Congenital abnormalities

Amazia

Congenital absence of the breast may occur on one (Fig. 46.13) or both sides. It is sometimes associated with absence of the sternal portion of the pectoralis major (Poland’s syndrome). It is more common in males.

Polymazia

Accessory breasts (Fig. 46.14) have been recorded in the axilla (the most frequent site), groin, buttock and thigh. They have been known to function during lactation.

Mastitis of infants

Mastitis of infants is at least as common in the male as in the female. On the 3rd or 4th day of life, if the breast of an infant is pressed lightly, a drop of colourless fluid can be expressed; a few days later there is often a slight milky secretion, which disappears during the 3rd week. This is popularly known as ‘witch’s milk’. It is due to stimulation of the foetal breast by maternal prolactin, thus is essentially physiological.

Diffuse hypertrophy

Diffuse hypertrophy of the breasts occurs sporadically in otherwise healthy girls at puberty and, much less often, during the first pregnancy. The breasts attain enormous dimensions (Fig. 46.15) and may reach the knees when the patient is sitting. The condition is rarely unilateral. This tremendous overgrowth is apparently due to an alteration in the normal sensitivity of the breast to oestrogenic hormones, and some success in treating it with antioestrogens has been reported. Treatment is otherwise by reduction mammoplasty.

Injuries of the breast

Haematoma

Haematoma, particularly a resolving haematoma, gives rise to a lump which, in the absence of overlying bruising, is difficult to diagnose correctly unless it is aspirated or incised.

Traumatic fat necrosis

Traumatic fat necrosis may be acute or chronic, and usually occurs in stout, middle-aged women. Following a blow, or even indirect violence (e.g. contraction of the pectoralis major), a lump, often painless, appears. This may mimic a carcinoma, even displaying skin tethering and nipple retraction, and biopsy is required for diagnosis. A history of trauma is not diagnostic as this may merely have drawn the patient’s attention to a pre-existing lump.

Acute and subacute inflammations of the breast

Bacterial mastitis

Bacterial mastitis is the commonest variety of mastitis and nearly always commences acutely. Although associated with lactation in the majority of cases, it is not necessarily so. Of 100 consecutive cases of breast abscess, 32 occurred in women who were not lactating (De Jode). Some of these will be associated with an infected haematoma or with periductal mastitis and this will be discussed later.

  Aetiology. Lactational mastitis is seen far less frequently than in former years. Most cases are caused by Staphylococcus aureus and, if hospital-acquired, are likely to be penicillin resistant. The intermediary is usually the infant; after the second day of life 50 per cent of infants harbour staphylococci in the nasopharynx.

‘Cleansing the baby’s mouth’ with a swab is also an aetiological factor. The delicate buccal mucosa is excoriated by the process; it becomes infected, and organisms in the infant’s saliva are inoculated on to the mother’s nipple.

Whilst ascending infection from a sore and cracked nipple may initiate the mastitis, in many cases the lactiferous ducts will first become blocked by epithelial debris leading to stasis — this theory is supported by the relatively high incidence of mastitis in women with a retracted nipple. Once within the ampulla of the duct, staphylococci cause clotting of milk and within this clot organisms multiply.

Clinical features. The affected breast, or more usually a segment of it, presents the classical signs of acute inflammation. Early on this is a generalised cellulitis, but later an abscess will form.

Treatment. During the cellulitic stage the patient should be treated with an appropriate antibiotic, e.g. flucloxacillin, and the breast rested, with feeding on the opposite side only. The infected breast should be emptied of milk using a breast pump. Support of the breast, local heat and analgesia will help to relieve pain.

If an antibiotic is used in the presence of undrained pus, an ‘antibioma’ may form. This is a large, sterile brawny oedematous swelling which takes many weeks to resolve.

The breast should be incised and drained if the infection does not resolve within 48 hours, or if, after being emptied of milk, there is an area of tense induration or other evidence of an underlying abscess.

