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Editor,—Epithelioid haemangioma (EH) is the preferred name used to describe a rare primary, neoplastic disorder of vascular endothelium with a variable and secondary inflammatory response.1 It is part of a spectrum of epithelioid vascular tumours and has been reported under a number terms, in particular angiolymphoid hyperplasia with eosinophilia. The term Kimura’s disease has been used interchangeably with these terms but this should now be considered a separate disorder.23 We report a case in which EH involved the lacrimal gland in isolation, without systemic eosinophilia or other features of Kimura’s disease.
A 77-year-old white woman with no previous medical history presented with a 1 year history of fullness in the right orbit and gradual right ptosis. She had developed epiphora 6 months before presentation and reported a non-specific visual disturbance in the same eye.
On examination corrected visual acuity was 6/9 right and 6/6 left. There was a 3 mm right non-axial proptosis with 2 mm of globe depression but no diplopia. A firm, non-tender mass was palpable in the lateral aspect of the right upper lid with a 1 mm ptosis. Ocular examination was otherwise normal.
A computed tomography scan of the orbit (Fig 1) showed a well defined homogeneous mass enlarging the right lacrimal gland, with no evidence of bone erosion or deformity of the adjacent globe. The left lacrimal gland was normal. The appearance was of a benign lacrimal tumour, most likely a pleomorphic adenoma. Routine haematological investigation was normal, with a white cell count of 5.2 × 109/l and 1.9% eosinophils.
A right lateral orbitotomy was performed to improve surgical access and the lacrimal gland was excised with a margin of normal tissue. The proptosis resolved postoperatively; however, a 2 mm ptosis remained and she experienced diplopia on extreme right gaze because of right lateral rectus underaction.
Histological examination of the 25 mm mass showed a well circumscribed lesion with the typical features of EH (Fig 2A and B). Multiple thick walled vascular channels with plump endothelial cells were associated with an infiltrate of inflammatory cells, particularly eosinophils and plasma cells. The endothelial cells did not show cytological atypia, and mitoses were not evident. Lymphoid follicles were present in the periphery of the lesion.
Epithelioid haemangioma reported under the term angiolymphoid hyperplasia with eosinophilia (ALHE) is an exceptional entity in the orbit,2 and usually involves the skin of the head and neck.4 The differential diagnosis includes angiosarcoma, eosinophilic granuloma, and granuloma faciale.25 Because of its benign nature its management is by local excision alone.5 Recurrences have been treated with radiotherapy.2
Epithelioid haemangioma shows some histological features with Kimura’s disease. Both have an inflammatory infiltrate of eosinophils, lymphocytes, and plasma cells, often in association with lymphoid follicles. However, the plump epithelioid cells that characterise EH are not found in Kimura’s disease which also tends to show more fibrosis and to contain eosinophil microabscesses. Kimura’s disease usually affects young Asian males, is associated with eosinophilia, and shows features of an autoimmune disorder. EH and Kimura’s disease should therefore be considered separate clinicopathological entities.23
Epithelioid haemangioma, under the term ALHE, has been previously reported in the lacrimal gland.67 However, these two cases were of young patients who had features of Kimura’s disease and their initial presentation was with non-orbital involvement and later lacrimal gland enlargement. Both patients, one of Asian descent and the other Afro-Caribbean, had raised serum eosinophil counts. Our reported case of EH involves the lacrimal gland in isolation and affects an elderly white female without systemic eosinophilia or other characteristics of Kimura’s disease.