Gigantomastia – a classification and review of the literature

Journal of Plastic, Reconstructive & Aesthetic Surgery (2008) 61, 493e502

REVIEW

Gigantomastia e a classification and review of the literature Anne Dancey*, M. Khan, J. Dawson, F. Peart Department of Plastic and Reconstructive Surgery, Selly Oak Hospital, Birmingham, UK Received 8 December 2006; accepted 18 October 2007

KEYWORDS Gigantomastia; Breast reduction; Mastectomy

Summary Introduction: Gigantomastia is a rare, psychologically and physically disabling condition characterised by excessive breast growth. To date, there is no universal classification or accepted definition for this condition. Many authors cite gigantomastia as breast enlargement that requires reduction of over 1500 g per breast. However, there is discordance in the literature with the weight of reduction ranging from 0.8 to 2 kg, or even a D cup bra size. Practically this is a postoperative definition which is of little use to the clinician in terms of patient management or prognosis. Method: We conducted a literature review and meta analysis of all published cases of gigantomastia. We combined this with seven cases managed at Selly Oak Hospital, Birmingham, to give us a series of 115 patients. In order to devise a universally accepted definition of gigantomastia, a survey of 150 plastic surgery consultants within Europe was conducted. Participants were e-mailed a questionnaire, requesting their definition of gigantomastia with an appropriate excision weight. They were also asked to comment on any preoperative measurements taken to establish this predicted weight. Conclusion: We propose a classification of gigantomastia based on the cause, management and prognosis of the disease. ª 2008 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

Gigantomastia is a rare condition characterised by excessive breast growth and can be physically and psychosocially disabling for the patient. Symptoms include mastalgia, ulceration/infection, postural problems, back pain and chronic traction injury to 4th/5th/6th intercostal nerves * Corresponding author. Address: Department of Plastic and Reconstructive Surgery, 20 Navigation Loop, Stone, Staffs Birmingham ST15 8YU, UK. Tel.: þ44 7808078276. E-mail address: [email protected] (A. Dancey).

with resultant loss of nipple sensation. It is also associated with decreased foetal growth during pregnancy. To date, there is no universal classification or accepted definition for gigantomastia. Many authors cite gigantomastia as breast enlargement that requires reduction of over 1500 g per breast. However there is discordance in the literature with the weight of reduction ranging from 0.8 to 2 kg, or even a D cup bra size. We propose a classification of gigantomastia based on the cause, management and prognosis of the disease. We support this with an analysis

1748-6815/$ - see front matter ª 2008 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.bjps.2007.10.041

494 of the literature and questionnaire survey of plastic surgery consultants in Europe. We then validate the proposed criteria with our experience of seven cases at Selly Oak Hospital, Birmingham.

Method A comprehensive literature search was undertaken using Pubmed, Medline and CINAHL search engines. The search terms used were gigantomastia, macromastia and breast hypertrophy. All relevant articles were retrieved and pertinent details recorded including age at presentation, BMI, aetiology, management, weight excised and outcome. This was combined with seven cases of gigantomastia managed in our unit. In order to devise a universally accepted definition of gigantomastia, a survey of 150 plastic surgery consultants within Europe was conducted. We randomly selected participants using the members’ directory for the British Association of Aesthetic Plastic Surgeons, British Association of Plastic, Reconstructive and Aesthetic Surgeons and the European Society of Plastic, Reconstructive and Aesthetic Surgery. As the majority of plastic surgeons performing breast reductions have various sub-specialties, we did not merely select those surgeons expressing an interest in breast surgery. Participants were e-mailed a questionnaire, requesting their definition of gigantomastia with an appropriate excision weight. They were also asked to comment on any preoperative measurements taken to establish the predicted excision weight. A classification was then devised according to aetiology and prognosis.

