Figure 1
A 73-year-old woman presents with a palpable mass in the left iliac fossa. She has a past medical history of malignant phyllodes tumour in the left breast in 2003 (treated by mastectomy) and recurrence in the left axilla in 2012 and 2015 (treated by excision and axillary node clearance respectively).
Metastasis of malignant phyllodes tumour in the abdominal wall.
Metastasis of melanoma
Metastasis of lymphoma
A targeted ultrasound of the site of the patient's concern demonstrated a well-circumscribed and oval-shaped mass with lobulated borders, measuring 36 x 25 mm, in the subcutaneous soft tissues of the abdominal wall in the left iliac fossa. The mass is heterogeneous, but predominately hypoechoic (Fig. 1). The mass is not invading the overlying skin or underlying abdominal wall muscle (Fig. 1, 2 and 3). Core biopsy of the mass was performed. Histology examination revealed a metastatic malignant phyllodes tumour. The patient underwent staging CT of the chest, abdomen and pelvis, and it did not identify further metastatic disease. The patient was treated by surgical excision of the mass. A consequent histology examination confirmed the metastasis of the malignant phyllodes tumour.
Background
Phyllodes tumour (PT) of the breast is a rare fibroepithelial neoplasm, with an incidence of less than 1% of all breast neoplasms [1]. The spectrum of PT ranges from benign to borderline and malignant. Malignant PT comprises 20-25% of all PTs [3, 4], making it a rare histopathological entity. The rate of distant metastasis of malignant PT was reported as 9-27% [17].
Clinical Perspective
There are no specific clinical or radiological characteristics that enable reliable differentiation of benign and malignant PT [1, 5, 8]. Malignant tumours are often larger and faster growing, but size and growth rate are poor predictors of the histopathological type [1]. The triple assessment of the patients by clinical, radiological, and cytological or histological examinations forms the essential diagnostic pathway for all breast lesions.
Imaging Perspective
Mammography: PT appears as a dense mass, with circumscribed or lobulated margins. Partially indistinct borders favour malignant PT [2]. Malignant PT with osteosarcomatous differentiation is associated with intratumoral macrocalcifications. However, calcifications are generally rare in PTs because of their rapid growth [7].
Ultrasound breast: PT appears as a hypoechoic, round or oval mass, with circumscribed or lobulated borders, resembling fibroadenoma. Lobulation, heterogeneous hypoechoic internal echoes and absence of calcifications favour PT [9]. Intramural cystic areas are seen more often in malignant than in benign PT [2, 8, 9].
MRI breast: PTs are hypointense or isointense compared with adjacent breast tissue on T1-weighted images, and can be hyperintense or hypointense on T2-weighted images. Heterogeneous inner structure and internal septations are frequently seen in PTs. There may be increased T2 signal in the tissue surrounding PT [10, 11]. 33% of PT have contrast enhancement characteristics suggestive of malignancy [11].
CT: The primary tumour and metastases may appear as a heterogeneous enhancing mass [10].
Outcome
The 5-year overall survival rate is 84% and 5-year cause-specific survival is 91% for malignant PT after initial surgical management [4]. Tumour size and surgical margins are the principal determinants of local recurrence and distant metastasis [13]. Patient prognosis is extremely poor after metastatic spread [17].
Metastases of malignant PT spread mostly by the haematogenous route [17] to the lungs (most frequent site) [12], mediastinum, skeleton [3, 7], cardiac chambers [14, 15], kidney [16], brain, GI tract and uterus [17].
Take Home Message
There are no pathognomonic clinical or radiological characteristics that enable reliable identification of the malignant PT. Metastasis of malignant PT is rare, but has been reported to various organs of the body.
Phyllodes tumour (PT) of the breast is a rare fibroepithelial neoplasm, with an incidence of less than 1% of all breast neoplasms [1]. The spectrum of PT ranges from benign to borderline and malignant. Malignant PT comprises 20-25% of all PTs [3, 4], making it a rare histopathological entity. The rate of distant metastasis of malignant PT was reported as 9-27% [17].
Clinical Perspective
There are no specific clinical or radiological characteristics that enable reliable differentiation of benign and malignant PT [1, 5, 8]. Malignant tumours are often larger and faster growing, but size and growth rate are poor predictors of the histopathological type [1]. The triple assessment of the patients by clinical, radiological, and cytological or histological examinations forms the essential diagnostic pathway for all breast lesions.
Imaging Perspective
Mammography: PT appears as a dense mass, with circumscribed or lobulated margins. Partially indistinct borders favour malignant PT [2]. Malignant PT with osteosarcomatous differentiation is associated with intratumoral macrocalcifications. However, calcifications are generally rare in PTs because of their rapid growth [7].
Ultrasound breast: PT appears as a hypoechoic, round or oval mass, with circumscribed or lobulated borders, resembling fibroadenoma. Lobulation, heterogeneous hypoechoic internal echoes and absence of calcifications favour PT [9]. Intramural cystic areas are seen more often in malignant than in benign PT [2, 8, 9].
