Most of the diagnostic and treatment paradigms that we apply in the care of children with ovarian tumors are still based on the now somewhat dated concepts that were developed for women with epithelial ovarian cancer. Although there some similarities, the biology of germ cell tumors is typically different than that of epithelial cancers and therefore they should probably be approached differently. The unique psychosocial needs of adolescents and the importance of preserving sexual function and fertility are also factors to consider when determining the correct surgical approach. Nevertheless, based on the results of more recent clinical trials and the experience of many pediatric surgical oncologists, these protocols are gradually being updated and appropriately individualized.
As a rule, every ovarian mass in a young girl should be assumed to be malignant until proven otherwise. This even applies to large ovarian cysts (larger than 8 cm or growing rapidly) and mature teratomas, which though benign can contain immature (glial) elements that can seed the peritoneum (gliomatosis peritonei) or harbor a malignancy. In addition, a benign neoplasm (mucinous cystadenoma) can look just like a simple benign cyst but is associated with a risk of recurrence if incompletely excised or if its contents are spilled. Consequently, when treating a young patient with an ovarian mass: (1) surgical intervention should be undertaken without excessive delay, (2) serum tumor markers (AFP, βHCG) should be sent as part of the preoperative work up in all cases, and (3) the surgical approach must not put the patient at risk for spillage and subsequent up-staging of the tumor. This sometimes makes it difficult to consider a minimally invasive approach, which greatly increases the risk of spillage. As a general rule, laparoscopy should be used only in cases in which spillage is thought to be unlikely (small mature teratoma) or harmless (thin-walled simple cyst). When in doubt, it is recommended that a Pfannenstiel or lower midline incision be used and that precautions be taken to avoid even microscopic spillage. Tumors should be removed en bloc and with meticulous technique. To avoid a big incision when dealing with a large cyst, many surgeons will drain the cyst prior to performing the resection. This is technically considered spillage regardless of what precautions are taken. Some have resorted to creative maneuvers such as using cyanoacrylate adhesive to attach sterile plastic sheeting to the capsule of the tumor and thus create a barrier that is presumably impervious to spillage. Nevertheless, there is on-going debate as to the best way to balance the need to avoid harm (tumor spread) and the desire to minimize scarring.
Despite a lack of supportive data, traditional gynecology oncology guidelines often recommend ipsilateral salpingo-oophorectomy in all patients with an ovarian tumor. For most tumor types seen in girls, however, this is probably excessive. The approach recommended for young women with an ovarian mass is to preserve the fallopian tube unless it is directly involved with tumor and, when possible, to preserve part of the capsule of the ovary, which is where the ova reside. When the nature of the mass is not known, one can remove the entire mass using an ovary-sparing technique and send it for frozen-section analysis before deciding whether the ovary needs to be removed. When there is obvious metastatic disease, spillage is less of an issue and cyst drainage or incisional biopsy are acceptable. Although some surgical oncologists cringe at the concept of “tumor debulking,” ovarian cancer is one of the few tumors for which reducing the gross volume of tumor is palliative and, in some cases, might also improve survival. Inspection and selective biopsy of suspicious iliac and para-aortic lymph nodes should be performed as part of the staging process but formal lymph node dissection is unlikely to be therapeutic and is associated with significant complications.