Skip to main content

Advertisement

Log in

Imaging of non-neurogenic peripheral nerve malignancy—a case series and systematic review

  • Scientific Article
  • Published:
Skeletal Radiology Aims and scope Submit manuscript

Abstract

Objective

To evaluate the frequency, clinico-pathologic and imaging features of malignant tumors in peripheral nerves which are of non-neurogenic origin (non-neurogenic peripheral nerve malignancy—PNM).

Materials and methods

We retrospectively reviewed our pathology database for malignant peripheral nerve tumors from 07/2014–07/2019 and performed a systematic review. Exclusion criteria were malignant peripheral nerve sheath tumor (MPNST). Clinico-pathologic and imaging features, apparent diffusion coefficient (ADCmin), and standard uptake values (SUVmax) are reported.

Results

After exclusion of all neurogenic tumors (benign = 196, MPNST = 57), our search yielded 19 non-neurogenic PNMs (7%, n = 19/272), due to primary intraneural malignancy (16%, n = 3/19) and secondary perineural invasion from an adjacent malignancy (16%, n = 3/19) or metastatic disease (63%, n = 12/19). Non-neurogenic PNMs were located in the lumbosacral plexus/sciatic nerves (47%, n = 9/19), brachial plexus (32%, n = 6/19), femoral nerve (5%, n = 1/19), tibial nerve (5%, n = 1/19), ulnar nerve (5%, n = 1/19), and radial nerve (5%, n = 1/19). On MRI (n = 14/19), non-neurogenic PNM tended to be small (< 5 cm, n = 10/14), isointense to muscle on T1-W (n = 14/14), hyperintense on T2-WI (n = 12/14), with enhancement (n = 12/12), low ADCmin (0.5–0.7 × 10–3 mm2/s), and variable metabolic activity (SUVmax range 2.1–13.1). A target sign was absent (n = 14/14) and fascicular sign was rarely present (n = 3/14). Systematic review revealed 89 cases of non-neurogenic PNM.

Conclusion

Non-neurogenic PNMs account for 7% of PNT in our series and occur due to metastases and primary intraneural malignancy. Although non-neurogenic PNMs exhibit a non-specific MRI appearance, they lack typical signs of neurogenic tumors such as the target sign. Quantitative imaging features identified by DWI (low ADC) and F18-FDG PET/CT (high SUV) may be helpful clues to the diagnosis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6

Similar content being viewed by others

References

  1. Wilson TJ, Amrami KK, Howe BM, Spinner RJ. Clinical and radiological follow-up of intraneural perineuriomas. Neurosurgery. 2019;85(6):786–92.

    PubMed  Google Scholar 

  2. Murphey MD, Smith WS, Smith SE, Kransdorf MJ, Temple HT. From the archives of the AFIP. Imaging of musculoskeletal neurogenic tumors: radiologic-pathologic correlation. Radiographics. 1999;19(5):1253–80.

    CAS  PubMed  Google Scholar 

  3. Kim DH, Murovic JA, Tiel RL, Moes G, Kline DG. A series of 397 peripheral neural sheath tumors: 30-year experience at Louisiana State University Health Sciences Center. J Neurosurg. 2005;102(2):246–55.

    PubMed  Google Scholar 

  4. Ahlawat S, Fayad LM. Imaging cellularity in benign and malignant peripheral nerve sheath tumors: utility of the "target sign" by diffusion weighted imaging. Eur J Radiol. 2018;102:195–201.

    PubMed  Google Scholar 

  5. Demehri S, Belzberg A, Blakeley J, Fayad LM. Conventional and functional MR imaging of peripheral nerve sheath tumors: initial experience. AJNR Am J Neuroradiol. 2014;35(8):1615–20.

    CAS  PubMed  PubMed Central  Google Scholar 

  6. Ahlawat S, Khandheria P, Del Grande F, Morelli J, Subhawong TK, Demehri S, et al. Interobserver variability of selective region-of-interest measurement protocols for quantitative diffusion weighted imaging in soft tissue masses: comparison with whole tumor volume measurements. J Magn Reson Imaging. 2016;43(2):446–54.

    PubMed  Google Scholar 

  7. Treglia G, Taralli S, Bertagna F, Salsano M, Muoio B, Novellis P, et al. Usefulness of whole-body fluorine-18-fluorodeoxyglucose positron emission tomography in patients with neurofibromatosis type 1: a systematic review. Radiol Res Pract. 2012;2012:431029.

