Hamartome neuromusculaire de l’orbite : rapport d’un cas et discussion de la place de corticothérapie dans le traitement

Introduction: Neuromuscular choristoma (NC) is a rare tumor composed of striated muscle fibers admixed with nerve fibers that often affects large peripheral nerve trunks in children. To the best of our knowledge, this is the first reported case in the literature of a NC of the ophthalmic nerve in an adult. Case description: A 27-year-old woman presented with a 20-day history of left periorbital headache radiating to the frontotemporal region, with associated tearing, nausea, and vomiting. The examination was normal, except for hypoesthesia in the distribution of the first trigeminal nerve branch (V1). Cranial computerized tomography showed a lesion in the left orbit and cavernous sinus with widening of the superior orbital fissure. On magnetic resonance imaging, the lesion was well-defined with fusiform morphology and showed a central cystic component and peripheral enhancement. An extradural approach was performed with subtotal tumor resection. Biopsy showed proliferation of striated muscle cells admixed with nerve fascicles and Schwann cells. A pathological diagnosis of NC of V1 was determined. Six months after surgery, left hypotropia with limitation of supraduction was observed, as well as hypoesthesia and paralysis of the left forehead with ipsilateral eyebrow ptosis. The rest of the examination remained within normal limits. Conclusion: NC should be considered in the differential diagnosis of a young patient with periorbital headache and hypoesthesia in the V1 region.

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“…9 Its appearance in cranial nerves is unusual, and only six cases of a NC of cranial nerve V have been reported (Table 1). 1,2,5–8…”

Section: Discussionmentioning

confidence: 99%

“…1,6–8 In the case of NCs of cranial nerve V, two of the six cases reported (2/6, 33.3%) corresponded to adult cases, with an age range from 2 to 47 years. 1,2,5–8…”

Section: Discussionmentioning

confidence: 99%

Orbital neuromuscular choristoma of the ophthalmic nerve

Bermudez-Castellanos

Marqués-Fernández

González

et al. 2021

European Journal of Ophthalmology

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“…Finding neuromuscular hamartoma in the orbit is unusual; there have been only five reported cases [ 1 , 3 – 6 ]. After a review of the literature (Table.…”

Section: Discussionmentioning

confidence: 99%

“…Neuromuscular hamartoma, also known as neuromuscular choristoma or benign triton tumor, is a rare benign tumor with well-differentiated, mature, striated muscle and nerve fibers [ 1 ]. In most cases, it involves large nerves, such as the brachial plexus or sciatic nerve [ 2 ], and typically appears in infants and young children.…”

Section: Introductionmentioning

confidence: 99%

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Incidental Hamartoma in an elderly patient: a case report

et al. 2020

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Background: Neuromuscular hamartoma is a very rare tumor; with only five cases reported in the orbit. It often occurs in infants and young children and involves large peripheral nerves, but there has been only few reports of occurrence in the orbit and adults. Case presentation: This paper describes a 70-year-old man with an incidental orbital mass detected by an imaging test and who later developed associated symptoms. The mass was diagnosed as neuromuscular hamartoma. Superior rectus muscle recession and inferior rectus muscle resection were performed in the right eye for hypertropia secondary to postoperative inferior rectus muscle palsy. Hypertropia in the right eye and diplopia improved after surgery, and regular follow-up is underway. Conclusion: This is the first case of an incidentally detected orbital mass diagnosed by histologic examination as a neuromuscular hamartoma in an older patient whose proptosis progressed after a long period of inactivity.

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Hamartome neuromusculaire de l’orbite : rapport d’un cas et discussion de la place de corticothérapie dans le traitement

Cited by 2 publications

References 17 publications

Orbital neuromuscular choristoma of the ophthalmic nerve

Orbital neuromuscular choristoma of the ophthalmic nerve

Incidental Hamartoma in an elderly patient: a case report

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