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CASE REPORT
Year : 2022 | Volume
: 13
| Issue : 3 | Page : 257-259
Pleomorphic liposarcoma of breast, presenting as a recurrent breast tumor
Durre Aden, Sufian Zaheer, Divita Saxena, Sunil Ranga
Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
Correspondence Address:
Sufian Zaheer
Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, 4th Floor, College Building, New Delhi - 110 029
India
Source of Support: None, Conflict of Interest: None
CheckDOI: 10.4103/jmh.jmh_17_22
Abstract
Primary pleomorphic liposarcoma of the breast is a very rare mesenchymal tumor. Sarcoma arising in the breast constitutes <1% of all malignant breast tumors, and liposarcoma of the breast has an incidence of 0.3% of all mammary sarcomas. It is a very aggressive tumor with a high incidence of recurrences and distant metastasis. They can mimic primary invasive breast cancer clinically as well as radiologically. Histology and radiological evaluation of the patient help correctly type and grade this malignancy. On microscopy, the tumor is composed of pleomorphic tumor cells with atypical lipoblast with areas of necrosis and mitosis. We report one such rare case here.
Keywords: Histopathology, liposarcoma breast, recurrences
Introduction 
Breast cancer is the most common neoplasm in women globally.[1] Invasive breast carcinoma – No Special Type (NST) is the most common subtype. In rare cases, breast tumor mass does not show epithelial differentiation, and is primary mesenchymal sarcomas which constitute less than 0.1% of breast malignancies, with an incidence estimated at 2–5 cases/100,000 yearly.[2] The most common primary breast sarcoma is angiosarcoma. Liposarcoma of the breast is a very rare and aggressive mesenchymal breast tumor. At times, exact diagnosis may pose a significant challenge since there are over 50 subtypes of soft-tissue sarcoma (STS), which determine their prognostic and therapeutic features. Liposarcoma can either present as a pure form or may arise in malignant phyllodes tumors. To diagnose primary liposarcoma of the breast, extensive sampling of the specimen is required.[3],[4] Imaging modalities identify liposarcoma often as invasive breast carcinoma. Diagnosing the type of sarcoma and the degree of differentiation is important for surgical management and prognosis. Surgery with a wide local excision is the treatment of choice. They can recur locally or metastasize. Very few cases of pleomorphic liposarcoma breast have been reported in the past.[2] We report an unusual case of pleomorphic liposarcoma presenting as recurrent invasive breast cancer and the diagnostic challenge it imposes in diagnosing it.
Case Report A 24-year-old female had a history of a lump in the right breast of size 23 cm × 21 cm × 8 cm; the overlying skin was ulcerated. She had no family history of cancer. On mammography, the lesion was scored BIRADS 5, and a clinicoradiological diagnosis of invasive breast carcinoma was made. Radical mastectomy and lumpectomy was performed, and the specimen was sent for histopathological workup. The contralateral breast was unremarkable. There was no palpable lymph node. It was a fungating growth of 21 cm × 8 cm with a smooth surface. On gross examination, a gray-tan-to-yellow tumor measuring 23 cm × 21 cm × 8 cm with hemorrhagic areas was observed [Figure 1]a. Histopathologic examination showed a tumor composed of mature adipocytes of variable size with highly pleomorphic cells with vesicular nuclei, prominent nucleoli, and scant cytoplasm [Figure 1]b. A fair number of lipoblasts with a few bizarre and multinucleated forms were also seen [Figure 1]c. Tumor cells showed marked pleomorphism with the spindle-to-oval nuclei with brisk mitosis (20–25/10 hpf) with extensive areas of necrosis [Figure 1]d. There were no glandular or heterologous elements seen, although areas with marked hemorrhage and necrosis were also noted. Immunohistochemistry (IHC) showed tumor cells strongly positive for vimentin and focally for S100 [Figure 2]a and [Figure 2]b, and negative for pan-cytokeratin (CK), epithelial membrane antigen (EMA), P63, smooth muscle actin (SMA), desmin, estrogen receptor, progesterone receptor, HER2 neu, and CD 34. Ki 67 labeling index was very high (60%–70%). Histopathological as well as immunohistochemical findings were those of pleomorphic liposarcoma. The deep resection margin was involved, and all other close resection surgical margins were free of tumors. The patient had a history of a lump at a similar site 2 years ago, which was initially 1 cm × 1 cm, but increased in size rapidly, for which she went for local excision of the tumor. There was a recurrence of the lump in the same breast within a year with the involvement of the overlying nipple and skin. Thus, it was reported as pleomorphic liposarcoma of the breast. Because of the involved deep resection margins, a wide excision was recommended and performed. At present, the patient is planning for repeat surgery.
