Malignant peripheral nerve sheath tumor overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]

Overview

Malignant peripheral nerve sheath tumor (MPNST) is a form of cancer of the connective tissue surrounding nerves. On gross pathology, a large firm mass with necrosis and hemorrhage is the characteristic finding of malignant peripheral nerve sheath tumor. On microscopic histopathological analysis, spindle cells, nuclear atypia, mitoses, and herring bone pattern are characteristic findings of malignant peripheral nerve sheath tumor.[1] Neurofibromatosis type I (NF1) gene is involved in the pathogenesis of malignant peripheral nerve sheath tumor.[2] Malignant peripheral nerve sheath tumor must be differentiated from neurofibroma and schwannoma.[3] The incidence of malignant peripheral nerve sheath tumor is approximately 0.1 per 100,000 individuals worldwide.[4] The incidence of malignant peripheral nerve sheath tumor increases with age.[4] Malignant peripheral nerve sheath tumor affects men and women equally.[3] Common risk factors in the development of malignant peripheral nerve sheath tumor are pre-existing plexiform neurofibromas, pre-existing perineuriomas, radiation therapy, and neurofibromatosis type 1.[5][6][7] Symptoms of malignant peripheral nerve sheath tumor include peripheral edema, difficulty in moving the extremity, pain, and numbness.[8] MRI may be diagnostic of malignant peripheral nerve sheath tumor. Findings on MRI suggestive of malignant peripheral nerve sheath tumor include isointense on T1 weighted image and low signal on T2 weighted image.[3] The predominant therapy for malignant peripheral nerve sheath tumor is surgical resection. Adjunctive chemotherapy and radiation therapy may be required. Common complication of malignant peripheral nerve sheath tumor is metastasis. Prognosis is generally poor.[9]

Classification

There is no classification system established for malignant peripheral nerve sheath tumor.

Pathophysiology

On gross pathology, a large firm mass with necrosis and hemorrhage is the characteristic finding of malignant peripheral nerve sheath tumor. On microscopic histopathological analysis, spindle cells, nuclear atypia, mitoses, and herring bone pattern are characteristic findings of malignant peripheral nerve sheath tumor.[1] Neurofibromatosis type I (NF1) gene is involved in the pathogenesis of malignant peripheral nerve sheath tumor.[2]

Causes

Malignant peripheral nerve sheath tumor may be caused by a mutation on neurofibromatosis type I gene.[10][2]

Differential Diagnosis

Malignant peripheral nerve sheath tumor must be differentiated from neurofibroma and schwannoma.[3]

Epidemiology and Demographics

The incidence of malignant peripheral nerve sheath tumor is approximately 0.1 per 100,000 individuals worldwide.[4] The incidence of malignant peripheral nerve sheath tumor increases with age.[4] Malignant peripheral nerve sheath tumor affects men and women equally.[3]

Risk Factors

Common risk factors in the development of malignant peripheral nerve sheath tumor are pre-existing plexiform neurofibromas, pre-existing perineuriomas, radiation therapy, and neurofibromatosis type 1.[5][6][7]

Screening

According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for Malignant peripheral nerve sheath tumor.[11]

Complication and prognosis

Common complication of malignant peripheral nerve sheath tumor is metastasis. Prognosis is generally poor.[9]

Diagnosis

Staging

There is no established system for the staging of malignant peripheral nerve sheath tumor.

Symptoms

Symptoms of malignant peripheral nerve sheath tumor include peripheral edema, difficulty in moving the extremity, pain, and numbness.[8]

Physical Examination

Common physical examination findings of malignant peripheral nerve sheath tumor include edema, paresthesia, and weakness.

Laboratory Findings

There are no laboratory findings associated with malignant peripheral nerve sheath tumor.

X Ray

There are no X-ray findings associated with malignant peripheral nerve sheath tumor.

CT

CT scan may be diagnostic of malignant peripheral nerve sheath tumor. Finding on CT scan suggestive of malignant peripheral nerve sheath tumor is a rapid growth mass with irrefular border.[3]

MRI

MRI may be diagnostic of malignant peripheral nerve sheath tumor. Findings on MRI suggestive of malignant peripheral nerve sheath tumor include isointense on T1 weighted image and low signal on T2 weighted image.[3]

Ultrasound

There are no ultrasound findings associated with malignant peripheral nerve sheath tumor.

