Difficult Management of a Rapidly Growing Benign Phyllodes Tumor in a 49-Year-Old Woman

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1 ase Report Difficult Management of a Rapidly Growing enign Phyllodes Tumor in a 49-Year-Old Woman Stamatia V. Destounis, MD, ortney Vogt, S, ndrea L. rieno, S, Renee. Morgan, RT(R)(M) P hyllodes breast tumors are rare, representing 0.3% to 1.0% of all breast tumors. 1,2 These tumors present a difficult management issue because of the chance of recurrence, which has been reported to occur in 15% of patients overall; therefore, careful clinical observation of these patients is recommended after surgical excision. Hajdu et al 3 reported a high incidence of local relapse of phyllodes breast tumors, with an incidence of 18%. Phyllodes tumors are difficult to diagnose because these tumors can be extremely difficult to differentiate from fibroadenomas. These tumors may masquerade as fibroadenomas and may be difficult to assess, even at core biopsy. The patients affected by these tumors vary in age at presentation, most typically 30 to 70 years old. 4,5 n article by Yohe and Yeh 6 discussed features suggesting phyllodes tumors that the pathologist can look for when analyzing a specimen and stated that increased sampling of a large tumor will likely yield a more accurate diagnosis. We report a case of a rapidly growing phyllodes tumor in a 49-year-old patient. In this case, the tumor grew very quickly in a short time and initially required wide surgical excision; fast recurrence led to a mastectomy for complete removal and clear margins. Even in the spectrum of what may be considered benign disease, a phyllodes tumor can locally recur and can be troublesome to treat. 2,3 This can lead to multiple diagnostic and invasive procedures and, as in this patient, mastectomy. ase Report bbreviations MRI, magnetic resonance imaging Received February 18, 2010, from Elizabeth Wende reast are, LL, Rochester, New York US. Manuscript accepted for publication March 10, We thank Fadi Hatem, MD (Department of Pathology and Laboratory Medicine, Rochester General Hospital, Rochester, NY), for interpretation of the core biopsy slides. ddress correspondence to Stamatia V. Destounis, MD, Elizabeth Wende reast are, LL, 170 Sawgrass Dr, Rochester, NY US. sdestounis@ewbc.com 49-year-old woman presented for evaluation of a palpable lump in the right superior breast 5 months after a screening mammogram showed normal findings. t the time of presentation, the patient explained that the lump was at least 2 to 3 in and involved most of the upper breast. The patient reported normal menses. ilateral digital mammography was performed, and an area of asymmetry was noted in comparison to her previous study, involving the right superior aspect 11- to 12- o clock region, measuring approximately 5 6 cm in diameter (Figure 1) by the merican Institute of Ultrasound in Medicine J Ultrasound Med 2010; 29: /10/$3.50

2 Management of Phyllodes Tumor Physical examination and visual inspection showed the right breast to be moderately larger than the left breast. 6 6-cm area of palpable thickening was present in the superior aspect of the right breast. The right breast was firm to palpation. There was no axillary or supraclavicular lymphadenopathy or skin changes. On sonography, an irregular hypoechoic mass was seen involving the right superior breast (Figure 2). 12-gauge vacuum-assisted sonographically guided needle core biopsy was performed, and the pathologic diagnosis was cellular fibroepithelial neoplasm; favor fibroadenoma, but cannot completely exclude a benign phyllodes Figure 1., Diagnostic right mediolateral oblique view showing dense tissue with an area of increased density of the right breast in the 11- to 12-o clock region., Diagnostic right craniocaudal mammographic view showing dense tissue with an area of increased density in the right superior aspect (continued) J Ultrasound Med 2010; 29:

3 Destounis et al tumor (Figure 3). The patient proceeded to undergo surgical excision of the area, at which time pathologic diagnosis was benign phyllodes tumor with positive margins. The patient returned 3 months after surgical excision for a follow-up examination. She stated that a lump in her right breast developed that was hard and sizable, and she believed that the phyllodes tumor had returned. Digital mammography was performed and revealed extensive nodularity involving the entire right central aspect. The area of nodularity extended over cm (Figure 4). Physical examination and visual inspection revealed a well-healed scar in the right superior aspect. Firmness was palpated within the lateral aspect of the right breast, from the 9- to 12- o clock region, extending to the right breast upper inner quadrant 2- to 3-o clock region. This area measured 9 cm in diameter. Sonography Figure 1. (continued), Right tangential magnification mammographic view of the palpable region. revealed a hypoechoic mass involving the entire central and superior aspect of the right breast. The size of the mass was difficult to assess but measured approximately 11 cm (Figure 5). vacuum-assisted sonographically guided needle core biopsy was performed, and pathologic evaluation revealed a cellular fibroepithelial neoplasm; favor fibroadenoma, but cannot completely exclude a benign phyllodes tumor (Figure 6). efore any further surgery, it was recommended that the patient undergo a breast magnetic resonance imaging (MRI) examination to assess the extent of disease. The right breast revealed a mass involving the entire superior and central breast extending cm (Figure 7). The mass did abut the pectoralis fascia very closely, and involvement of the pectoralis could not be completely excluded. Figure 2., Sonogram of the right breast showing an irregular hypoechoic mass involving the right superior aspect., Sonogram of the right breast showing a hypoechoic mass involving the right superior aspect. J Ultrasound Med 2010; 29:

4 Management of Phyllodes Tumor Figure 3., Low-power view from the first core biopsy showing biphasic epithelial proliferation. The epithelial component encompasses all of the elongated and irregular benign ductal structures., Medium-power view from the first core biopsy showing biphasic stromal proliferation. The stromal component is the cellular tissue containing and surrounding the ducts. The stroma has pink fibrous tissue and small elongated cells, which are fibroblasts., High-power view from the first core biopsy focusing on the stromal cells (fibroblasts) to show there is no atypia or mitosis, indicating a benign lesion. Figure 4., Diagnostic mediolateral oblique bilateral mammographic views showing dense glandular tissue with extensive density involving the entire right central aspect., Diagnostic craniocaudal bilateral mammographic views showing widespread density involving the entire right central aspect (continued) J Ultrasound Med 2010; 29:

5 Destounis et al The findings of the MRI examination were discussed with the patient. It was explained that phyllodes tumors do recur, which makes these tumors a difficult management issue. ecause the patient s mass recurred so quickly and was large, it was recommended that a mastectomy would be the best option to excise the mass and obtain clear margins. Discussion Phyllodes tumors are rare fibroepithelial breast tumors, which can be classified as benign, borderline, or malignant. This is based on microscopic features such as stromal cellularity and distribution, pleomorphism, mitotic activity, and cell margin appearance. 1,4,6 9 Malignant phyllodes tumors are considered breast cancer but account for less than 1%. Even if benign, they have the potential to become malignant. They only develop in the breast, as they have never been found in other parts of the body. pproximately 20% to 30% of these tumors rapidly increase in size in as little as a few weeks. ecause of their fast-growing nature, the skin can become semitransparent, reddish, and warm to the touch around the affected area. However, nipple and areola involvement are not usually found. phyllodes tumor is quite large and can average 2 in, but larger tumors have been found. On manual examination, the tumor moves freely within the breast and has a smooth, firm texture; however, the tumor is usually not painful. In phyllodes tumors, the stroma is more cellular, and the cells vary in shape and size. They are composed of polyclonal epithelial cells and monoclonal stromal cells and have leaflike projections into cystic spaces. 7 Figure 5., Sonogram of the palpable abnormality showing an irregular mass measuring approximately 11 cm extending throughout the entire superior breast., Sonogram of the palpable abnormality showing an irregular hypoechoic mass extending throughout the entire superior breast. Figure 4. (continued), Right tangential magnification mammographic view showing the new enlarging palpable lump. J Ultrasound Med 2010; 29:

6 Management of Phyllodes Tumor Figure 6., Low-power view from the second core biopsy showing biphasic epithelial proliferation., Medium-power view from the second core biopsy showing biphasic stromal proliferation., High-power view from the second core biopsy focusing on the stromal cells (fibroblasts) to show there is no atypia or mitosis, indicating a benign lesion. Figure 7., xial fat-suppressed gadolinium-enhanced MRI of the right breast showing an enhancing mass involving the entire superior to inferior medial aspect, extending cm., Sagittal T1-weighted fat-suppressed gadolinium-enhanced MRI of the right breast showing an enhancing mass involving the entire superior to inferior medial aspect J Ultrasound Med 2010; 29:

