Sir,

We describe a case of choroidal carcinoid metastases in a young patient and briefly address the considerations and controversy involved in selection of appropriate therapy.

Case report

A systemically well, 22-year-old female presented with blurred vision OS. Visual acuities (VA) were 6/5 OD and 6/6 OS. Anterior segment evaluation was normal OU.

Posterior segment examination confirmed a single, large, amelanotic, choroidal mass OS (6.8 mm ultrasound thickness) (Figure 1a) and two amelanotic choroidal lesions OD measuring 1.8 and 1.2 mm thickness. Systemic work-up revealed a solid mediastinal mass (Figure 2).

Figure 1
figure 1

(a) Wide-field fundus photograph OS at initial assessment. (b) Wide-field fundus photograph OS post treatment.

Figure 2
figure 2

CT thorax demonstrating mediastinal mass.

Fine needle biopsy of the choroidal lesion OS provided insufficient aspirate for diagnosis. Biopsy of the mediastinal lesion demonstrated typical bronchial carcinoid of low proliferative index (Ki-67<2%). Octreotide scintigraphy was performed 1 month following initial assessment. A functioning neuroendocrine tumour (NET) at the site of the right hilar mass (Figure 3a) in addition to a further site of active disease in the left sphenoid bone (Figure 3b) were demonstrated. Management decisions were made in conjunction with oncology and endocrinology services.

Figure 3
figure 3

Octreotide scan demonstrating mediastinal (a) and sphenoid (b) lesions.

Intraretinal fluid accumulation at the left macula caused a reduction in VA to 6/18 OS 8 months following presentation. Neither intravitreal bevacizumab (1.25 mg in 0.05 ml) nor subsequent systemic treatment reduced this fluid.

At 13 months following diagnosis, four targeted (177) Lutetium-DOTA-octreotate treatments were administered over a 7-month period in a Swedish endocrine oncology unit. This was followed by a reduction from 6.2 to 5.9 cm in primary bronchial tumour diameter and from 10.4 to 8 mm in the left choroidal metastasis (Figure 1b). There were no changes in the size of the choroidal metastases OD. At 39 months follow-up, VA was 6/5 OD, 6/30 OS and no new systemic or choroidal lesions had developed.

Comment

Response of choroidal carcinoid metastases to radiolabelled somatostatin analogues has not been described previously. A recent report of metastatic conjunctival carcinoid catalogued relentless tumour progression despite use of radiopharmaceuticals.1 However, in a large case series of metastatic NETs, endoradiotherapy was shown to prolong the lives of treatment-responsive patients.2 Haematological toxicities coupled with nephrotoxicity, which increases with successive treatments and over time, have the potential to limit the scope for retreatment.2 External radiotherapy, reserved in this case for progressive disease, has also been used for surgically inaccessible NETs.3 Reports of long-term stability without treatment mean that choosing to observe rather than intervene is also a viable option in managing carcinoid metastatic to the choroid.3, 4