Results The results of the literature search are shown in Table 1. One hundred and eight patients were identified dating from 1910 to 2006 and seven patients were treated at our unit in Birmingham. This gives a series of 115 patients with an age range from 10 to 56 years (median 18). Aetiology included 57 cases of juvenile gigantomastia, 41 cases of pregnancy-induced gigantomastia, 13 cases of idiopathic gigantomastia and four drug-induced gigantomastias (oestrogen, neothetazone, cyclosporine, penicillamine and bucillamine). Ten patients were noted to have concurrent immunological diseases (myasthenia gravis, Hashimoto’s thyroiditis, rheumatoid arthritis, psoriasis). In eight cases the gigantomastia was noted to be familial and these all presented as juvenile gigantomastias. The BMI was only documented in 11 patients (six from our series). Twenty-nine patients underwent primary mastectomy, of which three patients required further excision and one patient required three further excisions. Forty-six patients had a single reduction procedure. Six patients underwent two reduction procedures. Eleven patients had breast reduction with a subsequent mastectomy, although three of these patients required further excisions following the mastectomy. Fifteen patients were managed without surgery. The median total weight of breast tissue excised (from both breasts) was 6.7 kg (1.35 to 24.5 kg). The outcomes in all cases were eventually good, with the exception of two patients who died as a result of the disease.

A. Dancey et al. One hundred and fifty questionnaires were e-mailed to a random selection of plastic surgery consultants across the Europe. We received 35 replies, which is a 23% response rate. The definitions contained a minimum weight of excision ranging from 200 g to 2 kg per side. Nine consultants knew of no minimum weight criteria. Of those who used a criteria, 44% considered resections of >1 kg per side to be representative of gigantomastia, 25% used 500 g, 19% used 2 kg and 12% used 1.5 kg. The majority used no method of preoperative weight assessment apart from experience and clinical judgement. One respondent used a water displacement method.

Discussion There is no universally accepted definition for gigantomastia. A thorough literature search yielded definitions ranging from a D-cup bra size, to breast enlargement requiring reduction of over 0.8e2 kg.1,2 The first paper we could find using the term gigantomastia was by Lewison et al. in 1960.3 However there is still no concordance in the literature, with terms used including macromastia and hypertrophy. Furthermore, there is a wealth of terms used to describe the causes of gigantomastia. Pregnancy-induced gigantomastia is also referred to as ‘gravid’ or ‘gestational’, and juvenile gigantomastia is often called ‘puberty induced’ or ‘virginal’. We only received 35 replies to our questionnaire, which is a 23% response rate. However, we felt this was sufficient to indicate the variability in predicted excision weight. The expected resection weight ranged from 200 g to 2 kg for each side, with 1 kg being the mode. It is immediately apparent that these definitions are of little use to a clinician. The weight of breast tissue or indeed the size of the breasts is intimately related to a patient’s BMI and yet few authors present these details. Thus a large patient may well have appropriately large and heavy breasts, which would be considered excessive in a smaller patient. Therefore we feel that the concept of percentage body weight is a more useful term to assess the breast size. Unfortunately the amount of breast tissue removed is a postoperative assessment and cannot therefore be used to classify these patients, establish outcome or indeed influence management. The amount of breast tissue excised is also dependent on the operation performed (i.e. mastectomy verses breast reduction). All respondents to the questionnaire, bar one, used no physical method to preoperatively determine the excision weight. To resolve some of the confusion surrounding this condition we attempted to subclassify gigantomastia according to aetiology. Examining patient records, there would appear to be three subgroups of gigantomastia, which are shown in Table 2 and illustrated in Figs. 1e7. The first group is an idiopathic, spontaneous condition of excessive breast growth with quiescence at the time of presentation. It can be further subdivided into patients who are overweight (group 1a) and patients who are of normal weight (group 1b). This condition is by far the commonest presentation but the majority of these cases are not reported in the literature, as they are notable only by the amount of breast tissue excised. This subgroup is best managed by breast reduction and has

Gigantomastia e a classification and review of the literature Table 1

495

Results of literature search on gigantomastia

Reference Author

Year Age BMI

Familal Total breast Cause tissue excised /Kg

Management

69 51

Albert Simpson

1910 14 1920 32

NK NK

No No

24.5 NA

Juvenile Pregnancy

70 71 72

Honan Keyser Greig

1920 16 NK 1921 15 NK 1922 14.5 NK

No No No

NK 6.7 NA

Juvenile Juvenile Juvenile

75 75 75 75 73 74 76 77 52

Wakeley Wakeley Wakeley Wakeley Goodman Gaines Fisher Harris Burslem

1934 1934 1934 1934 1934 1937 1943 1945 1952

NK NK NK NK NK NK NK NK NK

No No No No No No No No No

7.6 9.77 13.07 14.32 18.9 5 15.9 NK NA

Juvenile Juvenile Juvenile Juvenile Juvenile Juvenile Juvenile Juvenile Pregnancy

