| Abstract|| |
Pure mesenchymal sarcomas and phyllodes tumor with sarcomatous differentiation of the breast are rare tumors. Primary breast sarcomas are a heterogenous group of neoplasms which include malignant fibrous histiocytoma, fibrosarcoma, angiosarcoma, leiomyosarcoma, osteosarcoma, rhabdomyosarcoma, and liposarcoma. Sarcomatous differentiation in phyllodes tumor is seen in the form of angiosarcoma, chondrosarcoma, leiomyosarcoma, osteosarcoma, rhabdomyosarcoma, and liposarcoma. Here, we report a case of a 28-year-old pregnant woman with a breast lump during her second trimester which drastically increased in size in the postpartum period. Wide local excision revealed a malignant phyllodes tumor with liposarcomatous differentiation. Later, the patient underwent completion mastectomy which showed residual tumor.
Keywords: Breast, liposarcoma, phyllodes tumor, sarcomatous
|How to cite this article:|
Narla SL, Stephen P, Kurian A, Annapurneswari S. Well-differentiated liposarcoma of the breast arising in a background of malignant phyllodes tumor in a pregnant woman: A rare case report and review of literature. Indian J Pathol Microbiol 2018;61:577-9
|How to cite this URL:|
Narla SL, Stephen P, Kurian A, Annapurneswari S. Well-differentiated liposarcoma of the breast arising in a background of malignant phyllodes tumor in a pregnant woman: A rare case report and review of literature. Indian J Pathol Microbiol [serial online] 2018 [cited 2019 Aug 19];61:577-9. Available from: http://www.ijpmonline.org/text.asp?2018/61/4/577/242969
| Introduction|| |
Phyllodes tumor of the breast accounts for <1% of all breast tumors. They are a type of fibroepithelial neoplasms. Phyllodes tumor can develop de novo or can arise from fibroadenomas. A number of histomorphological parameters are considered while categorizing them into benign, borderline, and malignant subtypes. Malignant transformation in the form of sarcomas arising from phyllodes tumor of the breast can occur in <6% of the cases. Sarcomas of the breast are uncommon tumors and constitute <1% of all breast malignancies. Liposarcomas are extremely rare within the breast. We present a case of liposarcoma arising from phyllodes tumor in a pregnant woman.
| Case Report|| |
A 28-year-old pregnant woman presented with a lump in the left breast. On examination, it was a 5 cm × 3 cm × 1 cm lump in the upper outer quadrant, which was firm and freely mobile. Overlying skin and nipple areola were normal. There was no palpable axillary lymphadenopathy. She was completely asymptomatic. The right breast was unremarkable. Radiological investigations were not performed as the patient was pregnant. Fine-needle aspiration cytology of the left breast lump was performed, and 700–800 ml of blood tinged fluid was aspirated. Microscopic examination of the fluid showed rare scattered ductal epithelial cell clusters against a background of foamy macrophages. Needle core biopsy of the lump was suggestive of borderline phyllodes tumor. Surgery was not performed as the patient's estimated date of delivery was not far off. Meanwhile, the patient delivered a preterm baby. The patient noticed a sudden increase in the size of the lump in her puerperium. Wide local excision biopsy of the breast lump was performed and was sent for histopathological examination. Macroscopic examination revealed a variegated solid cystic mass measuring 14 cm × 14 cm × 8 cm with gray-white, yellowish, necrotic, and hemorrhagic areas. Microscopic examination revealed a biphasic lesion with a leaf-like pattern [Figure 1]a, [Figure 1]b, [Figure 1]c composed of varying sized, compressed, and dilated ducts against a cellular stroma with moderate-to-severe nuclear atypia and significant mitoses (29–30/10 HPF). There were foci showing lipomatous differentiation [Figure 1]d and [Figure 1]e. Lipoblasts with scalloped hyperchromatic nuclei with uni- and multi-vacuolated clear cytoplasm were seen admixed with focal myxoid areas [Figure 1]f. Lactational changes were extensively noted. A diagnosis of well-differentiated liposarcoma arising in a background of malignant phyllodes tumor was rendered. The patient further underwent completion mastectomy which showed residual tumor. Positron emission tomography-computed tomography scan did not show fluorodeoxyglucose avidity anywhere else in the body. After the surgery, unfortunately, the patient was lost to follow-up.
|Figure 1: (a-c) Malignant phyllodes tumor with a leaf-like pattern (H and E, a: ×40, b: ×40, c: ×100); (d and e) liposarcomatous differentiation (H and E, d: ×100, e: ×400); (f) lipoblasts (H and E, ×400)|
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| Discussion|| |
Breast sarcomas are malignant neoplasms arising from the mesenchymal elements of the mammary parenchyma and are uncommon tumors constituting <1% of all breast malignancies., In 1862, Neuman reported a case of breast sarcoma. Fooate and Stewart were the first to describe the malignant change of heterologous elements within a phyllodes tumor. Breast sarcomas can be primary (arising de novo) or secondary (arising from preexisting fibroepithelial lesions). Primary breast sarcomas are a heterogenous group of neoplasms, which include malignant fibrous histiocytoma, fibrosarcoma, angiosarcoma, leiomyosarcoma, osteosarcoma, rhabdomyosarcoma, and liposarcoma. Liposarcomas are the second most common sarcomas preceded by malignant fibrous histiocytoma (<0.3% of the all breast sarcomas). Phyllodes tumor has been broadly divided into benign, borderline, and malignant by the World Health Organization depending on the stromal cellularity, cellular pleomorphism, mitoses, infiltrative margins, etc., Sarcomatous differentiation in phyllodes tumor is seen in the form of angiosarcoma, chondrosarcoma, leiomyosarcoma, osteosarcoma, rhabdomyosarcoma, and liposarcoma. These tumors have a predilection to recur and metastasize; therefore, it is imperative to get a good surgical clearance. Liposarcomas have a predilection to lower extremities, pelvic girdle, and retroperitoneum. Other rare locations include peritoneal cavity, axilla, vulva, spermatic cord, and breast. A review of literature has revealed few case reports of liposarcomas arising in a background of phyllodes; however, since the entity of malignant phyllodes tumor with dedifferentiation itself is a rare entity, a very few case series exist. Differential diagnosis of lipomatous neoplasms in the breast include primary or secondary liposarcoma and lipoma. In the present case, microscopic examination revealed phyllodes tumor intermingled with areas of abrupt transition into liposarcomatous areas. Hence, the differentials of primary liposarcoma and benign lipomas are excluded. The mainstay of treatment for sarcomas is wide local excision with an at least 2-cm all-round clearance. Mastectomy is advised when the margins are involved or if there is positive nodal metastasis. Here, our patient eventually required a completion modified radical mastectomy with axillary clearance due to the positive margin status on lumpectomy. The indication for the use of adjuvant chemotherapy and radiation therapy is inability to obtain negative surgical resection status, higher grade liposarcoma, or size >5 cm., Prognosis of breast liposarcomas is not known due to the rarity of the condition.
| Conclusion|| |
Phyllodes tumor with malignant lipomatous transformation should be considered as secondary liposarcomas. Liposarcomas of the breast are rare tumors and must be considered in the differential diagnosis of all malignant mesenchymal tumors.
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Conflicts of interest
There are no conflicts of interest.
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Swetha Lakshmi Narla
Department of Histopathology, Apollo Cancer Institutes, 320, Anna Salai, Chennai - 600 035, Tamil Nadu
Source of Support: None, Conflict of Interest: None