To further investigate, a literature research was performed using PubMed database to look for published cases of carcinoma arising within a phyllode tumour. Including this case study, 47 published cases were analysed (Table 1) (2, 4–23, 23–41). All of them were female patients aged between 19 and 80 years old (mean age = 47.6 years old and median age = 49 years old). The size of the PTs ranged from 1.4 cm to 21 cm (mean = 7 cm, median = 5.4 cm). The most common carcinoma subtypes reported include DCIS (n = 22), followed by mixed in-situ and invasive carcinoma (n = 10) and invasive ductal carcinoma (IDC) (n = 7). There were 17 malignant PTs, 8 borderline PTs and 22 benign PTs. Invasive carcinomas were found in 7 (41%) cases of malignant PTs, 5 (63%) cases of borderline PTs and 8 (36%) cases of benign PTs. Of note, report of usual ductal hyperplasia within PTs is also most common in borderline PTs (83%), followed by benign PTs (74%) and malignant PTs (51%) (1).
Table 1
published data of carcinoma arising within a phyllodes tumour.
Case | First authors | Year | Age | Phyllodes tumour | Carcinoma component | Presences of microcalcifications (characteristics) | Management | Outcome | Follow-up period (months) |
Type | Size (cm) | Type | Size (cm) | Resection | Axillary dissection | Lymph node status | Adjuvant therapy | Status | Recurrence | Metastasis |
1 | Leong et al (5) | 1980 | 51 | Benign | 6.0 | LCIS + ITC | Not available (N/A) | Yes (not available) | Mastectomy (MX) | Yes | Negative | Radiotherapy (RT) | Alive | No | No | 21 |
2 | Cole-Beuglet et al (6) | 1983 | 55 | Benign | 1.4 | DCIS + LCIS | N/A | N/A | N/A | No | - | N/A | N/A | N/A | N/A | N/A |
3 | Cole-Beuglet et al (6) | 1983 | 60 | Benign | 3.0 | IDC | N/A | N/A | N/A | No | - | N/A | N/A | N/A | N/A | N/A |
4 | Grove et al (7) | 1986 | 71 | Benign | 19.0 | DCIS | N/A | No | MX | Yes | Negative (0/11) | N/A | Alive | No | No | 4 |
5 | Ishida et al (8) | 1984 | 41 | Benign | 5.6 | IDC | Focal | No | MX | No | N/A | N/A | Alive | No | No | 30 |
6 | Kundsen et al (9) | 1987 | 71 | Benign | 7.0 | DCIS + LCIS | Multifocal | N/A | MX | Yes | Negative | No | N/A | N/A | N/A | N/A |
7 | Yasumura et al (10) | 1988 | 47 | Benign | 13.0 | IDC | N/A | N/A | MX | Yes | Negative (0/13) | Chemotherapy (CT) | Alive | No | No | 66 |
8 | Kodama et al (11) | 2003 | 47 | Benign | 17.0 | LCIS + ILC | N/A | No | MX | No | - | N/A | Alive | No | No | 108 |
9 | Parfitt et al (12) | 2004 | 26 | Benign | 3.3 | DCIS + IDC | Focal | N/A | Local excision (LoEx) | Yes | Positive (4/13) | CT + RT + Hormonal therapy (HT) | Alive | No | No | 36 |
10 | Ramdass et al (13) | 2006 | 59 | Benign | N/A | Squamous cell carcinoma (SCC) + IDC | N/A | N/A | N/A | N/A | N/A | N/A | N/A | N/A | N/A | N/A |
11 | Yamaguchi et al (14) | 2008 | 54 | Benign | 15.0 | DCIS | Focal | No | MX | No | - | HT | Alive | No | No | 12 |
12 | Nio et al (15) | 2011 | 53 | Benign | 3.5 | DCIS | 0.5 | N/A | LoEx | No | - | RT | Alive | No | No | 24 |
13 | Shirah et al (16) | 2011 | 49 | Benign | 4.8 | LCIS + ILC | 0.2 (ILC) | N/A | LoEx | No | - | No | N/A | N/A | N/A | N/A |
14 | Shin et al (17) | 2013 | 42 | Benign | 1.8 | DCIS | 1.2 | No | LoEx | No | - | RT | Alive | N/A | N/A | 8 |
15 | Ghosh et al (18) | 2014 | 42 | Benign | 2.2 | DCIS | N/A | No | LoEx | No | - | N/A | N/A | N/A | N/A | N/A |
16 | Colakoglu et al (19) | 2014 | 19 | Benign | 1.8 | DCIS | 0.8 | N/A | LoEx | No | - | RT + HT | Alive | N/A | N/A | 36 |
17 | Chopra et al (20) | 2016 | 23 | Benign | 5.0 | DCIS | N/A | N/A | LoEx | No | - | N/A | N/A | N/A | N/A | N/A |
18 | Lui et al (21) | 2017 | 19 | Benign | 5.1 | DCIS | N/A | Yes (specks described as benign looking) | LoEx | Sentinel lymph node biopsy (SLNB) | Negative (0/2) | HT | Alive | No | No | N/A |
19 | Panko et al (22) | 2017 | 70 | Benign | 2.3 | DCIS + IDC | 0.5 (IDC) | N/A | LoEx | SLNB | Negative | RT + HT | Alive | No | No | N/A |
20 | Co et al (2) | 2017 | 44 | Benign | 5 | DCIS | Focal | No | Mx | No | - | N/A | Alive | No | No | N/A |
21 | Co et al (2) | 2017 | 25 | Benign | 2.5 | DCIS | Multifocal | No | LoEx | No | - | RT | Alive | No | No | N/A |
22 | Sun et al (23) | 2019 | 30 | Benign | 1.5 | DCIS | 0.35 | No | LoEx | No | - | No | Alive | No | No | 1 |
23 | Deodhar et al (24) | 1997 | 51 | Borderline | 14.0 | DCIS | Focal | N/A | LoEx | N/A | N/A | N/A | N/A | N/A | N/A | N/A |
24 | Kuo et al (25) | 2010 | 24 | Borderline | 10.0 | DCIS + IDC | 2.5 | N/A | MX | SLNB | Isolated tumour cells (1/2) | CT + HT | Alive | No | No | 15 |
25 | Trabelsi et al (26) | 2010 | 52 | Borderline | 15 | DCIS + IDC | Foci | N/A | MX | No | - | No | Alive | N/A | N/A | 38 |
26 | Quinlan-Davidson et al (27) | 2011 | 53 | Borderline | 6.5 | LCIS + ITC | 2.4 | Yes (not available) | MX | SLNB | Negative (0/3) | No | N/A | N/A | N/A | N/A |
27 | Wu et al (28) | 2014 | 52 | Borderline | 3.0 | IDC | Focal | N/A | MX | Yes | Positive (1/21) | CT + RT | Alive | No | No | 23 |
28 | Co et al (2) | 2017 | 54 | Borderline | 9 | DCIS | Focal | No | MX | No | - | N/A | Alive | No | No | N/A |
29 | Fischer et al (29) | 2017 | 40 | Borderline | 4.2 | LCIS + ILC | 1.4 (ILC) | No | MX | Yes | Negative | HT | Alive | No | No | 6 |
30 | Present study | 2020 | 42 | Borderline | 6.5 | DCIS | x | Yes | MX | Yes | Negative | X | Alive | No | No | 96 |
31 | Seemayer et al (30) | 1975 | 27 | Stromal sarcoma | 6.0 | DCIS | Focal | N/A | MX | No | - | N/A | N/A | N/A | N/A | N/A |
32 | Klausner et al (31) | 1983 | 60 | Malignant | 4.0 | IDC | Focal | N/A | MX | Yes | Negative | N/A | N/A | N/A | N/A | N/A |
33 | Ward et al (32) | 1986 | 55 | Malignant | 4.0 | LCIS | N/A | N/A | MX | N/A | N/A | N/A | Alive | No | No | 87 |
34 | Padmanabhan et al (33) | 1997 | 47 | Malignant | 7.5 | LCIS | Focal | N/A | MX | Yes | Negative (0/4) | No | Alive | No | No | 6 |
35 | Nishimura et al (34) | 1997 | 80 | Malignant | 10.5 | DCIS | N/A | N/A | MX | No | - | No | Deceased | No | Lung metastasis | 3 |
36 | Lim et al (35) | 2005 | 45 | Malignant | 12.0 | DCIS | 0.6 | N/A | MX | No | - | N/A | Deceased* | No | No | 108 |
37 | Nomura et al (36) | 2006 | 75 | Malignant | 3.5 | DCIS | N/A | N/A | MX | Yes | Negative | No | Alive | No | No | 26 |
38 | Sugie et al (37) | 2007 | 54 | Malignant | 8.0 | SCC | N/A | N/A | MX | Yes | Negative | CT | Deceased | No | Lung + bone metastasis | 40 |
39 | Korula et al (38) | 2008 | 51 | Malignant | 21.0 | DCIS | N/A | No | MX | Yes | Positive (2/12) | CT + RT + HT | Alive | No | No | 11 |
40 | Macher-Goeppinger et al (39) | 2010 | 70 | Malignant | 6.0 | IDC | 2.5 | N/A | MX | Yes | Negative | No | N/A | N/A | N/A | N/A |
41 | Abdul Aziz et al (4) | 2010 | 43 | Malignant | 3.5 | DCIS + ITC | 0.2 (ITC) | Yes (not available) | LoEx | No | - | RT | Alive | No | No | 12 |
42 | Choi et al (40) | 2012 | 62 | Malignant | 10.0 | Invasive cribriform carcinoma | 6.0 | N/A | MX | Yes | Negative | CT | Alive | No | No | 24 |
