GIANT BREAST TUMORS Mohammed B. Hawary, FRCSC; Eufemiano Cardoso, MD; Sultan Mahmud, MD; Jamal Hassanain, MD For a variety of reasons, giant breast tumors continue to pose a challenge in diagnosis and management. These tumors are poorly understood because of their rarity and unpredictable behavior. Their rapid growth, associated with skin congestion and ulceration, and tendency to recur, gives rise to a suspicion of malignancy.1,2 In addition, owing to the varied histological features seen in these tumors, there have been widely varying interpretations and diagnoses by pathologists.3 This has led to inappropriate, and at times unnecessarily radical, surgical therapy. In the 1950s, breasts were amputated for this relatively non-threatening condition.4 However, the present trend is towards more conservative management. In order to ensure proper surgical management, an understanding of the natural history of the disease and its biologic behavior is essential. We present six cases of giant breast tumors treated at King Khalid University Hospital over a 15-year period. Our aim is to increase awareness of the existence of this rare condition in the Middle East. The literature is reviewed to elucidate principles of management of these tumors. All six cases studied presented with unilateral, rapidly growing breast mass over a period of six to eighteen months. The patients were in good health, and their menstrual cycles were regular. There was no family history of breast disease in any of them. The clinical features, relevant investigations and the surgical management of representative cases are summarized below. Case 1 A 14-year-old Saudi girl complained of an excessive increase of her right breast over a period of 12 months. Examination revealed a diffusely enlarged right breast, about three times the size of the left. The mass was firm, non-tender, and did not have any discrete masses in it. Dilated veins were present on the surface. An incisional biopsy showed hyperplasia of stroma and the epithelial From the Department of Surgery, Division of Plastic Surgery, King Khalid University Hospital, Riyadh, Saudi Arabia. Address reprint requests and correspondence to Dr. Hawary: Department of Surgery, King Khalid University Hospital, P.O. Box 7805, Riyadh 11472, Saudi Arabia. Accepted for publication 21 November 1998. Received 19 August 1998. lining the ducts. The histological report was inconclusive, and a diagnosis of unilateral virginal hypertrophy was made. A reduction mammoplasty using an inferior pedicle 174 Annals of Saudi Medicine, Vol 19, No 2, 1999 technique was carried out, and 780 g of breast tissue was excised. Histology of the excised tissue showed a giant fibroadenoma. Follow-up at six months showed right breast engagement due to a recurrence. She was readmitted a year later and complete excision of the tumor was done. Excision margins were confirmed to be negative microscopically. No recurrence was noted at the eight-year follow-up. Case 2 An 11-year-old Saudi girl presented with swelling of the right breast. It started soon after her menarche and grew rapidly over a nine-month period. The right breast was 2½ times larger than the left. The swelling was diffuse but not tender. There were no discrete lumps. The overlying skin was stretched and showed engorged veins. Mammography showed a homogenous mass occupying the whole breast and the normal breast tissue compressed into a thin rim at the periphery. After open biopsy confirmed the tumor to be a giant fibroadenoma, the tumor (15x12x12 cm) was enucleated through an inframammary incision. Follow-up showed no recurrence over a 10-year period. Case 3 An 18-year-old Saudi female had normal breasts until the age of 16, when the left breast began to grow rapidly over a 16-month period. The left breast was about three times the size of the right one. The swelling was diffuse, non-tender and firm with no discrete masses. The veins over the surface were distended. Mammography findings were identical to those of Case 2. The mass was confirmed to be a giant fibroadenoma by aspiration biopsy. At operation, the tumor (18x16x12 cm) was enucleated through a submammary incision. The thinned-out, wrinkled excess skin was excised. There was no recurrence over a six-year follow-up period. Case 4 A 17-year-old Saudi female complained of a painless large swelling in her right breast that had been growing for 16 months. Local examination showed a solitary, nontender, firm lump about 9 cm in