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Disseminated malignant phyllodes: Presentation after a decade

1 Department of Pathology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
2 Department of Medical Oncology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India

Date of Submission30-Apr-2021
Date of Acceptance06-Jun-2021
Date of Web Publication06-Jan-2022

Correspondence Address:
Leena Dennis Joseph,
Department of Pathology, Sri Ramachandra Institute of Higher Education and Research, Chennai - 600 116, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcrt.jcrt_715_21

 > Abstract 

Phyllodes tumors are rare biphasic fibroepithelial lesions of the breast and account for 0.3%–0.5% of primary breast tumors. Malignant phyllodes tumor has a 10%–26% risk of distant metastasis. The most common site of metastasis is lungs followed by bone and soft tissue. This is a rare case of a 42-year-old female with a previous history of malignant phyllodes tumor breast. She presented after 10 years with metastases to multiple sites including lung, abdominal wall, retroperitoneum, bone, and brain. These tumors have a poor overall survival. Accurate diagnosis and aggressive management of malignant phyllodes tumors can help in effective treatment at diagnosis and for close follow-up of the patients.

Keywords: Distant metastases, follow-up, malignant phyllodes

How to cite this URL:
Sherin S B, Joseph LD, Pavithra V, Manickavasagam M. Disseminated malignant phyllodes: Presentation after a decade. J Can Res Ther [Epub ahead of print] [cited 2022 Dec 4]. Available from: https://www.cancerjournal.net/preprintarticle.asp?id=335105

 > Introduction Top

Phyllodes tumors are rare fibroepithelial neoplasms of the breast that constitute for only 0.3%–0.5% of primary breast tumors.[1] The incidence is higher in women of age group of 42–45 years. They are subclassified histologically as benign, borderline, and malignant, based on features such as tumor margins, stromal cellularity, and mitotic index. The majority of phyllodes tumors are benign (60%–75%).[2] Malignant phyllodes tumors have aggressive behavior and most of the distant metastases develop from malignant tumors (10%–26%).[3] The most common sites of metastases are the lungs, soft tissue, bone, and pleura.[4] Hematogenous spread is most frequently reported to the lungs (70%–80%).[5] Distant metastases in phyllodes can have varying presentations. It can present as a synchronous lesion or it can present within 10 years of diagnosis. Rarely, the metastases can present even after a decade.[3] We report herein a case of metastatic malignant phyllodes tumor presenting as disseminated disease 10 years after the initial diagnosis.

 > Case Report Top

A 42-year-old female presented with chief complaints of cough with expectoration for 4 months. She had a previous history of modified radical mastectomy for phyllodes tumor of the right breast done 10 years back. On evaluation, computed tomography (CT thorax) showed a 10.2 cm × 9.1 cm × 4.4 cm mass in the right lung lower lobe with severe compression of bronchus intermedius and complete obstruction of right lung lower lobe. A right middle and lower lobe lobectomy was performed and was sent for histopathological examination. Gross examination of the lobectomy specimen revealed a well-defined solid gray–white lesion in the lung and was measuring 9 cm × 9 cm × 7 cm [Figure 1]. Microscopic examination of the lobectomy specimen showed infiltration of the lung parenchyma by a malignant spindle cell tumor with increased cellularity [Figure 2]. The tumor cells were predominantly arranged in fascicles [Figure 3]. The cells had spindle cell morphology with elongated, hyperchromatic, blunt-ended nuclei showing moderate nuclear atypia, and high mitotic rate (10 mitoses in 10 high-power fields) [Figure 4] and [Figure 5]. By immunohistochemistry, the tumor cells were positive for Vimentin and smooth muscle actin. Ki67 labeling index was 20%. With a known history of malignant phyllodes tumor in the breast, the lobectomy specimen was reported as metastasis from phyllodes with a heterologous component of leiomyosarcoma.
Figure 1: Gross image of the lobectomy specimen showing a well-defined solid, grey white, firm lesion in the lung with focal grey black areas

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Figure 2: Low power view showing a cellular lesion infiltrating the lung parenchyma (marked in blue arrow). Adjacent cartilage (marked in star) is noted in the background (H and E, ×100)

