In the late 1980s, my supervising physician and I saw Mary (not her real name), a 20-year-old who called her breasts Pride and Joy. She was referred by a medical oncologist who was evaluating changes in Mary's right breast that were suspicious for inflammatory breast cancer. The patient also had a large mass in the left breast. We were asked to perform bilateral breast biopsies. The patient was reluctant to undergo any other surgical procedures, although she was willing to have neoadjuvant chemotherapy. There was no known family history of breast cancer.

On examination, a mobile, nontender, 4-cm mass was palpable in the upper outer quadrant of the left breast. The right breast was erythematous, twice the size of the left breast, with peau d'orange. Two enlarged lymph nodes were palpable in the right axilla and one in the left axilla. On xerograms performed to rule out additional lesions, the right breast showed diffuse thickening and edematous changes without a discrete mass; the left breast showed dense tissue throughout without suspicious masses. The diagnosis ofwas made in the operating room by incisional breast biopsies. Frozen sections showed invasive lobular cancer in the left breast and confirmed inflammatory breast cancer involving the dermis in the right breast.

Mary did very well during chemotherapy and was proud of not missing a day of work at Pizza Hut. The erythema of the right breast resolved after chemotherapy, but the skin remained thickened and the breast was firmer than the left breast. The left breast mass was no longer palpable. The lymph nodes on each side also responded to the chemotherapy; they were still palpable but were not as large.

Mary was a feisty and defiant young woman who refused the modified radical mastectomy that was the recommended treatment for inflammatory breast cancer in 1987. We also suggested a partial mastectomy with axillary dissection for the left breast. However, the patient did not want additional scars on her breasts, nor did she did want to lose her breast, although she did agree to undergo mammography every 6 months. Just shy of her 1-year diagnosis, small pink nodules began to appear across the right breast incision. She refused biopsy of the nodules and any further treatment. Mary died of metastatic breast cancer when she was 22 years old.

We were all shaken by that experience. Historically, breast cancer was thought of as a disease of older
women; only 4% to 5% of women younger than 40 years will develop breast cancer. When breast cancer did occur in a young woman, the disease tended to be more aggressive and more difficult to treat, even with the most compliant patients. On one hand, we understood how important this young woman's self-image was to her, but her insistence on avoiding surgery prevent-ed her from receiving the treatment that might have increased her survival. We were not given a chance to help her fight her disease. She did everything she could to preserve her breasts, but in the end she did very little to keep the rest of herself safe from the disease they harbored.
 
A few years later, I met Janice (not her real name), a 20-year-old college student who found a mass in her right breast. The lesion did not feel like a cyst or a fibroadenoma. Ultrasonography did not show a solid or a fluid-filled lesion. Mammography revealed a spiculated density deep in the upper outer quadrant. Both Janice and her mom, Mrs. J., were very frightened since several women in the family had had breast cancer and the family was of Ashkenazi descent (women with inherited mutations have a 36% to 85% lifetime risk of breast cancer.) The frozen section from material obtained from a needle-localized excisional breast biopsy was positive for infiltrating duct cancer.

In the recovery room, Janice and her parents were very emotional during the difficult discussion—as were we, thinking back to our experience with Mary. Yet Janice and her parents were determined to do what was necessary so that Janice could beat the cancer and get on with her education and life. The next week, Janice underwent evaluation for metastasis, which was negative, and she met with the medical and radiation oncologist. Her tumor was 1.5 cm and negative for estrogen and progesterone receptors, so she was a good candidate for a partial mastectomy, axillary lymph node dissection, chemotherapy, and radiation therapy. Her margins were negative, and 15 lymph nodes were negative for metastatic disease. Janice was always upbeat and did very well throughout her treatments. The surgical scarring of her par-were barely noticeable.

During a routine follow-up appointment 2 years later, a new abnormality was found in Janice's left breast. Once again, a needle localized excisional breast biopsy was performed, and she was found to have a 1-cm infiltrating duct tumor. Janice and her mother agreed that Janice would have breast conservation surgery, a different regimen of chemotherapy, and radiation therapy. Again, she did very well. The medical oncologist recommended newly available genetic testing, which came back positive for BRCA1 while she was in the midst of chemotherapy. Janice was a 22-year-old new college graduate with a steady boyfriend, and she hoped to have children in the future. Eventually, she would confront reducing her breast cancer recurrence risk, but for now she did not want bilateral mastectomy and salpingoophorectomy. She was willing to take tamoxifen (Nolvadex) for 5 years. We continued to follow her every 6 months.

BREAST CANCER IN 2007

Janice's mom, Mrs. J., accompanied her for most of her clinic appointments. Several years after Janice's diagnosis, her mom telephoned about wanting to be checked for a mass underneath her right arm. She thought the mass resulted from a shaving nick, but it had not gone away after several weeks. Within the past 3 months, she had undergone bilateral digital mammography* that was negative, with dense tissue reported bilaterally. However, on ultrasound, a 1.5-cm lymph node was found high in her right axilla. Instead of performing an open biopsy in the operating room, the radiologist agreed to perform an ultrasound-guided core biopsy, the result of which was positive for infiltrating duct cancer. Tests for estrogen and progesterone receptors, as well as for the HER2/neu protein, were also positive. Breast MRI was recommended because of the family history of BRCA1 coupled with the axillary manifestation of breast cancer. A suspicious lesion was found in the central portion of he right breast.

Because Janice carried the BRCA1 gene, Janice's mom, who was postmenopausal, was referred for genetic counseling. A left prophylactic mastectomy with sentinel lymph node biopsy and bilateral alpingoophorectomy were easy choices for her to make. She also underwent a right modified radical mastectomy and chemotherapy port placement for breast cancer treatment. A 2-cm invasive duct cancer was found in the right breast, and lobular carcinoma in situ was found in the left breast. Two of 20 lymph nodes were positive for metastatic breast cancer on the right side; the left sentinel lymph node biopsy was negative. Following chemotherapy that included docetaxel (Taxotere), Mrs. J. had bilateral deep inferior epigastric perforator flaps, which use fatty tissue and the blood supply of the abdomen for breast reconstruction. She took trastuzumab (Herceptin) for 1 year, and she is currently taking an aromatase inhibitor.

I continue to see Janice and Mrs. J. for follow-up appointments approximately every 3 months. At our recent visit, Janice and her mom were very excited about Janice's upcoming wedding. I was gratified to see them through their very difficult experiences with breast cancer and their recoveries.

THEN AND NOW

Both young patients had breast cancer before the availability of many of the advances in diagnosis and treatment that enabled Janice's mother to have a single surgical experience. Genetic testing, taxanes, Herceptin, aromatase inhibitors, and prophylactic mastectomy to reduce breast cancer risk are now offered routinely. Guided breast biopsies using ultrasound and stereotactic techniques are now offered with MRI.

Another difference is that Mary was treated in a time when patients were called noncompliant if they did not follow physician recommendations. Fortunately, compliance has been replaced by adherence and the patient-centered medicine movement, which allowed Janice and her mother to be participants in their own care. Their wishes were respected, and they were happy with their treatment and results.

While my son and I were waiting in an airport check-in line, Janice, her new husband, and her mom joined the same line. Her mom was dropping off the newlyweds for their honeymoon. My belief was affirmed that I was meant to help people like Janice and her mother overcome breast cancer and go on to lead happy and healthy lives. Comparing their experiences with Mary's shows how advances in breast cancer therapy have indeed made things better for patients. I wonder: if Mary had seen Janice's and her mom's results, would she have been more willing to let us treat her? If she had agreed to social services support, surgery, radiation therapy, and our caring as health providers, would that have made a difference? JAAPA

* Bold phrases refer to ideas, therapies, and diagnoses that did not exist in 1987.

Janet Rigberg-Mayes works for Virginia Surgery Associates in Fairfax, Virginia.