|Year : 2015 | Volume
| Issue : 1 | Page : 40-42
Giant malignant phyllodes tumor with liposarcomatous differentiation
I Yusuf1, AA Sheshe2, S Raphael1
1 Department of Histopathology, Aminu Kano Teaching Hospital, Bayero University, Kano, Nigeria
2 Department of Surgery, Aminu Kano Teaching Hospital, Bayero University, Kano, Nigeria
|Date of Web Publication||13-Mar-2015|
Department of Histopathology, Aminu Kano Teaching Hospital, Bayero University, PMB 3452, Kano
Source of Support: None, Conflict of Interest: None
Malignant phyllodes tumor may occur with heterelogous differentiation. Sarcomatous stromal elements that may be seen in these tumors include angiosarcoma, chondrosarcoma, leiomyosarcoma, osteosarcoma, rhabdomyosarcoma, and liposarcoma. However, liposarcomatous stromal differentiation in malignant phyllodes tumor is very rare. We report a case of an 18-year-old lady who presented with a 4-months history of recurrent huge rapidly growing ulcerated left breast mass. A clinical impression of ulcerated malignant phyllodes tumor was made. Simple mastectomy was necessary due to the recurrent nature of the lesion, presentation, and available facilities. Histological findings revealed a lesion exhibiting leaf-like pattern and composed of a highly cellular stroma demonstrating myxoid changes, liposarcomatous differentiation including lipoblasts and bizarre tumor giant cells. A histologic diagnosis of malignant phyllodes tumor with liposarcomatous differentiation was made. She had an un-eventful post-operative period and was discharged to follow up.
Keywords: Giant Phyllodes tumor, liposarcomatous Phyllodes differentiation, malignant breast disease
|How to cite this article:|
Yusuf I, Sheshe A A, Raphael S. Giant malignant phyllodes tumor with liposarcomatous differentiation. Arch Int Surg 2015;5:40-2
| Introduction|| |
Phyllodes tumors (PT) of the breast are a group of circumscribed biphasic tumors analogous to fibroadenomas, characterized by double-layered epithelial component arranged in clefts surrounded by an overgrowing hypercellular mesenchymal component typically organized in a leaf-like structure. , Classification into benign, borderline, and malignant is based on histological characteristics that include degree of stromal hypercellularity, stromal cytologic atypia, mitotic activity, stromal overgrowth, and infiltrative margins.  Malignant PTs have infiltrative rather than pushing margins. The stroma shows frankly sarcomatous, usually fibrosarcomatous changes. Heterelogous differentiation such as liposarcoma, chondrosarcoma, or rhabdomyosarcoma may be seen. ,, All types of phyllodes tumors are characteristically associated with high and rapid recurrence rates when incompletely excised. 
| Case Report|| |
The patient was an 18-year-old single lady who presented to our hospital with 4-months history of progressive painless left breast mass. No other breast symptoms. She had no family history of breast disease, no history of trauma to the breast and has never used hormonal contraceptives. She was initially investigated and counselled for an excision biopsy. However, the patient absconded to avoid surgical intervention. She had several traditional treatments including scarification that developed into an ulcer. She re-presented 3 months later with complains of progressive left breast swelling, ulceration, and intermittent bleeding. She had lost weight remarkably.
On examination, she was moderately pale,febrile, and anicteric. She had a huge left breast mass 26 × 20 × 18 cm size, compressing the whole breast. The nipple was inverted and shrunken. The mass was ulcerated in the upper outer quadrant. The ulcer was covered with traditional concoctions producing foul smelling purulent discharge. The edge of the ulcer was raised and everted with fleshy masses in the floor. The skin was stretched and shiny with visible distended veins over the mass but no peau'd orange. There was a single palpable non-tender, firm left median axillary lymph node 1 × 1 × 0.5 cm in size. Her abdominal and chest findings were essentially normal. The vital signs were within normal limits.
An impression of Ulcerated Cystosarcoma Phyllodes with probably malignant transformation was made and the patient was admitted, placed on analgesics and antibiotics pending results of investigations. She had initial breast cytology (FNAB) of the mass during the first presentation which was reported as suspicious probably benign (C3). Her blood sample was taken for repeat full blood counts, differentials, and electrolytes, the results of which were all within normal ranges. Her chest radiograph and abdominal scan were normal. She was counselled and had left simple mastectomy because of the huge size of the mass and the florid clinical features of malignancy.
The mastectomy specimen measured 19 × 16 × 12 cm and weighed 2 kg. The skin over the mass was focally ulcerated and the nipple was retracted. Cut sections show a delineated tumor, 14 cm in its widest diameter with grayish white to yellowish solid surfaces with tiny cleft-like spaces. Necrotic areas were also observed. Microscopy showed a lesion exhibiting a leaf-like pattern and composed of multiple, compressed slit-like ductules lined by attenuated cuboidal epithelium [Figure 1]. The abundant cellular stroma show focal myxoid changes, liposarcomatous differentiation, including lipoblasts with enlarged, scalloped, anaplastic nuclei, and abundant clear cytoplasm [Figure 2] and [Figure 3]. Several bizarretumor giant cellsand areas of necrosis were also seen. A diagnosis of malignant phyllodes tumor with liposarcomatous differentiation was made. The patient did well post operatively and was subsequently discharged a week after surgery. She was lost to follow-up.
