Phyllodes tumors of the breast in 2 sisters

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Abstract

Rationale:

Phyllodes tumors (PT) of the breast are rare neoplasm originating from fibroepithelial component. To our knowledge, our report is the first reported case of PT in 2 sisters.

Patient concerns:

We presented 2 cases of PT of the breast involving in 2 sisters. On physical examination of the younger sister, a firm mass measuring approximately 3 cm in diameter was identified in upper inner quadrant of the right breast. Physical examination of the elder sister revealed a 3 cm lump in upper outer quadrant of the left breast.

Diagnoses:

Histopathology of the younger sister revealed a malignant PT. The elder sister was diagnosed with borderline PT.

Interventions:

The younger sister with malignant PT underwent right mastectomy. The elder sister with borderline PT was scheduled for wide resection of the mass in the left breast.

Outcomes:

After a follow-up of 23 months, no local or distant recurrence was observed.

Lessons:

Our cases indicate that genetic factor may contribute to the risk of PT of the breast. Markers such as p53 and Ki-67 may have some correlation with PT malignancy.

Related collections

Most cited references 32

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The treatment and prognosis of patients with phyllodes tumor of the breast: an analysis of 170 cases.

B A Stelmach, J. W. Mituś, K Smolak … (1996)

The study addresses the controversial prognostic and therapeutic aspects of phyllodes tumor of the breast. Records of 170 women with phyllodes tumor of the breast were reviewed. On the basis of the criteria proposed by Azzopardi and Salvadori et al., including estimation of tumor margin, growth of the connective tissue component, mitoses, and cellular atypia, the entire series was divided into three histotypes of phyllodes tumor, i.e., benign (92 cases, 54.1%), borderline (19 cases, 11.2%), and malignant (59 cases, 34.7%). Ninety-eight patients (57.6%) were treated by wide local excision (79 benign, 15 borderline, and 4 malignant), 43 (25.3%) by simple mastectomy (13 benign, 4 borderline, and 26 malignant), and 29 (17.1%) by radical mastectomy (all malignant). Of the 170 treated patients, 141 (82.9%) survived 5 years without evidence of disease. In the Cox multivariate analysis the histotype of the tumor was the only independent prognostic factor: 5-year NED survival was observed in 95.7% of the patients with benign phyllodes tumor, 73.7% with borderline phyllodes tumor, and 66.1% with malignant phyllodes tumor. After a wide local excision 98.7% of the patients with benign tumor, and 80% with borderline tumor, were cured. Local recurrence was found in 14 patients (8.2%) (4 benign, 3 borderline, and 7 malignant); 10 of these underwent reoperation (7 wide local excision, 3 radical mastectomy) and survived 5 years NED. The histotype of phyllodes tumor (benign, borderline, and malignant), assessed on the basis of the criteria proposed by Azzopardi and Salvadori et al., was the only prognostic factor in our group of patients. Based on the data from literature and our own observations, we observed that a wide local excision, with an adequate margin of normal breast tissue, is the preferred initial therapy for phyllodes tumor of the breast.

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Phyllodes tumor of the breast.

Yann Malard, Isabelle Lacroix, Edouard Lagneau … (2008)

To better identify prognostic factors for local control and survival, as well as the role of different therapeutic options, for phyllodes tumors, a rare fibroepithelial neoplasm of the breast. Data from 443 women treated between 1971 and 2003 were collected from the Rare Cancer Network. The median age was 40 years (range, 12-87 years). Tumors were benign in 284 cases (64%), borderline in 80 cases (18%), and malignant in 79 cases (18%). Surgery consisted of breast-conserving surgery (BCS) in 377 cases (85%) and total mastectomy (TM) in 66 cases (15%). Thirty-nine patients (9%) received adjuvant radiotherapy (RT). After a median follow-up of 106 months, local recurrence (LR) and distant metastases rates were 19% and 3.4%, respectively. In the malignant and borderline group (n = 159), RT significantly decreased LR (p = 0.02), and TM had better results than BCS (p = 0.0019). Multivariate analysis revealed benign histology, negative margins, and no residual disease (no RD) after initial treatment and RT delivery as independent favorable prognostic factors for local control; benign histology and low number of mitosis for disease-free survival; and pathologic tumor size < or = 3 cm and no tumor necrosis for overall survival. In the malignant and borderline subgroup multivariate analysis TM was the only favorable independent prognostic factor for disease-free survival. This study showed that phyllodes tumor patients with no RD after treatment have better local control. Benign tumors have a good prognosis after surgery alone. In borderline and malignant tumors, TM had better results than BCS. Thus, in these forms adjuvant RT should be considered according to histologic criteria.

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Phyllodes tumours.

Megan Parker, Catherine S Harries (2001)

Phyllodes tumours are rare fibroepithelial lesions that account for less than 1% of all breast neoplasms. With the non-operative management of fibroadenomas widely adopted, the importance of phyllodes tumours today lies in the need to differentiate them from other benign breast lesions. All breast lumps should be triple assessed and the diagnosis of a phyllodes tumour considered in women, particularly over the age of 35 years, who present with a rapidly growing "benign" breast lump. Treatment can be by either wide excision or mastectomy provided histologically clear specimen margins are ensured. Nodal metastases are rare and routine axillary dissection is not recommended. Few reliable clinical and histological prognostic factors have been identified. Local recurrence occurs in approximately 15% of patients and is more common after incomplete excision. It can usually be controlled by further surgery. Repeated local recurrence has been reported without the development of distant metastases or reduced survival. Approximately 20% of patients with malignant phyllodes tumours develop distant metastases. Long term survival with distant metastases is rare. The role of chemotherapy, radiotherapy, and hormonal manipulation in both the adjuvant and palliative settings remain to be defined.

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Author and article information

Journal

Journal ID (nlm-ta): Medicine (Baltimore)

Journal ID (iso-abbrev): Medicine (Baltimore)

Journal ID (publisher-id): MEDI

Title: Medicine

Publisher: Wolters Kluwer Health

ISSN (Print): 0025-7974

ISSN (Electronic): 1536-5964

Publication date Collection: November 2017

Publication date (Electronic): 17 November 2017

Volume: 96

Issue: 46

Electronic Location Identifier: e8552

Affiliations

[a ]Department of Thyroid Surgery

[b ]Department of Pathology

[c ]Department of Pancreatic Surgery

[d ]Department of Breast Surgery, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China.

Author notes

[∗ ]Correspondence: Xiaoqin Yang, Department of Breast Surgery, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China (e-mail: xiaoqinyang1977@ 123456163.com ).

Article

Publisher ID: MD-D-17-03707 Accession ID: 08552

DOI: 10.1097/MD.0000000000008552

PMC ID: 5704806

PubMed ID: 29145261

SO-VID: a865b08f-a3f3-4f43-9b08-35f31de44134

License:

This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc-nd/4.0

History

Date received : 14 June 2017

Date revision received : 10 October 2017

Date accepted : 12 October 2017

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Keywords: breast,diagnosis,genetic predisposition,phyllodes tumor,therapy

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Keywords: breast, diagnosis, genetic predisposition, phyllodes tumor, therapy

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Phyllodes tumors of the breast in 2 sisters : Case report and review of literature

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Abstract

Rationale:

Patient concerns:

Diagnoses:

Interventions:

Outcomes:

Lessons:

Related collections

Hematology and Oncology Discovery

Most cited references 32

The treatment and prognosis of patients with phyllodes tumor of the breast: an analysis of 170 cases.

Phyllodes tumor of the breast.

Phyllodes tumours.

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