Her first three pregnancies had been uneventful. However, during the last (fourth) pregnancy (30 months earlier), she had noticed a progressive painful enlargement of the right breast during the second trimester which worsened till she delivered a full-term normal baby. Pain and swelling in the right breast had slowly decreased during the 3 months after delivery but it continued to be bigger than the left breast.
Clinical examination revealed a young healthy woman who was in distress because of pain in the right breast. Her pulse was 82 beats/min and blood pressure 120/80 mmHg; she weighed 55 kg and had a height of 155 cm. She had massive enlargement of the right breast, which was firm, lobulated, tender and pinkish in appearance with a small ulcerated area. The left breast was normal (figure 1). There was no discharge from either breast. Her chest, cardiovascular and neurological examination was unremarkable. Abdominal examination revealed a fundal height of 22 ± 2 weeks.
Investigations revealed a normal blood count. Serum chemistry revealed hypoalbuminaemia (serum albumin, 3.1 g/dl). Ultrasonography revealed a single live foetus with gestational age of 20 weeks. Aspiration of the right breast revealed marked epithelial hyperplasia. Hormone estimation revealed a mild elevation of total tri-iodothyronine (T3), total thyroxine (T4) and luteinising hormone (LH) levels (T3, 2.53 ng/ml; T4, 13.7 μg/dl; LH, 127.1 IU/l, with upper limits of normal values in non-pregnant state being 2.5, 13.5 and 64, respectively). Serum prolactin level was 126.3 ng/ml (normal value in second trimester of pregnancy <116 ng/ml). Serum levels of growth hormone, thyroid-stimulating hormone, and follicle-stimulating hormone were within normal limits. Serum progesterone was 77.91 ng/ml (normal value >30 ng/ml).
A diagnosis of unilateral gestational macromastia was made. The patient did not satisfactorily respond to breast support and analgesia. Ultimately, in consultation with the patient and her husband, she underwent reduction mammoplasty (figure 2). The breast tissue removed was firm, fleshy and weighed 4.3 kg. Histopathological examination of the specimen revealed lobular hypertrophy. Acini showed vacuolation in the inner epithelium. Some lobules also showed eosinophilic secretions in dilated lumina. This was consistent with glandular proliferation of pregnancy.
To distinguish macromastia from cases of moderate to minimal breast enlargement, the usual practice is to limit the diagnosis to non-obese women in whom the weight bearing itself is uncomfortable or in whom stretching of overlying skin causes ulceration. The first case of macromastia in the English literature was described by Simpson in 1920.6 Gestational macromastia is exceedingly rare; Lowinson reported two cases in 56 796 deliveries.4
Our patient noticed progressive, painful, massive enlargement of the right breast during her fourth pregnancy with significant symptomatic relief after delivery and recurrence of symptoms and signs during the subsequent pregnancy which makes unilateral gestational macromastia the most logical diagnosis. Gestational macromastia can manifest either in first pregnancy or after a previous normal pregnancy.7 Manifestations usually begin in the first trimester and appear to have no relationship to family and menstrual history.7 Several cases of mammary gigantism have been reported following D-penicillamine therapy, including unilateral gynaecomastia in a man.8
The cause of this disorder is not understood. The breast is a target for most hormones that act in concert to promote mammogenesis, lactogenesis and galactopoiesis. Glandular proliferation which begins at puberty and is intensified during pregnancy is dependent on progesterone. Oestrogen has a greater influence on the ductular growth. Prolactin, which is essential for lactogenesis, also appears to co-ordinate the effects of oestrogen and progesterone on breast tissue.7 9 Other hormones including T4, growth hormone, cortisol, insulin and human placental lactogen have permissive roles in the developmental or pathogenetic states of breast. Lafreniereet al demonstrated elevated serum prolactin levels in their patient and some earlier cases reviewed.7Our patient also had markedly increased serum prolactin. Whether this finding is aetiologically related to gestational macromastia is still unknown. Although it has been postulated that end-organ responsiveness to various hormones may be aetiologically responsible, this response has not been quantified and remains only a hypothesis. The unilateral nature of this disorder in our patient lends support to the concept that local factors play a more important role in the causation of this disorder.
gestational macromastia is a rare disorder and unilateral disease has not been previously reported
the condition may start in any pregnancy but recurs in subsequent pregnancies
gestational macromastia is due to increased responsiveness of breast tissue to normal or increased levels of growth-promoting hormones
hyperprolactinaemia is a common finding
termination of pregnancy is not helpful; total mastectomy is the only possible treatment
Our patient had noticed significant relief of symptoms and some decrease in the size of the breast after her fourth delivery. Studies have documented either some regression or no relief at all following delivery.10 11 In only one patient, breasts returned to normal size post-partum.4 Because of lack of effectiveness in obtaining breast regression, termination of pregnancy is not recommended.5 7 Total bilateral mastectomy is recommended before morbid complications set in. Since this condition recurs with each successive pregnancy, partial mastectomy is not recommended unless no further pregnancies are anticipated. This couple gave consent only for reduction mammoplasty and decided to undergo tubal ligation after delivery. The unilateral nature of disease in this patient and documentation of hyperprolactinaemia add a new dimension to this rare pathological condition.