Adolescent Health Care: A Practical Guide

Chapter 59

Breast Disorders

Heather R. Macdonald

Adolescence is a time of significant physiological and psychological changes for young adults. Many of these changes center around a patient's developing reproductive capability. Although serious breast disorders are rare in this age-group, breast complaints and anxieties regarding normal development are common. To identify breast pathology, and reassure patients concerned about normal breast development, health care providers must understand normal breast development and its variations, common breast complaints, and warning signs of serious disease. Equally important is sensitivity to the complex emotions these physical changes can illicit in a teenage patient. In the course of evaluating and treating a breast complaint or disorder, her or his feelings and anxieties should be addressed. Table 59.1 lists common breast complaints in adolescence.

Table 59.1 Common Breast Complaints in Adolescents

Congenital Anomalies Disorders of Development Benign Breast Disease Polythelia Asymmetry Mastalgia Polymastia Macromastia Nipple discharge Supernumerary Hypoplasia Mastitis breast Tuberous breast Abscess Amastia deformity Mass Inverted nipple Gynecomastia

Normal Development

Breast tissue begins as ectoderm-derived mammary bands apparent in embryos 4 mm in length (Rebar, 1999). By 4 weeks of embryonic life or 7 mm in embryonic length, breast tissue has coalesced into a ridge (mammary crest or milk line) running from the axilla to inguinal region along each ventrolateral side of the body wall. The thoracic part of this ridge thickens to form the primordial mammary buds by 10 to 12 mm in embryonic length as the caudal ridges regress. By the 6 to 12 weeks, the nipple and areola form over the breast buds.

Breast development remains quiescent until the fifth gestational month when the primitive mammary bud forms 15 to 25 secondary buds that will eventually develop into the ductal system of the mature breast, with each duct opening separately into the nipple. As the secondary buds elongate to form cords and then lumen within cords, two types of epithelial cells become evident—a secretory inner layer and myoepithelial outer layer. By the time of birth, these epithelial ducts will have undergone limited proliferation, likely due to maternal hormones in the fetal circulation as pregnancy reaches term. Breast glands in newborns are still rudimentary, but can respond to high maternal prolactin levels at birth and secrete “witch's milk,” a natural phenomenon. Parents should be discouraged from expressing the milky discharge as repeated stimulation encourages production and delays resolution. The discharge and associated breast enlargement is self-limiting, if left alone.

Common breast complaints in adolescents are outlined in Table 59.1. Occasionally, a prepubertal boy or girl can present with a breast “mass.” Physical examination finds a soft mobile nodule behind one or both areolas, and no signs of pubertal initiation. This prepubertal breast enlargement is not a cause for concern and resolves spontaneously. Excisional biopsy of these masses is not indicated because removal of the child's breast bud will prevent normal breast development. The child may be monitored for signs of precocious puberty, but no other further action should be taken.

Until puberty, male and female breast development is equivalent. Puberty is a complex endocrinological event involving multiple hormones and varying classes of receptors. At puberty, a different hormonal milieu develops in boys and girls. Figures 1.5 and 1.10 demonstrate the stages of breast development as described by Tanner. The first sign of puberty is accelerated growth followed by thelarche, the onset of secondary breast development. Normal and abnormal pubertal development including breast development is discussed inChapters 1 and 8.

Anatomy

The milk-producing alveolus, or terminal duct, is the primary unit of the breast and it drains through a branching duct system to the nipple. Each lobule consists

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of approximately 10 to 100 alveoli and the lobules drain into lactiferous ducts that merge to form a sinus beneath the nipple. The stroma, consisting of fibrous tissue, surrounds and supports the lobules and ducts. Other structures in the breast are lymphatics, fat tissue, and nerves. Most benign breast diseases and almost all breast cancers begin within the terminal duct lobular unit. In pubertal females, circulating estrogens, estradiol in particular, stimulate duct development whereas progesterone influences lobular and alveolar development.

Women of reproductive age tend to have breasts that have a nodular texture representing the glandular units or lobules of the breast. During each menstrual cycle, these units undergo proliferative changes under hormonal stimulation. This nodularity can increase, particularly with lobular enlargement and edema that may occur toward the end of menstrual cycles. This process may vary from a feeling of breast fullness to distinct masses suggestive of a pathological process.

Congenital Anomalies

Aberrant Breast Tissue

Failure of the primordial milk crest to regress leads to the persistence of breast tissue along the milk line and occurs in 2% to 6% of women (Graydanus et al., 1989). Polythelia, or accessory nipple, is the most common congenital breast anomaly in males or females. Polymastia, or the presence of accessory breast tissue along the milk line, is differentiated from a supernumerary breast, or the presence of a nipple and underlying breast tissue. All three terms describe aberrant breast tissue found most often along the milk line, although aberrant tissue has been reported in other locations. Isolated case reports exist of carcinoma found in accessory breast tissue (Smith and Greening, 1972). Previous association between accessory breast tissue and renal anomalies were likely overstated. Larger studies show that 10% to 15% of patients with supernumerary breast tissue will have major structural renal anomalies if two or more other phenotypic anomalies are present (Hersh et al., 1987). Isolated accessory nipples with or without breast tissue do not appear to be associated with other congenital anomalies.

