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9 March 2011 Case of the Week #199
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Case of the Week #199
This 44 year old female was initially seen at our outpatient clinic in 2005 for a skin "lump" near the dorsum and ankle of the left foot. There was no history of trauma and a local excision was done, revealing irregular, non-encapsulated, subcutaneous fibrofatty tissue. The diagnosis was soft tissue angiolipoma. Four years later, the lesion recurred (see images below).
What is your diagnosis?
Hemosiderotic fibrohistiocytic lipomatous tumor
Microscopically, the lesion is characterized by two well defined tissue components, fatty tissue and spindle cells. The spindle cells are arranged in a dissecting pattern between adipocytic lobules and along the septae (figs 1, 2). A minor focal inflammatory infiltrate is present (fig 3). Abundant hemosiderin pigment is present in the cytoplasm of the spindle cells as well in numerous macrophages located around blood vessels (figs 4, 5). The pigment is positive for the Prussian blue iron stain (fig 7). Some small sized vessels are hyalinized (fig 6). An SMA immunostain highlights prominent vessels (fig 8), but does not stain the spindled cells, which are strongly positive for CD34 (fig 9).
Hemosiderotic fibrohistiocytic lipomatous tumor was first described by Marshall-Taylor and Fanburg-Smith (1). They described a peculiar soft tissue and subcutaneous lesion composed of CD34 positive fibroblastic cells, with a prominent vascularity and abundant hemosiderin within the cytoplasm of the spindle cells and many histiocytes. An associated lipomatous component was present. Eight patients were female and two were male. The mean age was 51 years. Almost all lesions were located in the foot, in the dorsum and around the ankle. Follow up in eight patients revealed local recurrences in four (50%) over a three year period. No patients developed metastatic disease. There was a history of local trauma in seven patients with ankle distortion and two of the women reported tight-fitting, uncomfortable shoes.
Kazakov et. al. (2) reported two cases associated with venous stasis. They hypothesized that the proliferation of the spindle fibroblastic cell component along with erythrocyte extravasation and formation of hemosiderin in the adipose tissue may simply represent an exaggerated tissue response to venous stasis. Browne and Fletcher (3) reported 13 new cases, and West et al (4) emphasized the clinical location in the foot, the unique histopathological pattern and described the MRI characteristics. Recently, Wettach et al (5) found a clonal reciprocal translocation between chromosomes 1 and 10, with a further chromosomal rearrangement in chromosomes 1 and 3, supporting a neoplastic and not reactive origin for this lesion.
Hemosiderotic fibrohistiocytic lipomatous tumor is rare. In a recent review of the literature, Oliveira et al (6) found only 29 cases reported. All were biologically benign with no metastasis recorded. However, late local recurrences are frequent, and pathologists should be aware of this lesion (7).
This lesion may be related to or represent a precursor lesion of the pleomorphic hyalinizing angiectatic tumor of soft tissue, due to its vascular hyalinization (8-10).
The differential diagnosis includes several cutaneous and subcutaneous pigmented lesions of alleged fibrohistiocytic derivation: pigmented aneurysmal dermatofibroma (nodular, well localized storiform pattern), pigmented variant of dermatofibrosarcoma protuberans (called Bednar tumor, prominent cartwheel pattern, melanin pigment), Kaposi sarcoma-plaque stage (RBC extravasation, PAS positive globules, some HHV-8 nuclear positivity), and plexiform fibrous histiocytoma (mutinodular, storiform pattern, numerous multinucleated giant cells, scanty hemosiderin).
1. 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.
2. Kazakov DV, Sima R, Michall M. Hemosiderotic fibrohistiocytic lipomatous lesion. Clinical correlation with venous stasis. Virchows Arch 2005;447:103
3. Browne TJ, Fletcher CDM. Haemosiderotic fibrolipomatous tumor (so-called haemosiderotic fibrohistiocytic lipomatous tumor): analysis of 13 new cases in support of a distinct entity. Histopathology 2006;48:453
4. West AT, Toms AP, Murphy J, Sulton M. Haemosiderotic fibrohistiocytic lipomatous lesion / tumor of the foot. MRI and histopathology. Skeletal Radiol 2008;37:71
5. Wettach G, Boyd L, Lance R, et al. Cytogenetic analysis of a hemosiderotic fibrolipomatous tumor. Cancer Genetics and Cytogenetics 2008;182:140
6. Oliveira-Ramalho AR, Nara-Nunes M, Jorge-Adad S, et al. Hemosiderotic fibrohistiocytic lipomatous lesion: case report and review of the literature. Sao Paulo Med J 2009;127:174
7. Prud'homme A, Rousselot C, de Pinieux G,et al. Hemosiderotic fibrohistiocytic lipomatous lesion: a new entity you must remind. Ann Chir Plast Esthet 2007;52:616
8. Luzar B, Gasljevic G, Juricic V, Bracko M. Hemosiderotic fibrohistiocytic lipomatous lesion: early pleomorphic hyalinizing angiectatic tumor? Pathol Int 2006;56:283
9. Michal M, Kazakov DV. Relationship between pleomorphic hyalinizing angiectatic tumor and hemosiderotic fibrohistiocytic lipomatous lesion. Am J Surg Pathol 2005;29:1256
10. Folpe AL, Weiss SW. Pleomorphic hyalinizing angiectatic tumor: analysis of 41 cases supporting evolution from a distinctive precursor lesion. Am J Surg Pathol 2004;28:1417
Nat Pernick, M.D., President
and Liz Parker, B.A., Associate Medical Editor
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