Abstract
Malignant peripheral nerve sheath tumours (MPNSTs) are malignant tumours arising from a peripheral nerve or displaying nerve sheath differentiation. Most MPNSTs are found on the head, body trunk and extremities, whereas cases in the gastrointestinal are extremely rare. About half arise in neurofibromatosis type 1 patients and 10% arise post-irradiation. This is probably the first small bowel MPNST post-radiation therapy case reported. A 72-year-old female who received radiotherapy 30 years ago for cervical cancer was admitted with progressive abdominal pain and weight loss. Computed tomography revealed a mass with inhomogeneous enhancement in the lumen of the small intestine. Tumour excision was performed with ileocecal and sigmoid colon resection due to suspicion for peripheral tissue invasion. Histopathological examination revealed spindle-shaped cells with focal cartilage differentiation. Together with immunochemistry stain showing complete loss of H3K27me3, a final diagnosis of MPNST was made. The patient is presently under regular follow-ups, and has remained disease-free for 24 months.
Similar content being viewed by others
Data availability
All data underlying the results are available as part of the article and no additional source data are required.
References
Anghileri M, Miceli R, Fiore M et al (2006) Malignant peripheral nerve sheath tumors: prognostic factors and survival in a series of patients treated at a single institution. Cancer 107:1065–1074. https://doi.org/10.1002/cncr.22098
Ducatman BS, Scheithauer BW, Piepgras DG et al (1986) Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases. Cancer 57:2006–2021. https://doi.org/10.1002/1097-0142(19860515)57:10%3c2006::aid-cncr2820571022%3e3.0.co;2-6
LaFemina J, Qin L-X, Moraco NH et al (2013) Oncologic outcomes of sporadic, neurofibromatosis-associated, and radiation-induced malignant peripheral nerve sheath tumors. Ann Surg Oncol 20:66–72. https://doi.org/10.1245/s10434-012-2573-2
Mavrogenis AF, Pala E, Guerra G, Ruggieri P (2012) Post-radiation sarcomas. Clinical outcome of 52 Patients. J Surg Oncol 105:570–576. https://doi.org/10.1002/jso.22122
Yamanaka R, Hayano A (2017) Radiation-induced malignant peripheral nerve sheath tumors: a systematic review. World Neurosurg 105:961-970.e8. https://doi.org/10.1016/j.wneu.2017.06.010
Zhu LB, Li PF, Xiao WH et al (2017) A distal ileum malignant peripheral nerve sheath tumor causing intussusception in a patient in China: a case report. World J Surg Oncol. https://doi.org/10.1186/s12957-017-1101-z
Louis DN, Ohgaki H, Wiestler OD et al (2007) The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol 114:97–109. https://doi.org/10.1007/s00401-007-0243-4
Farid M, Demicco EG, Garcia R et al (2014) Malignant peripheral nerve sheath tumors. Oncologist 19:193–201. https://doi.org/10.1634/theoncologist.2013-0328
Nozu T, Takahashi A, Asakawa H et al (1995) Malignant intestinal schwannoma: a case report and a review of the literature in Japan. Intern Med 34:1101–1105. https://doi.org/10.2169/internalmedicine.34.1101
Hansen D, Pedersen A, Pedersen KM (1990) Malignant intestinal schwannoma. Case report. Acta Chir Scand 156:729–732
Wada Y, Hiramatsu K, Katou T et al (2016) A case of a rapidly growing malignant peripheral nerve sheath tumor of the small intestine presenting with bowel obstruction. Nihon Rinsho Geka Gakkai Zasshi (J Jpn Surg Assoc) 77:1116–1121. https://doi.org/10.3919/jjsa.77.1116
Cahan WG, Woodard HQ, Higinbotham NL et al (1998) Sarcoma arising in irradiated bone: report of eleven cases. 1948. Cancer 82:8–34. https://doi.org/10.1002/(sici)1097-0142(19980101)82:1%3c8::aid-cncr3%3e3.0.co;2-w
Bergstrom R (1962) Changes in peripheral nerve tissue after irradiation with high energy protons. Acta Radiol 58:301–312. https://doi.org/10.3109/00016926209169572
Cavanagh JB (1968) Effects of x-irradiation on the proliferation of cells in peripheral nerve during Wallerian degeneration in the rat. Br J Radiol 41:275–281. https://doi.org/10.1259/0007-1285-41-484-275
Scheithauer BW, Erdogan S, Rodriguez FJ et al (2009) Malignant peripheral nerve sheath tumors of cranial nerves and intracranial contents: a clinicopathologic study of 17 cases. Am J Surg Pathol 33:325–338. https://doi.org/10.1097/PAS.0b013e31818d6470
Talamonti MS, Goetz LH, Rao S, Joehl RJ (2002) Primary cancers of the small bowel: analysis of prognostic factors and results of surgical management. Arch Surg 137:564–570. https://doi.org/10.1001/archsurg.137.5.564. (discussion 570-1)
Sordillo PP, Helson L, Hajdu SI et al (1981) Malignant schwannoma–clinical characteristics, survival, and response to therapy. Cancer 47:2503–2509. https://doi.org/10.1002/1097-0142(19810515)47:10%3c2503::aid-cncr2820471033%3e3.0.co;2-3
Rodriguez FJ, Folpe AL, Giannini C, Perry A (2012) Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems. Acta Neuropathol 123:295–319. https://doi.org/10.1007/s00401-012-0954-z
WHO Classification of Tumours Editorial Board (2020) WHO classification of tumors, soft tissue and bone tumours, 5th edn. IARC Press, Lyon
Karamchandani JR, Nielsen TO, van de Rijn M, West RB (2012) Sox10 and S100 in the diagnosis of soft-tissue neoplasms. Appl Immunohistochem Mol Morphol 20:445–450. https://doi.org/10.1097/PAI.0b013e318244ff4b
Prieto-Granada CN, Wiesner T, Messina JL et al (2016) Loss of H3K27me3 expression is a highly sensitive marker for sporadic and radiation-induced MPNST. Am J Surg Pathol 40:479–489. https://doi.org/10.1097/PAS.0000000000000564
Schaefer I-M, Fletcher CD, Hornick JL (2016) Loss of H3K27 trimethylation distinguishes malignant peripheral nerve sheath tumors from histologic mimics. Mod Pathol 29:4–13. https://doi.org/10.1038/modpathol.2015.134
Lee W, Teckie S, Wiesner T et al (2014) PRC2 is recurrently inactivated through EED or SUZ12 loss in malignant peripheral nerve sheath tumors. Nat Genet 46:1227–1232. https://doi.org/10.1038/ng.3095
Pekmezci M, Cuevas-Ocampo AK, Perry A, Horvai AE (2017) Significance of H3K27me3 loss in the diagnosis of malignant peripheral nerve sheath tumors. Mod Pathol 30:1710–1719. https://doi.org/10.1038/modpathol.2017.97
Otsuka H, Kohashi K, Yoshimoto M et al (2018) Immunohistochemical evaluation of H3K27 trimethylation in malignant peripheral nerve sheath tumors. Pathol Res Pract 214:417–425. https://doi.org/10.1016/j.prp.2017.12.015
Mustapar N, Zawawi MSF, Tuan Sharif SE (2020) The value of H3K27me3 immunohistochemistry in differentiating malignant peripheral nerve sheath tumour with its histologic mimickers. Asian Pac J Cancer Prev 21:699–705. https://doi.org/10.31557/APJCP.2020.21.3.699
James AW, Shurell E, Singh A et al (2016) Malignant peripheral nerve sheath tumor. Surg Oncol Clin N Am 25:789–802. https://doi.org/10.1016/j.soc.2016.05.009
Wong WW, Hirose T, Scheithauer BW et al (1998) Malignant peripheral nerve sheath tumor: analysis of treatment outcome. Int J Radiat Oncol Biol Phys 42:351–360. https://doi.org/10.1016/s0360-3016(98)00223-5
Wanebo JE, Malik JM, VandenBerg SR et al (1993) Malignant peripheral nerve sheath tumors. A clinicopathologic study of 28 cases. Cancer 71:1247–1253. https://doi.org/10.1002/1097-0142(19930215)71:4%3c1247::aid-cncr2820710413%3e3.0.co;2-s
Kahn J, Gillespie A, Tsokos M et al (2014) Radiation therapy in management of sporadic and neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors. Front Oncol 4:324. https://doi.org/10.3389/fonc.2014.00324
Zou C, Smith KD, Liu J et al (2009) Clinical, pathological, and molecular variables predictive of malignant peripheral nerve sheath tumor outcome. Ann Surg 249:1014–1022. https://doi.org/10.1097/SLA.0b013e3181a77e9a
Gladdy RA, Qin L-X, Moraco N et al (2010) Do radiation-associated soft tissue sarcomas have the same prognosis as sporadic soft tissue sarcomas? J Clin Oncol 28:2064–2069. https://doi.org/10.1200/JCO.2009.25.1728
Watson KL, Al Sannaa GA, Kivlin CM et al (2017) Patterns of recurrence and survival in sporadic, neurofibromatosis type 1-associated, and radiation-associated malignant peripheral nerve sheath tumors. J Neurosurg 126:319–329. https://doi.org/10.3171/2015.12.JNS152443
Author information
Authors and Affiliations
Corresponding author
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
About this article
Cite this article
Siripongvutikorn, Y., Noura, S., Nakata, K. et al. A distal ileum malignant peripheral nerve sheath tumour after abdominal radiation therapy: case report of a rare tumour. Int Canc Conf J 13, 1–5 (2024). https://doi.org/10.1007/s13691-023-00625-7
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s13691-023-00625-7