Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Abstract
To the Editor. —Alveolar soft-part sarcoma is an uncommon neoplasm with a marked propensity for local recurrence and metastases. Primary sites include the extremities, trunk, retroperitoneum, orbit, and tongue.1 In addition, one case of primary choroidal alveolar soft-part sarcoma has been reported.2 Metastases have been documented in the lungs, brain, skeleton, and lymph nodes but have not been reported in the eye.1 We report a case of choroidal metastases of alveolar soft-part sarcoma.

https://jamanetwork.com/journals/jamaop ... act/636758


Unusual Orange-Colored Choroidal Metastases

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3691979/

INTRODUCTION

Choroidal metastases constitute the most frequent intraocular malignancy among adults, yet in practice, they are less frequently encountered than choroidal melanomas since the majority of cases have far advanced systemic metastases drawing attention away from ophthalmic screening.1,2 Nearly one-third of patients with choroidal metastases have no history of cancer and the ophthalmologist may be the first physician to detect the primary tumor.1,3

The color of these lesions may help localize the primary tumor; breast and lung cancers which constitute more than two-thirds of choroidal metastases, are typically yellowish-white in color whereas metastatic cutaneous melanoma is usually brown.2,3 Orange-colored choroidal metastases have previously been reported to occur secondary to carcinoid tumors, thyroid cancer and renal cell carcinoma.4-8 It is of importance to distinguish unifocal orange-colored metastases from a choroidal hemangioma or a highly vascular amelanotic melanoma to avoid misdiagnosis.

The objective of the current report is to describe two patients with metastatic choroidal lesions whose primary cancers were not previously reported to be associated with orange-colored choroidal metastases, and to add them to the differential diagnoses of such lesions.

Case 1

Case 2

A 38-year-old African female was referred for blurred vision of 2 weeks’ duration. With a diagnosis of alveolar soft part sarcoma of the right leg five years earlier, she had initially received chemotherapy followed by below- knee amputation of her right leg. Multiple pulmonary metastases, appearing the following year, were surgically resected from five lung lobes. Three years later, she developed brain metastases that were treated with whole brain irradiation sparing the eyes, with total dose of 3000 centiGray (cGy) in 10 fractions.

On ocular examination, visual acuity was 20/20 and 20/50 in the right and left eyes, respectively. Funduscopy revealed a single bright orange-colored choroidal mass, 3.2 mm in diameter, superotemporal to the foveola, with exudation at its base extending into the fovea (Fig. 1C).

Ultrasonography displayed a small dome- shaped choroidal mass that appeared acoustically echogenic on B-scan (Fig. 1D), with moderate to high internal reflectivity on A-scan, similar to that observed with choroidal hemangiomas.

Fluorescein angiography showed very faint, mottled fluorescence of the lesion 21 seconds after dye injection, no significant increase in later phases of the angiogram and reaching maximum fluorescence at 5 minutes and (Figures 2 D, E, and F).

These findings indicated limited vascularity of the lesion as an explanation for its bright orange color, as compared to the typical fluorescein angiography features of a circumscribed choroidal hemangioma (Figures 2 G, H, and I).

The choroidal mass was treated with 2000 cGy stereotactic irradiation delivered in 5 fractions which caused eventual shrinkage of the lesion into a gray-white gliotic sca