Rare Occurrence of an Intraocular Choroidal Solitary Fibrous Tumor/Hemangiopericytoma

Lorenzo Rinaldo, Sarah Chao Ying Xu, Scott Daniel Eggers, Diva R. Salomão, John Chen, Aditya Raghunathan

Research output: Contribution to journalArticle

Abstract

Purpose: Tumors previously diagnosed as solitary fibrous tumors (SFT) and hemangiopericytomas (HPC) are characterized by the NAB2-STAT6 fusion gene, leading to nuclear STAT6 expression, and are now considered part of one SFT/HPC tumor entity by the 2016 World Health Organization Classification of Tumors of the Central Nervous System. We present the first primary choroidal SFT/HPC with the diagnosis confirmed by STAT6 expression. Procedures: A 51-year-old man underwent enucleation for a choroidal mass, which revealed a spindle cell neoplasm involving the optic nerve, without extrascleral extension. Immunohistochemical stains for S-100, melan-A, tyrosinase, and HMB45 were all negative; however, detection of monosomy 3 by FISH favored a choroidal spindle cell melanoma. Four years later, he presented with hepatic metastases of a spindle cell tumor, and a year later with an epithelioid malignancy involving the calvarium. Results: The calvarial tumor showed nuclear STAT6 immunoreactivity, supporting the diagnosis of SFT/HPC. Retrospectively, the choroidal and hepatic masses were also found to demonstrate nuclear STAT6 expression, supporting the diagnosis of a primary choroidal SFT/HPC with metachronous metastases to the liver and calvarium. Conclusions: This case highlights the significance of considering SFT/HPC in the diagnosis of intraocular spindle cell tumors and the importance of STAT6 immunohistochemistry in the evaluation of such tumors.

Original languageEnglish (US)
Pages (from-to)213-219
Number of pages7
JournalOcular Oncology and Pathology
Volume4
Issue number4
DOIs
StatePublished - Jun 1 2018

Fingerprint

Solitary Fibrous Tumors
Hemangiopericytoma
Neoplasms
Optic Nerve Neoplasms
Skull
Liver
MART-1 Antigen
Neoplasm Metastasis
Monosomy
Central Nervous System Neoplasms
Monophenol Monooxygenase
Gene Fusion
Melanoma
Coloring Agents
Immunohistochemistry

Keywords

  • Choroidal lesion
  • Hemangiopericytoma
  • Intraocular tumor
  • NAB2-STAT6
  • Solitary fibrous tumor

ASJC Scopus subject areas

  • Nursing(all)

Cite this

Rare Occurrence of an Intraocular Choroidal Solitary Fibrous Tumor/Hemangiopericytoma. / Rinaldo, Lorenzo; Xu, Sarah Chao Ying; Eggers, Scott Daniel; Salomão, Diva R.; Chen, John; Raghunathan, Aditya.

In: Ocular Oncology and Pathology, Vol. 4, No. 4, 01.06.2018, p. 213-219.

Research output: Contribution to journalArticle

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AB - Purpose: Tumors previously diagnosed as solitary fibrous tumors (SFT) and hemangiopericytomas (HPC) are characterized by the NAB2-STAT6 fusion gene, leading to nuclear STAT6 expression, and are now considered part of one SFT/HPC tumor entity by the 2016 World Health Organization Classification of Tumors of the Central Nervous System. We present the first primary choroidal SFT/HPC with the diagnosis confirmed by STAT6 expression. Procedures: A 51-year-old man underwent enucleation for a choroidal mass, which revealed a spindle cell neoplasm involving the optic nerve, without extrascleral extension. Immunohistochemical stains for S-100, melan-A, tyrosinase, and HMB45 were all negative; however, detection of monosomy 3 by FISH favored a choroidal spindle cell melanoma. Four years later, he presented with hepatic metastases of a spindle cell tumor, and a year later with an epithelioid malignancy involving the calvarium. Results: The calvarial tumor showed nuclear STAT6 immunoreactivity, supporting the diagnosis of SFT/HPC. Retrospectively, the choroidal and hepatic masses were also found to demonstrate nuclear STAT6 expression, supporting the diagnosis of a primary choroidal SFT/HPC with metachronous metastases to the liver and calvarium. Conclusions: This case highlights the significance of considering SFT/HPC in the diagnosis of intraocular spindle cell tumors and the importance of STAT6 immunohistochemistry in the evaluation of such tumors.

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