Choroidal Metastasis as the Initial Manifestation of a Pigmented Neuroendocrine Tumor

Abstract

W e report the case of a 77-year-old woman in whom choroidal metastasis was the initial manifestation of a primary neoplasm presumed to be a pigmented pulmonary carcinoid tumor. The tumor initially was misdiagnosed cytologically and pathologically as a choroidal melanoma because it contained intrinsic melanin pigment. Positive immunoreactivity for cytokeratin, synaptophysin, chromogranin, and calcitonin and the presence of dense-core neurosecretory vesicles disclosed by electron microscopy established that the metastasis was a neuroendocrine tumor. Findings from systemic evaluation suggested that the primary tumor was located in the lung. The patient subsequently developed an intradural paraspinal metastasis, which also contained melanin pigment. The latter observation confirmed that the melanin in the uveal metastasis was intrinsic and did not represent secondary phagocytosis by tumor cells. Metastases from pigmented tumors of nonmelanocytic derivation are exceedingly rare but present a major diagnostic challenge to ocular pathologists and cytopathologists if the diagnosis is not suspected. Confirmatory immunohistochemical analysis should be obtained when a pigmented choroidal tumor thought to be a melanoma has atypical features.

Fine Needle Aspiration Biopsy with Adjunct Immunohistochemistry in Intraocular Tumor Management

“…Neuroendocrine tumors metastatic to the eye usually originate in the lung, and, as in this patient, the intraocular metastasis may be detected before the primary tumor. 38,39 Approximately onethird of patients presenting with intraocular metastases do not have a history of cancer. 36 One patient presented with a unilateral cataract and a heavily pigmented ciliary body mass, presumed to be melanoma.…”

“…46 There have been few subsequent reports of IHC applied to material from intraocular FNABs. 11,12,39,[47][48][49][50] The most systematic use of IHC on intraocular FNAB reported in the literature was by Eide et al, 12 who performed IHC on cytospins from over half the patients in their series.…”

Metastatic Small-Cell Neuroendocrine Carcinoma Simulating Circumscribed Choroidal Hemangioma

“…We are aware of only 3 prior case reports, all of which differ from ours in that the lesions were pigmented [2] , multiple and bilateral [3] or associated with a known primary carcinoma [4] . Most choroidal metastases are unifocal, and in one third of the cases, there is no known history of cancer at presentation [5] .…”

“…Neuroendocrine carcinoma metastatic to the choroid is extremely rare [2][3][4] . We are aware of only 3 prior case reports, all of which differ from ours in that the lesions were pigmented [2] , multiple and bilateral [3] or associated with a known primary carcinoma [4] .…”

Neuroendocrine tumours metastatic to the uvea: diagnosis by fine needle aspiration biopsy

“…These tumours can metastasise to various bodily sites, including the uvea. Uveal metastasis from a neuroendocrine tumour is rare [8] and can simulate other primary or metastatic uveal tumours, both clinically and cytomorphologically [6]. We describe four cases of uveal metastasis from a neuroendocrine tumour diagnosed after fine needle aspiration biopsy (FNAB) and cytomorphological-immunohistochemical analysis of the aspirates obtained.…”