Submit Your Article CMED MEACR meeting
Home Print this page Email this page Users Online: 959
Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 


 
 Table of Contents  
ORIGINAL ARTICLE
Year : 2014  |  Volume : 3  |  Issue : 4  |  Page : 326-328

Malignant phyllodes tumor with chondro and osteosarcomatous differentiation and secondaries in lungs


1 Department of Radiation Oncology, Acharya Tulsi Regional Cancer Treatment and Research Institute, Bikaner, Rajasthan, India
2 Department of Pathology, Sardar Patel Medical College and Associated Group of Hospitals, Bikaner, Rajasthan, India

Date of Web Publication16-Jun-2014

Correspondence Address:
Akhil Kapoor
Room No. 73, PG Boys Hostel, PBM Hospital Campus, Bikaner, Rajasthan
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-0513.134496

Rights and Permissions
  Abstract 

Phyllodes tumor is a rare breast tumor, with neoplastic epithelial and stromal components for <1% of all breast neoplasms. Phyllodes tumors form a spectrum from benign tumors, similar to fibroadenomas, of malignant tumors with a propensity for rapid growth and metastatic spread. High-grade malignant phyllodes tumor is a very rare, but aggressive breast malignancy and forms approximately 25% of all phyllodes tumors. We report a case of a 44-year-old female who presented with a painless left breast lump for 4 months duration. The mass was >10 cm in size and firm in consistency. She underwent left mastectomy. The histopathology revealed a malignant phyllodes tumor with sarcomatous stromal overgrowth and heterogeneous chondro and osteosarcomatous differentiation. At 2 months after surgery, she reported to us with secondaries in bilateral lungs. We planned palliative chemotherapy in view of good general condition of the patient and lung metastasis. Chemotherapy included ifosfamide, adriamycin and cisplatin as per standard regimen every 3 weekly. After three cycles, her lung metastasis cleared completely. It was planned to continue same chemotherapy for six cycles.

Keywords: Breast cancer, chondromatous, malignant, osteosarcomatous, phyllodes tumor


How to cite this article:
Narayan S, Kapoor A, Singhal MK, Bagri PK, Sharma N, Kumar V, Kumar HS. Malignant phyllodes tumor with chondro and osteosarcomatous differentiation and secondaries in lungs. Clin Cancer Investig J 2014;3:326-8

How to cite this URL:
Narayan S, Kapoor A, Singhal MK, Bagri PK, Sharma N, Kumar V, Kumar HS. Malignant phyllodes tumor with chondro and osteosarcomatous differentiation and secondaries in lungs. Clin Cancer Investig J [serial online] 2014 [cited 2019 Dec 12];3:326-8. Available from: http://www.ccij-online.org/text.asp?2014/3/4/326/134496


  Introduction Top


Phyllodes tumor is a rare breast tumor, with neoplastic epithelial and stromal components for <1% of all breast neoplasms. The terminology phyllodes tumor was given by World Health Organization and classifies it into benign, borderline, and malignant tumors according to histopathological features malignant phyllodes tumor with high-grade features is a very rare, but highly aggressive subgroup of this tumor. [1] It has neoplastic epithelial and stromal components. The stromal component may show homologous and heterologous sarcomatous elements, including chondrosarcomatous and osteosarcomatous differentiation. [2] As such tumors may present with an almost exclusively sarcomatous component, a pathologist should include this entity in the differential diagnosis of fine-needle aspirations of breast neoplasms showing sarcomatous differentiation. Such differentiation of a malignant phyllodes tumor is rare, accounting for 1.3% of phyllodes tumors in the breast. [3] This differentiation is also believed to increase the aggressiveness of the neoplasm manifold, particularly when large (>5 cm) or associated with an osteoclastic or osteoblastic osteosarcoma. We report a case of a 44-year-old female in which the histopathology revealed a malignant phyllodes tumor with sarcomatous stromal overgrowth and heterogeneous chondro and osteosarcomatous differentiation.