|Figure 1: Low power view showing leaf-like pattern characteristic of phyllodes tumor (H&E× 40)|
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|Figure 2: Stromal component showing slit-like ducts, myxoid change, and lipomatous differentiation (H&E× 100)|
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|Figure 3: High power view showing lipomatous change and malignant lipoblasts (H&E× 400)|
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| Discussion|| |
Fibroepithelial tumors of the breast represent heterogeneous group of biphasic neoplasms that range from benign to malignant and include fibroadenoma and PT.  PT are typically large, fast growing masses and account for less than 1% of all breast neoplasm. It occurs predominantly in middle-aged women with average age of presentation being 40-50 years but can also occur in young adults or even in adolescents. Therefore, the diagnosis cannot be excluded on the basis of age alone. ,
PT usually presents with unilateral, firm to hard painless palpable breast mass. , The average size of the mass is 4-5 cm but may range between 1-20 cm.  Sizes greater than 10 cm, such as seen in this case report, may stretch the skin with striking distention of superficial veins, rarely ulceration may occur.  The malignant variants tend to be larger and may invade and ulcerate the skin or extend into the chest wall. The mass is well-circumscribed but not encapsulated and cut section reveals a bulging, firm, gray-tan tissue that exhibit cleft-like spaces and may show areas of infarction, necrosis, or haemorrhage. ,
Malignant PTs have infiltrative rather than pushing margins and the stroma is frankly sarcomatous. Heterelogous stromal differentiation that may be seen in these tumors include angiosarcoma, chondrosarcoma, leiomyosarcoma, osteosarcoma, rhabdomyosarcoma, and liposarcoma. , Liposarcomatous stromal differentiation in malignant PTis very rare  and may consist of well-differentiated myxoid, round cell, and pleomorphic elements.  The pleomorphic subtype seen in this case report and in that reported by Isotalo et al.,  showed lipoblasts with pleomorphic, hyperchromatic, scalloped nuclei, and vacuolated cytoplasm.
The diagnosis of malignant phyllodes with liposarcomatous differentiation, rather than a primary liposarcoma of the breast was predicated on the age of the patient(liposarcomas are rare in that age-group), the gross findings of a solid tumor with cleft-like spaces, the microscopic features of myxoid stroma and slit-like, compressed ducts and ductules demonstrating a leaf-like pattern characteristic of a phyllodes tumor. The clinical behavior of malignant PT is difficult to predict. Liposarcomatous differentiation in malignant PT; however, does not appear to equate with more aggressive clinical behavior , and local recurrences are uncommon for completely excised tumors.
The fundamental principles of treatment is complete wide local excision to prevent local recurrences which may occur in both benign and malignant phylodes. ,, Other than surgery, there is no cure for malignant phyllodes, as chemotherapy and radiation therapy are not effective. The frequency of local recurrences and metastases correlate with the grade of the tumor. , Benign PT will not metastasize and has low probability for local recurrence. Both low and high grade malignant PT have probabilities of metastases and may recur locally unless widely excised. , However, despite wide excision a very high percentage of surgeries yielded incomplete excision margins that required revision surgery. 
This provided the ground for a simple mastectomy in this patient due to involvement of the whole breast. However, a recent study suggests a good outcome after breast conserving surgery, followed by radiation therapy in patients with borderline and malignant tumors. 
| References|| |
Bellocq JP, Magro G. Fibroepithelial tumours. In: Tavassoli FA, Devilee P, editors. Pathology and Genetics of tumours of the breast and female genital organs. World Health Organization of Tumours of the Breast and Female Genital Organs. Lyon: IARC Press; 2005. p. 99-103.
Uniev L, Maslovsky I, Vainshtein P, Yoffe B, Ben-Dor D. Malignant phyllodes tumour with liposarcomatous differentiation and tubular adenoma-like epithelial component. Int J Med Sci 2006;3:130-4.
Moffat CJ, Pinder SE, Dixon AP, Elston CW, Blarney RW, Ellis IO. Phyllodes tumours of the breast: A clinicopathological review of thirty-two cases. Histopathology 1995;27:205-18.
Breast. In: Rosai J, editor. Rosai and Ackerman's Surgical Pathology. 9 th
ed. Vol 2. Ch 20. India: Mosby; 2009. p. 1829-31.
Fibroepithelial Neoplasms. In: Rosen PP. editor. Rosen's Breast Pathology. 1 st
ed. Ch 8. Philadelphia: Lippincort-Raven; 1986. p. 155-72.
Austin RM, Dupree WB. Liposarcoma of the breast: A clinicopathologic study of 20 cases. Human Pathol 1986;7:906-13.
Isotalo PA, George RL, Walker R, Sengupta SK. Malignant phyllodes tumour with liposarcomatous differentiation. Arch Pathol Lab Med 2005;129:421-2.
Tan PH, Jayabaskar T, Chuah KL, Lee HY, Tan Y, Hilmy M, et al
. Phyllodes tumours of the breast: The role of pathologic parameters. Am J Clin Pathol 2005;123:529-40.
Guillot E, Couturaud B, Reyal F, Curnier A, Ravinet J, Laé M, et al
; Breast Cancer Study Group of the Institut Curie. Management of phyllodes breast tumors. Breast J 2011;17:129-37.
Barth RJ Jr, Wells WA, Mitchell SE, Cole BF. A prospective, multi-institutional study of adjuvant radiotherapy after resection of malignant phyllodes tumors. Ann Surg Oncol 2009;16:2288-94.
[Figure 1], [Figure 2], [Figure 3]