Patients with aberrant breast tissue usually present with complaints of a soft tissue mass along the primordial milk line. Work-up includes ultrasonography and biopsy if clinical examination does not definitively make the diagnosis. Excision is unnecessary unless the mass is increasing in size or the patient has cosmetic concerns. These masses may grow with pregnancy and lactation.

Absence of Breast Tissue (Amastia and Athelia)

Complete involution of the mammary ridge leads to amastia, or absence of breast tissue. This rare abnormality is usually unilateral. Poland syndrome presents with amastia, ipsilateral rib anomalies, webbed fingers, and radial nerve palsies. Amastia can be extremely disturbing to an adolescent, but can be surgically corrected, in one or staged surgeries. Tragically, a common cause of amastia is iatrogenic. In an adolescent who presents with failed breast development on one side and a surgical history of a breast biopsy as a young child for a breast mass, the breast biopsy removed a normal asymmetric breast bud. This child should be referred to a plastic surgeon for reconstruction of her breast. Athelia is the absence of the nipple on one or both sides. Surgical correction is an option for this condition.

Nipple Inversion

Nipple inversion is common in newborns and usually resolves within a few days of birth. Patients with persistent nipple inversion from birth should be reassured that this is a normal variant and poses no other problem than cosmetic. Surgery to correct the inversion is only indicated for chronic infection as surgical correction of nipple inversion is rarely successful.

Disorders of Development

Breast Asymmetry

Extremes of normal variants of breast development can present as disorders. It is normal for some female adolescents and women to exhibit a moderate amount of breast asymmetry. It is also normal for one breast to develop at a slightly different rate than the other breast. If the physical examination reveals breast asymmetry with normal breast tissue and no dominant masses, the appropriate treatment is reassurance. If the adolescent is pubertal, 75% of breast asymmetry will resolve completely by adulthood (Graydanus et al., 1989). However, if the asymmetry is marked and causes psychological distress for the patient it can be addressed with plastic surgery. In the discussion with the patient and her family, the health care provider must be sensitive to the adolescent's desire to be “normal” and not appear different than her peers. What may seem like a trivial issue to an adult may provoke shame and embarrassment in an adolescent. Breast asymmetry may also be caused by a large mass that distorts the normal breast tissue, such as a giant fibroadenoma; these masses are typically evident during a routine breast examination. Pseudoasymmetry is also a possibility, resulting from deformities of the rib cage such as a pectus excavatum.

Macromastia

Another extreme of normal development that presents in adolescence is macromastia, its further extreme gigantomastia, or virginal/juvenile hypertrophy. In patients with juvenile hypertrophy, pendulous breasts can reach 30 to 50 lb. This disorder may be unilateral or bilateral.

1. Etiology: The cause of juvenile hypertrophy is not well understood, but may represent an abnormal response of the breast to normal hormonal stimulation, especially by estrogen. Eighty percent of macromastia and gigantomastia present in adolescence, and are strongly associated with family history and obesity (Corriveau and Jacobs, 1990). There has been no evidence linking elevated hormone levels to excessive breast volume.
2. Clinical manifestations: While adult patients usually present with physical complaints of back and shoulder

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pain and limited activity, adolescent concerns tend to focus more on self image, and social and athletic limitations (Losee et al., 2004).

3. Treatment: No definite therapeutic guidelines have been developed. Although it is preferable to delay surgery until the breasts have fully matured, this may not be practical in some adolescents if their breast size is unbearable.
4. Surgical options: Once an underlying mass or tumor has been ruled out by physical examination with breast ultrasonography and biopsy if necessary, management for this problem is reassurance and plastic surgery if desired. Standard treatment is a reduction mastopexy. Owing to the risk of recurrence after surgery, some physicians have recommended subcutaneous mastectomy with reconstruction. Patients should be referred to plastic surgeons with experience in treating teens, and who are sensitive to the teens' particular body image concerns.
5. Psychological approaches: Teen girls with excessive breast volume are at risk for a secondary eating disorder, a dysfunctional attempt to achieve more “normal” body proportions (Kreipe et al., 1997; Losee et al., 2004). Girls who present with macromastia or gigantomastia should be evaluated carefully for a distorted body image or eating disorder and treated appropriately. Studies have shown that reduction mammaplasty may play an important role in treating these psychiatric disorders by helping these girls achieve more acceptable breast size (Kreipe et al., 1997) and improving quality of life (Blomqvist et al. 2004).
6. Hormonal manipulation: Hormonal treatment has been attempted in these patients using medroxyprogesterone, dydrogesterone, or danazol. Teratogenic and carcinogenic effects have been a concern with these medications.
7. Combination of medications and surgery: Post-operative treatment with dydrogesterone has been used to prevent recurrences.

Breast Hypoplasia

Breast hypoplasia may also present in adolescence. Underlying causes include iatrogenic injury as previously mentioned, trauma, malnutrition, or it may be an idiopathic condition. Hypoplasia may occur with aggressive weight loss or athletic activity. Teens with breast hypoplasia should be carefully screened for eating disorders, as breast underdevelopment may be a presenting symptom of anorexia or bulimia in a girl with low self-esteem. Other disorders in the differential diagnosis include premature ovarian failure, androgen excess (from tumor or exogenous anabolic steroid use), and chronic diseases—like diabetes mellitus, inflammatory bowel disease and others—that lead to weight loss. Work-up consists of a careful physical examination to document the condition as well as search for underlying causes listed in the preceding text. Treatment is targeted at the underlying primary disorder. Idiopathic breast hypoplasia can be addressed by a plastic surgeon.