    PubMed  PubMed Central  Google Scholar 

  8. Kurtz KA, Hoffman HT, Zimmerman MB, Robinson RA. Perineural and vascular invasion in oral cavity squamous carcinoma: increased incidence on re-review of slides and by using immunohistochemical enhancement. Arch Pathol Lab Med. 2005;129(3):354–9.

    PubMed  Google Scholar 

  9. Mendenhall WM, Amdur RJ, Williams LS, Mancuso AA, Stringer SP, Price MN. Carcinoma of the skin of the head and neck with perineural invasion. Head Neck. 2002;24(1):78–83.

    PubMed  Google Scholar 

  10. Chang PC, Fischbein NJ, McCalmont TH, Kashani-Sabet M, Zettersten EM, Liu AY, et al. Perineural spread of malignant melanoma of the head and neck: clinical and imaging features. AJNR Am J Neuroradiol. 2004;25(1):5–11.

    PubMed  Google Scholar 

  11. Maru N, Ohori M, Kattan MW, Scardino PT, Wheeler TM. Prognostic significance of the diameter of perineural invasion in radical prostatectomy specimens. Hum Pathol. 2001;32(8):828–33.

    CAS  PubMed  Google Scholar 

  12. Liebig C, Ayala G, Wilks JA, Berger DH, Albo D. Perineural invasion in cancer: a review of the literature. Cancer. 2009;115(15):3379–91.

    CAS  PubMed  Google Scholar 

  13. Capek S, Howe BM, Amrami KK, Spinner RJ. Perineural spread of pelvic malignancies to the lumbosacral plexus and beyond: clinical and imaging patterns. Neurosurg Focus. 2015;39(3):E14.

    PubMed  Google Scholar 

  14. Marek T, Howe BM, Amrami KK, Spinner RJ. Perineural spread of nonmelanoma skin cancer to the brachial plexus: identifying anatomic pathway(s). World Neurosurg. 2018;114:e818–23.

    PubMed  Google Scholar 

  15. Crush AB, Howe BM, Spinner RJ, Amrami KK, Hunt CH, Johnson GB, et al. Malignant involvement of the peripheral nervous system in patients with cancer: multimodality imaging and pathologic correlation. Radiographics. 2014;34(7):1987–2007.

    PubMed  Google Scholar 

  16. Advani P, Paulus A, Murray P, Jiang L, Goff R, Pooley R, et al. A rare case of primary high-grade large B-cell lymphoma of the sciatic nerve. Clin Lymphoma Myeloma Leuk. 2015;15(6):e117–20.

    PubMed  Google Scholar 

  17. Aghion DM, Capek S, Howe BM, Hepel JT, Sambandam S, Oyelese AA, et al. Perineural tumor spread of bladder cancer causing lumbosacral plexopathy: an anatomic explanation. Acta Neurochir. 2014;156(12):2331–6.

    PubMed  Google Scholar 

  18. Alter RY, Wamsley CC, Mullen JT, Haile WZ, Goldsmith JD, Kasper EM. Peripheral nerve fibromyxoid sarcoma. J Neurosurg. 2014;121(3):576–9.

    PubMed  Google Scholar 

  19. Babu MA, Spinner RJ, Dyck PJ, Amrami KK, Nathan MA, Kawashima A, et al. Recurrent prostatic adenocarcinoma with perineural spread to the lumbosacral plexus and sciatic nerve: comparing high resolution MRI with torso and endorectal coils and F-18 FDG and C-11 choline PET/CT. Abdom Imaging. 2013;38(5):1155–60.

    PubMed  Google Scholar 

  20. Bang JS, Adsul N, Lim JH, Jang IT. Extra-osseous Ewing sarcoma of sciatic nerve masquerading as benign nerve sheath tumor and presented as lumbar radiculopathy: case report and review of literature. World Neurosurg. 2018;115:89–93.

    PubMed  Google Scholar 

  21. Cabrilo I, Burkhardt K, Schaller K, Tessitore E. Renal carcinoma relapse presenting as a peripheral nerve sheath tumor: a case report and brief review of the literature. Neurochirurgie. 2013;59(3):128–32.

