Wilms tumor of the ovary: Review of the literature and report of 2 cases Journal Article


Authors: Turashvili, G.; Fix, D. J.; Soslow, R. A.; Park, K. J.
Article Title: Wilms tumor of the ovary: Review of the literature and report of 2 cases
Abstract: Primary extrarenal Wilms tumor of the gynecologic tract is extremely rare with scattered case reports occurring in the ovary, uterine corpus and cervix. Only 9 cases of primary ovarian Wilms tumor have been reported to date. Here, we provide an extensive literature review and describe 2 patients with ovarian Wilms tumor: a 36-yr-old female (patient 1) and a 16-yr-old female (patient 2), both presenting with abdominal pain and suspected ovarian torsion. They were each found to have unilateral ovarian masses measuring >15 cm in size which were removed by unilateral salpingo-oophorectomy. Microscopically, the tumors exhibited the typical triphasic histology of Wilms tumor. In addition, the tumor from patient 1 contained elements of mature cystic teratoma, while an extensive rhabdomyosarcomatous component was identified in patient 2. Both tumors were diffusely and strongly positive for WT1 with variable staining for other biomarkers. The cases were diagnostically challenging and referred to our center for an expert opinion. Teratoid Wilms tumor in patient 1 is the second reported case of ovarian Wilms tumor arising in association with teratoma. Recognition of primary ovarian Wilms tumor requires a high index of suspicion and exclusion of other entities based on tumor morphology and immunohistochemical studies. Copyright © 2018 by the International Society of Gynecological Pathologists.
Keywords: immunohistochemistry; adolescent; adult; clinical article; human tissue; protein expression; review; microscopy; cancer recurrence; case report; cancer combination chemotherapy; cancer radiotherapy; salpingooophorectomy; computer assisted tomography; ovary cancer; transcription factor gata 3; differential diagnosis; cyclophosphamide; vincristine; abdominal pain; ovary; emergency ward; cancer cell; dactinomycin; epithelium cell; cytoplasm; spindle cell; teratoma; cytokeratin 7; wt1 protein; rhabdomyosarcoma; estrogen receptor; progesterone receptor; frozen section; transcription factor fli 1; epithelioid cell; granulocyte colony stimulating factor; cd99 antigen; nephroblastoma; cytokeratin ae1; cytokeratin ae3; vimentin; common acute lymphoblastic leukemia antigen; wilms tumor; transcription factor pax8; cd56 antigen; ovarian bleeding; steroidogenic factor 1; human; female; priority journal; myod1 protein; homeobox protein nkx 2.1; forkhead box protein l2; blastema; ovary torsion
Journal Title: International Journal of Gynecological Pathology
Volume: 39
Issue: 1
ISSN: 0277-1691
Publisher: Lippincott Williams & Wilkins  
Date Published: 2020-01-01
Start Page: 72
End Page: 78
Language: English
DOI: 10.1097/pgp.0000000000000565
PUBMED: 30516617
PROVIDER: scopus
PMCID: PMC6546561
DOI/URL:
Notes: Review -- Export Date: 3 February 2020 -- Source: Scopus
Altmetric
Citation Impact
BMJ Impact Analytics
MSK Authors
  1. Kay Jung Park
    308 Park
  2. Robert Soslow
    797 Soslow
  3. Daniel Jonas Fix
    10 Fix