J Korean Med Sci.  2006 Dec;21(6):1136-1138. 10.3346/jkms.2006.21.6.1136.

Schwannomatosis Involving Peripheral Nerves: A Case Report

• Kim DH
• Hwang JH
• Park ST
• Shin JH

• KMID: 1713136
• DOI: http://doi.org/10.3346/jkms.2006.21.6.1136

Abstract

Schwannomatosis or neurilemmomatosis has been used to describe patients with multiple nonvestibular schwannomas with no other stigmata of neurofibromatosis type-2 (NF-2). In our case, schwannomatosis, multiple schwannomas were present in a 21-yr-old woman with no stigmata or family history of NF-1 or NF-2. She had no evidence of vestibular schwannoma or other intracranial tumors. Multiple peripheral tumors were found in the carotid space of the neck, and soft tissue of posterior shoulder, lower back, ankle and middle mediastinum. All of those tumors were completely limited to the right side of the body. All surgically removed tumor specimens in this patient proved to be schwannomas.

Keyword

Peripheral Nerves; Peripheral Nervous System Neoplasms; Neuromas; Neurilemmoma

MeSH Terms

Treatment Outcome
Peripheral Nervous System Neoplasms/*diagnosis/*surgery
Neurilemmoma/*diagnosis/*surgery
Humans
Female
Adult

Figure

• 

  Fig. 1 A 21-yr-old woman with schwannomatosis. (A) Enhanced axial T1-weighted MR image shows a well enhanced mass (arrow) in right carotid space of the neck. (B) Enhanced axial T1-weighted MR image shows another small enhanced mass (arrow) in the right psoas muscle of L2 level. (C) Contrast-enhanced chest CT scan shows well-defined and lobulated slightly enhancing mass (arrow) in the mediastinum right lateral to the heart. (D) Sagittal T1-weighted MR image of the right ankle shows well-circumscribed hypointense mass (arrow) below the medial malleolus (asterisk). (E) Enhanced coronal T1-weighted MR image of the right ankle represents an enhancing mass (arrow) located below the medial malleolus. (F) Histological appearance of tumor in the right carotid space. The tumor reveals schwannoma consisting of Antoni A containing crowded spindle cells with palisading nuclei (arrow) and Antoni B containing loosely arranged spindle cells in abundant myxomatous matrix (asterisk) areas. Tumor tissue was counterstained with hematoxylin and eosin (×400).

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