diameter in the right upper quadrant. It was freely mobile. The left breast was normal. Mammography was not done. Open biopsy revealed that the BRIEF REPORT: GIANT BREAST TUMORS mass was a giant fibroadenoma. At operation, the tumor (10x8x8 cm) was enucleated through a lateral inframammary incision. There was no evidence of recurrence for 12 years. Case 5 A 28-year-old Saudi female, married with four children, presented with swelling of the right breast of 18 months’ duration. The swelling was painless and had increased in size rapidly. On examination, a firm non-tender, mobile mass measuring about 15 cm in diameter was noted in the inferior half of the breast. The superficial veins were distended. Mammography and ultrasound showed a homogenous well-defined mass, which was confirmed by an open biopsy to be a benign cystosarcoma phylloides. Using a submammary incision, the tumor (15x14x15 cm) was excised with a margin of normal breast tissue. Magnification (3x) was used, and redundant skin was excised. The patient was followed up for 2½ years without evidence of recurrence. Case 6 A 32-year-old Palestinian female, married with three children, was referred to the Plastic Surgery Clinic with an enormous swelling of the left breast which had grown rapidly for 12 months, and an ulcer with a serosanguinous discharge for two months. A solitary mass about 18 cm in diameter, non-tender and firm was found occupying the lateral half of the breast. An ulcer 1.5 cm in diameter and 0.6 cm deep was found over the center of the mass. The mass was fixed to the skin at the site of the ulcer, but it was freely mobile over the underlying muscles. Dilated veins were present over the surface. Axillary lymph nodes were not palpable. Mammogram confirmed a homogenous mass infiltrating the skin at the site of the ulcer. Aspiration biopsy of the mass revealed benign cystosarcoma phylloides. Using the submammary approach and magnification (3x), the tumor was excised with a margin of normal breast tissue. A large ellipse of skin centered over the ulcer was excised together with the tumor. The breast was reconstructed after excision of the excess skin. There was no evidence of recurrence after one year. The patient failed to attend follow-up after this period. Discussion Giant breast tumors are rapidly growing breast masses with diameters exceeding 5 cm and/or weights of more than 500 g.1,5 They can grow to immense proportions, resulting in congestion and ulceration of skin by centrifugal pressure.1 Such an enlargement of the breast can be due to giant fibroadenoma, cystosarcoma phylloides or virginal hypertrophy, occurring in that order of frequency. 5-7 These tumors are believed to be closely related variants of a similar pathologic process.7 They are characterized by proliferation of epithelial and connective tissue elements in varying proportions. The exact etiology is not known. Hormonal influences are thought to be contributory factors. Excessive estrogen stimulation and/or receptor sensitivity, or lack of estrogen antagonists have been implicated in the pathogenesis.7-10 Nevertheless, the fact that these tumors are unilateral and have an apparent geographical distribution suggests that other factors, possibly genetic and environmental, could be involved. Benign virginal hypertrophy is usually bilateral, but on rare occasions may occur unilaterally. Typically, there is a diffuse enlargement of breast without any associated dilated cutaneous veins. Histological differentiation between virginal hypertrophy and giant fibroadenoma is often difficult.5 We experienced similar difficulty in Case 1. Reduction mammoplasty is the treatment of choice, although in rare cases the disease may recur from the residual breast tissue. Giant fibroadenoma occurs predominantly in adolescent Blacks and in the Oriental race.1-3 The disease is often confined to one breast as a solitary mass occupying part or the whole breast. In rare cases, it may be multifocal and involve both breasts. The tumor is well-encapsulated and has histologic features of a fibroadenoma, with a variable growth pattern of epithelial and connective tissue elements. The condition is normally benign, and the potential to grow decreases with age. Simple enucleation of the tumor is all that is required to control the disease. Tailoring of skin envelope may be indicated in a massive breast enlargement. Cystosarcoma phylloides occur in the older age group, with a mean age of 40 years.5,11 They are rare in adolescents and appear to have no racial predisposition.11,12 Generally, they present as a solitary mass confined to one breast. It is rare to find bilateral breast involvement.3,12 In older patients, 5%-10% of these tumors may be malignant,5,12 but those that occur in adolescence are rarely malignant.1,2,13 Malignant degeneration has not been reported to occur in a benign cystosarcoma.3 The diagnosis of benign or malignant cystosarcoma is based on a combination of histologic features, such as stromal cellularity, pleomorphism, mitotic rate per 10 high-power field, stromal overgrowth and infiltrating, as opposed to pushing, borders.3 A high mitotic rate (>4 per 10 high-power field), stromal overgrowth and infiltrating margins are indicators of malignancy.3,13 Malignant cystosarcoma phylloides, however, are low-grade locally invasive tumors which rarely metastasize to regional lymph nodes. Since these tumors do not have a true capsule,2,12 they must be excised with a margin of normal tissue. Excision with a wider margin of normal tissue is indicated in malignant cystosarcoma phylloides.3 In adolescent females, however, cystosarcoma phylloides may be locally excised without sacrificing the normal breast tissue. Mastectomy may be indicated as a last resort in the unusual instance of repeated recurrences or a biologically virulent tumor.3 The nature of surgical therapy demands considerable familiarity with the pathological process of a disease. Huge masses growing rapidly inside the breast cause pressure atrophy of the surrounding normal breast tissue. This Annals of Saudi Medicine, Vol 19, No 2, 1999 175 HAWARY ET AL phenomenon was evident in five of our patients who had tumors of 15 cm diameter or greater. But in all of them, the affected breast regained volume spontaneously and matched the opposite normal breast in six to nine months. Giant breast tumors, including the malignant cystosarcoma phylloides, usually behave in a non-virulent manner. Surgical removal of these large tumors is accomplished in most instances through a submammary incision. Complex reconstructive procedures are unnecessary and also inappropriate. It is worth emphasizing that sacrifice of breast in such young patients should be avoided unless the tumor is unusually aggressive. All patients with giant breast tumors, and those with cystosarcoma in particular, should be followed up for recurrence. This could occur in the first two years after the primary excision.3 In most instances, this results from incomplete excision of tumor, as was the case with one of our patients. Complete excision should be confirmed histologically by looking for tumor-free margins. Because of the rarity of this disease, our experience of six cases over a period of 15 years is too limited to draw any definite conclusions. However, by treating these cases along the guidelines suggested, we were able to control the disease without mutilation of the breast. References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 176 Raganoonan C, Fairbain JK, Williams S, Hughes LE. Giant breast tumours of adolescence. Aust NZ J Surg 1987;57:243-7. Carl D, Patel V. Surgical problems in the management of the breast. Am J Obstet Gynecol 1985;152:1010-5. Hart J, Layfield LJ, Trembull WE, et al. Practical aspects in the diagnosis and management of cystosarcoma phylloides. Arch Surg 1988;123:1079-83. McDonald JR, Harrington SW. Giant fibroadenoma of the breast (cystosarcoma phylloides). Ann Surg 1950;131-234. Musio F, Mozingo D, Otchy DP. Multiple giant fibroadenoma. Am Surg 1991;57:438-41. Amiel C, Tramier D, Marck MF, et al. Le fibro-adenome mamaire geant. J Gynaecol Obstet Biol Reprod 1993;22:764-5. Kier LC, Hickey RC, Keetal WC, et al. Endocrine relationships in benign lesions of the breast. Ann Surg 1952;135:782-7. Nambier R, Kutty MK. Giant fibroadenoma in adolescent females: a clinicopathologic study. Br J Surg 1974;61:113-7. Wulsin J. Large breast tumours in adolescent female. Ann Surg 1960;152:151-9. Oberman HA. Breast tumours in the adolescent female. Pathol Ann 1979;14:175-201. Pike A, Oberman H. Juvenile adenofibromas: a clinicopathologic study. Am J Surg Pathol 1985;9:730-6. Briggs RM, Walters M, Rosenthal D. Cystosarcoma phylloides in adolescent female patients. Am J Surg 1983;146:172-14. Hart WR, Bauer RC, Oberman HA. A clinico-pathologic study of 25 hypercellular periductal stromal tumours of the breast. Am J Clin Pathol 1978;70:211-6. Annals of Saudi Medicine, Vol 19, No 2, 1999