Tuberous Breast Deformity

A rare disorder of breast development is tuberous breast deformity. Teens present with long narrow ptotic breasts that appear to be an overdevelopment of the nipple-areolar complex with an underdevelopment of the breast mound. Treatment of choice is plastic surgery; reassurance is also an option in this condition for the milder cases.

Benign Breast Disease

Mastalgia

Moderate to severe breast pain can be a distressing problem to a patient and is not uncommon in teens. It must be distinguished from physiological tenderness and swelling that occurs with most women's menses, due to proliferative lobular changes induced by hormonal stimulation. Mastalgia can be cyclic pain, worse immediately before menses, or noncyclic pain, mastodynia. Pathophysiology is unknown. Pain charts will assist the patient in characterizing the nature and timing of her breast pain. A thorough physical examination, with imaging and biopsy studies if any abnormalities are found, is indicated to rule out underlying pathology. Other causes of breast pain include breast pathology, costochondritis, rib fracture, and gastritis. Once the diagnosis of mastalgia or mastodynia has been made by exclusion, treatment includes analgesics (nonsteroidal anti-inflammatory drugs [NSAIDs]), good bra support (both night and day if needed), and reassurance that the pain is not a symptom of a more serious illness. Most breast pain resolves spontaneously within 3 to 6 months. Medication used to treat severe pain in adults, bromocriptine and tamoxifen, have not been studied in teens. Evening primrose oil has shown minimal symptom relief (Hindle, 1999) but may take months to show an effect.

Nipple Discharge

Nipple discharge can be caused by endocrine disorders, as well as breast pathology. High serum prolactin levels can be due to prolactinoma-induced galactorrhea (see Chapter 57), a bilateral milky discharge. Review of systems should include menstrual irregularities and headaches. In addition to a breast examination, the patient should undergo visual field testing. Prolactinomas can impinge on the optic nerve, causing visual field defects. Work-up for a suspected prolactinoma includes measurement of serum prolactin levels and magnetic resonance imaging (MRI) of the brain. If identified, many patients can be treated medically with bromocriptine, or be surgically resected.

Galactorrhea can also occur during pregnancy, post-abortion, and postpartum. Medications can induce galactorrhea as well. If no underlying cause is identified, the diagnosis of physiological galactorrhea is made by exclusion. Patients should be reassured that they have no underlying breast pathology and counseled to reduce stimulation to the breast. The condition is usually self-limiting.

Nonmilky discharges usually indicate an underlying breast or nipple problem. Physical examination may reveal a local contact dermatitis of the nipple that can give rise to serous, purulent, or bloody discharge. Culprits include soap, clothes, clothing detergent, or lotion used by the patient. Treatment is identification and discontinuation of the offending agent, with topical steroid cream for symptom relief.

Purulent discharge indicates infection. It should be cultured and the patient treated with the appropriate antibiotics and warm compresses. If the infection fails to

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respond to antibiotic therapy, incision and drainage may be indicated.

Serous or bloody discharge from a single duct may be due to an intraductal papilloma and should be examined by ductogram. Any abnormality found should be excised. Cytological analysis has not been shown to be cost effective in teens. Usually, serous discharge is a self-limiting condition and may be related to fibrocystic change. Again, patients should be counseled to limit stimulation of the breast. Bloody nipple discharge, although a matter of concern in adults, is usually not related to underlying carcinoma in teens due to the extremely low incidence of breast cancer in this population. Again, intraductal papilloma and duct ectasia are more common causes.

Intraductal Papilloma

This is a pathology rarely seen in teens. It can present either as a diffuse thickening of the breast or a nipple discharge, usually bloody. It represents a focal hyperplasia of the ductal epithelium invaginating into the duct on a vascular stalk. Disruption of this stalk leads to bloody discharge. If the proliferation of duct epithelium grows large enough it creates a palpable mass. This is an infrequent finding in an adolescent, although a palpable mass associated with a bloody nipple discharge has a 95% probability of being an intraductal papilloma, even in teens. Treatment is surgical excision.

Montgomery Tubercles

Discharge may be associated with Montgomery or Morgagni tubercles. These are enlarged sebaceous glands around the areola associated with lactiferous ducts. They present as soft papules that may enlarge with pregnancy and lactation, and elicit an episodic thin clear or brown discharge. Breast examination may reveal a soft mass beneath the areola. This condition resolves spontaneously within several months.

Breast Infection

Mastitis or breast abscesses present with an acute history of a red inflamed breast.

