Twenty five year old woman, married for one year, presented in December 2000 to the Ob/Gyn Outpatient department with pain in the abdomen of 3 months duration. She suffered from irregular menstrual cycles lasting for 5-10 days occurring every 15 days to 2 months. Intraoperatively, there was a 25x20 cm mass replacing the left ovary. Uterus appeared normal. Bowel, kidneys, omentum and undersurface of the diaphragm were normal.

Gross: The left ovarian tumor sent for histopathology consisted of a partly cystic and solid mass measuring 21x14 cm with a large defect on the surface. The cut surface showed variegated consistency with hemorrhage, necrosis and multiloculated cystic areas. Histopathology images are shown in figures labeled A to G.

This patient was lost to follow up after discharge, but presented again in December 2001 with complaints of pain in the abdomen and mass. Preoperatively, there was hemorrhagic ascites, and a fleshy growth arising from the omentum measuring 15x12cm and adherent to the serosa of sigmoid colon. Uterus, right ovary and other abdominal organs were normal.

Gross: Multiple gray brown friable masses of tissue were received, largest measured 13x10x7 cm. On cut surface, there were areas of myxoid appearance, hemorrhage, and necrosis . (Figs H to L)

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Krishnan R. Iyengar, MD, DNB
JIPMER Hospital
Pondicherry, India

The 13 responses I received may be categorized broadly into:

Germ cell tumors: 5
i. Mixed Germ Cell Tumour with features of Endodermal Sinus tumour and Embryonal Carcinoma,
ii. Ovarian choriocarcinoma,
iii. endodermal sinus tumor(yolk sac tumor) with polyvesicular vitelline pattern and syncytiotrophoblastic component,
iv. DD 1- dysgerminoma ; 2-malignant change in the neural component of a pre existing dermoid,
v. Germ cell tumors:1.Yolk sac tumor 2.Dysgerminoma

Mixed mullerian tumor: 3
i. Malignant Mixed Mullerian tumour of ovary with metastasis ; DD - Metastatic Lobular Carcinoma
ii. 1- LEIOMIOSARCOMA MIXOIDE PLEOMORFICO
2- TUMOR MULLERIANO MIXTO MALIGNO.
iii. carcinosarcoma

Sex cord stromal tumor: 3
i. Poorly differentiated Sertoli Leydig cell tumor with heterologous elements.
ii. Malignant granulosa cell tumor
iii. granular cell tumor (a typographic error, I presume}

MFH: 1

Undifferentiated carcinoma: 1

Our own diagnosis in the earlier biopsy was a mixed germ cell tumor. However, with the biopsy in 2001, the earlier slides were also reviewed. The glomeruloid structures (depicted in images C and D) were very striking. The tubule formation is fairly obvious in several fields. Images B, I and J show the small round cell appearance of primitive blastema. The spindle cell stroma is seen in image E. Heterologous elements like osteoid and spindle cell sarcoma-like areas are present in the other images. With all these features and the fact that the kidneys were found to be normal during surgery, we considered a Primary Ovarian Wilm's tumor.

These tumors are rare, and do occur in adults. The closest differentials are teratoma with wilm's tumor-like areas and sertoli-Leydig cell tumor. Mixed mullerian tumors are not expected to have small round cell areas. The hyaline globules that one sees in yolk sac tumors were also conspicuously absent.

References:
1. Sahin A, Benda JA. Primary ovarian Wilms' tumor. Cancer 1988 Apr 1;61(7):1460-3.
2. Isaac MA, Vijayalakshmi S, Madhu CS, Bosincu L, Nogales FF. Pure cystic nephroblastoma of the ovary with a review of extrarenal Wilms' tumors. Hum Pathol 2000 Jun;31(6):761-4.
3. Pereira F, Carrascal E, Canas C, Florez L. Extrarenal Wilms tumor of the left ovary: a case report. J Pediatr Hematol Oncol 2000 Jan-Feb;22(1):88-9.

Krishnan.