Peripheral primitive neuroectodermal tumors: A retrospective analysis of 89 cases and literature review (Review) Erratum in /10.3892/ol.2020.11385

Gao,Liming; Zhu,Yingying; Shi,Xiaohua; Gao,Zhiqiang; Chen,Xingming

doi:10.3892/ol.2019.11011

Peripheral primitive neuroectodermal tumors: A retrospective analysis of 89 cases and literature review (Review)

Erratum in: /10.3892/ol.2020.11385

Authors:
- Liming Gao
- Yingying Zhu
- Xiaohua Shi
- Zhiqiang Gao
- Xingming Chen
View Affiliations

Affiliations: Department of Otolaryngology‑Head and Neck Surgery, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, P.R. China, Department of Pathology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, P.R. China
Published online on: October 18, 2019 https://doi.org/10.3892/ol.2019.11011
Pages: 6885-6890

Metrics: Total Views: 0 (Spandidos Publications: | PMC Statistics: )

Metrics: Total PDF Downloads: 0 (Spandidos Publications: | PMC Statistics: )

Cited By (CrossRef): 0 citations Loading Articles...

Abstract

Peripheral primitive neuroectodermal tumors (pPNETs) are rare, small cell carcinomas with a poor prognosis. The aim of the present study was to describe therapeutic approaches, outcomes and probable prognostic factors. The clinical features, treatments, and outcomes of 89 consecutive patients with pPNET treated at the Peking Union Medical College Hospital from 1999 to 2018 were retrospectively reviewed. A total of 43 males and 46 females were included in the study, with a median age of 25 years (range, 5‑73 years). The predominantly affected regions were the abdomen and pelvis, followed by the thoracopulmonary region. The mean primary tumor size was 12.6 cm (range, 1‑30 cm). A total of 16 patients (18%) initially presented with metastasis. A total of 46 patients received combined therapy, 35 received mono‑therapy, and 8 underwent only biopsy with no further treatment. The period of observation ranged from 1‑232 months. The median overall survival (OS) time was 15 months [95% confidence interval (CI), 9‑21 months], with 3‑ and 5‑year OS rates of 32 and 25%, respectively. Large tumor size [adjusted hazard ratio (aHR)=3.65; 95% CI, 2.07‑6.42; P<0.001), metastasis at initial presentation (aHR=4.34, 95% CI, 2.23‑8.42; P<0.001), and combined modality treatment (aHR=0.16, 95% CI, 0.06‑0.39; P<0.001) were significantly associated with OS. The prognosis of pPNET is, overall, poor. Large tumor size and metastasis at initial presentation are associated with poorer outcomes. This highly malignant tumor requires an aggressive combination of radical resection, chemotherapy and radiotherapy, when indicated.

