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Neurilemmoma

Schwannoma

Neurilemomma, also known as schwannoma, is a benign, encapsulated neurogenic tumor originating from neural crest derived Schwann cells of any nerve in the body. They present as asymptomatic palpable masses or may induce late neurologic complaints such as chronic neuropathic pain. Diagnosis is usually established 5 years after disease onset.


Presentation

Neurilemommas are usually located on the flexor surfaces of the limbs (wrist, elbow, knee), and may involve cranial or spinal nerves or even small nerve twigs, causing different types of symptoms [1]. Their peak incidence is between ages 30 and 60 and sex ratio is 1. If a spinal nerve is involved, symptoms mimic disk hernia [2] [3].

The tumor itself may cause no pain or it can be tender on palpation. It is usually well defined, slow growing and mobile relative to the underlying and overlying structures. Nerve compression may cause distal paresthesia, carpal tunnel or tarsal tunnel syndrome, discogenic back discomfort or sciatic pain. Cranial nerve involvement has also been described. The cause of these symptoms may not be easily detected because lesions may be located proximally and cause distal complaints. Patient complaints are present in 33% of cases [4].

Different types of neurilemmoma have been described: plexiform [5], cellular, ancient, solitary, melanotic [6] [7] and plexiform melanocytic [8]. Less frequently encountered variants include malignant epithelioid schwannoma without neurofibromatosis [9], cutaneous pseudoglandular schwannoma [10], neuroblastomalike/rosetoid schwannoma [11] and plexiform multinodular neurilemomma [12]. Lesions can also be found as part of genetic syndromes: schwannomatosis, type 1 or 2 neurofibromatosis and the Carney complex. The tumor may compress neighboring structures, causing various symptoms. Rare locations include the subungal [13] and lip [14] areas, whereas commonly involved nerves are vagus, cervical, ulnar and peroneal. Both sensory and motor nerves may be affected and functional deficit may be encountered.

Neurilemmomas are benign tumors with no malignancy potential, except for 10% of the melanotic type.

Soft Tissue Swelling
  • The tumor presented as a slowly growing soft tissue swelling. Magnetic resonance imaging disclosed a very infiltrative, 9-cm mass involving the subcutis and deep soft tissues of the right cheek.[ncbi.nlm.nih.gov]
Cough
  • A 9-year-old girl was admitted because of recurrent cough, dyspnea, and tachypnea for over 3 months, which had become worse for the preceding week. A chest X-ray revealed a mass in the lumen of the trachea.[ncbi.nlm.nih.gov]
  • They are often presented with a prolonged cough and shortness of breath. Most tracheal tumors in adults are cancerous (80% to 90%). Benign tracheal tumors are rare in adult patients.[ncbi.nlm.nih.gov]
  • A rare case of giant neurilemmoma is presented with a large swelling in the right side of the neck associated with breathlessness and paroxysmal cough.[ncbi.nlm.nih.gov]
Paroxysmal Cough
  • A rare case of giant neurilemmoma is presented with a large swelling in the right side of the neck associated with breathlessness and paroxysmal cough.[ncbi.nlm.nih.gov]
Sore Throat
  • Herein we describe the case of intraoral neurilemmoma in a 9-year-old girl complaining only of a sore throat. She had an exophytic multilobulated mass on the base of the tongue.[ncbi.nlm.nih.gov]
Tachypnea
  • A 9-year-old girl was admitted because of recurrent cough, dyspnea, and tachypnea for over 3 months, which had become worse for the preceding week. A chest X-ray revealed a mass in the lumen of the trachea.[ncbi.nlm.nih.gov]
Dysphagia
  • . • Dysarthria, dysphagia, and, finally, cerebellar seizures and respiratory distress. In view of the currently available diagnostic and therapeutic armamentarium, this sequence now rarely ever reaches its end.[scielo.br]
Jaundice
  • Neurilemmoma should be considered in the differential diagnosis of obstructive jaundice.[ncbi.nlm.nih.gov]
Heel Pain
  • In the present cases, surgical excision of the tumors resulted in immediate and complete relief of chronic heel pain. Surgeons should consider neurilemmoma as a cause of persistent chronic heel pain despite the rarity of the disease.[ncbi.nlm.nih.gov]
  • A 42-year-old man presented with left medial heel pain radiating to the lateral sole for 6 months. On examination, there was positive Tinel sign over the medial heel with pain radiating to the lateral sole.[ncbi.nlm.nih.gov]
Neck Swelling
  • The tumor was noted as a asymptomatic left lower neck swelling 9 months before admission. Ultrasound scan showed the solid lesion of predominantly hypoechoic structure measuring 17.2 x 12 x 26 mm within the left lobe.[ncbi.nlm.nih.gov]
Limb Weakness
  • A 29-year-old man who experienced middle back pain with prominent right lower limb weakness, and an 11-year-old boy who suffered from sudden onset neck pain with left arm weakness and hand clawing for 2 weeks before admission to our department were included[ncbi.nlm.nih.gov]
Sexual Dysfunction
  • Several cases were accompanied by sexual dysfunction. Most histologic studies were consistent, with a benign schwannoma that showed a palisading Antoni A and Antoni B pattern without malignant changes in cell morphology.[ncbi.nlm.nih.gov]
Pollakisuria
  • Postoperatively, the patient's subjective state was excellent, with normal blood pressure values, and without pollakisuria.[ncbi.nlm.nih.gov]
Adnexal Mass
  • A 35-year-old woman presented with an adnexal mass which came out as a leiomyosarcoma of the broad ligament arising in a pre-existing pure neurilemmoma-like leiomyoma.[ncbi.nlm.nih.gov]

