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Published in: Pathology & Oncology Research 4/2012

01-10-2012 | Letter to Editor

Primitive Neuroectodermal Tumor of Ovary in a Young Lady, Confirmed with Molecular and Cytogenetic Results—A Rare Case Report with a Diagnostic and Therapeutic Challenge

Authors: Vikas Ostwal, Bharat Rekhi, Vanita Noronha, Ranjan Basak, Sangeeta B. Desai, Amita Maheshwari, Kumar Prabhash

Published in: Pathology & Oncology Research | Issue 4/2012

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Excerpt

Primitive neuroectodermal tumor (PNET) is a small round cell tumor of neuroectodermal origin. It is the most differentiated form of PNET/Ewing’s family of tumors (EFT) [1]. It is the second most common sarcoma among children and usually occurs in the bone and soft tissues [2]. Ewing’s sarcoma/PNET is characterized by a t (11; 22) (q24; q12) chromosomal translocation that leads to formation of a chimeric transcript EWS-FLI1 in 85% cases, presence of which confirms its diagnosis, especially at non-conventional sites [3]. It has been uncommonly documented at sites other than musculoskeletal system, such as kidneys [4]. PNET has been rarely documented in the female genital system, including ovary, with only few cases confirmed by molecular and / or molecular cytogenetic analysis [512]. …
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Literature
1.
go back to reference Enzinger FM, Weiss SW (1995) Primitive neuroectodermal tumors and related lesions. In: Enzinger FM, Weiss SW (eds) Soft tissue tumors, 3rd edn. Mosby-Year Book, St Louis, pp 929–964 Enzinger FM, Weiss SW (1995) Primitive neuroectodermal tumors and related lesions. In: Enzinger FM, Weiss SW (eds) Soft tissue tumors, 3rd edn. Mosby-Year Book, St Louis, pp 929–964
2.
go back to reference Grier HE (1997) The Ewing family of tumors: Ewing’s sarcoma and primitive neuroectodermal tumors. Pediatr Clin North Am 44:991–1004PubMedCrossRef Grier HE (1997) The Ewing family of tumors: Ewing’s sarcoma and primitive neuroectodermal tumors. Pediatr Clin North Am 44:991–1004PubMedCrossRef
3.
go back to reference Aurias A, Rimbaut C, Buffe D, Dubousett MA (1983) Chromosomal translocations in Ewing’s sacoma. New Eng J Med 309:496–497 Aurias A, Rimbaut C, Buffe D, Dubousett MA (1983) Chromosomal translocations in Ewing’s sacoma. New Eng J Med 309:496–497
4.
go back to reference Marley EF, Liapis H, Humphrey PA (1997) Primitive neuroectodermal tumor of the kidney-another enigma: a pathologic, immunohistochemical, and molecular diagnostic study. Am J Surg Pathol 21:354–359PubMedCrossRef Marley EF, Liapis H, Humphrey PA (1997) Primitive neuroectodermal tumor of the kidney-another enigma: a pathologic, immunohistochemical, and molecular diagnostic study. Am J Surg Pathol 21:354–359PubMedCrossRef
5.
go back to reference Rekhi B, Qureshi S, Basak R, Menon S, Desai S, Medhi S, Kurkure P, Maheshwair AM, Jambhekar NA (2010) A primary vaginal Ewing’s sarcoma/Primitive neuroectodermal tumor (PNET) in a young girl with t (11; 22) (q24; q12) (EWSR1-FLI1) positive results: a case report. J Med Case Report 4:88CrossRef Rekhi B, Qureshi S, Basak R, Menon S, Desai S, Medhi S, Kurkure P, Maheshwair AM, Jambhekar NA (2010) A primary vaginal Ewing’s sarcoma/Primitive neuroectodermal tumor (PNET) in a young girl with t (11; 22) (q24; q12) (EWSR1-FLI1) positive results: a case report. J Med Case Report 4:88CrossRef
6.
go back to reference Lawlor ER, Murphy JI, Sorensen PH, Fryer CJ (1997) Metastatic primitive neuroectodermal tumour of the ovary: successful treatment with mega-dose chemotherapy followed by peripheral blood progenitor cell rescue. Med Pediatr Oncol 29:308–312PubMedCrossRef Lawlor ER, Murphy JI, Sorensen PH, Fryer CJ (1997) Metastatic primitive neuroectodermal tumour of the ovary: successful treatment with mega-dose chemotherapy followed by peripheral blood progenitor cell rescue. Med Pediatr Oncol 29:308–312PubMedCrossRef
