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Am J Ophthalmol. 2019 May 9. pii: S0002-9394(19)30208-9. doi: 10.1016/j.ajo.2019.04.027. [Epub ahead of print]

CONJUNCTIVAL MYXOID LESIONS: CLINICAL-PATHOLOGIC MULTIPARAMETRIC ANALYSIS, INCLUDING MOLECULAR GENETICS (AN AMERICAN OPHTHALMOLOGICAL SOCIETY THESIS).

Author information

1
Department of Ophthalmology, Thomas Jefferson University, Philadelphia, PA, USA; Department of Pathology, Wills Eye Hospital, Philadelphia, PA, USA. Electronic address: tmilman@willseye.org.
2
Department of Laboratory Medicine and Pathology, Mayo Clinic, Mayo Clinic College of Medicine and Science, Rochester, MN, USA.
3
Orbit and Oculoplastics Department, Hospital de la Luz, Mexico City, Mexico.
4
Department of Ophthalmology, Ocular Oncology and Pathology Section, Emory Eye Center, Emory University School of Medicine, Atlanta, GA.
5
Department of Ophthalmology, Thomas Jefferson University, Philadelphia, PA, USA; Biostatistics Consulting Core, Vickie and Jack Farber Vision Research Center, Wills Eye Hospital, Philadelphia, PA.
6
Department of Ophthalmology, Thomas Jefferson University, Philadelphia, PA, USA; Ocular Oncology Service, Wills Eye Hospital, Philadelphia, PA, USA.
7
Department of Ophthalmology, Thomas Jefferson University, Philadelphia, PA, USA; Cornea Service, Wills Eye Hospital, Philadelphia, PA, USA.
8
Department of Ophthalmology, Thomas Jefferson University, Philadelphia, PA, USA; Department of Pathology, Wills Eye Hospital, Philadelphia, PA, USA.

Abstract

PURPOSE:

To evaluate the clinical and pathologic characteristics of conjunctival myxoid lesions, with specific focus on PRKAR1A studies, in order to distinguish neoplastic conjunctival myxoma from other myxoid conjunctival lesions.

METHODS:

A retrospective, interventional, multicenter study of all patients diagnosed with conjunctival myxoma, conjunctival stromal tumor, and reactive fibromyxoid proliferation between 1988 and 2018. Patient medical and family histories and clinical and pathologic characteristics of excised lesions were assessed.

RESULTS:

There were 28 patients with conjunctival myxoid lesions diagnosed as "myxoma" (16/28), "conjunctival stromal tumor" (10/28), and "reactive fibromyxoid proliferation" (2/28). Lesions with abundant myxoid matrix (14/28, 50%) occurred in younger patients (mean 49 years, range 23-68) compared to 14 lesions with scant-to-moderate myxoid matrix (mean 61, range 18-82; p=0.04), more likely contained predominantly stellate cells (6/14, 43% vs. 0/14, 0%; p=0.05) and fibrillar collagen (13/14, 93% vs. 2/14, 14%; p<0.0001), conforming to the traditional morphologic definition of myxoma. Absence of PRKAR1A protein expression was found in 2 lesions with morphologic features of myxoma (2/14, 14%), one of which demonstrated a pathogenic mutation in PRKAR1A gene. There was no difference between the lesions with respect to other clinical and pathologic parameters.

CONCLUSIONS:

PRKAR1A plays a role in the development of a subset of conjunctival myxomas, particularly in tumors fulfilling stringent morphologic criteria for myxoma. With the exception of PRKAR1A studies, current immunohistochemical panels cannot reliably distinguish between the neoplastic conjunctival myxoma and other myxoid lesions, underscoring the importance of morphology in establishing accurate diagnosis.

KEYWORDS:

COST; Carney complex; Conjunctiva; PRKAR1A; conjunctival fibromyxoid tumor; conjunctival myxoid stromal tumor; conjunctival myxoma; conjunctival stromal tumor; myxoma

PMID:
31078543
DOI:
10.1016/j.ajo.2019.04.027

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