52

Burslem

1952 NK

NK

No

NA

Pregnancy

53

Williams

1957 31

NK

No

NK

Pregnancy

54 3

Blaydes Lewison

1958 23 1960 20

NK NK

No No

NK NA

Pregnancy Pregnancy

3

Lewison

1960 18

NK

No

NA

Pregnancy

84 55

Erich Nolan

1960 13 1962 22

NK NK

No No

NK NK

Juvenile Pregnancy

56

Ramsden

1963 20

NK

No

NK

Pregnancy

5

Dingman

1963 12

NK

No

5.57

Juvenile

57

Greeley

1965 29

NK

No

NK

Pregnancy

81 24

Wilkins Kapur

1965 10 1968 37

NK NK

No No

NK 12

Juvenile Pregnancy

8 17 78 78 6

Scott Ship Fisher Fisher Hollingsworth

1970 1971 1971 1971 1973

NK 16 10 18 14

NK NK NK NK NK

no No No No No

NA 3.3 4 5 4.85

Neothetazone Juvenile Juvenile Juvenile Juvenile

10 15 25 79 46 80 80 80 27 26

Sperling Mayl Kullander Goldwyn Cardoso Oberman Oberman Oberman Hedberg Miller

1973 1974 1976 1976 1977 1979 1979 1979 1979 1979

12 11 24 11 12 11 13 17 26 26

NK NK NK NK 30.5 NK NK NK NK NK

No No No No No No No No No No

NK 1.8 2.7 8 10 NK NK NK NA NK

Juvenile Juvenile Pregnancy Juvenile Juvenile Juvenile Juvenile Juvenile Pregnancy Pregnancy

Mastectomy Died prior to surgery Mastectomy Mastectomy Died prior to surgery Mastectomy Mastectomy Mastectomy Mastectomy Mastectomy BBR Mastectomy Mastectomy Post delivery some regression Post delivery some regression Mastectomy plus further excision axillary breasts Mastectomy Post delivery some regression Post delivery regressed to normal BBR Post delivery some regression Spontaneous abortion followed by BBR Mastectomy plus further excision Therapeutic abortion followed by BBR Mastectomy spontaneous abortion followed by mastectomy Stopped medication BBR & Mastectomy BBR BBR & Mastectomy Mastectomy plus further excision BBR(x2) plus Progesterogen BBR(x2) plus Gynonest BBR & 2 Br- Alpha Ergocryptin BBR(x2) BBR & Mastectomy BBR BBR BBR & Mastectomy Bromocriptine Therapeutic abortion some regression (continued on next page)

16 15 18 17 14.5 14 11 15 27

496

A. Dancey et al.

Table 1 (continued) Reference Author

Year Age BMI

Familal Total breast Cause tissue excised /Kg

Management

26

Miller

1979 22

NK

No

NK

Pregnancy

28

1981 30

NK

No

4.76

Pregnancy

82 48

WolnerHanssen Furnas Boyce

Therapeutic abortion some regression BBR

1982 11 1984 24

NK NK

No No

7.07 6.22

29

Lafreniere

1984 18

NK

No

7.51

Juvenile Juvenile and then pregnancy Pregnancy

30 31 5 83 33 33 32 34

Gargan Stavrides Dingman Samuelov Beischer Beischer Jackson Tchabo

1986 1987 1988 1988 1989 1989 1989 1989

25.1 NK NK NK NK NK NK NK

No No No No No No No No

13.6 3.55 5.95 8.2 NK 8.4 NK 3.8

Pregnancy Pregnancy Juvenile Juvenile Pregnancy Pregnancy Pregnancy Pregnancy

44 35

Sagot Propper

1990 11 1991 24

NK NK

No No

1.8 N/A

Juvenile Pregnancy

5 5 39 85 85 85 85 43 43 43 63 36 45 58 58

Kupfer Kupfer Colon Gliosci Gliosci Gliosci Gliosci Sridhar Sridhar Sridhar Koger Wolf Szczurowicz ohlsen ohlsen