MRI breast: PTs are hypointense or isointense compared with adjacent breast tissue on T1-weighted images, and can be hyperintense or hypointense on T2-weighted images. Heterogeneous inner structure and internal septations are frequently seen in PTs. There may be increased T2 signal in the tissue surrounding PT [10, 11]. 33% of PT have contrast enhancement characteristics suggestive of malignancy [11].
CT: The primary tumour and metastases may appear as a heterogeneous enhancing mass [10].
Outcome
The 5-year overall survival rate is 84% and 5-year cause-specific survival is 91% for malignant PT after initial surgical management [4]. Tumour size and surgical margins are the principal determinants of local recurrence and distant metastasis [13]. Patient prognosis is extremely poor after metastatic spread [17].
Metastases of malignant PT spread mostly by the haematogenous route [17] to the lungs (most frequent site) [12], mediastinum, skeleton [3, 7], cardiac chambers [14, 15], kidney [16], brain, GI tract and uterus [17].
Take Home Message
There are no pathognomonic clinical or radiological characteristics that enable reliable identification of the malignant PT. Metastasis of malignant PT is rare, but has been reported to various organs of the body.
References
[1] Parker SJ, Harries SA. (2001) Phyllodes tumours. Postgraduate Medical Journal 77:428-435 (PMID: 11423590)
[2] Berg WA, Birdwell RL et al. (2006) Diagnostic Imaging: Breast. Amirsys, Salt Lake City, Utah
[3] El Ochi MR, Toreis M, Benchekroun M et al. (2016) Bone metastasis from malignant phyllodes breast tumor: report of two cases. BMC Clinical Pathology 16:4. (PMID: 26933383)
[4] Macdonald OK, Lee CM, Tward JD et al. (2006) Malignant phyllodes tumor of the female breast. Cancer 107: 2127–2133. (PMID: 16998937)
[5] Santamaría G, Velasco M, Bargalló X et al. (2010) Radiologic and Pathologic Findings in Breast Tumors with High Signal Intensity on T2-weighted MR Images. Radiographics 30:2, 533-548. (PMID: 20228333)
[6] Vijaykumar A, Ajitha MB, Shivaswamy BS et al. (2012) A Systematic Study on Fibroadenoma of the Breast. Eur J Surg Sci 3(3):80-85.
[7] Phalak KA, Sedgwick EL, Dhamne S et al. (2013) AIRP Best Cases in Radiologic-Pathologic Correlation: Malignant Phyllodes Tumor with Osteosarcomatous Differentiation. RadioGraphics 33:5, 1377-1381. (PMID: 24025930)
[8] Liberman L, Bonaccio E, Hamele-Bena D et al. (1996) Benign and malignant phyllodes tumors: mammographic and sonographic findings. Radiology Jan, 198(1):121-4. (PMID: 8539362)
[9] Chao TC, Lo YF, Chen SC et al. (2002) Sonographic features of phyllodes tumors of the breast. Ultrasound in Obstetrics & Gynecology 20: 64–71, Volume 20, Issue 1. (PMID: 12100421)
[10] Singer A, Tresley J, Velazquez-Vega J et al. (2013) Unusual Aggressive Breast Cancer: Metastatic Malignant Phyllodes Tumor. J Radiol Case Rep Feb; 7(2): 24–37. (PMID: 23705037)
[11] Wurdinger S, Herzog AB, Fischer DR et al. (2005) Differentiation of Phyllodes Breast Tumors from Fibroadenomas on MRI. American Journal of Roentgenology 185: 1317-1321. (PMID: 16247156)
[12] Augustyn A, Sahoo S, Wooldridge RD. (2015) Large Malignant Phyllodes Tumor of the Breast with Metastases to the Lungs. Rare Tumors May 5; 7(2): 5684. (PMID: 26266007)
[13] Kapiris I, Nasiri N, Hern RA et al. (2001) Outcome and predictive factors of local recurrence and distant metastases following primary surgical treatment of high-grade malignant phyllodes tumours of the breast. European Journal of Surgical Oncology Volume 27, Issue 8, December. (PMID: 11735168)
[14] Yoshidaya F, Hayashi N, Takahashi K et al (2015) Malignant phyllodes tumor metastasized to the right ventricle: a case report. Surg Case Rep Dec; 1: 121. (PMID: 26943445)
[15] Bhambhani A, Ayyagari S, Mohapatra T et al. (2014) Malignant phyllodes tumor of the left atrium. Indian Heart Journal Volume 66, Issue 2, March–April, Pages 241-243. (PMID: 24814127)
[16] Karczmarek-Borowska B, Bukala A, Syrek-Kaplita K et al. (2015) A Rare Case of Breast Malignant Phyllodes Tumor With Metastases to the Kidney. Medicine (Baltimore) 94(33): e1312. (PMID: 26287414)
[17] Koh VCY, Thike AA, Tan PH (2017) Distant metastases in phyllodes tumours of the breast: an overview. Applied Cancer Research 37:15.
Case information
| URL: | https://www.eurorad.org/case/15057 |
| DOI: | 10.1594/EURORAD/CASE.15057 |
| ISSN: | 1563-4086 |
License
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Figure 1
Ulster Hospital, Northern Ireland
Figure 2
Ulster Hospital, Northern Ireland
Figure 3
Ulster Hospital, Northern Ireland