View Figures

View References

1	Virani MJ and Jain S: Primary intraspinal primitive neuroectodermal tumor (PNET): A rare occurrence. Neurol India. 50:75–80. 2002.PubMed/NCBI
2	Dehner LP: Peripheral and central primitive neuroectodermal tumors. A nosologic concept seeking a consensus. Arch Pathol Lab Med. 110:997–1005. 1986.PubMed/NCBI
3	Coffin CM and Dehner LP: Peripheral neurogenic tumors of the soft tissues in children and adolescents: A clinicopathologic study of 139 cases. Pediatr Pathol. 9:387–407. 1989. View Article : Google Scholar : PubMed/NCBI
4	Ambros IM, Ambros PF, Strehl S, Kovar H, Gadner H and Salzer-Kuntschik M: MIC2 is a specific marker for Ewing's sarcoma and peripheral primitive neuroectodermal tumors. Evidence for a common histogenesis of Ewing's sarcoma and peripheral primitive neuroectodermal tumors from MIC2 expression and specific chromosome aberration. Cancer. 67:1886–1893. 1991. View Article : Google Scholar : PubMed/NCBI
5	Fletcher C: Peripheral neural tumorsFletcher CDM: Diagnostic histopathology of tumors. Edinburgh: CHurchil Livingstone; pp. 1239–1241. 1995
6	Hachitanda Y, Tsuneyoshi M, Enjoji M, Nakagawara A and Ikeda K: Congenital primitive neuroectodermal tumor with epithelial and glial differentiation. An ultrastructural and immunohistochemical study. Arch Pathol Lab Med. 114:101–105. 1990.PubMed/NCBI
7	Votta TJ, Fantuzzo JJ and Boyd BC: Peripheral primitive neuroectodermal tumor associated with the anterior mandible: A case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 100:592–597. 2005. View Article : Google Scholar : PubMed/NCBI
8	Schmidt D, Herrmann C, Jurgens H and Harms D: Malignant peripheral neuroectodermal tumor and its necessary distinction from Ewing's sarcoma. A report from the Kiel Pediatric Tumor Registry. Cancer. 68:2251–2259. 1991. View Article : Google Scholar : PubMed/NCBI
9	Kalantari M, Deyhimi P and Kalantari P: Peripheral Primitive Neuroectodermal Tumor (pPNET) of the Parotid: Report of a rare case. Arch Iran Med. 18:858–860. 2015.PubMed/NCBI
10	Ghosh A, Saha S, Pal S, Saha PV and Chattopadhyay S: Peripheral primitive neuroectodermal tumor of head-neck region: Our experience. Indian J Otolaryngol Head Neck Surg. 61:235–239. 2009. View Article : Google Scholar : PubMed/NCBI
11	Hashimoto H, Enjoji M, Nakajima T, Kiryu H and Daimaru Y: Malignant neuroepithelioma (peripheral neuroblastoma). A clinicopathologic study of 15 cases. Am J Surg Pathol. 7:309–318. 1983. View Article : Google Scholar : PubMed/NCBI
12	Schmidt D, Harms D and Burdach S: Malignant peripheral neuroectodermal tumours of childhood and adolescence. Virchows Arch A Pathol Anat Histopathol. 406:351–365. 1985. View Article : Google Scholar : PubMed/NCBI
13	Jürgens H, Bier V, Harms D, Beck J, Brandeis W, Etspüler G, Gadner H, Schmidt D, Treuner J, Winkler K, et al: Malignant peripheral neuroectodermal tumors. A retrospective analysis of 42 patients. Cancer. 61:349–357. 1988. View Article : Google Scholar : PubMed/NCBI
14	Llombart-Bosch A, Terrier-Lacombe MJ, Peydro-Olaya A and Contesso G: Peripheral neuroectodermal sarcoma of soft tissue (peripheral neuroepithelioma): A pathologic study of ten cases with differential diagnosis regarding other small, round-cell sarcomas. Hum Pathol. 20:273–280. 1989. View Article : Google Scholar : PubMed/NCBI
15	Marina NM, Etcubanas E, Parham DM, Bowman LC and Green A: Peripheral primitive neuroectodermal tumor (peripheral neuroepithelioma) in children. A review of the St. Jude experience and controversies in diagnosis and management. Cancer. 64:1952–1960. 1989. View Article : Google Scholar : PubMed/NCBI
