Neurilemmoma (Schwannoma)

Neurilemmoma (Schwannoma)

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Neurilemmomas (neurilemomas) are benign, encapsulated tumors of the nerve sheath. Their cells of origin are thought to be Schwann cells derived from the neural crest (see the image below); accordingly, they are often referred to as schwannomas. These masses usually arise from the side of a nerve, are well encapsulated, and have a unique histologic pattern.

This benign lesion essentially manifests itself with cosmetic deformity, a palpable mass, symptoms similar to a compressive neuropathy, or some combination of these. Neurologic symptoms tend to present late. Symptoms can be vague, and there is an average interval of up to 5 years before the diagnosis is established.

The cause of these neoplasms is unknown. [1] Neurilemmoma can be associated with von Recklinghausen disease; when this is the case, multiple tumors often are present.

Neurilemmoma is the most common neurogenic tumor, but precise prevalence figures are not available. They affect persons aged 20-50 years. No racial or sex predilection is recognized. Common locations for the tumors are, in order of decreasing frequency, the head and flexor surfaces of the upper and lower extremities and the trunk.

Recurrence is unlikely after complete resection. Patients usually have rapid and complete relief of pain, with excellent long-term results. [2]

Rare descriptions exist of malignant change in long-standing neurilemmomas, usually in patients with an underlying diagnosis of neurofibromatosis. Malignant change is extremely rare in isolated lesions.

Kano et al evaluated tumor control and hearing preservation relating to tumor volume, imaging characteristics, and nerve and cochlear radiation dose after stereotactic radiosurgery with a Gamma Knife (Elekta, Stockholm, Sweden) in patients with acoustic neuroma. [3]

At a median of 20 months after surgery, none of the patients required further treatment. [3]  Serviceable hearing was preserved in 71% of all patients and in 89% of patients with Gardner-Robertson (GR) class I hearing. Patients who received a radiation dose lower than 4.2 Gy to the central cochlea had significantly better hearing preservation of the same GR class, and all 12 patients younger than 60 years who received a cochlear radiation dose lower than 4.2 Gy retained serviceable hearing at 2 years after surgery.

Roche PH, Bouvier C, Chinot O, Figarella-Branger D. Genesis and biology of vestibular schwannomas. Prog Neurol Surg. 2008. 21:24-31. [Medline].

Cui H, Li P, Lu C, Huang X, Chen L, Liu N, et al. [Clinical diagnosis and treatment of primary retroperitoneal schwannoma: a report of 109 cases]. Zhonghua Yi Xue Za Zhi. 2015 Jun 9. 95 (22):1755-8. [Medline].

Kano H, Kondziolka D, Khan A, Flickinger JC, Lunsford LD. Predictors of hearing preservation after stereotactic radiosurgery for acoustic neuroma. J Neurosurg. 2009 Oct. 111 (4):863-73. [Medline].

Guerrissi JO. Solitary benign schwannomas in major nerve systems of the head and neck. J Craniofac Surg. 2009 May. 20 (3):957-61. [Medline].

Bakar B, Sumer MM, Cila A, Tekkok IH. An extreme lateral lumbar disc herniation mimicking L4 schwannoma. Acta Neurol Belg. 2009 Jun. 109 (2):155-8. [Medline].

Ichinose T, Takami T, Yamamoto N, Tsuyuguchi N, Ohata K. Intratumoral hemorrhage of spinal schwannoma of the cauda equina manifesting as acute paraparesis–case report. Neurol Med Chir (Tokyo). 2009 Jun. 49 (6):255-7. [Medline].

Strasilla C, Sychra V. [Imaging-based diagnosis of vestibular schwannoma]. HNO. 2017 May. 65 (5):373-380. [Medline].

Fortnum H, O’Neill C, Taylor R, Lenthall R, Nikolopoulos T, Lightfoot G, et al. The role of magnetic resonance imaging in the identification of suspected acoustic neuroma: a systematic review of clinical and cost effectiveness and natural history. Health Technol Assess. 2009 Mar. 13 (18):iii-iv, ix-xi, 1-154. [Medline].

Wu S, Liu G, Tu R. Value of ultrasonography in neurilemmoma diagnosis: the role of round shape morphology. Med Ultrason. 2012 Sep. 14 (3):192-6. [Medline].

Yousem SA, Colby TV, Urich H. Malignant epithelioid schwannoma arising in a benign schwannoma. A case report. Cancer. 1985 Jun 15. 55 (12):2799-803. [Medline].

Tan M, Myrie OA, Lin FR, Niparko JK, Minor LB, Tamargo RJ, et al. Trends in the management of vestibular schwannomas at Johns Hopkins 1997-2007. Laryngoscope. 2010 Jan. 120 (1):144-9. [Medline].