Workup

With noncontributory hematological and biochemical workup, neurilemmoma diagnosis relies on history and clinical evaluation, as well as imaging and histologic examination.

Plain radiographies often reveal non specific aspects, as these tumors are radiolucent, while ultrasonography is a reliable diagnosis method [15]. More information is added by computerized tomography or magnetic resonance scanning, that describe a usually round mass, no bigger than 2,5 cm that uniformly enhances when gadolinium contrast is administered.

If doubt persists and a biopsy is needed or symptoms dictate excision, histology provides further description. The tumor is unilocular and surrounded by a vascularized capsule composed of epineurium. Neurilemmomas contain benign spindle cells (Antoni A areas) and connective tissue (Antoni B areas) with no mitotic figures, and may contain calcification or hemorrhage areas or degenerative cysts. The grade of the tumor depends on how aggressive the growth is. Immunohistochemistry is positive for vimentin, keratin, glial fibrillary acid protein, neuron-specific enolase and myelin basic protein [16] [14] and negative for masson trichrome stain. Leu7, S-100 protein and epithelial membrane antigen are present [17]. In degenerate tumors, the most valuable diagnostic tool is S-100 protein antibody stain, as it highlights beyond doubt the presence of Schwann cells in the tumor.

Dirofilaria Immitis
  • Human pulmonary dirofilariasis (HPD) is a rare zoonotic infection caused by Dirofilaria immitis. Dogs are the definite hosts and humans are infected occasionally via a vector, generally a mosquito.[ncbi.nlm.nih.gov]

Treatment

  • Complete surgical excision is the treatment of choice. Malignant transformation and recurrence following this treatment are rare. A 20-year-old woman presented with a slow-growing mass over the back of her tongue first noticed 8 months before.[ncbi.nlm.nih.gov]
  • Magnetic resonant imaging (MRI) plays a central role in diagnosing vagal nerve neoplasm and in fact, provides important pre-operative information useful in planning optimal surgical treatment.[ncbi.nlm.nih.gov]
  • When they do occur; treatment is simple. However; diagnosis is invariably delayed because of the vagueness of symptoms.[ncbi.nlm.nih.gov]
  • A review of the literature is presented and the differential diagnosis, prognosis and treatment of this benign neurogenic tumor are discussed.[ncbi.nlm.nih.gov]
  • Your specialist will discuss the risks of any treatment with you.[cancerresearchuk.org]

Prognosis

  • A review of the literature is presented and the differential diagnosis, prognosis and treatment of this benign neurogenic tumor are discussed.[ncbi.nlm.nih.gov]
  • The overall prognosis is excellent, as was the case for our patient.[ncbi.nlm.nih.gov]
  • We describe our management of these 2 cases, and we discuss what has been published in the literature regarding the presentation, diagnosis, histopathology, treatment, and prognosis for patients with glossal neurilemmoma.[ncbi.nlm.nih.gov]
  • Despite the rarity of this condition, physicians should consider schwannoma as a differential diagnosis for a mass over the tongue, as there can be a favorable outcome and prognosis for the patient when this condition is correctly identified.[ncbi.nlm.nih.gov]
  • Complete surgical resection is the treatment of choice with excellent prognosis, as the tumour was benign, and recurrence is nearly unknown, so it is possible and indeed recommended to preserve nerve integrity with careful dissection.[ncbi.nlm.nih.gov]

Etiology

  • On routine ultrasonography of the pelvis confirmed a pelvic tumor of uncertain etiology, with no abdominal lymph node enlargement. No signs of metastasis were found.[ncbi.nlm.nih.gov]
  • Abstract Neurilemmoma (schwannoma) is a benign, encapsulated perineural tumor of neuroectodermal derivation that originates from the Schwann cells of the neural sheath of motor and sensitive peripheral nerves; the etiology is still unknown.[ncbi.nlm.nih.gov]
  • TERMINOLOGY Definitions Encapsulated, benign peripheral nerve sheath tumor composed predominantly of Schwann cells ETIOLOGY/PATHOGENESIS Molecular Aberrations Somatic NF2 gene mutations present in most tumors Bilateral vestibular schwannomas occur in[basicmedicalkey.com]
  • Etiology 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.[emedicine.medscape.com]