1. Predisposing conditions include pregnancy, lactation, history of recent cessation of breast-feeding, preexisting cyst, and breast trauma.
2. Patients may or may not present with constitutional symptoms of malaise or fever. Physical examination reveals an edematous erythematous breast. Purulent discharge may or may not be identified. Any drainage or discharge should be sent for culture and sensitivity.
3. Treatment is as follows:
4. Treatment for mastitis without abscess is antibiotics and warm compresses, begun as soon as the condition is clinically suspected.
5. Antibiotic therapy should be targeted at the most likely pathogen, skin flora including Staphylococcus aureus. Occasionally, streptococci or hemophilus species are found at culture. Dicloxacillin or cepha-lexin provides adequate coverage of skin pathogens. For penicillin allergic–patients, clindamycin provides good abscess penetration.
6. Breast-feeding: If patients are breast-feeding, expression of milk from the affected side should continue to prevent milk stasis. Infants can continue breast-feeding from the affected side.
7. Persistent infection: Patients should be re-examined within several days to confirm response to therapy. Persistent infection despite antibiotic therapy should be evaluated with ultrasonography for an underlying abscess.
8. Abscess: Any area of fluctuance on physical examination implies an underlying abscess. Drainage can be attempted in the office with a large-bore needle. Injecting a solution of normal saline with lidocaine into the abscess will serve two functions—pain relief and dilution of viscous purulent material, making aspiration easier. If needle aspiration is unsuccessful or if the abscess reaccumulates, the patient should be referred for incision and drainage. Incisions should be as small as possible to limit resulting distortion. Material obtained at aspiration or incision and drainage should be cultured.
9. Surgery: Infections that fail to respond to adequate antibiotic therapy should also be referred for surgical management. These patients should be screened for underlying immunosuppressive disease that may interfere with their ability to clear the infection, like diabetes mellitus or human immunodeficiency virus (HIV) infection.

Breast Masses

The presence of a dominant breast mass in a female patient of any age causes concern, but in adolescence, breast masses are overwhelmingly benign (Table 59.2). Surgical studies show that 86% of excisional biopsies are fibroadenomas (68%), and fibrocystic changes (18%) or other benign findings such as cysts and mastitis. Malignancies comprised less than 1% of excisional biopsies performed. A review of the adolescent literature in 1994 found 0.89% of breast masses excised to be primary adenocarcinoma (Neinstein, 1994). Therefore, in addition to appropriately establishing the cause of a dominant mass, practitioners must reassure patients and their parents that it is very unlikely they have a malignant disease.

Work-up of a Breast Mass

Every breast mass that feels benign should be confirmed with imaging and biopsy. Physical examination is not sensitive enough to differentiate benign from malignant lesions (Chateil et al., 1998; Garcia et al., 2000; Hays et al., 1997). Mammography is of low yield in adolescents and young women as the breast density of these patients obscures pathological findings on mammogram (Williams et al., 1986). A targeted ultrasonography is a noninvasive nonpainful way to characterize a mass. However, a retrospective series of breast ultrasonograms in adolescent girls with metastatic malignancies in their breasts, found that malignant lesions had at least one benign characteristic on ultrasound evaluation (Chateil et al., 1998; Hays et al., 1997).

Although excisional biopsy was commonly performed previously, current management of breast disease seeks to establish a diagnosis without surgical excision, saving patients with benign lesions from an unnecessary surgery (Pacinda and Ramzy, 1998). This is achieved by

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either core needle biopsy or fine needle aspiration (FNA). For younger patients, FNA is a less painful way to obtain diagnosis, and is cost effective and quick. Studies of FNA in pediatric and adult patients have shown 99% correlation between excisional histology and the diagnostic triad of concordant physical examination, biopsy, and imaging (Silverman et al., 1991; Eisenhut et al., 1996; Vetto et al., 1996). (For further detail see “Breast Imaging” and “Breast Biopsy” sections.)

TABLE 59.2 Breast Masses in Adolescence

Benign Breast Masses—Common Benign Breast Masses—Rare Malignant Breast Masses—Extremely Rare Fibroadenoma Intraductal papilloma Adenocarcinoma Cyst Juvenile papillomatosis Cystosarcoma Abscess Tubular/lactational adenoma Rhabdomyosarcoma Cystosarcoma phyllodes (benign) Angiosarcoma Lymphoma Cystosarcoma phyllodes (malignant) Adenocarcinoma

Fibroadenomas

Fibroadenomas are well-circumscribed fibroepithelial lesions. Grossly, they appear as uniformly smooth or nodule white or tan lesions, sharply demarcated from surrounding tissue. They do not form a true capsule, but rather a pseudocapsule of compressed normal breast tissue pushed aside as the lesion grows. If the lesion outgrows its blood supply and becomes infarcted it could have a hemorrhagic appearance. Histologically, fibroadenomas show both an epithelial and stromal component. Ducts are usually compressed giving a classic branching or fern-like appearance. Components of fibrocystic change, epithelial hyperplasia, sclerosing adenosis, and other complex changes within a fibroadenoma are calledcomplex fibroadenomas (Dehner et al., 1999). A complex fibroadenoma is a benign lesion, although data suggest they may confer an elevated risk of primary breast cancer in later life (Dupont et al., 1994). A cellular fibroadenoma includes epithelial hyperplasia with complex architecture and cellular stroma, and is a benign finding that does not elevate future breast cancer risk. A fibroadenoma that grows to 8 cm has been called giant fibroadenoma, a distinction that is only clinical and carries no difference in histology or management (Dehner et al., 1999). Giant fibroadenomas occur more frequently in teens than in adults and more frequently in African-Americans.

