Ms. B, a 46-year-old Caucasian, presented to the obstetrics/gynecology clinic for her annual well-woman exam. She had a normal well-check about one year earlier. She had recently moved and was anxious about finding a new health-care provider. At this exam, she had no complaints but expressed a tremendous urgency to get on with her preventive care, especially her mammogram.
1. Examination
Ms. B’s medical history included hypothyroidism and situational anxiety; surgical history included a laser conization of the cervix 11 years earlier and biopsy of a benign thyroid nodule. She had been pregnant three times, resulting in two births (the first at age 23 years) and one miscarriage. Menarche was age 12 years, and she was not yet menopausal. Medications included levothyroxine (Synthroid) 0.05 mg daily, norethindrone/ethinyl estradiol (Loestrin), and alprazolam (Xanax) 0.05 mg for occasional anxiety. She does not use tobacco, has two to three drinks per week, and reports no recreational drug use. Family history includes multiple women with breast cancer, including a sister who was diagnosed at age 24 years and recently passed away after 20 cancer-free years.
Ms. B’s height was 62 in and weight 149 lbs. Her BP was 118/64 mm Hg and oral temperature was 98.4°F. Complete physical examination was normal, with a negative hemoccult. Pap smear and routine screening mammogram were negative. The patient was instructed to come back in one year for her annual exam, or sooner if necessary.
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Two months later, Ms. B called the office to express concerns about new pain and swelling in her left breast. She was brought in that day for an exam. During the evaluation, the patient explained that she first noted the pain one month earlier and attributed it to a minor injury she sustained while water-skiing at a local lake. She expected the pain to resolve with time, but it seemed to be worsening.
No palpable lumps or nipple discharge was reported, but the patient had recently noticed some redness on the same breast. She also reported that while her right breast had always been slightly larger than her left, the size-discrepancy had reversed since onset of the new pain and swelling.
Exam revealed a completely normal right breast without dominant masses, skin changes, or nipple discharge. Despite a completely normal exam three months earlier, Ms. B’s left breast was visibly erythematous along most of the inferior surface and the skin somewhat edematous, thickened, and irregularly dimpled. In addition to tenderness in the breast, the patient also reported pain in the axilla, where two small palpable lumps were noted.
A bilateral diagnostic mammogram with ultrasound was ordered, and the importance of a prompt consultation with a general surgeon was discussed with the patient. Ms. B was told that her condition did not seem to be an infectious process, and that such skin changes are extremely worrisome. She agreed to make the necessary appointments and follow up as soon as possible.
Ten days later, Ms. B phoned to report increased swelling in the left breast and tingling pain that radiated to her neck, back, and left flank. Calls were made to move up her mammography and surgical appointments.
2. Diagnosis
The diagnostic mammogram and ultrasound showed dense breast tissue but overall benign findings. There was no change from a mammogram she brought for comparison from three years earlier except for two axillary nodules measuring 5.0 mm and 1.6 mm, which appeared to be lymph nodes. MRI showed irregular axillary nodes and skin thickening, and irregular-appearing tissue was noted throughout the entire inferior portion of the breast. The surgeon informed Ms. B that a biopsy should be done immediately.
A biopsy was performed the following Monday, and the pathology report revealed an infiltrating ductal carcinoma with dermal lymphatic invasion. The specimen was estrogen-progesterone-receptor negative, and the patient was informed of the diagnosis of inflammatory breast cancer (IBC). Ms. B subsequently went on to receive several courses of chemotherapy.
Approximately eight months after her initial appointment, Ms. B had a left modified radical mastectomy and a right prophylactic mastectomy. At her one-month postoperative checkup, her incisions were well healed, and she was preparing to begin radiation therapy the following week. She reported to the surgeon that she had just noticed a submandibular lump on the left side, and admitted that some sinus congestion and drainage she was having seemed to coincide with her finding the lump. She was instructed to follow up with her family physician for treatment of the possible upper-respiratory infection and return in two weeks if the lump had not resolved.
She came back one week later stating that the lump was unchanged. The surgeon believed that this was likely a benign process, as the lump was soft, mobile, and corresponded to where one would commonly palpate a lymph node. As a precaution, she instructed the patient to return in two weeks for re-evaluation.
