Abstract
To present four rare cases of peripheral primitive neuroectodermal tumors of different sites of head and neck region. Four cases of different age (range 8–40 years) and sex (three female, one male) with rare primitive neuroectodermal tumor of sinonasal region and neck are presented. Treatment options, biological behavior and prognostic outcome are discussed herewith. Two patients succumbed to the disease within four to six months of treatment; other two patients are still under follow-up depicting the aggressive nature of the tumor. Primitive neuroectodermal tumor belongs to the class of malignant round cell tumor. Immunohistochemistry plays a pivotal role in differentiating this tumor entity. Chemoradiation was tried, but local and systemic spread occurs early and holds poor prognosis. This case series is an attempt to describe the aggressive behavior of this rare tumor with high mortality.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Stout AP (1918) A tumor of the ulnar nerve. Proc NY Pathol Soc 18:2–11
Jurgens H, Bier V, Harms D, et al. (1988) Malignant peripheral neuroectodermal tumors. A retrospective analysis of 42 patients. Cancer 61(2):349–357
Kushner BH, Hajdu SI, Gulati SC, et al. (1991) Extracranial primitive neuroectodermal tumors. The Memorial Sloan-Kettering Cancer Center experience. Cancer Apr 1 67(7): 1825–1829
Marina NM, Etcubanas E, Parham DM, et al. (1989) Peripheral primitive neuroectodermal tumor (peripheral neuroepithelioma) in children. A review of the St. Jude experience and controversies in diagnosis and management. Cancer 64(9):1952–1960
Dehner LP (1993) Primitive neuroectodermal tumor and Ewing’s sarcoma. Am J Surg Pathol 17:1–13
Sabate JM, Franquet I, Parellada JA, et al. (1994) Malignant neuroectodermal tumor of the chest wall (Askin tumor): CT and MR findings in eight patients. Clin Radiol 49: 634–638
Jones JE, McGill T (1995) Peripheral primitive neuroectodermal tumors of the head and neck. Arch Otolaryngol Head Neck Surg 121(12):1392–1395
Miser JS, Kinsella TJ, Triche TJ, et al. (1987) Treatment of peripheral neuroepithelioma in children and young adults. J Clin Oncol 5:1752–1758
Carvajal R, Meyers P (2005) Ewing’s sarcoma and primitive neuroectodermal family of tumors. Hematol Oncol Clin North Am 19(3):501–25, vi–vii
Kuttesch JF, Wexler LH, Marcus RB, et al. (1996) Second malignancies after Ewing’s sarcoma: radiation dose-dependency of secondary sarcomas. J Clin Oncol Oct 14(10): 2818–2825
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Ghosh, A., Saha, S., Pal, S. et al. Peripheral primitive neuroectodermal tumor of head-neck region: our experience. Indian J Otolaryngol Head Neck Surg 61, 235–239 (2009). https://doi.org/10.1007/s12070-009-0074-9
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12070-009-0074-9