Epidemiology

  • […] nerve sheath tumor composed predominantly of Schwann cells ETIOLOGY/PATHOGENESIS Molecular Aberrations Somatic NF2 gene mutations present in most tumors Bilateral vestibular schwannomas occur in setting of germline NF2 gene mutations CLINICAL ISSUES Epidemiology[basicmedicalkey.com]
  • “News on the genetics, epidemiology, medical care and translational research of Schwannomas”. J Neurol. vol. 253. 2006. pp. 1533-41. (A well-written and updated review on all aspects of schwannomas.)[clinicaladvisor.com]
  • Epidemiology 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.[emedicine.medscape.com]
Sex distribution
Age distribution

Prevention

  • Superficial temporal artery-middle cerebral artery anastomosis was performed to prevent critical reduction of cerebral blood flow in the left ICA territory; this was followed by tumor resection. The occluded ICA recanalized after tumor resection.[ncbi.nlm.nih.gov]
  • Although this neoplasm is benign in nature, surgical resection is warranted to prevent recurrence, and it is the sole means of treatment. The overall prognosis is excellent, as was the case for our patient.[ncbi.nlm.nih.gov]
  • For malignant or high-grade schwannomas, radiotherapy would be used to help prevent recurrence after surgery. Schwannoma chemotherapy: It is rare to treat slow-growing schwannoma tumours using chemotherapy drugs.[braintumourresearch.org]
  • Careful dissection is of paramount important during the surgery, to prevent unfortunate resection of the nerve.[austinpublishinggroup.com]
  • There is no way to prevent acoustic neuroma, but scientists are looking at ways of using gene therapy to control the overproduction of Schwann cells.[medicalnewstoday.com]

References

Article

  1. Guerrissi J. Solitary benign schwannomas in major nerve systems of the head and neck. J Craniofac Surg. 2009;20(3):957-961.
  2. Bakar B, Sumer M, Cila A, et al. An extreme lateral lumbar disc herniation mimicking L4 schwannoma. Acta Neurol Belg. 2009;109(2):155-158.
  3. Ichinose T, Takami T, Yamamoto N, et al. Intratumoral hemorrhage of spinal schwannoma of the cauda equina manifesting as acute paraparesis--case report. Neurol Med Chir. 2009;49(6):255-257.
  4. Rodríguez-Peralto J, Riveiro-Falkenbach E, Carrillo R. Benign cutaneous neural tumors. Semin Diagn Pathol. 2013;30(1):45-57.
  5. Kara M, Akyüz M, Yilmaz Aet al. Peripheral nerve involvement in a neurofibromatosis type 2 patient with plexiform neurofibroma of the cauda equina: a sonographic vignette. Arch Phys Med Rehabil. 2011;92(9):1511-1514.
  6. Kurtkaya-Yapicier O, Scheithauer B, Woodruff J. The pathobiologic spectrum of Schwannomas. Histol Histopathol. 2003;18(3):925-934.
  7. Humber C, Copete M, Hohn F. Ancient schwannoma of upper lip: case report with distinct histologic features and review of the literature. J Oral Maxillofac Surg. 2011;69(6):e118-122.
  8. Yeh I, Argenyi Z, Vemula S, et al. Plexiform melanocytic schwannoma: a mimic of melanoma. J Cutan Pathol. 2012;39(5):521-525.
  9. Manganoni A, Farisoglio C, Lonati Aet al. Cutaneous epithelioid malignant schwannoma: review of the literature and case report. J Plast Reconstr Aesthet Surg. 2009;62(9):e318-e321.
  10. Lisle A, Jokinen C, Argenyi Z. Cutaneous pseudoglandular schwannoma: a case report of an unusual histopathologic variant. Am J Dermatopathol. 2011;33(5):e63-e65.
  11. Goldblum J, Beals T, Weiss SW. Neuroblastoma-like neurilemoma. Am J Surg Pathol. 1994;18(3):266-273.
  12. Suchak R, Luzar B, Bacchi C, et al. Cutaneous neuroblastoma-like schwannoma: a report of two cases, one with a plexiform pattern, and a review of the literature. J Cutan Pathol. 2010;37(9):997-1001.
  13. Huntley J, Davie R, Hooper G. A subungual schwannoma. Plast Reconstr Surg. 2006;117(2):712-713.
  14. Baderca F, Cojocaru S, Lazar E, et al. Schwannoma of the lip: case report and review of the literature. Rom J Morphol Embryol. 2008;49(3):391-398.
  15. Wu S, Liu G, Tu R. Value of ultrasonography in neurilemmoma diagnosis: the role of round shape morphology. Med Ultrason. 2012;14(3):192-196.
  16. Rodríguez-Peralto J, Riveiro-Falkenbach E, Carrillo R. Benign cutaneous neural tumors. Semin Diagn Pathol. 2013;30(1):45-57.
  17. Noh S, Do JE, Park JM, et al. Cutaneous schwannoma presented as a pedunculated protruding mass. Ann Dermatol. 2011;23:S264-S266.

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Last updated: 2019-07-11 22:17