Other Imaging Findings

Scintigraphy may be diagnostic of malignant peripheral nerve sheath tumor. Finding on Gallium67 scintigraphy suggestive of malignant peripheral nerve sheath tumor is higher uptake.[3]

Other Diagnostic Studies

There are no other diagnostic study findings associated with malignant peripheral nerve sheath tumor.

Biopsy

Biopsy is helpful in the diagnosis of schwannoma.

Treatment

Medical Therapy

The predominant therapy for malignant peripheral nerve sheath tumor is surgical resection. Adjunctive chemotherapy and radiation therapy may be required.

Surgery

Surgery is the mainstay of treatment for malignant peripheral nerve sheath tumor.

Primary Prevention

There is no established method for prevention of malignant peripheral nerve sheath tumor.

Secondary Prevention

There are no secondary preventive measures available for malignant peripheral nerve sheath tumor.

References

  1. 1.0 1.1 Malignant peripheral nerve sheath tumor. Librepathology 2015. http://librepathology.org/wiki/index.php/Malignant_peripheral_nerve_sheath_tumour
  2. 2.0 2.1 2.2 Ferrari, Andrea; Gianni Bisogno; Modesto Carli (2007). "Management Of Childhood Malignant Peripheral Nerve Sheath Tumor". Pediatric Drugs. 9 (4): 239–48. PMID 17705563. doi:10.2165/00148581-200709040-00005. 
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 Malignant peripheral nerve sheath tumor. Radiopaedia 2015. http://radiopaedia.org/articles/malignant-peripheral-nerve-sheath-tumour
  4. 4.0 4.1 4.2 4.3 Bates JE, Peterson CR, Dhakal S, Giampoli EJ, Constine LS (2014). "Malignant peripheral nerve sheath tumors (MPNST): a SEER analysis of incidence across the age spectrum and therapeutic interventions in the pediatric population.". Pediatr Blood Cancer. 61 (11): 1955–60. PMID 25130403. doi:10.1002/pbc.25149. 
  5. 5.0 5.1 Perrin RG, Guha A (2004). "Malignant peripheral nerve sheath tumors.". Neurosurg Clin N Am. 15 (2): 203–16. PMID 15177319. doi:10.1016/j.nec.2004.02.004. 
  6. 6.0 6.1 Baehring JM, Betensky RA, Batchelor TT (2003). "Malignant peripheral nerve sheath tumor: the clinical spectrum and outcome of treatment.". Neurology. 61 (5): 696–8. PMID 12963767. 
  7. 7.0 7.1 Kim DH, Murovic JA, Tiel RL, Moes G, Kline DG (2005). "A series of 397 peripheral neural sheath tumors: 30-year experience at Louisiana State University Health Sciences Center.". J Neurosurg. 102 (2): 246–55. PMID 15739552. doi:10.3171/jns.2005.102.2.0246. 
  8. 8.0 8.1 Valeyrie-Allanore L, Ismaïli N, Bastuji-Garin S, Zeller J, Wechsler J, Revuz J; et al. (2005). "Symptoms associated with malignancy of peripheral nerve sheath tumours: a retrospective study of 69 patients with neurofibromatosis 1.". Br J Dermatol. 153 (1): 79–82. PMID 16029330. doi:10.1111/j.1365-2133.2005.06558.x. 
  9. 9.0 9.1 Neville H, Corpron C, Blakely ML, Andrassy R (2003). "Pediatric neurofibrosarcoma". J. Pediatr. Surg. 38 (3): 343–6; discussion 343–6. PMID 12632346. doi:10.1053/jpsu.2003.50105. 
  10. Albright, A (2008). Principles and practice of pediatric neurosurgery. New York: Thieme. ISBN 1588903958. 
  11. Malignant peripheral nerve sheath tumor. U.S. Preventive Service Task Force (USPSTF) 2015. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=Malignant+peripheral+nerve+sheath+tumor

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