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What is Phyllodes Tumor?
Cystosarcoma phyllodes (CSP) is a rare type of breast tumor. It is categorized by the National Cancer Institute (NCI) as a tumor subtype that occurs within the breast but is not considered a typical cancer. Its name comes from two Greek words that mean "fleshy tumor" and "leaflike," because its internal structure resembles a leaf when the tumor is cross-sectioned. The term phyllodes tumor is considered preferable to CSP as of the early 2000s because most of these tumors are benign. Phyllodes tumors are also known as giant fibroadenomas of the breast.
Phyllodes tumors develop only in the breast; they are never found in other parts of the body. They are formed within the stroma (connective tissue) of the breast and contain glandular as well as stromal tissue. Phyllodes tumors can grow noticeably within a matter of weeks, causing the overlying skin to become semi-transparent or reddish and warm to the touch. They do not, however, usually involve the nipple or areola.
A phyllodes tumor can be moved freely within the breast when the doctor performs a manual examination. The tumor has a firm, smooth texture, can be easily distinguished from the surrounding tissue, and may grow to be quite large and bulky. The average size of phyllodes tumors is about 2 in (5 cm), although tumors as large as 11.8 in (30 cm) have been reported. These tumors do not cause pain when touched. For reasons that are not yet understood, phyllodes tumors are more likely to develop in the left breast than the right.
There is some disagreement among specialists regarding the number of phyllodes tumors that prove to be malignant. Although figures of 16–30 percent are commonly given, some doctors think that the actual incidence may be higher, as more cases of malignant tumors have been reported in the early 2000s.
Phyllodes tumors account for less than 1 percent of all breast tumors. Almost all occur in women, although a few cases have been reported in men. Phyllodes tumors have been identified in women in all age groups but are uncommon in adolescents. They are most likely to occur in women over 35.
As far as is known, phyllodes tumors occur with the same frequency in women of all races and in all parts of the world.
Causes & Symptoms
The cause of phyllodes tumors is not known as of the early 2000s.
The symptoms of a phyllodes tumor include the rapid but painless growth of a smooth, bulky mass within the affected breast. The patient may notice that her entire breast is enlarged and its shape distorted. The skin over-lying the tumor may feel warm to the touch and develop a shiny appearance; it may also become translucent.
The diagnosis of a phyllodes tumor may be made when the patient notices a rapidly growing mass in her breast and consults her doctor. After palpating (feeling) the mass and evaluating the appearance of the overlying skin, the doctor will order imaging studies and an open breast biopsy. Although a mammogram or ultrasound study may be useful in evaluating the size and location of the tumor, these tests are not reliable in distinguishing among benign phyllodes tumors, fibroadenomas, and malignant phyllodes tumors. In addition, fine-needle aspiration does not usually confirm the diagnosis; an open biopsy is considered the definitive diagnostic test as of the early 2000s.
There are no tumor marker or other blood tests that can be used to diagnose phyllodes tumors as of 2005.
Clinical Staging, Treatments, and Prognosis
Phyllodes tumors are not staged in the usual sense; they are classified on the basis of their appearance under the microscope as benign, borderline (or indeterminate), or malignant. The pathologist makes the decision on the basis of the cells' rate of division (mitosis) and the number of irregularly shaped cells in the biopsy sample. In one series of 101 patients with phyllodes tumors, 58 percent were identified as benign, 12 percent as borderline, and 30 percent as malignant.
Surgical excision (removal) is the usual treatment for phyllodes tumors, whether benign or malignant. In the case of benign tumors, the surgeon will usually try to spare as much breast tissue as possible, generally removing about 1 in (2 cm) of normal breast tissue from the area around the tumor as well as the tumor itself. If the tumor is very large, however, the doctor may remove the entire breast.
Although radiation therapy has been tried as follow-up treatment after surgery, phyllodes tumors do not respond well to either radiotherapy or chemotherapy if they recur or metastasize. In addition, malignant phyllodes tumors do not respond to hormone therapy.
The prognosis for benign phyllodes tumors is good following surgical removal, although there is a 20–35 percent chance of recurrence, particularly in patients over the age of 45. Recurrence is usually treated with further surgery, either another local excision or a complete mastectomy.
The prognosis for patients diagnosed with borderline or malignant phyllodes tumors is more guarded. About 4 percent of borderline tumors will eventually metastasize. A Mayo Clinic study of 50 patients with malignant tumors found that 32 percent had a recurrence within two years after surgery; 26 percent developed metastases, and 32 percent of the group died from their malignancy. The most common sites for metastases from malignant phyllodes tumors are the lungs, bones, liver, and chest wall, although metastases to the lymph nodes have also been reported. Most patients with metastases from a malignant phyllodes tumor die within three years of their first treatment.
Alternative and Complementary Therapies
Women who have had surgery for removal of a phyllodes tumor appear to use CAM therapies as often and for the same reasons as women treated for breast cancer. According to the Behavioral Research Center of the American Cancer Society, breast cancer survivors are highly likely to use some form of alternative or complementary therapy during cancer treatment or within a year or two of completing conventional treatment. A survey of 608 longer-term (8 years or longer) breast cancer survivors reported in early 2005 that the majority were still using CAM therapies. The survey respondents gave four reasons for using alternative treatments:
- To maintain an active role in recovery from cancer.
- To reduce their stress level.
- To reduce the risk of recurrence.
- To maintain hope.
Specific CAM therapies mentioned by the women in the ACS survey included exercise, humor, self-help books (bibliotherapy), prayer or spiritual practice, vitamin treatments, relaxation exercises, and support groups. Dr. Kenneth Pelletier, the former director of the program in complementary and alternative medicine at Stanford University School of Medicine, lists hypnosis, visualization, naturopathy, and journaling as other alternative approaches that breast cancer patients find helpful. Acupuncture is frequently mentioned as a useful method of pain control.
Coping With Treatment
Coping with the aftereffects of surgery for a phyllodes tumor is similar to coping with the effects of surgery for breast cancer. Patients who have had a complete mastectomy may experience pain, limited range of motion or weakness in the affected arm, scarring, or swelling. Exercises, outpatient physical therapy, and massage help to relieve these side effects of breast surgery. In terms of follow-up, most patients treated for phyllodes tumors are scheduled for a postoperative visit with the surgeon 1–2 weeks after surgery, with periodic checkups thereafter.
The National Cancer Institute (NCI) is not conducting any clinical trials involving phyllodes tumors as of 2005. There is, however, an ongoing study at the Dartmouth-Hitchcock Medical Center in New Hampshire of the effectiveness of radiation therapy in preventing recurrences of borderline or malignant phyllodes tumors in patients who have been treated with local excision of the tumor. Women who have had a borderline or malignant phyllodes tumor removed within the past three months may wish to consider participating in this study.
There is no way to prevent phyllodes tumors as of the early 2000s because their cause is not yet known.
Questions to Ask Your Doctor
- What are the chances that my phyllodes tumor is either borderline or malignant?
- What are the chances of a recurrence?
- Would you recommend a total mastectomy rather than local excision to minimize the risk of recurrence?
The special concerns of patients with phyllodes tumors are similar to those of patients diagnosed with breast cancer, particularly concern about disfigurement, physical weakness, or recurrence if the entire breast has been removed.