1. Prevalence: Common fibroadenomas are the most common breast tumor found in adolescents in surgical reports, comprising 60% to 90% of benign breast lesions. Most adolescents with this condition are older than 14 years, with a large increase in prevalence in 15- and 16-year olds. The condition has been reported to be twice as common in African-Americans. There is no evidence of progression to cancer.
2. Clinical manifestations: On physical examination, they feel like a round or oval firm mass distinct from the rest of the breast. They can be multiple and bilateral.
3. Diagnosis: Usually, the diagnosis can be made with a combination of clinical examination, ultrasonography and FNA, and core biopsy or excisional biopsy. On ultrasonography, fibroadenomas appear as hypoechoic lesions with smooth round distinct borders, and are wider than tall.
4. Management: Once the diagnosis of fibroadenoma is confirmed, management of the mass is expectant with careful observation, physical examination, and ultrasonography every 6 months, or surgical excision. Patients who desire expectant management should be advised that their lesion might shrink and regress, or may grow, especially with subsequent pregnancy. For small fibroadenomas, <3 centimeters, minimally invasive surgical techniques allow patients to have their lesion removed with less anesthesia exposure and scarring. Under ultrasound guidance and using a large-bore needle, core biopsies of the lesion are taken until is has been removed. Alternatively, a small fibroadenoma can be cryoablated as an outpatient procedure.

Giant fibroadenomas may distort the affected breast and should the patient desire removal, simple surgical excision should be performed, sparing as much normal compressed breast tissue as possible. Preoperatively, consideration should be given to plastic surgical assistance to correct the resulting deformity. Some patients benefit from a staged surgery, with plastic surgical correction delayed until the normal compressed breast tissue has a chance to recover.

Cysts

A dominant breast mass may also be a simple cyst. The incidence of cysts is underrepresented in series of excisional adolescent breast biopsies because their management rarely requires surgery. Patients with breast cysts can present with multiple breast masses, or increasing size or tenderness of a mass associated with menses. Physical examination reveals a firm, well-circumscribed mass distinct from the surrounding breast. Ultrasonography reveals a round, well-demarcated hypoechoic structure with increased shine through transmission because of its fluid content. Cysts are easily treated with FNA of fluid and resolution of the mass. If fluid is yellow, green, or brown it can be discarded. Bloody fluid should be sent for cytology and should raise concern regarding malignancy. Any persistent solid mass after FNA expression of fluid implies a complex

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cyst, a more concerning lesion, and the patient should be referred for image-guided core biopsy. Patients should be warned that simple cysts can recur and can easily be reaspirated. Symptomatic cysts that recur after multiple aspirations should be referred for simple excision.

Fibrocystic Changes

Patients with symptomatic fibrocystic change complain of multiple masses or nodularity, with pain increasing with menses. If surgical excision is performed, histology reveals proliferative changes of ducts and lobules, duct dilatation and elongation, and terminal duct cyst formation (Vorherr, 1986).

1. Although the true prevalence of fibrocystic changes in adolescents is unknown, they are likely very common in women in the reproductive–age-group. The prevalence increases even during the third and fourth decade of life.
2. Pathophysiology is unknown but this probably represents changes associated with effects of estrogen and progesterone on breast tissue.
3. Clinical manifestations: On physical examination, nodularity is apparent, most commonly in the upper outer quadrant of one or both breasts. Masses may not be as mobile and distinct from surrounding breast as fibroadenomas or cysts. Symptoms, particularly tenderness are more common a week before menstruation and are often relieved by menstruation.
4. Ultrasonography fails to reveal a single underlying mass; it may show areas of increased fibrous tissue or microcysts.
5. Treatment: Treatment in adolescents includes mild analgesics, well supporting bras and a trial of oral contraceptives for those with more severe symptoms. Symptoms in teens are rarely severe and aggressive measures used in adults, such as oral danazol or tamoxifen, have not been studied in teens. A previously suggested correlation between caffeine intake and breast symptomatology has not been validated in larger studies.

Tubular and Lactating Adenoma

Additional histologies that can be found rarely at biopsy of a solitary mass in an adolescent are tubular and lactating adenomas. Tubular adenomas are a well-circumscribed collection of uniform tubular structures spaced closely together (Dehner et al., 1999). A lactating adenoma adds generalized lactational changes and hyperplastic lobules. Both are rare benign findings that do not require further management.

Juvenile Papillomatosis

This lesion presents as a unilateral solitary painless breast mass clinically consistent with a fibroadenoma. Fifty percent of patients with this diagnosis are younger than 20 years (Rosen and Kimmel, 1990). Grossly, it is a firm well-circumscribed mass with multiple cysts separated by fibrous septa. On histological inspection, simple cysts with cuboidal epithelial cells, papillary ductal hyperplasia, and apocrine metaplasia are found in varying proportions. Epithelial hyperplasia may exhibit papillary, micropapillary, and cribriform architecture. Some cysts may be filled with foamy macrophages and secretions. Fibrocystic changes are also seen, including lobular hyperplasia, sclerosing adenosis, and fibroadenomatoid hyperplasia.

The clinical significance of this lesion involves the controversy over its inclusion as a preneoplastic lesion. The initial study that identified this entity noted that 50% of patients with juvenile papillomatosis had a history of breast cancer in a first-degree relative (Rosen and Kimmel, 1990). Seven older patients in this series had concurrent breast cancer. Local recurrence after diagnosis does occur (Dehner et al., 1999). For these reasons, treatment is wide surgical excision and close clinical follow-up.

