Ovarian Tumors. Andrea Hayes-Jordan MD FACS, FAAP Section Chief, Pediatric Surgery/Surgical Onc. UT MD Anderson Cancer Center

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1 Ovarian Tumors Andrea Hayes-Jordan MD FACS, FAAP Section Chief, Pediatric Surgery/Surgical Onc. UT MD Anderson Cancer Center

2 Case 13yo female with abdominal pain Ultrasound shows huge ovarian mass Surgeon performs unilateral oophorectomy No further therapy Pathology reveals Yolk sac tumor Repeat imaging 3 months later shows diffuse carcinomatosis (The tumor was not reported ruptured initially)

3 Ovarian Masses Ovarian masses in children have at least a 10 to 20% incidence of malignancy o o o o Germ Cell/Yolk sac tumor Choriocarcinoma Embryonal Carcinoma Ovarian stromal tumors (sertoyli leydig, sex cord tumors)

4 Cancer, 2010 SEER

5 Ovarian Germ Cell Tumors Stage Extent of Disease I Limited to ovary (peritoneal evaluation should be negative). No clinical, radiographic, or histologic evidence of disease beyond the ovaries. (Note: The presence of gliomatosis peritonei does not result in changing Stage I disease to a higher stage.) II Microscopic residual; peritoneal evaluation negative. (Note: The presence of gliomatosis peritonei does not result in changing Stage II disease to a higher stage.) III Lymph node involvement (metastatic nodule); gross residual or biopsy only; contiguous visceral involvement (omentum, intestine, bladder); peritoneal evaluation positive for malignancy. IV Distant metastases, including liver.

6 Ovarian Germ Cell- COG Stage Strata Treatment 1 Low risk surgery alone, follow serum markers 2-3 Intermediate Risk surgery, chemotherapy with PEB 4 High Risk No open protocol

7 Ovarian Germ Cell tumors Preoperative (or Intraoperative): serum markers alpha fetoprotein, beta hcg. Surgery: o Abdominal exploration, o peritoneal fluid/washings for cytology, o intact oophorectomy, o uninvolved fallopian tube and uterus left alone.

8 Ovarian Germ Cell Tumors Intergroup trials demonstrated a yield of close to 25% positivity from peritoneal cytology and a low yield from biopsy of normal appearing lymph nodes, ommentum and contralateral ovary. Staging guidelines have been modified to reflect the yield of each portion of the staging procedure. Stage I tumors with incomplete surgical staging will upgrade to Stage II and receive chemotherapy.

9 Ovarian Germ Cell tumors Operative note must comment on presence of peritoneal implants, omental adherence, lymphadenopathy, tumor rupture, and contralateral ovary, or will be upstaged to Stage II. Stage I tumors treated with SURGERY ALONE

10 What the surgeon should have done Washings or send ascites Inspect pelvic lymph nodes, if enlarged biopsy Inspect other ovary Inspect and comment on ommentum and diaphragm Biopsy necessary only upon suspicion

11 Gliomatosis Peritonei Benign

12 Gliomatosis Peritonei Benign

13 Ovarian Germ Cell Tumors -Gliomatosis peritonei does not result in upstaging, does not need to be completely excised. -Placement of central venous access is not mandatory, left to discretion of practitioner. -Metal clips should not be used.

14 Bilateral ovarian disease Preservation of normal ovarian parenchyma encouraged. For discrete lesion with demarcated capsule, may be excised. If no evidence of normal ovarian tissue, remove larger ovarian tumor. If second ovary >10 cm, remove. If diagnosis of second ovary in doubt, biopsy and await permanent section.

15 Questions?