1991 1991 1991 1993 1993 1993 1993 1994 1994 1994 1994 1995 1996 1996 1996

NK NK 22.2 NK NK NK NK NK NK NK NK NK NK NK NK

Yes Yes No No No No No No No No No No No no no

5.57 7.4 8.7 NK 6.5 NK N/A 3 NA NK 4.4 9.4 NK 2.44 9.55

Juvenile Juvenile Pregnancy Juvenile Juvenile Juvenile Juvenile Juvenile Juvenile Juvenile Idiopathic Pregnancy Pregnancy Ostrogen Pregnancy

68

el-boghdadly

1997 36

NK

no

16.11

Pregnancy

37 38

cheung Windom

1997 34 1999 26

NK NK

No No

N/A N/A

Pregnancy Pregnancy

47 9 49 49 13

Zargar Cervelli O’Hare O’Hare Arscott

1999 1999 2000 2000 2001

30 38 12 14 12

22.5 NK NK NK NK

No No No No No

4.3 NK NA 1.35 15.4

Pregnancy Cyclosporine Juvenile Juvenile Juvenile

4 4 4 4 7

Baker Baker Baker Baker Sakai

2001 2001 2001 2001 2002

10 11 17 24 25

NK NK NK NK NK

No No No No No

NK NK NK NK 12

61

Skillman

2002 36

NK

No

12

Juvenile Juvenile Juvenile Pregnancy Rheumatoid Arthritis& Bucillamine Idiopathic

32 28 12 12 27 28 24 28

11 12 34 14 14 20 15 18 16 12 31 30 35 35 21

Mastectomy plus 3 further excisions Provera, Danocrine, Tamoxifen, Mastectomy (x2) prednisolone, bromocriptine, tamoxifen, Mastectomy BBR Bromocriptine & Mastectomy BBR(x2) BBR & Mastectomy BBR BBR Mastectomy termination, BBR & dihydroprogesterone BBR resolved secondary to vasculitis Mastectomy BBR(x2) Mastectomy BBR (x2) & dydrogesterone BBR & dydrogesterone BBR & dydrogesterone Dydogesterone (surgery declined) BBR Declined surgery BBR & medroxyprogesterone BBR BBR BBR BBR BBR & Mastetomy & axillary tissue removal Mastectomy þ axillary breast removal bromocryptine. Declined reduction patient died of high grade lymphoma Unilateral BR BBR tamoxifen, lost to follow up BBR BBR & near total Mastectomy then complete Mastectomy BBR & Mastectomy & Re-excision(x2) Left BR BBR NK Mastectomy

Mastectomy

Gigantomastia e a classification and review of the literature

497

Table 1 (continued ) Reference Author

Year Age BMI

Familal Total breast Cause tissue excised /Kg

Management

40 65 65 66 41 42 65 65 59 86 62 62 50

Agarwal Govrin Yehudain Govrin Yehudain Chrominski Ahcan Vidaeff Govrin Yehudain Govrin Yehudain wechselberger Sakai misirlioglu misirlioglu Touraine

2002 2003 2003 2003 2003 2003 2003 2003 2004 2005 2005 2005 2005

24 13 17 51 27 NK 12 14 15 23 39 48 12

20 NK NK NK NK NK NK NK NK NK NK NK NK

No Yes Yes No No no Yes Yes No No Yes Yes No

NA 3.9 4 9 10.23 11.41 11.6 NK 9.75 NK 4.1 4.3 NK

Bromocryptine BBR BBR & Mastectomy BBR Mastectomy mastectomy BBR & Mastectomy BBR(x2) BBR Unilateral BR BBR BBR BBR

50

Touraine

2005 13

NK

No

NK

50

Touraine

2005 13

NK

No

NK

50

Touraine

2005 13

NK

No

NK

50

Touraine

2005 26

NK

No

NK

50

Touraine

2005 28

NK

No

NK

50

Touraine

2005 35

NK

No

NA

50

Touraine

2005 44

NK

No

NK

23 51 67

Swelstad Kaviani Kulkarni selly oak selly oak selly oak selly oak selly oak selly oak selly oak