Breast Malignancy

Prevalence

Primary breast cancer is a rare phenomenon in adolescents. One 25-year survey of a tertiary children's hospital identified 18 patients with breast cancer, 16 females and 2 males (Rogers et al., 1994). Of these 18 tumors identified, 13 were metastatic disease, 2 were primary breast malignancy, and 3 were secondary malignancy. Breast carcinoma, so common a disease in older age, is extremely rare in adolescents. Neinstein's review of 1,791 breast excisions found five primary adenocarcinomas in teens (Neinstein, 1994). Dehner et al., found only 1 case of breast carcinoma in a review of 134 cases of breast pathology in patients through 20 years of age. History of prior radiotherapy to the chest may be a predisposing factor. Rogers et al., found three cases of breast carcinoma diagnosed in adulthood as secondary malignancies in patients with a history of Hodgkin lymphoma as teens (Rogers et al., 1994). The more common primary breast malignancies found in teens are rhabdomyosarcoma, angiosarcoma or cystosarcoma phyllodes, or lymphoma. Tumors that metastasize to the breast include rhabdomyosarcoma, lymphoma, and neuroblastoma.

History

Whenever possible the history should be taken with the patient dressed. She may feel more comfortable answering sensitive questions (e.g., her gynecological history or about illicit drug use), without the added vulnerability of being undressed.

1. History of symptoms: A detailed history of symptoms can give important clues to a patient's underlying diagnosis. For example, a pain chart can reveal cyclic or constant pain.
2. Change in size of mass: Masses that change in size with menstrual cycles may be cysts; those that do not are more likely solid masses. Sudden increase in growth is a concerning sign.
3. Medication history: Recent initiation of medication could explain mastodynia or nipple discharge. Although there have been obvious benefits of oral contraceptives in the risk of endometrial and ovarian cancer, their impact on breast cancer has been more controversial. Almost all epidemiological studies of present and past oral contraceptive users demonstrate risk ratios whose confidence intervals cluster around 1.0 or include 1.0 indicating no statistically significant increased risk. Some studies have indicated a slight increased risk in selected subgroups. Both the Centers for Disease

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Control and Prevention (CDC) Cancer and Steroid Hormone Study and a World Health Organization study of 5,000 women found no increased risk of breast cancer with duration of use. Overall, there is no conclusive evidence that oral contraceptives increase a woman's risk of breast cancer. See Chapter 43 for a more in-depth discussion.

4. History of trauma: Also important is a history of trauma; this may not only explain breast complaints, but also alert a clinician to a serious underlying social problem and other potential injuries.
5. Past medical history: Past medical history may identify factors that increase the risk of malignancy. Chest wall radiation, for example for lymphoma, increases the risk of subsequent breast cancer (Rogers et al., 1994). Patients with a history of malignancy that can metastasize to the breast (lymphoma, rhabdomyosarcoma) should raise concern. Any patient with a history of cancer must be followed up carefully for evidence of recurrence, including breast cancer recurrence.
6. Family history: Risk factors for malignancy should be queried of each patient although most patients with cancer have no identifiable risk factors (Cady et al., 1998). First-degree family members affected by cancer increase a patient's risk; the age of these family members should be carefully documented. Screening for breast disease should begin 10 years before the age at which the youngest close relative was diagnosed. Patients with strong family histories of breast cancer, especially if bilateral disease or in conjunction with ovarian or endometrial cancer, should be referred for genetic counseling. Estimates of breast cancer risk show as much as an eightfold increase if the disease developed before menopause or was bilateral (Cady et al., 1998). Genetic testing may be indicated in the affected family member.
7. Genetics: At least eight candidate-susceptibility genes have been identified. Probably the most important that lead to striking family clusters of breast cancer are mutations of BRCA1 and BRCA2, accounting for almost 80% of hereditary breast cancer and approximately 5% to 6% of all breast cancers (Greene, 1997; Calederon-Margalit and Paltiel, 2004;Evans et al., 2005). Although these mutations are rare in the general population (5–50 per 100,000), they may occur in approximately 1% of all Jewish women of Ashkenazic descent (Evans et al., 2005). Routine screening of BRCA1 is not warranted because of the difficulty in evaluating the multiple mutations, social discrimination issues such as insurance, and the lack of appropriate preventive strategies. However, genetic testing for cancer risk is likely to change dramatically in the coming years. Elger and Harding (2000) discuss testing for BRCA1 in the adolescent population, and the importance of making distinctions between testing children and adolescents. Samuel and Ollila (2005) review the difficult decisions faced by young women who carry a BRCA mutation regarding radiological screening and potential prophylactic surgery.

Breast Self-Examination

The question regarding instruction of breast self-examination engenders controversy. Breast self-examination has a sensitivity of only 30% in detecting breast disease. As sensitivity improves over time as patients learn what is normal in their own breasts, specificity decreases (O'Malley and Fetcher, 1987). Breast self-examination has not been shown to improve survival in breast cancer (O'Malley and Fetcher, 1987). Indeed, it has been shown to increase anxiety and depression in patients who believe they find an abnormality (Baxter, 2001;Ellman et al., 1993). Gaskie and Nashelsky, 2005 report that although breast self-examination has little impact on breast cancer mortality, careful clinical breast examination can affect breast cancer mortality rates. In young women, in whom malignant disease is rare, examinations are more likely to discover clinically insignificant benign disease than malignancy. However, there is benefit to breast self-examination in that it establishes a life-long habit of patients investing in their own breast health. Current recommendations from the American Cancer Society as listed on their Web site for the general public defers any recommendations for or against breast self-examination saying: “Women should be told about the benefits and limitations of breast self-examination.” The World Health Organization does not recommend teaching women younger than 20 years to do breast self-examination (World Health Organization, 1983).