7 Destounis et al Phyllodes tumors are often thought to be fibroadenomas. On mammography, they appear as large well-circumscribed oval or lobulated masses. On sonography, they tend to present as solid well-defined masses with heterogeneous echoes. 5 Sometimes a cyst is seen within the solid lesion, which usually is highly suggestive of a phyllodes tumor. On MRI, most phyllodes tumors present as oval, round, or lobulated masses with circumscribed margins and homogeneous high signal intensity. 8 needle biopsy cannot usually differentiate between fibroadenomas and phyllodes tumors. Typically an open surgical biopsy is necessary so the pathologist can accurately diagnose the mass. 9 The usual treatment for a phyllodes tumor is surgical excision because these types of tumors do not respond well to radiation, chemotherapy, or hormone therapies. If benign, the surgeon will try to spare as much of the unaffected breast tissue as possible by doing a wide margin excision (taking >1 cm of tissue around the primary tumor). However, if the tumor is too large (>5 cm) or malignant, a mastectomy may be the only treatment option. The prognosis for a benign phyllodes tumor is very good, and the chance of recurrence is very low. The prognosis for patients with borderline or malignant tumors varies. orderline tumors have the potential to become cancerous, and borderline and malignant phyllodes tumors have the potential to metastasize. The most common sites for this are the lungs, bones, liver, and chest wall. This can recur years after treatment. Most people in whom metastases develop die within 3 years of their first treatment. The prognosis based on the histologic class alone can be problematic. There have been rare occurrences of benign phyllodes tumors metastasizing and malignant phyllodes tumors not recurring or metastasizing. 9 Women between the ages of 30 and 70 years, many of whom may be premenopausal, are affected by these tumors. 4,5 There are instances of local recurrence occurring in less than 20% of benign phyllodes tumors and more than 25% of malignant phyllodes tumors. Distant recurrence occurs in less than 5% of borderline phyllodes tumors and up to 25% of malignant phyllodes tumors. 7 reast-conserving surgery with clear margins is the current treatment choice, but this method of treatment does not further diminish local recurrence effectively. 10 Phyllodes tumors are considered rare tumors with unpredictable behavior. 11 Therefore, treatment should be decided on a case-by-case basis dependent on whether clear surgical margins are obtained. References 1. Esposito NN, Mohan D, rufsky, Lin Y, Kapali M, Dabbs DJ. Phyllodes tumor: a clinicopathologic and immunohistochemical study of 30 cases. rch Pathol Lab Med 2006; 130: Noguchi S, Motomura K, Inaji H, Imaoka S, Koyama H. lonal analysis of fibroadenoma and phyllodes tumor of the breast. ancer Res 1993; 53: Hajdu SI, Espinosa MH, Robbins GF. Recurrent cystosarcoma phyllodes: a clinicopathologic study of 32 cases. ancer 1976; 38: Lifshitz OH, Whitman GJ, Sahin, Yang WT. Radiologicpathologic conferences of the University of Texas M. D. nderson ancer enter. Phyllodes tumor of the breast. JR m J Roentgenol 2003; 180: Farria DM, Gorczyca DP, arsky SH, Sinha S, assett LW. enign phyllodes tumor of the breast: MR imaging features. JR m J Roentgenol 1996; 167: Yohe S, Yeh IT. Missed diagnoses of phyllodes tumor on breast biopsy: pathologic clues to its recognition. Int J Surg Pathol 2008; 16: Giri D. Recurrent challenges in the evaluation of fibroepithelial lesions. rch Pathol Lab Med 2009; 133: Jacklin RK, Ridgeway PF, Ziprin P, Healy V, Hadjiminas D, Darzi. Optimising preoperative diagnosis in phyllodes tumour of the breast. J lin Pathol 2006; 59: Frey R. ystosarcoma Phyllodes. In: The Gale Encyclopedia of ancer: Guide to ancer and Its Treatments. 2nd ed. Detroit, MI: Thomson Gale; 2005: heng SP, hang Y, Liu TP, Lee JJ, Tzen Y, Liu L. Phyllodes tumor of the breast: the challenge persists. World J Surg 2006; 30: arrio, lark D, Goldberg JI, et al. linicopathologic features and long-term outcomes of 293 phyllodes tumors of the breast. nn Surg Oncol 2007; 14: J Ultrasound Med 2010; 29: