Background – Primitive neurocetodermal tumor / Ewing’s sarcoma (PNET/ES) of uterine cervix is extremely rare tumor and only 27 cases have so far been reported in the literature. We hereby present one case report diagnosed by histopathology and IHC and review of literature. Objective –A young girl aged 20 years was admitted. Her routine investigations and CT and MRI scan of chest, abdomen and pelvis were performed. Biopsy was taken from vaginal mass and histopathologist reported as round cell tumor. The slides were reviewed by another pathologist and found to be rhabdomyosarcoma. Thus immunohistopathology was done and a diagnosis of PNET/ES was established. Methods – The patient presented with complains of vaginal bleeding, generalized body ache, poor nutritional intake, found extensive loco regional disease with metastases to liver, lung and multiple bones, stage FIGO IV, performance score ECOG -4. Owing to her low general condition, we could not plan any anti-cancer treatment like chemotherapy or radiation therapy. She was discharged on symptomatic treatment. Results – Her biopsy and immunohistopathology turn out to be PNET/ES of uterine cervix. Conclusion - The rarity of this entity poses a challenge to pathologists and oncologists. The PNET/ES of uterine cervix can be potentially curable if presented at an early stage and found operable. IHC plays an important role in establishing this rare diagnosis.
| Published in | Cancer Research Journal (Volume 8, Issue 4) |
| DOI | 10.11648/j.crj.20200804.12 |
| Page(s) | 62-67 |
| Creative Commons |
This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited. |
| Copyright |
Copyright © The Author(s), 2024. Published by Science Publishing Group |
PNET, PNET Cervix Uteri, Immunohistochemistry, Peripheral, Neuroepithelioma, Uterine Cervix
| [1] | Sato S, Yajima A, Kimura N, Namiki T, Furuhashi N and Sakuma H: Peripheral Neuroepithelioma (Peripheral Primitive Neuroectodermal Tumor) of the Uterine Cervix. Tohoku J Exp Med 1996; 180 (2): 187-195. |
| [2] | Horn LC, Fischer U and Bilek K: Primitive neuroectodermal tumor of the cervix uteri. A case report. Gen Diagn Pathol 1997; 142: 227-230. |
| [3] | Cenacchi G, Pasquinelli G, Montanaro L, Cerasoli S, Vici M, Bisceglia M et al: Primary endocervical extra osseous Ewing's sarcoma/PNET. Int J Gynecol Pathol 1998; 17: 83-88. |
| [4] | Pauwels P, Ambros P, Hattinger C, Lammens M, Dal Cin P, Ribot J et al. Peripheral primitive Neuroectodermal tumour of the cervix. Virchows Arch 2000; 436: 68-73. |
| [5] | Tsao AS, Roth LM, Sandler A and Hurteau JA: Cervical primitive neuroectodermal tumor. Gynecol Oncol 2001; 83: 138-142. |
| [6] | Malpica A and Moran CA: Primitive neuroectodermal tumor of the cervix: A clinicopathologic and immunohistochemical study of two cases. Ann Diagn Pathol 2002; 6: 281-287. |
| [7] | Snijders-Keilholz A, EwingP, Seynaeve C and Burger CW: Primitive Neuroectodermal Tumor of the Cervix Uteri: A Case Report-Changing Concepts in Therapy. Gynecol Oncol 2005; 98 (3): 516-519. |
| [8] | Goda J, Mayur BN, Pramod PK and Udayan K: Primitive neuroectodermal tumour of the Cervix: A rare entity. Internet Journal of Radiology 2006; 6 (1): 1-5. |
| [9] | Farzaneh F, Rezvani H, Boroujeni PT and Rahimi F: Primitive neuroectodermal tumor of the cervix: A case report. J Med Case Rep 2011; 5: 489. |
| [10] | Arora N, Kalra A, Kausar H, Ghosh TK and Majumdar A: Primitive neuroectodermal tumour of uterine cervix-a diagnostic and therapeutic dilemma. J Obstet Gynaecol. 1996; 32: 711-713, 2012. |
| [11] | Masoura S, Kourtis A, KalogiannidisI, Kotoula V, Anagnostou E, Angelidou S et al. Primary Primitive Neuroectodermal Tumor of the Cervix Confirmed with Molecular Analysis in a 23-year-old woman: A case report. Pathol Res Pract 2012; 208 (4): 245-249. |
| [12] | Benbrahim Z, Haie-Medar C, Duvillard P, EL Mesbahi O, Cesne AL and Pautier P. Primitive neuroectodermal tumor of the cervix uteri: a case report and review of literature. Int. J Hematol Oncol Stem Cell Res 2012; 6: 4. |
| [13] | Li B, Ouyang L, Han X, Zhou Y, Tong X, Zhang S et al. Primary primitive neuroectodermal tumor of the cervix. Onco Targets Ther 2013; 6: 707-711. |