Physical Examination of the Breast

A thorough and careful breast examination is essential to the evaluation of any breast complaint. In the adolescent patient, a clinician must be sensitive to her feelings of embarrassment or modesty. Before the examination begins, she should be informed of its components and given the opportunity to be accompanied by a parent or friend. If she declines, a chaperone should accompany the examiner, especially if he is male. A discussion of what changes she can expect during breast development, with an emphasis on the wide range of what is normal, may reduce her anxiety of the examination.

The optimal time for a breast examination is within a week of completion of menses, when the breast is most quiescent and least tender. If an examination is indeterminant, repeating it at a different point in her menstrual cycle may be informative. Inspection is an important and often overlooked first step in the examination. This should be performed with arms outstretched, overhead, and resting at the waist to allow the clinician to see possible distortion in various positions, as well as dermatological conditions affecting the breasts. Attention should also be paid to comparing the two breasts for symmetry.

Palpation

Palpation should be performed both sitting and standing, especially in large-breasted patients. It does not matter with which technique the palpation is performed as long as it is thorough and systematic, covering each breast from the clavicle to inframammary fold, and from sternum to mid-axillary line. Studies show time spent on examination correlates best with detection (Fletcher et al., 1985). Uncovering only the breast being examined may help the patient feel more comfortable.

Palpation should include the axillae and supraclavicular fossa. These areas are best assessed while the patient is

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sitting. If she rests her upper arm on the arm of the examiner and relaxes her shoulder, deep palpation of the axilla can be performed with minimal discomfort.

The examiner can examine the breast in one of the following several ways:

1. Spokes of a wheel: In this method, the examiner uses a pattern similar to the spokes of a wheel. Starting with the tail of the breast in the axilla, the examiner moves in a straight line to the nipple. Using straight lines from the outer boundary of the breast to the nipple, the examiner can work around the whole breast.
2. Concentric circles: A second method involves covering the breast in either concentric circles or a spiral pattern around the breast.
3. Vertical strips: A third method involves covering the breast by examining vertical strips. In a study by Atkins et al. (1991) this was found to be the most effective method.

Whichever method is used, the entire anterior chest wall should be palpated, applying varying degrees of pressure with the pads of the second, third, and fourth fingers and rotating in small dime-sized circles. Particular attention should be given to the nipple and areolar areas, as 15% of breast cancers are located here. The areola should be compressed to elicit nipple discharge. Notation of nipple discharge if elicited should include its color, from which and how many ducts it appeared, and palpation of which quadrant of the breast elicited it. Using the analogy of a clock face to describe the offending duct or quadrant of breast from which it is elicited is the most reproducible way to locate the source of discharge. The examiner should also palpate for supraclavicular, infraclavicular, and axillary nodes. Occasionally, using talc in palpating the breast may be helpful.

If breast masses are appreciated, they too should be described with careful attention to their location. Only dominant masses with texture distinct from the surrounding tissue should be identified as masses; areas of increased nodularity without discrete masses should be identified as such. This distinction is important as one condition implies underlying pathology whereas the other is often a variant of normal. Notation should be made of the mobility and firmness of a mass, as well as tenderness.

When examining a patient complaining of an ill-defined or deep mass, or nipple discharge not elicited by the examiner, lotion, gel, soap, or lubrication on the examiners fingers can assist in deep or repeated palpation with less discomfort to the patient.

Once the examiner has completed their examination, it is useful to ask the patient to pinpoint the location of the complaint that brought her into the office. This will allow the examiner to form an independent opinion of the normality or abnormality of her examination, and then address her specific concern. Likewise, it is useful to begin the examination on the unaffected side, to gain an appreciation of what her normal breast tissue feels like, before evaluating an area of suspected pathology.

Breast Imaging

Breast imaging is indicated any time a clinician appreciates an abnormality on physical examination. Physical examination alone is not enough to rule out malignancy, despite its rarity in this age-group.

Mammography

Mammography is an excellent screening tool for breasts that are primarily fatty replaced. As the developing breast is more fibroglandular than fatty, mammography is not as sensitive as it is in an older population (Chateil et al., 1998). Another study found that mammograms failed to identify 75% of palpable masses in women younger than 30 (Williams et al., 1986). The problem is mass and calcifications, the two most important findings on mammography, appear white. Glandular tissue also appears white. In a young patient this means a white lesion on a white background. Fatty tissue is grey; in older women white lesions on a grey background are easier to see.

Ultrasonography

Ultrasonography is ideally suited to evaluating masses in the adolescent population as it is superior to mammography in characterizing lesions in fibroglandular breasts (Garcia et al., 2000). Ultrasonography is superior in characterizing masses and can distinguish a cystic mass from a solid one. It cannot distinguish a benign solid mass from a malignant one. The hallmarks of benign lesions in adult patients—smooth regular borders, multiple masses, and homogeneous solid masses—can also be seen in metastatic lesions to juvenile breasts (Chateil et al., 1998). Abscesses carry the same characteristics of malignant adult lesions—irregular borders, heterogeneous contents.

