Statistics from Altmetric.com
Editor,—Angiomatous tumours of the ciliary body and iris are very rare. Even metastatic foci of angiosarcomas are not seen often in this location.1 This is why we are publishing our case of disseminated haemangiosarcoma with metastatic involvement of the ciliary body and the iris. The eye maintained useful function until the patient’s death 1 year after the appearance of the focus in the eye.
A 53 years old woman was admitted to our eye clinic for deterioration of vision in her left eye and headaches. A tumorous focus involving the ciliary body and the radical part of the iris was revealed both clinically by ultrasound and with magnetic resonance imaging (MRI). The tumour had grown laterally at the 3 o’clock position (see Fig 1A and appeared to have a solid structure. The diameter of its base was about 6 mm. The vision was 6/12 when the patient was first examined but quickly deteriorated to light perception only, because of the blood in the anterior chamber. After removal of the blood from the anterior chamber, the vision improved again to 6/12. The intraocular pressure was normal (18–20 mm Hg) at all times.
Haemangiosarcoma of the right breast treated by ablation and actinotherapy was noticed in the patient’s medical history. The original tumour had grown within the breast stroma. Its diameter was about 3–4 cm. The histological pattern varied from relatively well formed vascular channels surrounded by multilayering endothelium to poorly differentiated solid areas formed by poorly differentiated cells with frequent mitoses. Epithelioid differentiation was not noticed. Some areas were haemorrhagic. There were no risk factors for angiosarcoma such as previous radiotherapy2 in the patient’s history. The patient was HIV negative. Because of tumorous dissemination in the skin and brain, ocular surgery was abandoned. Removal of blood from the anterior chamber was necessary once more before the patient’s death, 1 year later; otherwise, chemotherapy remained the only suitable treatment. However, the intraocular focus stopped its macroscopical growth and even partly regressed after chemotherapy by cyclophosphamide and vincristine (compare Fig 1A and B). The eye maintained useful function with measurable vision for 1 year until the patient’s death due to haemorrhage from metastasis in the brain at the end of July 1993. Postmortem examination of the left eye confirmed histologically the haemangiosarcomatous origin of the intraocular focus.
Gross examination of the eyeball
The enucleated left eyeball measured 23 × 23 × 22 mm with 4 mm of optic nerve. The pupil was slightly oval owing to lateral pressure on the nasal side. Transillumination and opening of the globe revealed approximately half rounded infiltrate which involved the lateral part of the ciliary body (see Figs 1B and 2A). The centre of this infiltrate was at the 3 o’clock position, its vertical diameter was 5 mm and horizontal diameter was 3.5 mm. The aqueous and vitreous were clear. The anterior chamber was shallow but still maintained. The retina was attached and the other parts of the globe were without remarkable macroscopically apparent changes.
The tumorous focus seen in the figures is apparently of an angiomatous nature, and involves only the ciliary body (see Fig 2A and B). Cellular atypias, mitoses, and several haemorrhages are observable within the tumour (see Fig 2A and B). No clearly tumorous structures are present in the radical part of the iris (1 year before death there was a clinically observed and documented expansion of the tumour (see Fig 1A).There is no major growth of tumorous tissue from the focus within the ciliary body to the other surrounding structures of the eye. There is angle closure in the region of the tumour as a result of larger anterior synechiae (see Fig 2A). However, the angle of the anterior chamber remains narrow but without closure for the major part of the circumference of the anterior chamber. The other parts of the globe are without remarkable changes. Immunohistochemical positivity for factor VIII related antigen was later revealed in both primary tumour and ocular and skin metastases (particularly in the relatively more vasoformative areas), confirming the vascular nature of tumour.3 4
Angiomatous tumours of the iris and ciliary body, both primary and secondary foci, are reported generally as very rare. Even in the breast angiosarcoma is a relatively rare tumour.5 6 After histological examination, the angiosarcomatous nature of the described tumorous focus was confirmed. The rarity of this type of intraocular tumorous metastatic focus makes this case remarkable. The secondary intraocular foci of various carcinomas are reported more often.1 Some rules observed in cases of intraocular metastatic cancer are common with other histological types of malignancies. Metastatic foci in the anterior segment of the eye are reported as much more rare than in the posterior segment.1 7 On the other hand, the breast (our case) and the lung are the most frequent primary sites of the intraocular metastases.1 Median survival time of patients with metastasis to the anterior segment is only 5.4 months, which is significantly worse than with metastasis to the orbit (15.6 months) or posterior pole of the eye (7.2 months).8 The overall survival of patients with any form of angiosarcoma is very limited.9 Since survival of our patient was 1 year after diagnosis of the intraocular metastasis, chemotherapy seems to have been relatively beneficial for our patient.10
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.