Autopsy and Case Reports
https://app.periodikos.com.br/journal/autopsy/article/doi/10.4322/acr.2024.499
Autopsy and Case Reports
Clinical Case Report

Bilateral primary ovarian Ewing sarcoma recurring as left submandibular lymphadenopathy diagnosed on cytology

Shaivy Malik; Neha Kawatra Madan; Meetu Agrawal; Rajni Yadav; Adarsh Barwad

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Abstract

Ewing sarcoma (ES) is a highly malignant and aggressive small round-cell tumor originating from primitive neuroepithelium and mesenchymal stem cells. It is usually seen in children and adolescents with a male predilection and a preponderance to occur in long bones. Although skeletal/soft tissue ES is encountered in clinical practice, primary ES of the genital tract, particularly bilateral primary ovarian ES, is highly uncommon, with only a handful of cases reported worldwide. Ovarian ES is occasionally reported to involve para-aortic and pelvic lymph nodes in advanced stages. Still, cervical lymph node metastasis from ovarian ES is an infrequent clinical occurrence and, when present, indicates a worse prognosis. Here, we present an intriguing case of bilateral peripheral primary ovarian ES in an adult female, recurring as metastasis in the left submandibular lymph node. This case underlines the importance of keeping metastasis from ES as a possible differential while diagnosing metastatic small round cell tumors in peripheral lymph nodes. It also highlights the usefulness of a minimally invasive diagnostic modality of fine needle aspiration cytology and cell block preparation with applied ancillary techniques of immunohistochemistry and confirmatory molecular testing by fluorescence in-situ hybridization (FISH), for an accurate and quick diagnosis of such entities. The cytological diagnosis of our patient helped in the prompt and early initiation of chemotherapy without requiring any invasive procedure.

Keywords

Ovary, Cytology, Neoplasm Metastasis, Lymph Nodes, Molecular Conformation

References

Yoshida A. Ewing and Ewing-like sarcomas: a morphological guide through genetically-defined entities. Pathol Int. 2023;73(1):12-26. http://doi.org/10.1111/pin.13293. PMid:36484765.

Gupta D, Ahuja US, Puri N, Singh S, Kumar G. Ewing’s sarcoma of mandible: a case report with review. Int J Clin Pediatr Dent. 2019;12(5):470-4. http://doi.org/10.5005/jp-journals-10005-1665. PMid:32440057.

Lee ES, Hwangbo W, Kim I. Ewing’s sarcoma/primitive neuroectodermal tumor of the uterine corpus. J Pathol Transl Med. 2015;49(1):66-70. http://doi.org/10.4132/jptm.2014.10.14. PMid:25812660.

Gao L, Zhu Y, Shi X, Gao Z, Chen X. Peripheral primitive neuroectodermal tumors: a retrospective analysis of 89 cases and literature review. Oncol Lett. 2019;18(6):6885-90. http://doi.org/10.3892/ol.2019.11011. PMid:31788128.

Purkayastha A, Sharma N, Kapur A, Sahai K. Primitive neuroectodermal tumor of lung in adult with hemorrhagic brain metastasis: an extremely rare case scenario. Indian J Med Paediatr Oncol. 2017;38(1):84-7. http://doi.org/10.4103/0971-5851.203491. PMid:28469346.

Ng TL, O’Sullivan MJ, Pallen CJ, et al. Ewing sarcoma with novel translocation t(2;16) producing an in-frame fusion of FUS and FEV. J Mol Diagn. 2007;9(4):459-63. http://doi.org/10.2353/jmoldx.2007.070009. PMid:17620387.

Li YP, Chang K, Chen TW, Lee SP, Chen CA, Cheng WF. Primary ewing family of tumor arising in the ovary: a case report. Int J Gynecol Pathol. 2019;38(5):470-3. http://doi.org/10.1097/PGP.0000000000000536. PMid:30085939.

Modi S, Vasdev N, Gupta P, Goyal H. Ewing sarcoma arising in colon and ovary: two rare sites of presentation. Indian J Pathol Microbiol. 2022;65(2):433-6. PMid:35435388.

Takeshima N, Hirai Y, Umayahara K, Fujiwara K, Takizawa K, Hasumi K. Lymph node metastasis in ovarian cancer: difference between serous and non-serous primary tumors. Gynecol Oncol. 2005;99(2):427-31. http://doi.org/10.1016/j.ygyno.2005.06.051. PMid:16112718.

Li J, Sun P, Ma L, et al. Ewing Sarcoma and Primitive Neuroectodermal Tumor of the Thoracic Esophagus: Case Report and Comprehensive Literature Review. Case Rep Oncol. 2022;15(1):267-76. http://doi.org/10.1159/000522152. PMid:35529299.

World Health Organization (WHO). WHO classification of tumours of soft tissue and bone. 5th ed. Lyon: IARC Press; 2020.

Delattre O, Zucman J, Melot T, et al. The Ewing family of tumors--a subgroup of small-round-cell tumors defined by specific chimeric transcripts. N Engl J Med. 1994;331(5):294-9. http://doi.org/10.1056/NEJM199408043310503. PMid:8022439.

Chiang S, Snuderl M, Kojiro-Sanada S, et al. Primitive neuroectodermal tumors of the female genital tract: a morphologic, immunohistochemical, and molecular study of 19 cases. Am J Surg Pathol. 2017;41(6):761-72. http://doi.org/10.1097/PAS.0000000000000831. PMid:28296680.

Chen S, Deniz K, Sung YS, Zhang L, Dry S, Antonescu CR. Ewing sarcoma with ERG gene rearrangements: a molecular study focusing on the prevalence of FUS-ERG and common pitfalls in detecting EWSR1-ERG fusions by FISH. Genes Chromosomes Cancer. 2016;55(4):340-9. http://doi.org/10.1002/gcc.22336. PMid:26690869.

Kilpatrick SE, Reith JD, Rubin B. Ewing Sarcoma and the history of similar and possibly related small round cell tumors: from whence have we come and where are we going? Adv Anat Pathol. 2018;25(5):314-26. http://doi.org/10.1097/PAP.0000000000000203. PMid:29911999.

Sadiq M, Ahmad I, Shuja J, Ahmad K. Primary Ewing sarcoma of the kidney: a case report and treatment review. CEN Case Rep. 2017;6(2):132-5. http://doi.org/10.1007/s13730-017-0259-0. PMid:28509141.

Guo AW, Liu YS, Li H, Yuan Y, Li SX. Ewing sarcoma of the ileum with wide multiorgan metastases: a case report and review of literature. World J Gastrointest Oncol. 2022;14(8):1585-93. http://doi.org/10.4251/wjgo.v14.i8.1585. PMid:36160753.

Askri A, Farhat LB, Ghariani B, et al. Extraskeletal Ewing sarcoma of the abdominal wall. Cancer Imaging. 2008;8(1):156-8. http://doi.org/10.1102/1470-7330.2008.0023. PMid:18818133.

Chen L, Long C, Liu J, Xing F, Duan X. Characteristics and prognosis of pelvic Ewing sarcoma: a SEER population-based study. PeerJ. 2019;7:e7710. http://doi.org/10.7717/peerj.7710. PMid:31576245.

Kabata P, Kaniuka-Jakubowska S, Kabata W, et al. Primary ewing sarcoma of the thyroid-eight cases in a decade: a case report and literature review. Front Endocrinol. 2017;8:257. http://doi.org/10.3389/fendo.2017.00257. PMid:29163353.

Das S, Mishra SS, Rakshit K, Agarwal R. Case report: primary Ewing sarcoma of the penis. Indian J Surg Oncol. 2023;14(3):752-4. http://doi.org/10.1007/s13193-023-01793-x. PMid:37900645.

Chao X, Bi Y, Li L. Ovarian primary primitive neuroectodermal tumor: a review of cases at PUMCH and in the published literature. Orphanet J Rare Dis. 2019;14(1):147. http://doi.org/10.1186/s13023-019-1106-5. PMid:31217017.

Wu YC, Kao YC, Chang CW. Primary uterine Ewing sarcoma: a case report. Taiwan J Obstet Gynecol. 2021;60(1):142-4. http://doi.org/10.1016/j.tjog.2020.11.022. PMid:33494989.