Ms. B returned as directed. The surgeon noted that the lump had grown and was now a 1.0-cm x 1.5-cm firm area. There was no palpable axillary adenopathy. An ultrasound-guided fine-needle aspiration was ordered and performed two weeks later. The pathology report showed metastatic carcinoma. The patient was seen one more time at the surgeon’s office where she indicated that she would be receiving experimental treatment as part of a clinical trial. She was subsequently lost to follow-up.
3. Discussion
Despite the immediate referral to a general surgeon and relatively quick diagnosis, this patient’s unfortunate outcome of early metastatic disease illustrates that IBC is an extremely aggressive and difficult-to-treat form of breast cancer. It is critical that diagnosis not be delayed, which happens frequently because the signs and symptoms are commonly mistaken for mastitis. All patients being treated for mastitis should be instructed to follow up 48 hours after initiating antibiotics, and a high index of suspicion should be present for any patient with mastitislike symptoms not associated with lactation. Like other breast cancers, IBC may be missed with mammography and ultrasound. Patients with clinically suspicious findings should be seen right away by a general surgeon, regardless of benign-looking results on imaging studies.
IBC accounts for between 1% and 5% of all breast cancers in the United States.1 The name comes from the red and swollen appearance of the skin on the affected breast. Cancer cells block the skin’s lymphatic vessels, often creating a dimpled orange-peel (peau d’orange) surface texture. Other symptoms may include pink, reddish-purple, or bruised-appearing skin; ridges or pitting in the skin; warmth; nipple inversion; increased breast size and tenderness; and a sense of heaviness, burning, or aching in the breast. Tender or swollen lymph nodes may be noted in the axilla and/or supraclavicular area on the side of the affected breast. Symptoms usually have a rapid onset of one to three months. Diagnosis is primarily made on clinical grounds, with mammogram, ultrasound, and biopsy for confirmation. Because a dominant mass is rarely palpable or visible on imaging, biopsy should always include a full-thickness segment of skin.2
Since IBC is a locally advanced breast cancer, all cases will be classified as stage III or IV.3 Surgery has been shown to be ineffective as a first-line treatment and is technically difficult, with widespread involvement of the breast’s skin, underlying tissue, and lymphatics. Standard treatment involves administering chemotherapy first.4 Modified radical mastectomy with lymph node dissection is standard procedure and is followed by radiation to the chest wall. Sometimes hormonal therapy is recommended, depending on hormone receptor status.4
Supportive care to assist with such iatrogenic side effects as lymphedema can positively impact quality of life. Despite advances in treatment, prognosis for patients with IBC remains poor. Historically, treatment was limited to local control, with a median survival of less than two years and five-year survival rate of 5%. The use of modern multimodality treatment has improved five-year survival rates to 25% to 50%, which is still significantly lower than the average for other types of breast cancer.5
Ms. Ellis is a physician assistant at Desert Jewel Obstetrics & Gynecology, Scottsdale, Ariz.
References
1. Merajver SD, Sabel MS. Inflammatory breast cancer. In: Harris JR, Lippman ME, Morrow M, Osborne CK, eds. Diseases of the Breast. 3rd ed. Philadelphia, Pa.: Lippincott Williams and Wilkins; 2004:971-982.
2. Ahearne PM, Feig BW, Leach SD. Invasive breast cancer. In: Feig BW, Berger DH, Fuhrman GM, eds. The M.D. Anderson Surgical Oncology Handbook. 2nd ed. Philadelphia, Pa.: Lippincott Williams and Wilkins; 1999:23-59.
3. Anderson WF, Schairer C, Chen BE, et al. Epidemiology of inflammatory breast cancer (IBC). Breast Dis. 2005-2006;22:9-23. Available at www.ncbi.nlm.nih.gov/pmc/articles/pmid/16735783/.
4. National Cancer Institute. Inflammatory breast cancer: questions and answers. Available at www.cancer.gov/cancertopics/factsheet/Sites-Types/IBC.
5. Chittoor SR, Swain SM. Locally advanced breast cancer: Role of medical oncology. In: Bland KI, Copeland EM, eds. The Breast: Comprehensive Management of Benign and Malignant Diseases. Vol 2. 2nd ed. Philadelphia, Pa.: W.B. Saunders Company; 1988.
All electronic documents accessed November 15, 2010.