    CAS  PubMed  Google Scholar 

  22. Candy N, Young A, Allinson K, Carr O, McMillen J, Trivedi R. Metastatic neuroendocrine carcinoma of unknown origin arising in the femoral nerve sheath. World Neurosurg. 2017;104:1047.e1041–6.

    Google Scholar 

  23. Capek S, Hebert-Blouin MN, Puffer RC, Martinoli C, Frick MA, Amrami KK, et al. Tumefactive appearance of peripheral nerve involvement in hematologic malignancies: a new imaging association. Skelet Radiol. 2015;44(7):1001–9.

    Google Scholar 

  24. Capek S, Howe BM, Tracy JA, Garcia JJ, Amrami KK, Spinner RJ. Prostate cancer with perineural spread and dural extension causing bilateral lumbosacral plexopathy: case report. J Neurosurg. 2015;122(4):778–83.

    PubMed  Google Scholar 

  25. Capek S, Sullivan PS, Howe BM, Smyrk TC, Amrami KK, Spinner RJ, et al. Recurrent rectal cancer causing lumbosacral plexopathy with perineural spread to the spinal nerves and the sciatic nerve: an anatomic explanation. Clin Anat. 2015;28(1):136–43.

    PubMed  Google Scholar 

  26. Daher A, Kamiya-Matsuoka C, Woodman K. Patient with 2 hematologic malignancies presenting as neurolymphomatosis. J Clin Neuromuscul Dis. 2018;19(3):124–30.

    PubMed  Google Scholar 

  27. Del Grande A, Sabatelli M, Luigetti M, Conte A, Granata G, Rufini V, et al. Primary multifocal lymphoma of peripheral nervous system: case report and review of the literature. Muscle Nerve. 2014;50(6):1016–22.

    PubMed  Google Scholar 

  28. Descamps MJ, Barrett L, Groves M, Yung L, Birch R, Murray NM, et al. Primary sciatic nerve lymphoma: a case report and review of the literature. J Neurol Neurosurg Psychiatry. 2006;77(9):1087–9.

    CAS  PubMed  PubMed Central  Google Scholar 

  29. Di Tommaso L, Magrini E, Consales A, Poppi M, Pasquinelli G, Dorji T, et al. Malignant granular cell tumor of the lateral femoral cutaneous nerve: report of a case with cytogenetic analysis. Hum Pathol. 2002;33(12):1237–40.

    PubMed  Google Scholar 

  30. Green RA, O’Donnell P, Briggs TW, Tirabosco R. Two malignant peripheral nerve lesions of non-neurogenic origin. J Med Imaging Radiat Oncol. 2012;56(3):305–9.

    PubMed  Google Scholar 

  31. Hebert-Blouin MN, Amrami KK, Myers RP, Hanna AS, Spinner RJ. Adenocarcinoma of the prostate involving the lumbosacral plexus: MRI evidence to support direct perineural spread. Acta Neurochir. 2010;152(9):1567–76.

    PubMed  Google Scholar 

  32. Howe BM, Amrami KK, Nathan MA, Garcia JJ, Spinner RJ. Perineural spread of cervical cancer to the sciatic nerve. Skelet Radiol. 2013;42(11):1627–31.

    Google Scholar 

  33. Kahraman S, Sabuncuoglu H, Gunhan O, Gurses MA, Sirin S. A rare reason of foot drop caused by primary diffuse large b-cell lymphoma of the sciatic nerve: case report. Acta Neurochir. 2010;152(1):125–8.

    PubMed  Google Scholar 

  34. Kichari JR, Hussain SM, Den Hollander JC, Krestin GP. MR imaging of the brachial plexus: current imaging sequences, normal findings, and findings in a spectrum of focal lesions with MR-pathologic correlation. Curr Probl Diagn Radiol. 2003;32(2):88–101.

    PubMed  Google Scholar 

  35. Kosutic D, Gajanan K. Rare case of a liposarcoma in the brachial plexus. Ann R Coll Surg Engl. 2016;98(7):e106–8.

    CAS  PubMed  PubMed Central  Google Scholar 

  36. Ladha SS, Spinner RJ, Suarez GA, Amrami KK, Dyck PJ. Neoplastic lumbosacral radiculoplexopathy in prostate cancer by direct perineural spread: an unusual entity. Muscle Nerve. 2006;34(5):659–65.

    PubMed  Google Scholar 

  37. Larque AB, Bredella MA, Nielsen GP, Chebib I. Synovial sarcoma mimicking benign peripheral nerve sheath tumor. Skelet Radiol. 2017;46(11):1463–8.

    Google Scholar 

  38. Lavorato A, Titolo P, Vincitorio F, Cofano F, Garbossa D. Intraneural Ewing sarcoma of fibular nerve: case report, radiologic findings and review of literature. World Neurosurg. 2019;123:212–5.

    PubMed  Google Scholar 

  39. Lim AT, Clucas D, Khoo C, Parameswaran BK, Lau E. Neurolymphomatosis: MRI and (18) FDG-PET features. J Med Imaging Radiat Oncol. 2016;60(1):92–5.

    PubMed  Google Scholar 

  40. Mathys J, Vajtai I, Vögelin E, Zimmermann DR, Ozdoba C, Hewer E. Desmoplastic small round cell tumor: a rare cause of a progressive brachial plexopathy. Muscle Nerve. 2014;49(6):922–7.

    CAS  PubMed  Google Scholar 

  41. Mitchell BD, Fox BD, Viswanathan A, Mitchell AH, Powell SZ, Cech DA. Ewing sarcoma mimicking a peripheral nerve sheath tumor. J Clin Neurosci. 2010;17(10):1317–9.

    CAS  PubMed  Google Scholar 

  42. Nagao E, Nishie A, Yoshimitsu K, Irie H, Shioyama Y, Naito S, et al. Gluteal muscular and sciatic nerve metastases in advanced urinary bladder carcinoma: case report. Abdom Imaging. 2004;29(5):619–22.

    CAS  PubMed  Google Scholar 

  43. Oktay K, Guzel E, Bitiren M, Simsek S, Yildirim M, Guzel A. Lung adenocarcinoma metastasis mimicking peripheral nerve sheath tumor: case report and review of literature. World Neurosurg. 2018;120:490–4.

    PubMed  Google Scholar 

  44. Quinones-Hinojosa A, Friedlander RM, Boyer PJ, Batchelor TT, Chiocca EA. Solitary sciatic nerve lymphoma as an initial manifestation of diffuse neurolymphomatosis. Case report and review of the literature. J Neurosurg. 2000;92(1):165–9.

    CAS  PubMed  Google Scholar 

  45. Raveendran S, Kota AA, Stephen E, Pallapati SCR, Thomas BP. Synovial sarcoma of the brachial plexus - a rare tumor in a rare area: a case report. J Med Case Rep. 2018;12(1):334.

    PubMed  PubMed Central  Google Scholar 

  46. Reddy CG, Mauermann ML, Solomon BM, Ringler MD, Jerath NU, Begna KH, et al. Neuroleukemiosis: an unusual cause of peripheral neuropathy. Leuk Lymphoma. 2012;53(12):2405–11.

    PubMed  Google Scholar 

  47. Scheithauer BW, Amrami KK, Folpe AL, Silva AI, Edgar MA, Woodruff JM, et al. Synovial sarcoma of nerve. Hum Pathol. 2011;42(4):568–77.

    PubMed  Google Scholar 

  48. Schulz M, Lamont D, Muthu T, Hussain Z, Balakrishnan V. Metastasis of breast cancer to a lumbar spinal nerve root ganglion. Spine (Phila Pa 1976). 2009;34(20):E735–9.

    Google Scholar 

  49. Sideras PA, Matthews J, Sakib SM, Ofikwu F, Spektor V. Neurolymphomatosis of the peripheral nervous system: a case report and review of the literature. Clin Imaging. 2016;40(6):1253–6.

    PubMed  Google Scholar 

  50. Song EJ, Park JS, Ryu KN, Park SY, Jin W. Perineural spread along spinal and obturator nerves in primary vaginal carcinoma: a case report. World Neurosurg. 2018;115:85–8.

    PubMed  Google Scholar 

  51. Stone JJ, Adamo DA, Khan DZ, Packard AT, Broski SM, Nathan MA, et al. Multimodal imaging aids in the diagnosis of perineural spread of prostate cancer. World Neurosurg. 2019;122:e235–40.

    PubMed  Google Scholar 

  52. Swanson AA, Giannini C, Folpe AL, Van Dyke DL, Amrami KK, Michalak WA, et al. Low-grade fibromyxoid sarcoma arising within the median nerve. Neuropathology. 2018;38(3):309–14.

    CAS  PubMed  Google Scholar 

  53. Turgut M, Gokpinar D, Barutca S, Erkus M. Lumbosacral metastatic extradural Merkel cell carcinoma causing nerve root compression--case report. Neurol Med Chir (Tokyo). 2002;42(2):78–80.

    Google Scholar 

  54. Uchida K, Kobayashi S, Yayama T, Muramatsu J, Kurokawa T, Imamura Y, et al. Metastatic involvement of sacral nerve roots from uterine carcinoma: a case report. Spine J. 2008;8(5):849–52.

    PubMed  Google Scholar 

  55. van den Bent MJ, de Bruin HG, Beun GD, Vecht CJ. Neurolymphomatosis of the median nerve. Neurology. 1995;45(7):1403–5.

    PubMed  Google Scholar 

  56. van den Bent MJ, de Bruin HG, Bos GM, Brutel de la Riviere G, Sillevis Smitt PA. Negative sural nerve biopsy in neurolymphomatosis. J Neurol. 1999;246(12):1159–63.

    PubMed  Google Scholar 

  57. Wang T, Miao Y, Meng Y, Li A. Isolated leukemic infiltration of peripheral nervous system. Muscle Nerve. 2015;51(2):290–3.

    PubMed  Google Scholar 

  58. Wigfield CC, Hilton DA, Coleman MG, Whitfield PC. Metastatic adenocarcinoma masquerading as a solitary nerve sheath tumour. Br J Neurosurg. 2003;17(5):459–61.

    CAS  PubMed  Google Scholar 

  59. Ye BS, Sunwoo IN, Suh BC, Park JP, Shim DS, Kim SM. Diffuse large B-cell lymphoma presenting as piriformis syndrome. Muscle Nerve. 2010;41(3):419–22.

    PubMed  Google Scholar 

  60. Papachristou DJ, Palekar A, Surti U, Cieply K, McGough RL, Rao UN. Malignant granular cell tumor of the ulnar nerve with novel cytogenetic and molecular genetic findings. Cancer Genet Cytogenet. 2009;191(1):46–50.

  61. Wasa J, Nishida Y, Tsukushi S, Shido Y, Sugiura H, Nakashima H, et al. MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas. AJR Am J Roentgenol. 2010;194(6):1568–74.

    PubMed  Google Scholar 

  62. DeVries AH, Howe BM, Spinner RJ, Broski SM. B-cell peripheral neurolymphomatosis: MRI and (18)F-FDG PET/CT imaging characteristics. Skelet Radiol. 2019;48(7):1043–50.

    Google Scholar 

  63. Mazal AT, Ashikyan O, Cheng J, Le LQ, Chhabra A. Diffusion-weighted imaging and diffusion tensor imaging as adjuncts to conventional MRI for the diagnosis and management of peripheral nerve sheath tumors: current perspectives and future directions. Eur Radiol. 2019;29(8):4123–32.

    PubMed  Google Scholar 

  64. Bronstein Y, Tummala S, Rohren E. F-18 FDG PET/CT for detection of malignant involvement of peripheral nerves: case series and literature review. Clin Nucl Med. 2011;36(2):96–100.

    PubMed  Google Scholar 

  65. Yuh EL, Jain Palrecha S, Lagemann GM, Kliot M, Weinstein PR, Barbaro NM, et al. Diffusivity measurements differentiate benign from malignant lesions in patients with peripheral neuropathy or plexopathy. AJNR Am J Neuroradiol. 2015;36(1):202–9.

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Shivani Ahlawat.

Ethics declarations

Conflict of interest

Shivani Ahlawat has received support from Department of Defense 2018–2023 and Pfizer (2017–2018). Laura M. Fayad has received support from GERRAF 2008–-10 and Siemens 2011–12. There is no conflict of interest.

Ethical approval

We confirm that we have read the Journal’s position on issues involved in ethical publication and affirm that this report is consistent with those guidelines.

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Electronic supplementary material

ESM 1

(DOCX 25 kb)

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Luna, R., Fayad, L.M., Rodriguez, F.J. et al. Imaging of non-neurogenic peripheral nerve malignancy—a case series and systematic review. Skeletal Radiol 50, 201–215 (2021). https://doi.org/10.1007/s00256-020-03556-z

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00256-020-03556-z

Keywords

Navigation