Kyriazoglou A, Tsironis G, Liontos M, et al. Ewing’s sarcoma of the cervix: a case report of an unusual diagnosis in pregnancy treated with surgery, adjuvant VIDE and radiotherapy. Oncol Lett. 2019;17(6):5529-35. http://doi.org/10.3892/ol.2019.10267. PMid:31186774.

Dehner LP. Primitive neuroectodermal tumor and Ewing’s sarcoma. Am J Surg Pathol. 1993;17(1):1-13. http://doi.org/10.1097/00000478-199301000-00001. PMid:8383465.

López F, Rodrigo JP, Silver CE, et al. Cervical lymph node metastases from remote primary tumor sites. Head Neck. 2016;38(Suppl 1):E2374-85. http://doi.org/10.1002/hed.24344. PMid:26713674.

Dvoretsky PM, Richards KA, Angel C, et al. Distribution of disease at autopsy in 100 women with ovarian cancer. Hum Pathol. 1988;19(1):57-63. http://doi.org/10.1016/S0046-8177(88)80316-2. PMid:3335391.

Kim KJ, Jang BW, Lee SK, Kim BK, Nam SL. A case of peripheral primitive neuroectodermal tumor of the ovary. Int J Gynecol Cancer. 2004;14(2):370-2. http://doi.org/10.1136/ijgc-00009577-200403000-00027. PMid:15086740.

Ateser G, Yildiz O, Leblebici C, et al. Metastatic primitive neuroectodermal tumor of the ovary in pregnancy. Int J Gynecol Cancer. 2007;17(1):266-9. http://doi.org/10.1111/j.1525-1438.2006.00761.x. PMid:17291265.

Kuk JY, Yoon SY, Kim MJ, Lee JW, Kim BG, Bae DS. A case of primitive neuroectodermal tumor of the ovary. Korean J Obstet Gynecol. 2012;55(10):777-81. http://doi.org/10.5468/KJOG.2012.55.10.777.

Ahadi R, Tavakol N, Babaheidarian P, Abbasi Y. Ovarian Primitive Neuroectodermal Tumor (PNET): a case report. GinPolMedProject. 2018;3(49):23-6.

Jiménez-Heffernan JA, Rodríguez-García AM, Cima L, Gordillo CH, López-Ferrer P, Vicandi B. A comprehensive review of the “tigroid” background cytological concept: what, when, where and why? Pathologica. 2022;114(2):121-7. http://doi.org/10.32074/1591-951X-287. PMid:35481562.

Pasello M, Manara MC, Scotlandi K. CD99 at the crossroads of physiology and pathology. J Cell Commun Signal. 2018;12(1):55-68. http://doi.org/10.1007/s12079-017-0445-z. PMid:29305692.

Yoshida A, Sekine S, Tsuta K, Fukayama M, Furuta K, Tsuda H. NKX2.2 is a useful immunohistochemical marker for Ewing sarcoma. Am J Surg Pathol. 2012;36(7):993-9. http://doi.org/10.1097/PAS.0b013e31824ee43c. PMid:22446943.

Mulsow J, Jeffers M, McDermott R, Geraghty J, Rothwell J. Complete clinical response to neoadjuvant chemotherapy in a 54-year old male with Askin tumor. Thorac Cardiovasc Surg. 2010;58(5):306-8. http://doi.org/10.1055/s-0029-1186198. PMid:20680911.

Abboud A, Masrouha K, Saliba M, et al. Extraskeletal Ewing sarcoma: diagnosis, management and prognosis. Oncol Lett. 2021;21(5):354. http://doi.org/10.3892/ol.2021.12615. PMid:33747211.

Ohgaki K, Horiuchi K, Mizutani S, Sato M, Kondo Y. Primary Ewing’s sarcoma/primitive neuroectodermal tumor of the kidney that responded to low-dose chemotherapy with ifosfamide, etoposide, and doxorubicin. Int J Clin Oncol. 2010;15(2):210-4. http://doi.org/10.1007/s10147-010-0031-3. PMid:20186557.
 


Submitted date:
03/28/2024

Accepted date:
04/29/2024

Publication date:
07/12/2024

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