16	Kushner BH, Hajdu SI, Gulati SC, Erlandson RA, Exelby PR and Lieberman PH: Extracranial primitive neuroectodermal tumors. The memorial Sloan-Kettering cancer center experience. Cancer. 67:1825–1829. 1991. View Article : Google Scholar : PubMed/NCBI
17	Jones JE and McGill T: Peripheral primitive neuroectodermal tumors of the head and neck. Arch Otolaryngol Head Neck Surg. 121:1392–1395. 1995. View Article : Google Scholar : PubMed/NCBI
18	Ibarburen C, Haberman JJ and Zerhouni EA: Peripheral primitive neuroectodermal tumors. CT and MRI evaluation. Eur J Radiol. 21:225–232. 1996. View Article : Google Scholar : PubMed/NCBI
19	Kimber C, Michalski A, Spitz L and Pierro A: Primitive neuroectodermal tumours: Anatomic location, extent of surgery, and outcome. J Pediatr Surg. 33:39–41. 1998. View Article : Google Scholar : PubMed/NCBI
20	Zimmermann T, Blutters-Sawatzki R, Flechsenhar K and Padberg WM: Peripheral primitive neuroectodermal tumor: Challenge for multimodal treatment. World J Surg. 25:1367–1372. 2001.PubMed/NCBI
21	Thyavihally YB, Tongaonkar HB, Gupta S, Kurkure PA, Amare P, Muckaden MA and Desai SB: Primitive neuroectodermal tumor of the kidney: A single institute series of 16 patients. Urology. 71:292–296. 2008. View Article : Google Scholar : PubMed/NCBI
22	Demir A, Gunluoglu MZ, Dagoglu N, Turna A, Dizdar Y, Kaynak K, Dilege S, Mandel NM, Yilmazbayhan D, Dincer SI and Gurses A: Surgical treatment and prognosis of primitive neuroectodermal tumors of the thorax. J Thorac Oncol. 4:185–192. 2009. View Article : Google Scholar : PubMed/NCBI
23	Biswas B, Agarwala S, Shukla NK, Deo S, Sharma D, Thulkar S, Vishnubhatla S and Bakhshi S: Evaluation of outcome and prognostic factors in thoracic primitive neuroectodermal tumor: A study of 84 cases. Ann Thorac Surg. 96:2006–2014. 2013. View Article : Google Scholar : PubMed/NCBI
24	Sirivella S and Gielchinsky I: Treatment outcomes in 23 thoracic primitive neuroectodermal tumours: A retrospective study. Interact Cardiovasc Thorac Surg. 17:273–279. 2013. View Article : Google Scholar : PubMed/NCBI
25	Tan Y, Zhang H, Ma GL, Xiao EH and Wang XC: Peripheral primitive neuroectodermal tumor: Dynamic CT, MRI and clinicopathological characteristics-analysis of 36 cases and review of the literature. Oncotarget. 5:12968–12977. 2014. View Article : Google Scholar : PubMed/NCBI
26	Yi X, Liu W, Zhang Y, Xiao D, Yin H, Long X, Li L, Zai H, Chen M, Li W and Sun L: Radiological features of primitive neuroectodermal tumors in intra-abdominal and retroperitoneal regions: A series of 18 cases. PLoS One. 12:e01735362017. View Article : Google Scholar : PubMed/NCBI
27	Verrill MW, Judson IR, Harmer CL, Fisher C, Thomas JM and Wiltshaw E: Ewing's sarcoma and primitive neuroectodermal tumor in adults: Are they different from Ewing's sarcoma and primitive neuroectodermal tumor in children? J Clin Oncol. 15:2611–2621. 1997. View Article : Google Scholar : PubMed/NCBI
28	Rud NP, Reiman HM, Pritchard DJ, Frassica FJ and Smithson WA: Extraosseous Ewing's sarcoma. A study of 42 cases. Cancer. 64:1548–1553. 1989. View Article : Google Scholar : PubMed/NCBI
29	Wexler LH, DeLaney TF, Tsokos M, Avila N, Steinberg SM, Weaver-McClure L, Jacobson J, Jarosinski P, Hijazi YM, Balis FM and Horowitz ME: Ifosfamide and etoposide plus vincristine, doxorubicin, and cyclophosphamide for newly diagnosed Ewing's sarcoma family of tumors. Cancer. 78:901–911. 1996. View Article : Google Scholar : PubMed/NCBI
30	Shamberger RC, LaQuaglia MP, Gebhardt MC, Neff JR, Tarbell NJ, Marcus KC, Sailer SL, Womer RB, Miser JS, Dickman PS, et al: Ewing sarcoma/primitive neuroectodermal tumor of the chest wall: Impact of initial versus delayed resection on tumor margins, survival, and use of radiation therapy. Ann Surg. 238:563–568. 2003.PubMed/NCBI