2006 34 2006 19 2006 36 38 NK 56 28 44 55 26

NK NK NK 32 33 30 22 29 34 NK

No No No No No No No No No No

17 12 12 3 3.12 3.16 3.22 3.25 3.55 12.46

a good prognosis. Type 2 is related to an imbalance of endogenous hormone activity. This occurs at puberty (type 2a) and during pregnancy (type 2b). It is a rare pathological condition which may be familial. It is usually relentless in its course and mastectomy should certainly be considered. Hormonal profiles are invariably normal and the possibility of breast tissue receptor abnormalities should be considered. Type 3 is a drug-induced phenomenon and has been attributed to several pharmacological agents (bucillamine and penicillamine). This breast development usually resolves once the drug has been stopped, although breast

Pregnancy Juvenile Juvenile Idiopathic Pregnancy Pregnancy Juvenile Juvenile Juvenile Pregnancy Juvenile Juvenile Juvenile & Myaesthina Gravis Juvenile & Arthritis Juvenile & Hashimotos Thyroiditis Juvenile & Psoraisis Pregnancy & Myaesthina Gravis Pregnancy & Myaesthina Gravis Chronic Arthritis Penicillamine Idiopathic & Myaesthina Gravis Pregnancy Pregnancy Idiopathic Idiopathic Idiopathic Idiopathic Idiopathic Idiopathic Idiopathic Idiopathic & myaesthenia gravis

BBR BBR

BBR BBR BBR Stopped Penicillamine NK

Mastectomy BBR Mastectomy BBR BBR BBR BBR BBR BBR BBR & Mastectomy

reduction is normally required to return the breasts to their pre-morbid state.

Aetiology Idiopathic By far the majority of patients presenting with excessive breast growth, even of the magnitude of gigantomastia, have no obvious precipitating cause. Unfortunately, the published literature rarely quantifies the percentage of body weight excised in these patients. Anecdotally we

498

A. Dancey et al.

Table 2

Proposed classification of gigantomastia

Group

Characteristics

1a

Idiopathic, spontaneous condition of excessive breast growth in a patient with a BMI >30 Idiopathic, spontaneous condition of excessive breast growth in a patient with a BMI <30 Excessive breast growth related to an imbalance of endogenous hormone production occurring during puberty Excessive breast growth related to an imbalance of endogenous hormone production occurring during pregnancy Excessive breast growth induced by a pharmacological agent

1b

2a

2b

3

found that this group of patients can be divided into two groups according to their BMI. Those patients with a BMI of 30 or above have excessive breast growth which is partly dictated by their excess weight. This tends to be of insidious onset and will reduce to a greater or lesser degree with a programme of weight loss. The histological findings are of fibroglandular tissue, lymphocytic infiltration and venostasis. Under the current NHS cosmetic guidelines, we are unable to offer these patients surgical treatment until their weight has been optimised. In our unit this equates to a BMI

Figure 2

Type 1a gigantomastia lateral view.

of less than 30. The literature search found a total of 15 patients presenting with idiopathic gigantomastia. None of these patients had their BMI documented and therefore we were unable to subdivide this group according to BMI. The second group of patients are those who are of normal or low weight with excessive breast development.

Figure 1 Type 1a gigantomastia anteroposterior (AP) view. Overweight patient with idiopathic excessive breast growth.

Figure 3 Type 1b gigantomastia AP view. Patient of normal weight with idiopathic excessive breast growth.

Gigantomastia e a classification and review of the literature

Figure 4

Type 1b gigantomastia lateral view.

They too present with no obvious precipitating factors and all hormonal investigations appear normal. Endogenous hormone stimulation In some, gigantomastia is thought to be due to either a hypersensitivity of breast tissue by physiological levels of circulating hormones, or due to hormonal excess. Indeed the majority of published cases occur during either pregnancy or puberty, supporting the theory of abnormal stimulation of mammary tissue by circulating hormones. The incidence of pregnancy-induced gigantomastia is quoted as between 1 in 28 000 to 100 000 pregnancies. The true incidence of juvenile gigantomastia remains unknown. The majority of cases reported were of the juvenile type, although this may be a reporting bias. Patients with juvenile gigantomastia present with a history of alarmingly rapid breast growth, which can occur at any stage in the peripubertal period. This can be unilateral or bilateral. Growth is often particularly brisk for 6 months and is then superseded by a period of slower but sustained growth that can last for many years.4,22 Puberty is a sensitive time for any child and the derangement of the normal physical transformation can have profound compromise on their social and psychological wellbeing. Extreme self consciousness and poor self esteem may result.