| [14] | Khosla D, Rai B, Patel FD, Sreedharanunni S, Dey P and Sharma SC: Primitive neuroectodermal tumor of the uterine cervix diagnosed during pregnancy: A rare case with review of literature. J Obstet Gynaecol Res 2014; 40: 878-882. |
| [15] | Xiao C, Zhao J, Guo P, Wang D, Zhao D, Ren T et al: Clinical analysis of primary primitive neuroectodermal tumors in the female genital tract. Int J Gynecol Cancer 2014; 24: 404-409. |
| [16] | Al-Nueimy WMT and Mahamood SM. Primitive Neuroectodermal Tumor of the Cervix Uteri – A Case Report. Jordan Medical Journal 2014; 48 (4): 285-291. |
| [17] | Weissferdt A, Kalhor N and Moron CA. Ewing’s sarcoma with extensive neural differentiation: a clinicopathological, immunohistochemical, and molecular analysis of three cases. Am J Clin Pathol 2015; 143: 659-64. |
| [18] | Mashriqi N, Gujjarlapudi JK, Sidhu J, Zur M and Yalamanchili M: Ewing's sarcoma of the cervix, a diagnostic dilemma: A case report and review of the literature. J Med Case Rep 2015; 9: 255. |
| [19] | Bílek O, Holánek M, Zvaríková M, Fabian P, Robešová B, Procházková M et al. Extraosseus Ewing’s sarcoma, primary affection of uterine cervix-case report. Klin Onkol 2015; 28: 284-287. (In Czech). |
| [20] | Ahmed I, Chufal KS, Bhargava A and Bashir I. Primitive neurocetodermal tumour of the cervix: A rare diagnosis. BMJ Case Rep 2017; doi: 10. 1136/bcr-2016-217461. |
| [21] | Hu X, Liang J, Ling Y, Li P, Zheng S and Liu S. Pathologic characteristics of primary primitive Neurocetodermal tumor in the cervix: a small round cell malignant tumor with glial and epithelial differentiation after neoadjuvant chemotherapy. Int J Clin Exp Pathol 2017; 10 (7): 7898-7904. |
| [22] | Wang X, Gao Y, Xu Y, Liu Y and Qu P: Primary primitive neuroectodermal tumor of the cervix: A report of two cases and review of the literature. Mol Clin Oncol 2017; 6: 697-700. |
| [23] | Kyriazoglou A, Tsironis G, Liontos M, Papakosta A, Mahaira L, Thomakos N et al. Ewing's sarcoma of the cervix: A case report of an unusual diagnosis in pregnancy treated with surgery, adjuvant VIDE and radiotherapy. Oncology Letters 2019; 17 (6): 5529-5535. |
| [24] | Indelicato D, Marcus RB, Ewing’s Sarcoma. In: Lu JJ, Brady LW, eds. Decision Making in radiation Oncology. Berlin/Heidelberg: Springer Science & Business Media 2011; 1073-1088. |
| [25] | Meshram GG, Kaur N, Hura KS: Ewing’s sarcoma with distant metastases: A brief note on management and emerging therapies. Clinics and Practice 2019; 9 (3): 1111. |
| [26] | ESMO/European Sarcoma Network Working Group: Soft tissue and visceral sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol 2014; 25 (Suppl 3): iii102-iii112. |
| [27] | Xu J, Xie L, Sun X, Dong S, Tang X and Gyo W. Management of recurrent or refractory Ewing Sarcoma: A systematic review of phase II clinical trials in the last 15 years. Oncology Letters 2019; 18 (1): 348-358. |
| [28] | Bailey K, Cost C, Davis I, Glade-Bender J, Grohar P, Houghton P et al. Emerging novel agents for patients with advanced Ewing sarcoma: a report from the Children’s Oncology Group (COG) New Agents for Ewing Sarcoma Task Force [version 1; peer review: 3 approved]. F1000 Research 2019; 8: 493. |
| [29] | Li Q, Liu Y and Yu Y- Antiangiogenetic therapy for primitive neuroectodermal tumor with thalidomide. A case report and review of literature. Medicine 2017; 96: 51: 1-5. |
APA Style
Rajendra Kumar Tanwar, Bharti Saxena, Harsh Goyal, Shagufta Siddiqui, Rakesh Kumar Singh, et al. (2020). Primitive Neuroectodermal Tumor of Cervix: Report of a Rare Case and Review of Literature. Cancer Research Journal, 8(4), 62-67. https://doi.org/10.11648/j.crj.20200804.12
ACS Style
Rajendra Kumar Tanwar; Bharti Saxena; Harsh Goyal; Shagufta Siddiqui; Rakesh Kumar Singh, et al. Primitive Neuroectodermal Tumor of Cervix: Report of a Rare Case and Review of Literature. Cancer Res. J. 2020, 8(4), 62-67. doi: 10.11648/j.crj.20200804.12
AMA Style
Rajendra Kumar Tanwar, Bharti Saxena, Harsh Goyal, Shagufta Siddiqui, Rakesh Kumar Singh, et al. Primitive Neuroectodermal Tumor of Cervix: Report of a Rare Case and Review of Literature. Cancer Res J. 2020;8(4):62-67. doi: 10.11648/j.crj.20200804.12