Rutten I, Baumert BG, Seidel L, Kotolenko S, Collignon J, Kaschten B, et al. Long-term follow-up reveals low toxicity of radiosurgery for vestibular schwannoma. Radiother Oncol. 2007 Jan. 82 (1):83-9. [Medline].

Sade B, Mohr G, Dufour JJ. Vascular complications of vestibular schwannoma surgery: a comparison of the suboccipital retrosigmoid and translabyrinthine approaches. J Neurosurg. 2006 Aug. 105 (2):200-4. [Medline].

Strauss C, Prell J, Rampp S, Romstöck J. Split facial nerve course in vestibular schwannomas. J Neurosurg. 2006 Nov. 105 (5):698-705. [Medline].

Kondziolka D, Lunsford LD. Future perspectives in acoustic neuroma management. Prog Neurol Surg. 2008. 21:247-54. [Medline].

Pillai P, Sammet S, Ammirati M. Image-guided, endoscopic-assisted drilling and exposure of the whole length of the internal auditory canal and its fundus with preservation of the integrity of the labyrinth using a retrosigmoid approach: a laboratory investigation. Neurosurgery. 2009 Dec. 65 (6 Suppl):53-9; discussion 59. [Medline].

Park HH, Hong CK, Jung HH, Chang WS, Kim CH, Lee WS, et al. The Role of Radiosurgery in the Management of Benign Head and Neck Tumors. World Neurosurg. 2016 Mar. 87:116-23. [Medline].

Shin DW, Sohn MJ, Kim HS, Lee DJ, Jeon SR, Hwang YJ, et al. Clinical analysis of spinal stereotactic radiosurgery in the treatment of neurogenic tumors. J Neurosurg Spine. 2015 Oct. 23 (4):429-37. [Medline].

Kida Y, Yoshimoto M, Hasegawa T. Radiosurgery for facial schwannoma. J Neurosurg. 2007 Jan. 106 (1):24-9. [Medline].

Langlois AM, Iorio-Morin C, Masson-Côté L, Mathieu D. Gamma Knife Stereotactic Radiosurgery for Nonvestibular Cranial Nerve Schwannomas. World Neurosurg. 2018 Feb. 110:e1031-e1039. [Medline].

Yang L, Hu L, Zhao W, Zhang H, Liu Q, Wang D. Endoscopic endonasal approach for trigeminal schwannomas: our experience of 39 patients in 10 years. Eur Arch Otorhinolaryngol. 2018 Mar. 275 (3):735-741. [Medline].

Ian D Dickey, MD, FRCSC, LMCC Orthopedic Surgeon, Colorado Limb Consultants, Denver Clinic for Extremities at Risk; Medical Director, Denver Sarah Cannon Sarcoma Network; Staff Surgeon, Department of Orthopedics, Presbyterian/St Luke’s Hospital; Adjunct Professor, Department of Chemical and Biological Engineering, University of Maine

Ian D Dickey, MD, FRCSC, LMCC is a member of the following medical societies: Canadian Orthopaedic Association, Maine Society of Orthopaedic Surgeons, Mayo Clinic Alumni Association, Musculoskeletal Tumor Society, New England Orthopedic Society, Royal College of Surgeons of Canada

Disclosure: Received consulting fee from Stryker Orthopaedics for consulting; Received honoraria from Cadence for speaking and teaching; Received grant/research funds from Wright Medical for research; Received honoraria from Angiotech for speaking and teaching; Received honoraria from Ferring for speaking and teaching.

James Gerald Floyd, MD Assistant Professor, Department of Surgery, Division of Orthopedic Surgery, University of Alabama at Birmingham School of Medicine

James Gerald Floyd, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Medical Association, American Orthopaedic Foot and Ankle Society, National Medical Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Harris Gellman, MD Consulting Surgeon, Broward Hand Center; Voluntary Clinical Professor of Orthopedic Surgery and Plastic Surgery, Departments of Orthopedic Surgery and Surgery, University of Miami, Leonard M Miller School of Medicine; Clinical Professor of Surgery, Nova Southeastern School of Medicine

Harris Gellman, MD is a member of the following medical societies: American Academy of Medical Acupuncture, American Academy of Orthopaedic Surgeons, American Orthopaedic Association, American Society for Surgery of the Hand, Arkansas Medical Society, Florida Medical Association, Florida Orthopaedic Society

Disclosure: Nothing to disclose.

Howard A Chansky, MD Associate Professor, Department of Orthopedics and Sports Medicine, University of Washington Medical Center

Howard A Chansky, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons

Disclosure: Nothing to disclose.

Neurilemmoma (Schwannoma)

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