Figure 5 Type 2a gigantomastia AP view. 11-year-old girl presenting with puberty-induced excessive breast growth, which subsequently ulcerated.

499

Figure 6 Type 2b gigantomastia AP view. 28-year-old female presenting initially with spontaneous excessive breast growth which responded to breast reduction. Now returning with excessive breast growth induced by pregnancy.

In pregnancy-induced gigantomastia rapid and massive enlargement of the breasts closely resembles juvenile gigantomastia. It commonly occurs in the 20s to 30s age group. Once pregnancy-induced gigantomastia has occurred, subsequent pregnancies are likely to cause further recurrence, unless a mastectomy has been performed. On examination there is markedly disproportionate growth of one or both breasts. The breasts are ptotic in appearance with grossly dilated nipples and areola. The superficial veins are prominent and dilated, with thinning or ulceration of the skin as a result of excessive tension on the skin. The breasts are firm to palpation and often diffusely tender. Discrete nodules may also be present. In the overwhelming majority of cases, physical examination and laboratory investigations seldom demonstrate any abnormality to explain the cause. In cases of juvenile gigantomastia, the breast tissue shows varying degrees of stromal and ductal hyperplasia with dilatation. Collagenous fibrosis and cellular myxoid hyperplasia can sometimes be seen.5 There is often prominent ductal proliferation with cystic degeneration. Oedema is a characteristic finding in both the interstitium and periductal regions. There have also been reports of lymphatic dilatation.6 The histological appearance of pregnancy-induced

Figure 7

Type 2b gigantomastia lateral view.

500 gigantomastia is relatively similar to the juvenile form. The characteristic features are glandular hyperplasia, hyperplasia of the stromal elements and fibroadenomas. Isolated histological reports have shown an increase in oestrogen receptors in the glandular tissue, as well as receptor hypersensitivity of both oestrogen and progesterone receptors in cases of juvenile gynaecomastia. Breast tissue hypersensitivity to prolactin appears to be characteristic of pregnancy-induced gigantomastia, whilst juvenile gigantomastia is thought to be due to breast tissue hypersensitivity to hormones other than prolactin. A literature review revealed that eight out of 57 patients with juvenile gigantomastia have a strong family history of the condition. To our knowledge, there have been no reported genetic investigations in the literature.5 Drug induced Rare associations of gigantomastia include medicinal aetiologies such as penicillamine,7 neothetazone,8 and cyclosporine.9 The mechanism of action of these pharmacological agents remains unclear. Histological and immunological investigations have consistently failed to identify any specific abnormality. Cessation of the drug with or without a reduction procedure is the mainstay of treatment. An interesting subgroup of patients appear to have immunological risk factors for development of gigantomastia, such as myasthenia gravis, chronic arthritis and Hashimoto’s thyroiditis. Although these conditions are noted either during or preceding the diagnosis they are often found in conjunction with another risk factor for gigantomastia, i.e. pregnancy, or pharmacological factors. Touraine et al. published a series of eight patients presenting with inflammatory gigantomastia in the context of immune-mediated disease. They concluded that breast tissue is a target for autoimmune diseases although the precise mechanism of action cannot be individualised.

Treatment Once a diagnosis has been made, a treatment strategy must be devised. As one would expect there are several treatment options for this condition. These include breast reduction, mastectomy and reconstruction, hormonal treatment, or a combination of the above. Most authors conclude that gigantomastia cannot be influenced by conservative or medical treatment and only resolves with surgical manoeuvres. Hormonal therapy Multiple attempts have been made to control pre- and postoperative breast growth with hormonal therapy and reports of success are varied.23 Medicinal treatments include bromocriptine, medroxyprogesterone,10 tamoxifen,11 and danazole.12 Bromocriptine is an ergot-derived compound that acts as a dopamine agonist, resulting in a significant decrease in the release of prolactin from the anterior pituitary gland. High doses of bromocriptine can cause involution of the breasts with slowing or reversal of growth during pregnancy. Surgical treatment during pregnancy can thus be avoided until postpartum reduction mammaplasty.

