Lipomatous hemangiopericytoma - A rare variant of hemangiopericytoma that may be confused with liposarcoma

We describe the clinicopathologic features and biologic behavior of 16 cases of histologically benign hemangiopericytoma containing a variable amount of mature fat as an intrinsic part of the neoplasm. These so-called lipomatous hemangiopericytomas occurred primarily in men (12 men and 4 women) with a mean age of 54 years (range, 33-74 years). All occurred in deep soft tissue and had an average size of 10 cm when first detected. All were characterized by a relatively sharp border and typical histologic features of hemangiopericytomas, including oval to round cells surrounding a sinusoidal and staghorn vasculature often with perivascular hyalinization. Mature fat varied in amount but usually occupied approximately one quarter to three quarters of the area of tumor. Mitotic activity was low, with more than half the cases having no mitotic activity. Five cases showed moderate nuclear atypia. In four cases, the pericytic regions had sclerotic zones. In contrast to liposarcoma, neither lipoblasts nor isolated atypical hyperchromatic cells within mature fat, as are seen in well-differentiated liposarcoma, were present. Immunohistochemistry performed in four cases showed factor XIIIa in tumor cells and an intricate pattern of immunoreactivity around cells for type IV collagen. CD34 and smooth-muscle actins were identified in two of four cases. Follow-up in seven cases showed no recurrences or metastases within the follow-up period of 1 to 7 years. Because these lesions are located in deep soft tissue and contain large amounts of mature fat, they could be mistaken for well-differentiated liposarcomas in limited biopsy material, although the distinction is easily made in examining the entire specimen. The lipomatous hemangiopericytoma represents yet another example of a bimodal mesenchymal tumor containing mature fat and raises the question of whether a common cytogenetic abnormality can explain the emergence of two clonal populations normality can explain the in this hybrid tumor.