Ahmed, Z

Muzaffar, S

Shah, H U

Yaqoob, N

English

eCommons@AKU

eCommons@AKUDepartment of Pathology and Laboratory Medicine Medical College, PakistanOctober 2004Pleomorphic liposarcoma in a ten year old childZ AhmedAga Khan University, zubair.ahmad@aku.eduH U. ShahAga Khan UniversityN YaqoobAga Khan UniversityS MuzaffarAga Khan UniversityFollow this and additional works at: https://ecommons.aku.edu/pakistan_fhs_mc_pathol_microbiolPart of the Microbiology Commons, and the Pathology CommonsRecommended CitationAhmed, Z., Shah, H. U., Yaqoob, N., Muzaffar, S. (2004). Pleomorphic liposarcoma in a ten year old child. Journal of Pakistan MedicalAssociation, 54(10), 533-534.Available at: https://ecommons.aku.edu/pakistan_fhs_mc_pathol_microbiol/953IntroductionLiposarcoma is the second most common soft tissuesarcoma in adults.1 It is extremely uncommon in the pediatricage group. Although many cases so diagnosed in children inthe past are in reality examples of lipoblastomatosis,2 severalindubitable cases of liposarcoma have been recorded in ado-lescents and children.3-5 In children under ten years of age, itis extremely rare.6 Majority of liposarcomas in children aremyxoid lipsarcomas, while the pleomorphic subtype isexceptional.7 We report a case of pleomorphic liposarcoma ina 10 year old child. Case ReportWe received the specimen of a ten year old boy fromNawabshah coded as "mass left lower leg". There was a his-tory of trauma a month ago which was followed by thedevelopment of a mass along with a non-healing ulcer open-ing on the surface of the skin, on the lateral aspect of the leftlower leg. Interestingly, provisional clinical diagnosis wasgiant cell tumor of bone. Grossly, the specimen consisted ofmultiple nodular soft tissue fragments measuring togetherapproximately 12x8.5x6cms in aggregate. Multiple repre-sentative sections from the soft tissue fragments were sub-mitted. On microscopy, the tumor was seen to be composedof sheets of malignant lipoblasts with highly vacuolatedcytoplasm and severe nuclear hyperchromasia. Bizarretumor giant cells were seen and numerous mitotic figuresincluding abnormal ones were identified (Figures 1 and 2).In many cells, the nuclei were indented due to the presenceof intracytoplasmic lipid. On immuno histochemistry, thetumor cells were positive for S100 protein and weak posi-tive for vimentin. Tumor cells were negative for cytoker-atins, anti smooth muscle actin and CD34. Based on the his-tological features, a diagnosis of pleomorphic liposarcomawas made. DiscussionLiposarcoma of any type are rare in children, butpleomorphic subtype is exceptional.7 Only a handful ofcases are reported in literature.8-10 These cases occurred inneck8,9 shoulder9 inner chest wall10, roof of mesentery,7 fin-ger and urinary bladder.11 In our case, the tumor was locat-ed in the left lower extremity. To our knowledge, this is thefirst report of a childhood pleomorphic liposarcoma origi-nating in this anatomic location.Histological diagnosis of pleomorphic liposarcomadepends on the presence of pleomorphic malignantlipoblasts. These were easily identifiable in this case.Pleomorphic liposarcoma must be distinguished from poor-ly differentiated myxoid liposarcoma. The diffuse distribu-tion of pleomorphic lipoblasts and the absence of myxoid,spindle cell and round cell areas made this distinction pos-sible. In this case, tumour cells were positive for immunohistochemical stain S100 protein. This is usually seen inPleomorphic Liposarcoma in a ten year old ChildZ. Ahmed, H. U. Shah, N. Yaqoob, S. MuzaffarDepartment of Pathology and Microbiology, Aga Khan University, Karachi.Figure 2. High power view showing tumour giant cells and lipoblasts (H&E, Mag 40x).finger and urinary bladder.11 In our case, the tumorwas located in the left lower extremity. To our knowledge,this is the first report of a childhood pleomorphic liposarco-ma originating in this anatomic location.Histological diagnosis of pleomorphic liposarcomadepends on the presence of pleomorphic malignantlipoblasts. These were easily identifiable in this case.Pleomorphic liposarcoma must be distinguished from poor-ly differentiated myxoid liposarcoma. The diffuse distribu-tion of pleomorphic lipoblasts and the absence of myxoid,spindle cell and round cell areas made this distinction pos-sible. In this case, tumour cells were positive for immunohistochemical stain S100 protein. This is usually seen inliposarcoma, although more importantly it excludes thediagnosis of malignant fibrous histio cytoma.12 References1. Enterline HT, Culberson JD, Rochlin DB, et al. Liposarcoma: a clinical and patho-logical study of 53 cases. Cancer 1960;13:932-50. 2. Chung EB. Pitfalls in diagnosing benign soft tissue tumors in infancy and childhood.Pathol Annu 1985;20:323-86. 3. Castleberry RP, Kelly DR, Wilson ER, et al. Childhood liposarcoma: report of a caseand review of the literature. Cancer 1984;54:579-84. 4. La Quaglia MP, Spiro SA, Ghavimi F, et al. Liposarcoma in patients younger than orequal to 22 years of age. Cancer 1993;72:3114-19.5. Ferrari A, Casanova M, Spreafico F, et al. Childhood liposarcoma: a single institu-tional twenty year experience. Pediatr Hematol Oncol 1999;16:415-21.6. Schmookler BM, Enzinger FM. Liposarcoma occurring in children. An analysis of17 cases and review of the literature. Cancer 1983; 52:567-74. 8. Knowles CHR, Huggill PH. Liposarcoma: report of a case in a child. PatholBacteriol 1954;68:235-45. 9. Kauffman SL, Stout AP. Lipoblastic tumors of children. Cancer 1959;12:912-25. 10. Ali SZ, Teichberg S, Kahn E, et al. Intrathoracic pleomorpic liposarcoma in achild: study of an unusual case. Pediatr Pathol Lab Med 1996;16:99-105. 11. Sztaba R, Judycki W. Liposarcoma in children. Pediatr Pol 1981;56:655-61. 12. Hashimoto H, Daimaru Y, Enjoji M. S 100 protein distribution in liposarco-ma: an immuno peroxidase study with special reference to the distinction ofliposarcoma from myxoid malignant fibrous histio cytoma. Virchows Arch APathol Anat Histopathol 1984;405:1-10. 

Pleomorphic liposarcoma in a ten year old child

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Pleomorphic liposarcoma in a ten year old child

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