A. Dancey et al. It has been suggested that bromocriptine therapy should be given after delivery and continued for at least 6 months before contemplating surgical reduction. Despite its success in pregnancy-induced gigantomastia, the same results have not been achieved in juvenile cases.13 Tamoxifen is an anti-oestrogen that is a useful adjunct to reduction mammaplasty in juvenile gigantomastia. It actually causes regression of hypertrophied tissue,11 in contrast to dydrogesterone and medroxyprogesterone, which only slow or stop growth. Medroxyprogesterone is a lutenising hormone inhibitor with direct anti-oestrogenic effects. It has been shown to stop breast growth effectively, although it is associated with amenorrhoea and the formation of benign mammary nodules.14 Dydrogesterone is potent progesterone that reduces breast hypertrophy prior to surgery and prevents recurrence following breast reduction.15 It does not affect ovulation and is not carcinogenic. Reduction mammaplasty Breast reduction surgery is often used as a first line treatment, with or without hormonal therapy. This will remove a large portion of the hypertrophic breast thus alleviating discomfort and the risk of skin breakdown and infection. However breast reduction surgery is often not definitive and breasts continue to enlarge, requiring secondary reduction surgery or mastectomy and reconstruction. Indeed multiple case reports demonstrate the refractory nature of the condition to surgery alone.16e18 We feel that group 1 patients are best served with a breast reduction procedure. Anecdotally, we feel that subgroup 1B patients (those of normal weight) are more likely to require further reduction procedures than those in group 1A (who are overweight). However given the lack of published data, this is difficult to prove with confidence. These patients must be counselled as to the possible need for further reduction(s) with a view to proceeding to mastectomy. Group 3 patients may also benefit from breast reduction procedures, combined with cessation of the precipitating drug. In 1922, Thorek described standard free-nipple reduction mammaplasty in gigantomastia. Although this technique remains simple and reliable, it can produce a non aesthetic breast and nipple with poor projection. It has gradually been superseded by dermoglandular pedicle techniques, the most commonly used being the inferior pedicle. It was originally thought that inferior pedicle techniques gave a high incidence of nipple necrosis in comparison to nipple grafts.19e21 However, studies have shown very good results without the need to amputate the nipple and an inferior pedicle technique1 or three dermal pedicle technique is advocated.60 Mastectomy Reduction mammaplasty is considered by many as the technique of choice, as it preserves the native breast tissue without the need for implants. However, it is well recognised that these patients often require secondary procedures as a result of recurrent hypertrophy of the remaining breast tissue. Mastectomy is often used following reduction mammaplasty and avoids the use of prolonged hormone therapy. If incomplete removal of breast tissue has occurred then there is a risk that the remaining elements can continue to enlarge either spontaneously or

Gigantomastia e a classification and review of the literature in response to a hormonal stimulus, such as pregnancy. This is often to such an extent that it requires further surgical excision.17,18 Treatment options include subtotal mastectomy with breast implants, staged procedures employing tissue expanders or indeed autologous breast reconstruction. In addition to the psychological implications of mastectomy, the patient must be aware of the long term implications of an implant reconstruction. It would certainly appear that mastectomy should be considered as a first line treatment option in group 2 patients with pregnancy-induced or juvenile gigantomastia. The breast re-growth in this subgroup is often aggressive and unremitting. Those patients initially treated with reductions often proceed to a mastectomy, prolonging the disease course and patient morbidity. The differential diagnosis of gigantomastia should always include malignant breast carcinoma and fibroepithelial tumours such as fibroadenomas and cystosarcoma phyloides. Whilst the majority of cases of gigantomastia are bilateral and make a malignant process unlikely, unilateral cases have been described in the literature.4 It is important to investigate the patient fully with mammography, ultrasound and tissue biopsy as appropriate. In conclusion, we propose that gigantomastia is arbitrarily defined as excessive breast growth of over 1.5 kg per breast, which can be divided into three subgroups according to aetiology. Group 1 is idiopathic in nature, quiescent and can be managed with a breast reduction in the first instance and tend to have a good prognosis. Group 2 is a result of endogenous hormone imbalance (pregnancy or juvenile) and presents with very aggressive and unremitting breast growth. They often require multiple reductions and consideration should be given to a primary mastectomy with breast reconstruction. There is a familial tendency in juvenile gigantomastia and patients should therefore be made aware of the possible genetic implications. Group 3 is drug induced and responds well to cessation of therapy with or without breast reduction. We maintain that this rational provides a sound framework for further analysis, audit and future comparison.

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