7.
go back to reference Kawauchi S, Fukuda T, Miyamoto S, Yoshioka J, Shirahama S, Saito T, Tsukamoto N (1998) Peripheral primitive neuroectodermal tumor of the ovary confirmed by CD99 immunostaining, karyotypic analysis, and RT-PCR for EWS/FLI-1 chimeric mRNA. Am J Surg Pathol 22:1417–1422PubMedCrossRef Kawauchi S, Fukuda T, Miyamoto S, Yoshioka J, Shirahama S, Saito T, Tsukamoto N (1998) Peripheral primitive neuroectodermal tumor of the ovary confirmed by CD99 immunostaining, karyotypic analysis, and RT-PCR for EWS/FLI-1 chimeric mRNA. Am J Surg Pathol 22:1417–1422PubMedCrossRef
8.
go back to reference Shigeto K, Toshiro F, Shingo M, Jun-Ichi Y, Syuya S, Toshiaki S (1998) Peripheral primitive neuroectodermal tumor of the ovary confirmed by CD99 immunostaining, karyotypic analysis and RT-PCR for EWS/FLI-1 chimeric mRNA. Am J Surg Pathol 22:1417–1422CrossRef Shigeto K, Toshiro F, Shingo M, Jun-Ichi Y, Syuya S, Toshiaki S (1998) Peripheral primitive neuroectodermal tumor of the ovary confirmed by CD99 immunostaining, karyotypic analysis and RT-PCR for EWS/FLI-1 chimeric mRNA. Am J Surg Pathol 22:1417–1422CrossRef
9.
go back to reference Rangan A, Lobo FD, Rao AA (2003) Primary primitive neuroectodermal tumor of the ovary: a case report. Indian J Pathol Microbiol 46:58–59PubMed Rangan A, Lobo FD, Rao AA (2003) Primary primitive neuroectodermal tumor of the ovary: a case report. Indian J Pathol Microbiol 46:58–59PubMed
10.
go back to reference Kim KJ, Jang BW, Lee SK, Kim BK, Nam SL (2004) A case of peripheral primitive neuroectodermal tumor of the ovary. Int J Gynecol Cancer 14:370–372PubMedCrossRef Kim KJ, Jang BW, Lee SK, Kim BK, Nam SL (2004) A case of peripheral primitive neuroectodermal tumor of the ovary. Int J Gynecol Cancer 14:370–372PubMedCrossRef
11.
go back to reference Ateser G, Yildiz O, Leblebici C, Mandel NM, Unal F, Turna H, Arikan I, Colcaki D (2007) Metastatic primitive neuroectodermal tumor of the ovary in pregnancy. Int J Gynecol Cancer 17:266–269PubMedCrossRef Ateser G, Yildiz O, Leblebici C, Mandel NM, Unal F, Turna H, Arikan I, Colcaki D (2007) Metastatic primitive neuroectodermal tumor of the ovary in pregnancy. Int J Gynecol Cancer 17:266–269PubMedCrossRef
12.
go back to reference Chow SN, Lin MC, Shen J, Wang S, Jong YJ, Chien CH (2004) Analysis of chromosome abnormalities by comparative genomic hybridization in malignant peripheral primitive neuroectodermal tumor of the ovary. Gynecol Oncol 92:752–760PubMedCrossRef Chow SN, Lin MC, Shen J, Wang S, Jong YJ, Chien CH (2004) Analysis of chromosome abnormalities by comparative genomic hybridization in malignant peripheral primitive neuroectodermal tumor of the ovary. Gynecol Oncol 92:752–760PubMedCrossRef
13.
go back to reference Bridge RS, Rajaram V, Dehner LP, Pfeifer JD, Perry A (2006) Molecular diagnosis of Ewing sarcoma/primitive neuroectodermal tumor in routinely processed tissue: a comparison of two FISH strategies and RT-PCR in malignant round cell tumors. Mod Pathol 19:1–8PubMedCrossRef Bridge RS, Rajaram V, Dehner LP, Pfeifer JD, Perry A (2006) Molecular diagnosis of Ewing sarcoma/primitive neuroectodermal tumor in routinely processed tissue: a comparison of two FISH strategies and RT-PCR in malignant round cell tumors. Mod Pathol 19:1–8PubMedCrossRef
14.
go back to reference Kleinman GM, Young RH, Scully RE (1993) Primary neuroectodermal tumors of the ovary. A report of 25 cases. Am J Surg Pathol 17:764–778PubMedCrossRef Kleinman GM, Young RH, Scully RE (1993) Primary neuroectodermal tumors of the ovary. A report of 25 cases. Am J Surg Pathol 17:764–778PubMedCrossRef
15.