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Figure 3: Malignant spindle cell proliferation showing a hypercellular lesion with tumor cells arranged in fascicles (H and E, ×200)

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Figure 4: Spindle cells with moderate atypia and the cells have eosinophilic cytoplasm, hyperchromatic, and blunt ended nuclei (H and E, ×400)

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Figure 5: Malignant spindle cells with mitotic figures (marked in arrows) (H and E, ×400)

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The follow-up positron emission tomography CT done after 1 year revealed a disseminated disease with metastatic foci in the brain, omentum, retroperitoneum, anterior abdominal wall, and femur [Figure 6] and [Figure 7]. The anterior abdominal wall lesion was biopsied, and the histology was reported as metastatic phyllodes with a heterologous component of leiomyosarcoma [Figure 8]. The patient was started on palliative radiotherapy (RT). She received 30 Gy (10 fractions) of whole-brain radiation therapy. One cycle of concomitant chemotherapy was given with cyclophosphamide, vincristine, Adriamycin, and dacarbazine regimen. Magnetic resonance imaging was done after RT, and there was a significant interval decrease in the size of the brain lesion. However, our patient succumbed to the disease 2 months later. Clinical course of the patient is highlighted in the table [Table 1].
Figure 6: Positron emission tomography computed tomography showing few hyperenhancing FluoroDeoxyGlucose avid lesions (marked in white arrow)

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Figure 7: Positron emission tomography computed tomography showing large heterogeneously enhancing FDG-avid lesion (15.3 cm × 15.1 cm × 10.8 cm) in the retroperitoneum (marked in white arrow)

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Figure 8: Biopsy from the abdominal wall mass showing a spindle cell lesion with cells exhibiting moderate atypia

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Table 1: Clinical history of the patient

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 > Discussion Top

Phyllodes tumors are large, fast-growing masses that arise from the periductal stromal cells of the breast. These tumors can increase in size in just a few weeks and patients usually present with a firm, palpable mass. The tumor is relatively large with an average size of 4 cm.[6] The average age of presentation is 40%–50% years.[7] Benign phyllodes tumors present at a younger age when compared to borderline and malignant phyllodes tumors.[8]

Phyllodes tumors are biphasic fibroepithelial neoplasms characterized by the presence of an epithelial component along with stromal component. The tumor is graded based on the histologic changes of stromal proliferation. Therefore, histologically, it is classified into benign, borderline, and malignant grades. Around 85%–90% of phyllodes tumors are benign and 10%–15% are malignant.[9]

Grading of phyllodes tumors can help in the prognostication and thereby predict the clinical behavior. Benign tumors have a very low rate of local recurrence regardless of the margin status. Borderline tumors have the potential of local recurrence, and a very low risk of metastasis and malignant tumors have the highest risk of metastasis. The incidence of metastasis is 10%–26% in cases of malignant phyllodes.[3]

The most accepted theory on the pathogenesis of phyllodes tumors is epithelial–stromal interactions. The complex morphology of these tumors can be explained by the interdependence of growth between the epithelial and stromal components. Studies have also shown that the stromal dependence on epithelium is lost with increasing malignancy of the stromal elements.

The risk of metastases in malignant phyllodes is predetermined by tumor biology and can be correlated with the presence of malignant heterologous elements, necrosis, and size of the tumors.[6],[10] The involvement of axillary lymph nodes by metastases is very rare. The most common route of metastases is hematogeneous spread, and the most common site of involvement is lungs. Other sites of metastases include pleura, bone, central nervous system, visceral organs, and soft tissue.[11] Distant metastases usually occur within 10 years of diagnosis.[12]

Gregston et al. in their study have reported giant malignant phyllodes tumor in a 37-year-old female which has rapidly progressed and culminated in brain metastasis.[13] There are 10 reported cases of central nervous system metastases so far.[13] Another study by Friedman et al. reported a rare case of malignant phyllodes tumor metastasizing as giant abdominal mass.[14]

Histologically, malignant phyllodes tumor shows marked stromal cellularity and atypia, has permeative margins, and has mitotic activity of at least 10/10 HPFs. Stromal overgrowth is defined as stromal proliferation without any epithelial elements in at least 1 low-power field (×4). Metastases from malignant phyllodes show malignant stromal elements without accompanying epithelium. The presence of a malignant heterologous element such as liposarcoma, chondrosarcoma, or osteosarcoma puts the tumor into the malignant category regardless of the other histological parameters.