Breast Biopsy

Because of the limitations of physical examination and imaging, careful consideration should be given in obtaining a biopsy of any palpable mass. A FNA of the mass obtains important histological evidence without causing much discomfort to the patient. If an aspiration obtains nonbloody cyst fluid, the lesion can be diagnosed and treated with the same diagnostic test—it is a benign cyst. Cytology on the cyst fluid is not indicated unless it is bloody. If the cyst fails to resolve, but leaves a palpable mass, it needs to be biopsied.

If FNA reveals a solid mass, a smear of cells obtained can characterize the mass. FNA has been demonstrated to have a sensitivity of 90% and specificity of 100% in pediatric patients when diagnosing carcinoma (Silverman et al., 1991). If any doubt remains regarding the benign nature of a mass, despite a reassuring FNA, further tissue must be obtained for diagnosis. Previously, excisional biopsies were performed to diagnose and excise the lesion. However, the value of achieving a diagnosis through a minimally invasive core biopsy has merit—it can save a patient a surgery and a scar or breast deformity in a cosmetically sensitive area if the results are benign. If the results show metastatic disease, this procedure could save a patient an unnecessary surgery. If the results show primary breast cancer, like cystosarcoma, this allows for appropriate preoperative planning of one definitive surgery for the patient.

Once cytological or core biopsy results have been obtained, all three pieces of the diagnostic triad must be reviewed—physical examination, imaging, and biopsy. If all three concur the mass is benign, the health care provider can be 99% certain the mass is truly benign (Hindle, 1999). The patient may then safely decline further intervention.

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She should be followed up with serial examinations and ultrasonograms, every 6 months to a year, to establish stability of the lesion. Any sudden growth should raise suspicion of malignant transformation. If any one piece of the diagnostic triad is not in concordance, the lesion must be excised.

Management

Management of these tumors depends on the underlying primary disease. Primary breast carcinoma and breast lymphoma in teens should be treated as it is in adults.

Specific Breast Mass Lesions

Cystosarcoma Phyllodes

Cystosarcoma phyllodes are tumors more commonly found in the fourth and fifth decades of life, and can be benign or malignant. They are the most common breast sarcoma of adolescence (Rogers et al., 1994). They comprise 1% of breast neoplasms and 2.5% of fibroepithelial lesions (Rajan et al., 1998). Features that indicate malignant transformation include invasive tumor border, high stromal cell mitotic counts, cytological atypia, and necrosis. They are further divided into low and high grade. A series of 45 lesions in patients aged 10 to 24 found 34 benign lesions and 11 malignancies (Rajan et al., 1998). Treatment of cystosarcoma phyllodes and other primary breast sarcomas is wide local resection with achievement of negative margins being the surgical goal. Recent evidence shows teens may have less aggressive tumors and lower recurrence rates than adults (Rajan et al., 1998). These tumors do not metastasize to axillary lymph nodes, so no axillary procedure is indicated. The most common sites of metastases are lung and bone, so metastatic work-up should focus on these areas.

Rhabdomyosarcoma

Patients with a breast biopsy showing rhabdomyosarcoma should be screened for occult primary disease outside the breast. Metastatic rhabdomyosarcoma is more common than primary breast rhabdomyosarcoma. Isolated primary breast sarcoma should be resected aggressively as negative surgical margins are the only factors proved to influence survival (Pandey et al., 2004). Mastectomy is indicated only if the tumor-to-breast ratio does not favor breast conservation. Metastatic rhabdomyosarcoma to the breast carries an exceedingly poor prognosis as it frequently is a hallmark of widely disseminated disease (Hays et al., 1997). Treatment is chemotherapy.

Summary

As adolescence is a period of rapid and complex physical and psychological change, breast complaints in this age-group commonly center around discomfort (mastalgia), developmental variations (asymmetry), or developmental anomalies (hypo- or hyperplasia). It is the health care provider's role to reassure patients and their families regarding the spectrum of normal development, while appropriately intervening when developmental anomalies occur. Clinicians should not hesitate to involve mental health experts and plastic surgeons, when appropriate. Masses in this age-group are uncommon; malignant masses are thankfully rare. Persistent masses require evaluation. The physical examination, fine needle biopsy, and ultrasound imaging provide diagnostic information in a minimally invasive manner.

WEB SITES

For Teenagers and Parents

http://www.puberty101.com. A Web site with general information about puberty, including breast size and normal breast development.

http://www.youngwomenshealth.org. Section on breast health among glossary of terms; from Children's Hospital Boston Web site.

http://www.womenshealth.about.com. Contains a section on teen breast complaints, as well as provides question and answers.

http://www.goaskalice.Columbia.edu. Frank answers to common and uncommon teen questions from the Health Services at Columbia University, including those on breast health.

http://www.wdxcyber.com. Written by obstetrician-gynecologists. Has a breast section, written more specifically for general women's health, rather than adolescents. Advice from the Women's Diagnostic Cyber.

http://www.teenshealth.com. Detailed discussions regarding plastic surgery in teens, including excellent breast section.

For Health Professionals

http://www.cdc.gov/cancer/NBCCEDP. The CDC Women's Health page.

http://www.acog.org. Web site of the American College of Obstetrics and Gynecology.

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