43 | Co et al (2) | 2017 | 52 | Malignant | 10.0 | DCIS | Focal | No | MX | No | - | N/A | Alive | No | No | N/A |
44 | Co et al (2) | 2017 | 48 | Malignant | 5 | DCIS | Focal | No | MX | No | - | N/A | Alive | No | No | N/A |
45 | Co et al (2) | 2017 | 45 | Malignant | 4 | IDC | Focal | Yes (not available) | Mx | SLNB | Negative | N/A | Alive | No | No | N/A |
46 | Sun et al (23) | 2019 | 30 | Malignant | 3.2 | DCIS | 1.5 | Yes (punctated clusters, suspicious appearing) | LoEx | No | - | RT + HT | Alive | No | No | Almost 36 |
47 | Kaur et al (41) | 2019 | 26 | Malignant | 9 | Neuroendocrine carcinoma | 50% | N/A | LoEx | N/A | N/A | N/A | N/A | N/A | N/A | N/A |
The significance of microcalcifications were only discussed in 21 cases, where they were present in both in-situ and invasive carcinomas. Fourteen of them were pure DCIS lesions, in which 5 cases had microcalcifications (33.3%). The other 7 cases were involved by invasive carcinomas, 4 of which reported microcalcifications (57.1%) including 2 cases of lobular carcinoma in-situ (LCIS) + invasive tubular carcinoma (ITC), 1 case of DCIS + ITC and a case of IDC; the remaining 3 cases with invasive carcinoma involvement did not have microcalcifications (two cases of LCIS + invasive lobular carcinoma and a case of IDC). Both benign looking specks (21) and suspicious coarse punctuated clusters of microcalcifications (23), such as those seen in this case, had been described in carcinomas.
There were 20 cases with axillary surgery performed. Thirteen cases of which involved invasive carcinoma and 3 of them had lymph node metastasis (23%); 6 cases were diagnosed with in-situ carcinoma and 1 of them had lymph node metastasis (14%). All the metastases were of ductal type. This frequency of lymph node metastasis is comparable to that of ordinary invasive breast carcinoma not arising from a PT (42). None of the cases had sarcomatous lymph node involvement. The adjuvant modalities used to manage these patients were diverse. Two studies reported disease-related death as a result of distant metastasis of the sarcomatous component (34, 37). To date, no study has yet reported distant carcinomatous metastasis. Of the 32 cases with information on outcome and follow-up period available, there was no documented local recurrence (average follow-up period of 34.6 months).
Carcinoma, especially in-situ lesions, within a PT is often not suspected during the initial breast imaging (2). Thus, the carcinoma component is often only diagnosed incidentally on subsequent pathological examination of the core biopsy or excision specimen. The presence of microcalcifications, such as those seen in our case, is unusual for PTs and can alert the treating clinicians for the possibility of a carcinoma component within the PT. A targeted biopsy of the microcalcifications can therefore be performed.
The mainstay of treatment for these lesions is surgical excision followed by adjuvant therapies. The surgical approaches include local excision/lumpectomy (with margin of 1 cm advocated) (43–46) and mastectomy (47, 48). In contrast to true carcinosarcoma, it has been demonstrated that there is lack of clonality between the carcinoma and stromal neoplastic cells in carcinomas within PTs (39). Therefore, both the stromal and epithelial component of these lesions should be evaluated separately when formulating a management plan.