The presence of pus can be confirmed with a needle aspiration, and the pus analysed for the infection and for cytology. This has the advantage of allowing diagnosis on the smear of a rare inflammatory carcinoma (Fig. 46.16). In contrast to the majority of localised infections, fluctuation is a late sign and incision must not be delayed until it appears. Usually the area of induration is sector-shaped, and in early cases about one-quarter of the breast is involved (Fig. 46.17); in many late cases the area is more extensive (Fig. 46.18). When in doubt an ultrasound scan may clearly define an area ‘ripe’ for drainage.

Drainage of an intramammary abscess. The usual incision is sited in a radial direction over the affected segment, although if a circumareolar incision will allow adequate access to the affected area this should be preferred because of a better cosmetic result. The incision passes through the skin and the superficial fascia. A long haemostat is then inserted into the abscess cavity. Every part of the abscess is palpated against the point of the haemostat and its jaws are opened. All Ioculi that can be felt are entered.

Finally, the haemostat having been withdrawn, a finger is introduced and any remaining septa are disrupted. The wound may then be lightly packed with ribbon gauze or a drain inserted to allow dependent drainage.

Mastitis from milk engorgement

Mastitis from milk engorgement is liable to occur around weaning time, and sometimes in the early days of lactation when one of the lactiferous ducts becomes blocked with epithelial debris. In the latter instance only a sector of the breast becomes indurated and tender.

Chronic intramammary abscess

Chronic intramammary abscess which follows inadequate drainage or injudicious antibiotic treatment is often a very difficult condition to diagnose: when encapsulated within a thick wall of fibrous tissue, the condition cannot be distinguished from a carcinoma without the histological evidence from a biopsy.

Tuberculosis of the breast

Tuberculosis of the breast, which is comparatively rare, is usually associated with active pulmonary tuberculosis or tuberculous cervical adenitis.

Tuberculosis of the breast (Fig. 46.19) occurs more often in parous women and usually presents with multiple chronic abscesses and sinuses and a typical bluish attenuated appearance of the surrounding skin. The diagnosis rests on bacteriological and histological examination. Treatment is with antituberculous chemotherapy. Healing is usual although often delayed, and mastectomy should be restricted to patients with persistent residual infection.

Actinomycosis

Actinomycosis of the breast is rarer still. The lesions present the essential characteristics of faciocervical actinomycosis.

Syphilis of the breast

A primary chancre of the nipple has been referred to (above). Secondary lesions of syphilis include diffuse syphilitic mastitis.

Mondor’s disease

Mondor’s disease is thrombophlebitis of the superficial veins of the breast and anterior chest wall (Fig. 46.20) although it has also been encountered in the arm.

In the absence of injury or infection, the cause of thrombophlebitis —like that of spontaneous thrombophlebitis in other sites — is obscure. The pathognomonic feature is a thrombosed subcutaneous cord, usually attached to skin. When the skin over the breast is stretched by raising the arm, a narrow, shallow subcutaneous groove alongside the cord becomes apparent. The differential diagnosis is lymphatic permeation from an occult carcinoma of the breast. The only treatment required is restricted arm movements, and in any case the condition subsides within a few months without recurrence, complications or deformity.

Duct ectasia/periductal mastitis

Pathology

This is a dilatation of the breast ducts associated with periductal inflammation, the pathogenesis of which is obscure and almost certainly not uniform in all cases, although the disease is much more common in smokers.

The classical description of the pathogenesis of duct ectasia asserts that the first stage in the disorder is a dilatation in one or more of the larger lactiferous ducts which fill with a stag­nant brown or green secretion. This may discharge. These fluids then set up an irritant reaction in surrounding tissue leading to periductal mastitis or even abscess and fistula formation (Figs 46.21 and 46.22). In some cases a chronic indurated mass forms beneath the areola which mimics a carcinoma.

Fibrosis eventually develops which may cause slit-like nipple retraction.

