J Korean Neurosurg Soc.  2014 Aug;56(2):146-148. 10.3340/jkns.2014.56.2.146.

Adulthood Benign Triton Tumor Developed in the Orbit

• Bae DH
• Kim CH
• Cheong JH
• Kim JM

• KMID: 1956518
• DOI: http://doi.org/10.3340/jkns.2014.56.2.146

Abstract

Benign triton tumor (BTT) or neuromuscular hamartoma is an uncommon tumor composed of mature neural and well-differentiated striated muscular elements. Its development is exceptionally rare in the adult and head region. This report describes a case of adulthood BTT that occurred in the orbit. The patient was a 53-year-old woman who presented with right periorbital swelling and pain in eyeball over 2 months. Magnetic resonance imaging revealed a well-enhancing mass surrounding optic nerve and ocular muscles in the right retrobulbar area. The tumor was subtotally removed via transcranial approach. Its pathological diagnosis was confirmed to be a neuromuscular hamartoma. She developed diplopia postoperatively. Adulthood BTT should be considered in the differential diagnosis of head and neck tumors. It is also important to make adequate therapeutic strategy to avoid postoperative neural dysfunction.

Keyword

Adult; Benign triton tumor; Head; Neuromuscular hamartoma; Surgery

MeSH Terms

Adult
Diagnosis
Diagnosis, Differential
Diplopia
Female
Hamartoma
Head
Humans
Magnetic Resonance Imaging
Middle Aged
Muscles
Neck
Neptune*
Optic Nerve
Orbit*

Figure

• 

  Fig. 1 Preoperative orbital enhanced magnetic resonance (MR) images. A : Axial MR image showing a well-enhanced mass in the medial portion of right orbit. B : Coronal MR image demonstrating a well-enhanced mass in the supero-medial portion of right orbit.

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  Fig. 2 Photomicrograghs of the resected lesion. A : H & E staining shows nerve fibers admixed with skeletal muscle (×200). B : Immunohistochemical staining reveals diffuse immunoreactivity to S-100 protein suggesting neural elements (×200). C : Luxol-fast blue staining demonstrates dark bluish colored muscle fibers in the striated fashion (×200).

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