The proper diagnosis and accurate histological grading are very essential in phyllodes tumors because of their tendency to recur and the malignant potential. Most patients with metastases have a poor survival, and the median survival ranges from 4 to 17 months.[11] Early recognition of malignant phyllodes tumors can help in effective treatment at diagnosis and for close follow-up of the patients as these tumors have a substantial risk of metastasis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

 > References Top

Abe H, Teramoto A, Takei Y, Tanaka Y, Yoneda G. Malignant phyllodes tumor of the breast with rapid progression: A case report. Surg Case Rep 2020;6:308.  Back to cited text no. 1
Koh VC, Thike AA, Tan PH. Distant metastases in phyllodes tumours of the breast: An overview. Appl Cancer Res 2017;37:6-13.  Back to cited text no. 2
Asoglu O, Karanlik H, Barbaros U, Yanar H, Kapran Y, Kecer M, et al. Malignant phyllode tumor metastatic to the duodenum. World J Gastroenterol 2006;12:1649-51.  Back to cited text no. 3
Hawkins RE, Schofield JB, Fisher C, Wiltshaw E, McKinna JA. The clinical and histologic criteria that predict metastases from cystosarcoma phyllodes. Cancer 1992;69:141-7.  Back to cited text no. 4
Khanal S, Singh YP, Bhandari A, Sharma R. Malignant phyllodes tumor with metastases to lung, adrenal and brain: A rare case report. Ann Med Surg (Lond) 2018;36:113-7.  Back to cited text no. 5
Guillot E, Couturaud B, Reyal F, Curnier A, Ravinet J, Laé M, et al. Management of phyllodes breast tumors. Breast J 2011;17:129-37.  Back to cited text no. 6
Zhou ZR, Wang CC, Sun XJ, Yang ZZ, Chen XX, Shao ZM, et al. Prognostic factors in breast phyllodes tumors: A nomogram based on a retrospective cohort study of 404 patients. Cancer Med 2018;7:1030-42.  Back to cited text no. 7
Tan PH, Thike AA, Tan WJ, Thu MM, Busmanis I, Li H, et al. Predicting clinical behaviour of breast phyllodes tumours: A nomogram based on histological criteria and surgical margins. J Clin Pathol 2012;65:69-76.  Back to cited text no. 8
Jing P, Wei B, Yang X. Phyllodes tumor of the breast with nipple discharge: A case report. Medicine (Baltimore) 2018;97:e13767.  Back to cited text no. 9
Koh VC, Thike AA, Nasir ND, Yip GW, Bay BH, Tan PH. Size and heterologous elements predict metastases in malignant phyllodes tumours of the breast. Virchows Arch 2018;472:615-21.  Back to cited text no. 10
Ramakant P, Selvamani, Therese MM, Paul MJ. Metastatic malignant phyllodes tumor of the breast: An aggressive disease-analysis of 7 cases. Indian J Surg Oncol 2015;6:363-9.  Back to cited text no. 11
Grabowski J, Salzstein SL, Sadler GR, Blair SL. Malignant phyllodes tumors: A review of 752 cases. Am Surg 2007;73:967-9.  Back to cited text no. 12
Gregston AP, Metter DM, Osborne CR, Pippen J Jr., Giant malignant phyllodes tumor with metastasis to the brain. Proc (Bayl Univ Med Cent) 2019;32:116-8.  Back to cited text no. 13
Friedman G, Celestin A, Lum S, Reeves ME. Giant abdominal mass: A unique form of metastatic malignant phyllodes tumor of the breast. Am Surg 2014;80:E144-6.  Back to cited text no. 14


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]

  [Table 1]


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