  Case report Top


A 44-year-old perimenopausal woman had a painless left breast lump for 4 months duration which enlarged rapidly in last 2 months. She had no past history of malignancy or prior irradiation to the breast or thoracic region. Family history was also not significant. The lump was >10 cm and firm in consistency. The axillary lymph nodes were not palpable. Left sided mastectomy was done at a peripheral hospital. The histopathological specimen's cut surfaces showed irregular grey brown friable growth measuring 15 cm × 9 cm × 7 cm, reaching up to the distal resected margin. It was grey white glistening with slit-like areas. Grossly, the growth was close to all peripheral margins and 1 cm below the skin surface. No lymph node was identified. Microscopically, it was suggestive of malignant phyllodes tumor with sarcomatous stromal overgrowth and heterogeneous chondro and osteosarcomatous differentiation [Figure 1] and [Figure 2]. The mitotic rate was 28/10 high-power field (HPF) and remnants of benign phyllodes tumor were also seen. She reported to us after 2 months of surgery. Her computed tomography scan chest revealed bilateral lung metastasis. We planned palliative chemotherapy in view of good general condition of the patient and lung metastasis. Chemotherapy included ifosfamide, Adriamycin, and cisplatin as per standard regime of day 1-22. After three cycles, her lung metastasis cleared completely. She was planned to continue same chemotherapy for six cycles.
Figure 1: Microscopic view demonstrating osteosarcomatous components (H and E, ×4)

Click here to view
Figure 2: Photo micrograph demonstrating chondrosarcomatous components (H and E, ×4)

Click here to view



  Discussion Top


Phyllodes tumors are uncommon neoplasm that account for <1% of all breast tumors. [1],[4] High-grade malignant phyllodes tumor is a very rare, but aggressive breast malignancy and forms approximately 25% of all phyllodes tumors. [5] Most of the cases present between the ages of 35 and 55 years in women who haven't yet been through the menopause; adolescent and elderly women are also affected. There are no predisposing factors and the etiology of a phyllodes tumor is unknown. [6] However, its incidence is higher in whites in general, in Latin whites and East Asians in particular. Clinically it appears as a round, mobile, and painless mass. Axillary lymph nodes are usually not palpable at presentation, because metastatic spread of these tumors is primarily hematogenous; lungs, pleura, and bone have been the most common sites of metastasis. Metastasis to axillary lymph nodes is observed in only 2% cases. [7]

Histologically, the phyllodes tumor consists of epithelial cells and connective tissue with more stromal proliferation than that of fibroadenoma, often accompanied by cellular atypia. For malignant phyllodes tumor, they are further divided into borderline, low-grade, and high-grade on the basis of the following histological criteria: Tumor borders, mitotic activity, stromal atypia, and stromal overgrowth. Only the stromal cells have the potential for metastasis. The malignant character of the phyllodes tumor is therefore confirmed by the microscopic appearance of the stroma. [8]

Phyllodes tumors are clinically similar to fibroadenomas and both have common mammographic and sonographic characteristics. On ultrasound examination, they are lobulated with heterogeneous internal echoes and intramural cysts while mammographic finding is described as a sharply defined round or oval mass with lobulation. [9]

Difficulties with diagnosis of phyllodes tumor by fine-needle aspiration cytology (FNAC) have been reported, but when these tumors present with an almost exclusively sarcomatous component, it is important for the pathologist to include this entity in the diagnostic considerations of fine-needle aspirations of breast neoplasm showing sarcomatous differentiation. The cytologist reported phyllodes tumor in only 23% of cases where FNAC was done at the time of diagnosis. In cases where core biopsy was done, phyllodes tumor was correctly diagnosed in 65% of the cases. [7]

Surgical treatment is generally accepted as the most important and primary therapy for phyllodes tumors, regardless of its histological type. A lumpectomy with clear margins (more than 1-2 cm) is recommended for small lesions (up to a size of 3 cm). A segment resection also with clear margins is recommended for larger lesions (over 3 cm in size). A mastectomy should be performed in the case of even more extensive, borderline or infiltrative areas. An axillary lymphadenectomy should only be performed in clinically conspicuous cases, as phyllodes tumor has predominantly hematogenous metastases and axillary metastases occur in <10% of cases. [8],[10]

The role of radiotherapy remains unclear from published reports because of small patient numbers and a lack of controlled data. Pandey et al. suggested that adjuvant radiotherapy also improved the disease-free survival. August and Kearney recommended that adjuvant radiotherapy be considered for high-risk phyllodes tumors, including those >5 cm, with stromal overgrowth, with >10 mitoses/HPF, or with infiltrating margins. [7] If only local recurrence (LR), then repeat surgery once/twice may be considered. The role of external beam radiotherapy is limited to the condition only when surgery has been done twice/thrice and still recurrence occurs.

