Case studyAdult extrarenal Wilms tumor of the uterus with teratoid features
Section snippets
Case report
This nulliparous 62-year-old woman had an episode of profuse vaginal bleeding. She was referred to a regional hospital where she was seen to have a large uterine hemorrhagic polypoid mass protruding through the cervix. Surgery revealed an enlarged globular uterus without pelvic adhesions or ascites, and a total abdominal hysterectomy with salpingo-oophorectomy was performed. An initial histopathologic diagnosis of peripheral primitive neuroectodermal tumor (pPNET) was considered. The case was
Materials and methods
Tissues from 18 samples of tumor were fixed in 10% neutral buffered formalin and paraffin embedded. Five-microgram-thick sections were stained with hematoxylin and eosin. Immunohistochemical studies with an avidin-biotin peroxidase complex method used antibodies directed against the following: CD56 (Master Diagnostica [MD], Granada, Spain; monoclonal 56C04, diluted), CD57 -Leu-7- (MD, monoclonal HNK-1, diluted, CD99 MD, monoclonal 013 diluted), neu-n (Chemicon International, Temecula, CA, USA;
Pathology findings
Macroscopically, the uterus revealed an 8-cm sessile, friable, hemorrhagic mass in its fundus that protruded into the endometrial cavity and filled the cervical canal but on cut section was seen to invade only superficially a markedly dilated and thinned myometrium. The adnexa were involutive but otherwise unremarkable.
Microscopically, myometrial invasion was reduced to 3 mm. No areas of endometrioid differentiation were seen other than the normal endometrial glands embedded within the
Discussion
Extrarenal WTs are unusual neoplasms occurring preferentially in children, and they usually involve lumbosacral areas, retroperitoneum, and other midline locations [3], [7], being rare in the testis and adnexa. Most of them are pure tumors with only a small fraction originating from teratoma or germ cell neoplasms.
The female genital tract is nevertheless the second most frequent location, with 19 cases documented to date, 13 of which were found in the uterus, 4 in the ovary, and 2 in the cervix
Acknowledgment
The authors would like to thank Dr T González-Rocha for her assistance.
References (21)
- et al.
Pure cystic nephroblastoma of the ovary with a review of extrarenal Wilms' tumors
Hum Pathol
(2000) - et al.
Extrarenal Wilms' tumor of the uterine corpus
Gynecol Oncol
(2005) - et al.
Teratoid Wilms' tumor: report of a unilateral case
J Pediatr Surg
(2003) - et al.
Teratoid Wilms' tumor: a case report with literature review
J Pediatr Surg
(2006) - et al.
Wilms' tumor of the cervix: a case report and review of the literature
Gynecol Oncol
(2000) - et al.
Wilms' tumor of the ovary: a case report
J Pediatr Surg
(2002) - et al.
Immunohistochemistry of primary malignant neuroepithelial tumors of the kidney: a potential source of confusion? A study of 30 cases from the National Wilms Tumor Study Pathology Center
Hum Pathol
(2007) - et al.
Carcinosarcoma of the uterus with extensive neuroectodermal differentiation
Histopathology
(1996) - García-Galvis OF, Cabrera-Ozoria C, Aneiros-Fernández J, et al. Teratoid carcinosarcoma of the ovary with yolk sac and...
- et al.
Teratoid Wilms' tumor
Cancer
(1984)