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

Affiliations
  • 1Department of Neurosurgery, Hanyang University Guri Hospital, Guri, Korea. kch5142@hanyang.ac.kr

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.

  • 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).


Reference

1. Akimoto J, Fukami S, Hashimoto R, Haraoka J. Neuromuscular hamartoma is a possible primary pathology of oculomotor ophthalmoplegic migraine. Cephalalgia. 2012; 32:171–174. PMID: 22174363.
Article
2. Amita K, Shankar SV, Nischal KC, Basavaraj HB. Benign triton tumor : a rare entity in head and neck region. Korean J Pathol. 2013; 47:74–76. PMID: 23483800.
Article
3. Bonneau R, Brochu P. Neuromuscular choristoma. A clinicopathologic study of two cases. Am J Surg Pathol. 1983; 7:521–528. PMID: 6625049.
Article
4. Castro DE, Raghuram K, Phillips CD. Benign triton tumor of the trigeminal nerve. AJNR Am J Neuroradiol. 2005; 26:967–969. PMID: 15814954.
5. Daley TD, Darling MR, Wehrli B. Benign Triton tumor of the tongue. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2008; 105:763–766. PMID: 18299216.
Article
6. Demir Y, Uluoglu O, Ozmen S, Boyacioglu ZM, Atabay K. Neuromuscular hamartoma in the mental region. J Oral Maxillofac Surg. 2003; 61:397–400. PMID: 12618984.
Article
7. Louhimo I, Rapola J. Intraneural muscular hamartoma : report of two cases in small children. J Pediatr Surg. 1972; 7:696–699. PMID: 4635526.
8. Markel SF, Enzinger FM. Neuromuscular hamartoma--a benign "triton tumor" composed of mature neural and striated muscle elements. Cancer. 1982; 49:140–144. PMID: 7053811.
Article
9. Masson P. Tumeurs Humaines : Histologie, Diagnostics et Techniques. ed 2. Paris: Maloine;1956. p. 973–975.
10. Moore KL, Persaud TVN. The Developing Human : Clinically Oriented Embryology. ed 5. Philadelphia, PA: Saunders;1993. p. 375–423.
11. O'Connell JX, Rosenberg AE. Multiple cutaneous neuromuscular choristomas. Report of a case and a review of the literature. Am J Surg Pathol. 1990; 14:93–96. PMID: 2403760.
12. Oeppen RS, Harden SP, Argent JD. Neuromuscular hamartoma : imaging features of a rare paediatric craniofacial tumour. Pediatr Radiol. 2003; 33:50–52. PMID: 12497240.
Article
13. Tiffee JC, Barnes EL. Neuromuscular hamartomas of the head and neck. Arch Otolaryngol Head Neck Surg. 1998; 124:212–216. PMID: 9485116.
Article
14. Tobias S, Kim CH, Sade B, Staugaitis SM, Lee JH. Neuromuscular hamartoma of the trigeminal nerve in an adult. Acta Neurochir (Wien). 2006; 148:83–87. discussion 87. PMID: 16228126.
Article
15. Vajramani G, Devi I, Santosh V, Hegde T, Das BS, Das S, et al. Benign triton tumor of the trigeminal nerve. Childs Nerv Syst. 1999; 15:140–144. PMID: 10230673.
Article
16. Weiss SW, Goldblum JR, Enzinger FM. Enzinger and Weiss' Soft Tissue Tumors. ed 5. Philadelphia, PA: Mosby Elsevier;2008. p. 834p. 917–919.
Full Text Links
  • JKNS
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr