Recurrent polymorphous haemangioendothelioma

Sir: A 43-year-old woman was seen in May 1996 with a 2-month history of recurrent abdominal pain with lumbar irradiation. In July 1995, the patient had undergone surgery for a right ovarian cyst. Computed tomography (CT) scan disclosed a pelvic mass (Figure 1a), 40 mm in diameter, and a 30-mm nodule occupying the right Scarpa’s triangle. The retroperitoneal mass was surgically removed; the inguinal nodule was excised a year later. The pelvic mass, diagnosed as intravascular papillary endothelial hyperplasia (IPEH), comprised dilatated thin-walled veins containing multiple small vascular channels and papillary structures, lined by a single layer of endothelial cells with no nuclear atypia (Figure 1b,c). Examination of the inguinal nodule revealed an enlarged lymph node, 30 mm in diameter (Figure 2a), partially replaced by a neoplastic proliferation of intimately blended, cellular, solid masses and vascular structures (Figure 2b). The solid component was formed by diffuse sheets, anastomosing trabecules and cords of fairly uniform polygonal or spindle cells, which occasionally contained cytoplasmic vacuoles. The mitotic count was less than 1 ⁄ 10 high-power fields. Vascular areas consisted of cleft-like lumina and larger vascular spaces, with some papillary formation, lined by either plump or attenuated endothelial cells. Immunohistochemical studies showed diffuse positive labelling for CD31 (Figure 2c) and vimentin, and occasional positivity for factor VIII-Rag. A negative reaction was observed for CD34 and muscle-specific actin. These findings are consistent with polymorphous haemangioendothelioma (PH). Tumour growth was entirely limited to the lymph node with no involvement of the node capsule or extranodal spread; surgical margins contained no neoplastic cells. After 7 years of follow-up the patient presented with a painful nodule in the right inguinal area which was immediately excised. Two reddish 10-mm nodules were evident, showing a histological pattern resembling that described for the previous inguinal lesion (Figure 2d). CT scan failed to demonstrate lesions elsewhere; the lesion was therefore considered to be a local recurrence of the previous PH. Eight cases of PH have been reported since Chan et al. first described this rare condition. Although the first three were associated with lesions in inguinal, axillary and supraclavicular lymph nodes, involvement of central nodes and extranodal locations has also been reported. Thus, PH should not be classed solely as a peripheral lymph node tumour. Clinical presentation may range from an incidental finding at routine radiological examination to complications due to compression of related structures; enlarged lymph nodes are the most common clinical presentation. The term ‘polymorphous’ emphasizes the wide range of histological patterns displayed by this tumour; a variable proportion of solid, primitive vascular and angiomatous components replacing the whole node are typical. The solid component in PH comprises diffuse sheets with anastomosing cords and trabecules of Figure 1. a, Computed tomography scan showing a 40-mm mass occupying the right pelvic cavity. b, Low-power view of the pelvic mass showing dilatated thin-walled veins filled with papillary structures (· 2). c, Detail showing papillary structures covered by a single layer of endothelial cells with no nuclear atypia (· 200).