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Download@article{10.11648/j.crj.20200804.12,
author = {Rajendra Kumar Tanwar and Bharti Saxena and Harsh Goyal and Shagufta Siddiqui and Rakesh Kumar Singh and Ravindra Singh Gothwal and Madhu Saxena},
title = {Primitive Neuroectodermal Tumor of Cervix: Report of a Rare Case and Review of Literature},
journal = {Cancer Research Journal},
volume = {8},
number = {4},
pages = {62-67},
doi = {10.11648/j.crj.20200804.12},
url = {https://doi.org/10.11648/j.crj.20200804.12},
eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.crj.20200804.12},
abstract = {Background – Primitive neurocetodermal tumor / Ewing’s sarcoma (PNET/ES) of uterine cervix is extremely rare tumor and only 27 cases have so far been reported in the literature. We hereby present one case report diagnosed by histopathology and IHC and review of literature. Objective –A young girl aged 20 years was admitted. Her routine investigations and CT and MRI scan of chest, abdomen and pelvis were performed. Biopsy was taken from vaginal mass and histopathologist reported as round cell tumor. The slides were reviewed by another pathologist and found to be rhabdomyosarcoma. Thus immunohistopathology was done and a diagnosis of PNET/ES was established. Methods – The patient presented with complains of vaginal bleeding, generalized body ache, poor nutritional intake, found extensive loco regional disease with metastases to liver, lung and multiple bones, stage FIGO IV, performance score ECOG -4. Owing to her low general condition, we could not plan any anti-cancer treatment like chemotherapy or radiation therapy. She was discharged on symptomatic treatment. Results – Her biopsy and immunohistopathology turn out to be PNET/ES of uterine cervix. Conclusion - The rarity of this entity poses a challenge to pathologists and oncologists. The PNET/ES of uterine cervix can be potentially curable if presented at an early stage and found operable. IHC plays an important role in establishing this rare diagnosis.},
year = {2020}
}
TY - JOUR T1 - Primitive Neuroectodermal Tumor of Cervix: Report of a Rare Case and Review of Literature AU - Rajendra Kumar Tanwar AU - Bharti Saxena AU - Harsh Goyal AU - Shagufta Siddiqui AU - Rakesh Kumar Singh AU - Ravindra Singh Gothwal AU - Madhu Saxena Y1 - 2020/11/16 PY - 2020 N1 - https://doi.org/10.11648/j.crj.20200804.12 DO - 10.11648/j.crj.20200804.12 T2 - Cancer Research Journal JF - Cancer Research Journal JO - Cancer Research Journal SP - 62 EP - 67 PB - Science Publishing Group SN - 2330-8214 UR - https://doi.org/10.11648/j.crj.20200804.12 AB - Background – Primitive neurocetodermal tumor / Ewing’s sarcoma (PNET/ES) of uterine cervix is extremely rare tumor and only 27 cases have so far been reported in the literature. We hereby present one case report diagnosed by histopathology and IHC and review of literature. Objective –A young girl aged 20 years was admitted. Her routine investigations and CT and MRI scan of chest, abdomen and pelvis were performed. Biopsy was taken from vaginal mass and histopathologist reported as round cell tumor. The slides were reviewed by another pathologist and found to be rhabdomyosarcoma. Thus immunohistopathology was done and a diagnosis of PNET/ES was established. Methods – The patient presented with complains of vaginal bleeding, generalized body ache, poor nutritional intake, found extensive loco regional disease with metastases to liver, lung and multiple bones, stage FIGO IV, performance score ECOG -4. Owing to her low general condition, we could not plan any anti-cancer treatment like chemotherapy or radiation therapy. She was discharged on symptomatic treatment. Results – Her biopsy and immunohistopathology turn out to be PNET/ES of uterine cervix. Conclusion - The rarity of this entity poses a challenge to pathologists and oncologists. The PNET/ES of uterine cervix can be potentially curable if presented at an early stage and found operable. IHC plays an important role in establishing this rare diagnosis. VL - 8 IS - 4 ER -
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