go back to reference Anfinan NM, Sait KH, Al-Maghrabi JA (2008) Primitive neuroectodermal tumor of the ovary. Saudi Med J 29:444–446PubMed Anfinan NM, Sait KH, Al-Maghrabi JA (2008) Primitive neuroectodermal tumor of the ovary. Saudi Med J 29:444–446PubMed
16.
go back to reference Ambros IM, Ambros PF, Strehl S, Kovar H, Gardner H, Salzer-Kuntschik M (1991) MIC2 is a specific marker for Ewing’s sarcoma and peripheral primitive neuroectodermal tumors. Cancer 67:1886–1893PubMedCrossRef Ambros IM, Ambros PF, Strehl S, Kovar H, Gardner H, Salzer-Kuntschik M (1991) MIC2 is a specific marker for Ewing’s sarcoma and peripheral primitive neuroectodermal tumors. Cancer 67:1886–1893PubMedCrossRef
17.
go back to reference Gordon MD, Corless C, Renshaw AA, Beckstead J (1998) CD99, keratin, and vimentin staining of sex cord-stromal tumors, normal ovary, and testis. Mod Pathol 11:769–773PubMed Gordon MD, Corless C, Renshaw AA, Beckstead J (1998) CD99, keratin, and vimentin staining of sex cord-stromal tumors, normal ovary, and testis. Mod Pathol 11:769–773PubMed
18.
go back to reference Lin PP, Brody RI, Hamelin AC, Bradner JE, Healey JH, Ladanyi M (1999) Differential transactivation by alternative EWS-FLI1 fusion proteins correlates with clinical heterogeneity in Ewing’s sarcoma. Cancer Res 59:1428–1432PubMed Lin PP, Brody RI, Hamelin AC, Bradner JE, Healey JH, Ladanyi M (1999) Differential transactivation by alternative EWS-FLI1 fusion proteins correlates with clinical heterogeneity in Ewing’s sarcoma. Cancer Res 59:1428–1432PubMed
19.
go back to reference Giovannini M, Biegel JA, Serra M, Wang JY, Wei YH, Nycum L, Emanuel BS, Evans GA (1994) EWS-erg and EWS-Fli1 fusion transcripts in Ewing’s sarcoma and primitive neuroectodermal tumors with variant translocations. J Clin Invest 94:489–496PubMedCrossRef Giovannini M, Biegel JA, Serra M, Wang JY, Wei YH, Nycum L, Emanuel BS, Evans GA (1994) EWS-erg and EWS-Fli1 fusion transcripts in Ewing’s sarcoma and primitive neuroectodermal tumors with variant translocations. J Clin Invest 94:489–496PubMedCrossRef
20.
go back to reference Kovar H, Aryee DN, Jug G, Henockl C, Schemper M, Delattre O, Thomas G, Gadner H (1996) EWS/FLI-1 antagonists induce growth inhibition of Ewing tumor cells in vitro. Cell Growth Differ 7:429–437PubMed Kovar H, Aryee DN, Jug G, Henockl C, Schemper M, Delattre O, Thomas G, Gadner H (1996) EWS/FLI-1 antagonists induce growth inhibition of Ewing tumor cells in vitro. Cell Growth Differ 7:429–437PubMed
21.
go back to reference Toretsky JA, Connell Y, Neckers L, Bhat NK (1997) Inhibition of EWS-FLI-1 fusion protein with antisense oligodeoxynucleotides. J Neurooncol 31:9–16PubMedCrossRef Toretsky JA, Connell Y, Neckers L, Bhat NK (1997) Inhibition of EWS-FLI-1 fusion protein with antisense oligodeoxynucleotides. J Neurooncol 31:9–16PubMedCrossRef
22.
go back to reference Tanaka K, Iwakuma T, Harimaya K, Sato H, Iwamoto Y (1997) EWS-Fli1 antisense oligodeoxynucleotide inhibits proliferation of human Ewing’s sarcoma and primitive neuroectodermal tumor cells. J Clin Invest 99:239–247PubMedCrossRef Tanaka K, Iwakuma T, Harimaya K, Sato H, Iwamoto Y (1997) EWS-Fli1 antisense oligodeoxynucleotide inhibits proliferation of human Ewing’s sarcoma and primitive neuroectodermal tumor cells. J Clin Invest 99:239–247PubMedCrossRef
Metadata
Title
Primitive Neuroectodermal Tumor of Ovary in a Young Lady, Confirmed with Molecular and Cytogenetic Results—A Rare Case Report with a Diagnostic and Therapeutic Challenge
Authors
Vikas Ostwal
Bharat Rekhi
Vanita Noronha
Ranjan Basak
Sangeeta B. Desai
Amita Maheshwari
Kumar Prabhash
Publication date
01-10-2012
Publisher
Springer Netherlands
Published in
Pathology & Oncology Research / Issue 4/2012
Print ISSN: 1219-4956
Electronic ISSN: 1532-2807
DOI
https://doi.org/10.1007/s12253-012-9503-2

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