Clinicopathological features of peripheral nerve sheath tumors involving the eye and ocular adnexa

Mingjuan L. Zhang, Maria J. Suarez, Thomas Bosley, Fausto J Rodriguez

Research output: Contribution to journalArticle

Abstract

Peripheral nerve sheath tumors (PNSTs) are known to occur in the orbit and comprise 4% of all orbital tumors, but have not been well studied in contemporary literature. Ninety specimens involving the eye and ocular adnexa (1979-2015) from 67 patients were studied. The mean age was 32.5 years. Locations included orbit (58.9%), eyelid (60.0%), and other ocular adnexa. Most specimens were neurofibromas (70.0%), followed by schwannomas (11.1%), neuromas (11.1%), granular cell tumors (n = 4), nerve sheath myxomas (n = 2), and malignant PNST (n = 1). Fifty-six (88.9%) neurofibroma cases were neurofibromatosis 1 associated. Among neurofibromas, 31.7% were localized, 38.1% were plexiform, 25.4% were diffuse, and 4.8% were diffuse and plexiform. These tumors involved skin (31.7%), soft tissue (11.1%), skeletal muscle (22.2%), peripheral nerve (63.0%), lacrimal gland (20.6%), and choroid (n = 1). Other histologic findings included pseudo-Meissner corpuscles (27%), Schwann cell nodules (4.8%), prominent myxoid component (7.9%), melanin-like pigment (3.2%), and inflammation (14.3%). Available immunostains included S100 (+ in 15/15 cases), EMA (+ in 2/4 cases), CD34 (+ in 4/4 cases), and Ki-67 (<1% in 4/4 cases). Among 10 schwannomas, 8 were conventional and 2 were plexiform. Observed features included capsule (n = 5), hyalinized vessels (n = 5), Verocay bodies (n = 7), and Antoni B pattern (n = 5). Immunostaining included S100+ in 4 of 4 cases, and collagen IV+ and Ki-67 < 1% in 3 of 3 cases. Neurofibromas are the most common PNST involving the eye and ocular adnexa, and the majority are associated with neurofibromatosis 1. Plexiform and diffuse patterns and the presence of pseudo-Meissner corpuscles are relatively frequent in this area.

Original languageEnglish (US)
Pages (from-to)70-78
Number of pages9
JournalHuman Pathology
Volume63
DOIs
StatePublished - May 1 2017

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Nerve Sheath Neoplasms
Neurofibroma
Neurilemmoma
Mechanoreceptors
Neurofibromatosis 1
Orbit
Neurothekeoma
Granular Cell Tumor
Literature
Neuroma
Lacrimal Apparatus
Choroid
Schwann Cells
Melanins
Eyelids
Peripheral Nerves
Capsules
Neoplasms
Skeletal Muscle
Collagen

Keywords

  • Neurofibroma
  • Ocular
  • Orbit
  • Peripheral nerve sheath tumor
  • Schwannoma

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Clinicopathological features of peripheral nerve sheath tumors involving the eye and ocular adnexa. / Zhang, Mingjuan L.; Suarez, Maria J.; Bosley, Thomas; Rodriguez, Fausto J.

In: Human Pathology, Vol. 63, 01.05.2017, p. 70-78.

Research output: Contribution to journalArticle

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abstract = "Peripheral nerve sheath tumors (PNSTs) are known to occur in the orbit and comprise 4{\%} of all orbital tumors, but have not been well studied in contemporary literature. Ninety specimens involving the eye and ocular adnexa (1979-2015) from 67 patients were studied. The mean age was 32.5 years. Locations included orbit (58.9{\%}), eyelid (60.0{\%}), and other ocular adnexa. Most specimens were neurofibromas (70.0{\%}), followed by schwannomas (11.1{\%}), neuromas (11.1{\%}), granular cell tumors (n = 4), nerve sheath myxomas (n = 2), and malignant PNST (n = 1). Fifty-six (88.9{\%}) neurofibroma cases were neurofibromatosis 1 associated. Among neurofibromas, 31.7{\%} were localized, 38.1{\%} were plexiform, 25.4{\%} were diffuse, and 4.8{\%} were diffuse and plexiform. These tumors involved skin (31.7{\%}), soft tissue (11.1{\%}), skeletal muscle (22.2{\%}), peripheral nerve (63.0{\%}), lacrimal gland (20.6{\%}), and choroid (n = 1). Other histologic findings included pseudo-Meissner corpuscles (27{\%}), Schwann cell nodules (4.8{\%}), prominent myxoid component (7.9{\%}), melanin-like pigment (3.2{\%}), and inflammation (14.3{\%}). Available immunostains included S100 (+ in 15/15 cases), EMA (+ in 2/4 cases), CD34 (+ in 4/4 cases), and Ki-67 (<1{\%} in 4/4 cases). Among 10 schwannomas, 8 were conventional and 2 were plexiform. Observed features included capsule (n = 5), hyalinized vessels (n = 5), Verocay bodies (n = 7), and Antoni B pattern (n = 5). Immunostaining included S100+ in 4 of 4 cases, and collagen IV+ and Ki-67 < 1{\%} in 3 of 3 cases. Neurofibromas are the most common PNST involving the eye and ocular adnexa, and the majority are associated with neurofibromatosis 1. Plexiform and diffuse patterns and the presence of pseudo-Meissner corpuscles are relatively frequent in this area.",
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