Background: Pleomorphic hyalinizing angiectatic tumor (PHAT) and hemosiderotic fibrolipomatous tumor (HFLT) are low-grade neoplasms that share clinicopathologic features and recurring translocation t(1;10)(p22;q24) involving the TGFBR3 and MGEA5 genes. Coexistence of these tumors with a high-grade sarcoma is exceedingly rare and the cytologic features have not been widely described in the literature. Case: A 55-year-old female presented with a soft tissue tumor on the dorsum of the foot. Cytologic smears and corresponding core biopsies were composed of a population of markedly pleomorphic spindle cells seen singly and in loose clusters within a myxofibrous matrix and infiltrating fat, with coarse chromatin, prominent nucleoli, irregular nuclear contours and delicate to vacuolated cytoplasm. Intracytoplasmic hemosiderin granules and rare intranuclear cytoplasmic pseudoinclusions were identified. The histologic features of the excisional biopsy mirrored those of the cytologic preparations, but also demonstrated cellular foci of higher-grade sarcoma composed of markedly pleomorphic tumor cells with large vesicular nuclei and prominent nucleoli, exhibiting a mitotic index of 12 mitotic figures per 10 high-powered fields. Conclusion: While HFLT/PHAT generally can be managed by wide local excision, it is important to be aware of their capacity to harbor higher-grade lesions with metastatic potential which may require more radical surgical excision.

Keywords:
Pleomorphic hyalinizing angiectatic tumor, Hemosiderotic fibrolipomatous tumor, Pleomorphic undifferentiated sarcoma, Cytology, Histology
1.
Carter JM, Sukov WR, Montgomery E, Goldblum JR, Billings SD, Fritchie KJ, et al: TGFBR3 and MGEA5 rearrangements in pleomorphic hyalinizing angiectatic tumors and the spectrum of related neoplasms. Am J Surg Pathol 2014;38:1182-1992.
2.
Smith ME, Fisher C, Weiss SW: Pleomorphic hyalinizing angiectatic tumor of soft parts: a low-grade neoplasm resembling neurilemoma. Am J Surg Pathol 1996;20:21-29.
3.
Marshall-Taylor C, Fanburg-Smith JC: Hemosiderotic fibrohistiocytic lipomatous lesion: ten cases of a previously undescribed fatty lesion of the foot/ankle. Mod Pathol 2000;13:1192-1199.
4.
Kazakov DV, Pavlovsky M, Mukensnabl P, Michal M: Pleomorphic hyalinizing angiectatic tumor with a sarcomatous component recurring as high-grade myxofibrosarcoma. Pathol Int 2007;57:281-284.
5.
Elco CP, Marino-Enriquez A, Abraham JA, Dal Cin P, Hornick JL: Hybrid myxoinflammatory fibroblastic sarcoma/hemosiderotic fibrolipomatous tumor: report of a case providing further evidence for a pathogenetic link. Am J Surg Pathol 2010;34:1723-1727.
6.
Solomon DA, Antonescu CR, Link TM, O'Donnell RJ, Folpe AL, Horvai AE: Hemosiderotic fibrolipomatous tumor, not an entirely benign entity. Am J Surg Pathol 2013;37:1627-1630.
7.
Zreik RT, Carter JM, Sukov WR, Ahrens WA, Fritchie K, Montgomery EA, Weiss SW, Folpe AL: Myxoinflammatory fibroblastic sarcoma (MIFS) and hybrid hemosiderotic fibrolipomatous tumor (HFLT)/MIFS: related or not? Mod Pathol 2015;28(suppl 2):30A.
8.
Illueca C, Machado I, Cruz J, Almenar S, Noguera R, Navarro S, et al: Pleomorphic hyalinizing angiectatic tumor: a report of 3 new cases, 1 with sarcomatous myxofibrosarcoma component and another with unreported soft tissue palpebral location. Appl Immunohistochem Mol Morphol 2012;20:96-101.
9.
Yorita K, Ishihara A, Tokumitsu T, Minematsu E, Ohno A, Ikejiri H, et al: A case of a pleomorphic hyalinizing angiectatic tumor of soft parts with intracytoplasmic hemosiderin pigment apparent upon fine-needle aspiration cytology. Diagn Cytopathol 2015;43:407-411.
10.
Lin O, Crapanzano JP: Fine-needle aspiration cytology of pleomorphic hyalinizing angectatic tumor: a case report. Diagn Cytopathol 2005;32:238-242.
11.
Cimino-Mathews A, Ali SZ: Pleomorphic hyalinizing angiectatic tumor: cytomorphologic features on fine-needle aspiration. Diagn Cytopathol 2011;39:214-217.
© 2016 S. Karger AG, Basel
2016
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