Different chemotherapy regimens have been applied in malignant phyllodes tumors. Doxorubicin and ifosfamide-based chemotherapies have shown some efficacy in women with metastatic spread of phyllodes tumors. In one study of 101 patients, 4 patients were treated with chemotherapy and a role for adjuvant chemotherapy in patients with stromal overgrowth was considered. This recommendation has so far not been confirmed in literature. Altogether, there is no clear indication for adjuvant chemotherapy for patients with phyllodes tumors. [10]

Local recurrences are a common complication of high-grade lesions with a reported frequency of approximately 26% (12-65%). [3] Stromal overgrowth, larger tumor size, and involved margin were all significantly correlated with LR. Stromal overgrowth increased the probability of LR seven-fold, whereas if the margin was <1 cm, the risk of LR increased five-fold, and if tumor size was >10 cm, then the prevalence of LR was 4 times greater than for smaller tumors. The 5 and 10 years survival rates for malignant phyllodes tumor range from 54% to 82% and 23% to 42%, respectively. [7],[10]


  Conclusions Top


High-grade malignant phyllodes tumor with sarcomatous stromal overgrowth and heterogeneous chondro and osteosarcomatous differentiation is a very rare, but aggressive breast malignancy. Stromal overgrowth carries a grave prognosis. Either wide local excision with adequate margins or mastectomy is an appropriate treatment for such patients. Metastatic tumor may be benefitted from chemotherapy. When systemic therapy is used for malignant phyllodes tumor, treatment is based on guideline for treating sarcomas.

 
  References Top

1.Taira N, Takabatake D, Aogi K, Ohsumi S, Takashima S, Nishimura R, et al. Phyllodes tumor of the breast: Stromal overgrowth and histological classification are useful prognosis-predictive factors for local recurrence in patients with a positive surgical margin. Jpn J Clin Oncol 2007;37:730-6.  Back to cited text no. 1
    
2.Garcia FU, Soans S, Galindo LM. Malignant phyllodes tumor with chondrosarcomatous differentiation: Report of a case with cytological presentation. Diagn Cytopathol 1999;20:241-5.  Back to cited text no. 2
    
3.Silver SA, Tavassoli FA. Osteosarcomatous differentiation in phyllodes tumors. Am J Surg Pathol 1999;23:815-21.  Back to cited text no. 3
    
4.Parker SJ, Harries SA. Phyllodes tumours. Postgrad Med J 2001;77:428-35.  Back to cited text no. 4
    
5.Kapiris I, Nasiri N, A′Hern R, Healy V, Gui GP. Outcome and predictive factors of local recurrence and distant metastases following primary surgical treatment of high-grade malignant phyllodes tumours of the breast. Eur J Surg Oncol 2001;27:723-30.  Back to cited text no. 5
    
6.Mukherjee P, Kalish LH, Delprado W, Crea P. Telangiectatic osteosarcomatous differentiation in a phyllodes tumour. ANZ J Surg 2004;74:707-9.  Back to cited text no. 6
    
7.Asoglu O, Ugurlu MM, Blanchard K, Grant CS, Reynolds C, Cha SS, et al. Risk factors for recurrence and death after primary surgical treatment of malignant phyllodes tumors. Ann Surg Oncol 2004;11:1011-7.  Back to cited text no. 7
    
8.Kraemer B, Hoffmann J, Roehm C, Gall C, Wallwiener D, Krainick-Strobel U. Cystosarcoma phyllodes of the breast: A rare diagnosis: Case studies and review of literature. Arch Gynecol Obstet 2007;276:649-53.  Back to cited text no. 8
    
9.Foxcroft LM, Evans EB, Porter AJ. Difficulties in the pre-operative diagnosis of phyllodes tumours of the breast: A study of 84 cases. Breast 2007;16:27-37.  Back to cited text no. 9
    
10.Lenhard MS, Kahlert S, Himsl I, Ditsch N, Untch M, Bauerfeind I. Phyllodes tumour of the breast: Clinical follow-up of 33 cases of this rare disease. Eur J Obstet Gynecol Reprod Biol 2008;138:217-21.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2]


This article has been cited by
1 Borderline phyllodes tumor of breast in a premenarchal girl: A relatively common tumor at an uncommon age
Akhil Kapoor,Ishwar Charan,Mukesh Singhal,Harvindra Kumar
International Journal of Cancer Therapy and Oncology. 2016; 4(2): 4213
[Pubmed] | [DOI]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
Introduction
Case report
Discussion
Conclusions
References
Article Figures

 Article Access Statistics
    Viewed2334    
    Printed36    
    Emailed1    
    PDF Downloaded73    
    Comments [Add]    
    Cited by others 1    

Recommend this journal