An alternative theory suggests that periductal inflammation is the primary condition and anaerobic bacterial infection is found in some cases.

An association between recurrent periductal inflammation and smoking has been demonstrated which may suggest that arteriopathy is a contributing factor in its aetiology.

Clinical features

Nipple discharge (of any colour), a subareolar mass, abscess, mammary duct fistula and/or nipple retraction are the commonest symptoms (Fig. 46.23).

Treatment

In the case of a mass or nipple retraction, a carcinoma must be excluded by obtaining a mammogram and negative cytol­ogy or histology. If any suspicion remains the mass should be excised.

Antibiotic therapy may be tried, the most appropriate agents being flucloxacillin and metronidazole. However, surgery is often the only option likely to bring about cure of this notoriously difficult condition, and consists of excision of all of the major ducts (the Hadfield’s operation).

Aberrations of normal development and involution (ANDI)

Nomenclature

The nomenclature of benign breast disease is very confusing. This is because over the last century a variety of clinicians and pathologists has chosen to describe a mixture of physio­logical changes and disease processes according to a variety of clinical, pathological and aetiological terminology. As well as leading to confusion, patients were often unduly alarmed or overtreated by ascribing a pathological name to a variant of physiological development. To sort out this confusion, a new system has been developed and described by the Cardiff Breast Clinic2 — ANDI. (Many alternative terms have been applied to this condition including fibrocystic disease, fibroadenosis, chronic mastitis and mastopathy.)

A etiology

The breast is a dynamic structure which undergoes changes throughout a woman’s reproductive life, and superimposed upon this, cyclical changes throughout the menstrual cycle. This is illustrated in Fig. 46.24. The pathogenesis of ANDI involves disturbances in the breast physiology extending from an extreme of normality to well-defined disease processes. There is often little correlation between the histological appearance of the breast tissue and the symptoms.

Risk of malignancy developing in association with benign breast pathology

These relative risks according to different histological features found at biopsy are illustrated in Table 46.1.

Pathology

The disease consists essentially of four features which may vary in extent and degree in any one breast.

1. Cyst formation. Cysts are almost inevitable and very variable in size.

2.       Fibrosis. Fat and elastic tissue disappears and is replaced by dense white fibrous trabeculae. The interstitial tissue is infiltrated with chronic inflammatory cells.

3.       Hyperplasia of epithelium in the lining of the ducts and acini may occur with or without atypia.

4.      Papillomatosis. The epithelial hyperplasia may be so extensive that it results in papillomatous overgrowth within the ducts.

Clinical features

The symptoms of ANDI include an area of lumpiness (seldom discrete) and/or breast pain (mastalgia).

A benign discrete lump in the breast is commonly a cyst or fibroadenoma. True lipomas occur rarely.

Lumpiness may be bilateral, commonly in the upper outer quadrant, or less commonly confined to one quadrant of one breast. The changes may be cyclical, with an increase in both lumpiness and often tenderness before a menstrual period.

Noncyclical mastalgia is commoner in perimenopausal and postmenopausal women. It may be associated with ANDI or with periductal mastitis, or referred from, for example, a musculoskeletal disorder. About 10 per cent of breast cancers exhibit pain at presentation. Common breast symptoms are illustrated in Table 46.2.

 Treatment of lumpy breasts

If the clinician is confident that he or she is not dealing with a discrete abnormality (and clinical confidence may be buttressed by mammography or ultrasound scanning if appropriate), then initially the woman can be offered firm reassurance. It is perhaps worthwhile reviewing the patient at a different point in the menstrual cycle, say 6 weeks after the initial visit, and often the clinical signs will have resolved by that time. There is a tendency for women with lumpy breasts to be rendered unnecessarily anxious and to be submitted to multiple random biopsies because the clinician lacks the courage of his or her convictions.