Figure 1: Liposarcoma (a) Gross photograph showing a greyish yellow tumor in a mastectomy specimen (marked with arrow), (b)Histopathology shows a tumour composed of mature adipocytes of variable size along with highly pleomorphic cells with vesicular nuclei, prominent nucleoli and scant cytoplasm. There are lip blasts with few bizarre and multinucleated forms were also seen, (c) Tumour cells showed marked pleomorphism with spindle to oval nuclei and brisk mitosis (d) extensive areas of necrosis. (H and E, b:100X c&d: 400x)
Figure 2: Tumor cells are positive for (a) Vimentin, and (b) focally for S-100 (×400) Discussion Primary sarcomas of the breast tissue consist of approximately 0.1% of all malignant breast malignancies.[5] Of these, liposarcoma comprises <0.3% of all primary breast sarcomas.[2],[5] They are seen between 45 and 55 years of age and are generally unilateral.[6] They generally do not develop from lipomas. Irradiation to the chest wall is one of the risk factors. The WHO classifies liposarcoma into seven subtypes – well differentiated, dedifferentiated, myxoid, round cell, pleomorphic, mixed type, and not otherwise specified.[2] On mammography, these tumors often present as an ill-defined opacity with fat and soft tissue components.[2] They can either occur as pure primary liposarcoma or arise in a phyllode tumor.[3] In our case, the patient presented with a recurrent breast mass that was reported as primary liposarcoma of the breast. The malignant transformation of phyllodes shows fibrosarcomatous or other rare heterologous sarcomatous elements like rhabdoid, osteosarcoma, liposarcomatous, etc.[3] Lucas et al. reported 58 cases of well-differentiated liposarcoma, with 32 cases involving the extremities, 20 in the retroperitoneum, followed by four cases in the scrotum and one each of the abdominal wall and the cheek.[7] The prognosis depends on the histologic subtype and degree of differentiation of liposarcoma. The pleomorphic liposarcomas are aggressive malignancies with high metastatic potential, while well-differentiated and myxoid liposarcoma generally have a better outcome.[8] Liposarcoma must be distinguished from malignant phyllodes, metaplastic carcinoma, and other primary sarcomas of the breast. Pure sarcomas without pre-existing phyllodes are extremely rare, and it needs to be excluded.[4] In metaplastic carcinomas, the malignant epithelial component tends to merge with the spindle cell element, whereas in malignant phyllodes, the epithelial component is benign and is discrete from the spindle cell component. A panel of CK markers and p63 should be used to rule out metaplastic carcinoma. Extensive sampling and careful search for a biphasic pattern and epithelial differentiation with a panel of broadspectrum CK are needed to rule out metaplastic carcinoma. CD34 is expressed in the stroma of phyllodes but is absent in spindle cell metaplastic carcinoma, fibromatosis, or liposarcoma.[4] Our case showed negative staining for pan-CK and CD 34 but was focally positive for S100.[9] Sarcomas are spread by direct local invasion or through a hematogenous route. Since lymphatic dissemination is rare, neither sentinel node biopsy nor axillary lymph node dissection is recommended in the absence of evidence of lymph node involvement.[9],[10] The European Society for Medical Oncology (ESMO) and the European Sarcoma Network Working Group recommend treating breast sarcomas as any other STS by doing a breast-conserving surgery.[10] The negative margins are crucial for long-term survival.[3] Adjuvant radiotherapy consists of patients with large tumors (>5 cm); high-grade sarcoma is pleomorphic or round cells.[2] 5-year survival is 90% for well-differentiated types and 20% for the higher grades pleomorphic and round cells. Endocrine therapy, which is generally given in invasive breast carcinomas, is not recommended in breast sarcoma due to the lack of efficacy since they are hormone receptor-negative. Re-excision to obtain clear margins should be pursued whenever needed.
Conclusion Primary pleomorphic liposarcoma of the breast is a very rare malignancy of the breast. It generally presents as invasive breast cancer clinically and radiologically. Histopathological examination with IHC and tumor grading, helps in the proper diagnosis and management of the patient. Surgery with a wide local excision is the treatment of choice, but the role of adjuvant chemotherapy and radiotherapy is yet to be identified.
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The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed
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Conflicts of interest
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References 
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