Aggressive Ciliary Body Adenocarcinoma with Bilateral Lung Metastases: Histological, Molecular, Genetic and Clinical Aspects.

ABSTRACT

PURPOSE OF THE STUDY To describe the clinical and histopathological features of an aggressive ciliary body adenocarcinoma with pulmonary metastases and skull base spread. PROCEDURES AND RESULTS: A 45-year-old female patient presented with a post-traumatic phthisical eye that was eviscerated. This showed an unexpected carcinoma (positive for cytokeratins and melanocytic markers), the histological differential diagnosis for which included a primary ciliary body adenocarcinoma or a metastasis. The patient developed rapid post-surgical localized recurrence that required an orbital exenteration. This showed identical tumour to the evisceration specimen, with vascular invasion in orbital blood vessels and a contaminated orbital soft tissue margin. Staging imaging revealed multiple lung metastases, which were biopsied and shown to be a disseminated ciliary body adenocarcinoma rather than a disseminated primary lung carcinoma. The tumour spread locally to the skull base for which radiotherapy was given. Unfortunately, the patient passed away a few weeks later. CONCLUSIONS: To our knowledge, this is the first case of ciliary body adenocarcinoma with bilateral lung metastases. The malignant potential of these tumours should be considered as a possibility, and appropriate screening and staging tests should therefore be considered to guide appropriate management.