Treatment of mastalgia

  Pronounced cyclical mastalgia may become a significant clinical problem where the pain and tenderness interfere with the woman’s life, disturb her sleep and impair sexual activity. Initially, firm reassurance that the symptoms are not associated with cancer will help the majority of women. A patient symptom diary will help her to chart the pattern of pain throughout the month and thus determine whether this is cyclical mastalgia. If reassurance is inadequate, then a planned escalation of treatment (as shown in Table 46.3) could be advised. Oil of evening primrose, in adequate doses given over 3 months, will help more than half of these women. For those with intractable symptoms a prolactin inhibitor such as danazol may be given. Very rarely it is necessary to prescribe an antioestrogen, e.g. tamoxifen or a luteinizing hormone-releasing hormone (LHRH) agonist, to deprive the breast epithelium of oestrogenic drive.

For noncyclical mastalgia it is important to exclude extramammary causes such as chest wall pain, and it may be necessary to carry out a biopsy on a very localised tender area which might be harbouring a subclinical cancer. Treatment may be with nonsteroidal analgesics or by injection with local anaesthetic of a ‘trigger spot’.

Breast cysts

These occur most commonly in the last decade of reproductive life due to a nonintegrated involution of stroma and epithelium. They are often multiple, may be bilateral and can mimic malignancy. Diagnosis can be confirmed by aspiration and/or ultrasound.

Treatment

A solitary cyst or small collection of cysts can be aspirated. If they resolve completely, and if the fluid is not bloodstained, no further treatment is required. However, 30 per cent will recur and require reaspiration. Cytological examination of cyst fluid is no longer practised routinely. If there is a residual lump or if the fluid is bloodstained, a local excision for histological diagnosis is advisable, as is also the case if the cyst repeatedly reforms.

Galactocele

Galactocele, which is rare, usually presents as a solitary, subareolar cyst, and always dates from lactation. It contains milk and in long­standing cases its walls tend to calcify. It can become enormous (Fig. 46.25).

  Fibroadenoma

These usually arise in the fully developed breast during the 15—25-year period, although occasionally they occur in much older women. They arise from hyperplasia of a single lobule, and usually grow up to 2—3 cm in size. They are surrounded by a well-marked capsule and can thus be enucleated through a cosmetically appropriate incision. However, in a patient under 30 years these do not require excision unless associated with suspicious cytology, or if they become very large, or if the patient expressly desires the lump to be removed. Giant fibroadenomas occur occasionally during puberty. They are over 5 cm in diameter and are often rapidly growing, but in other respects are similar .to smaller fibroadenomas and can be enucleated through a submammary incision.

Phyllodes tumour

These benign tumours, previously sometimes known as serocystic disease of Brodie or cystosarcoma phyllodes, usually occur in women over the age of 40 but can appear in younger women (Fig. 46.26). They present as a large, sometimes massive tumour, with an unevenly bosselated surface. Occasionally ulceration of overlying skin occurs owing to pressure necrosis. In spite of their size they remain mobile on the chest wall. Histologically there is a resemblance to a fibroadenoma, but despite the name of cystosarcoma phyllodes they are rarely cystic and only very rarely develop features of a sarcomatous tumour. These may metastasise via the bloodstream.

  Treatment

Treatment for the benign type is enucleation in very young women or wide local excision. Massive tumours, recurrent tumours and those of the malignant type will require mastectomy.

  When the diagnosis of carcinoma is in doubt

There will always be cases where the clinician cannot be sure whether a particular lump in the breast is an area of mammary dysplasia, a benign tumour or an early carcinoma.

If there is doubt on either clinical, cytological or radio­logical examination it is essential to obtain a tissue diagnosis. This is often possible by needle biopsy. In the advent of a negative result, open biopsy of the mass is necessary. Because of the possibility of reporting errors, the authors suggest that frozen section reporting should rarely be used and certainly should not form the basis for a decision to undertake a mastectomy. Table 46.4 gives an algorithm for investigating any breast lump. Table 46.5 illustrates the features of three common lumps.