Sections

Additional Content

INTRODUCTION

• Pediatric ovarian tumors are commonly seen by all pediatric surgeons. 
• The majority of ovarian tumors are not malignant.
• Ovarian preservation is critical in these patients. 
• The frequency of ovarian sparing operations are increasing, but there are still oophorectomies being performed that could have been treated with ovarian preserving procedures.
• Overview of Ovarian Tumors:
• In large scale studies, there is a 10% overall risk of malignancy.
• Malignant tumors
• Germ cell
• This is the predominant type, occurring in 50-80% of cases.
• Most common type is yolk sac tumor.
• Also, embryonal, dysgerminomas and mixed tumors, like yolk saw with mature and immature teratomas.
• Sex chord stromal tumors
• Benign:
• Germ cell
• This also predominates in the benign category.
• Mature teratoma
• Occurs in up to 50% of cases.
• Immature teratoma
• Occurs in 10-15% of cases.
• Functional cysts
• Cystadenoma

CASE I: Ovarian cystic mass with some solid component

• Case I: A 4 year old girl presents with a 1-week history of abdominal pain and was found to have a large, predominantly cystic mass extending from her pelvis into her upper abdomen, with a 2 x 3 cm solid component with calcifications.
• History and Physical
• Always consider torsion in these patients, but this is less likely in a patient with a 1-week history of pain.
• If you see pubic hair in this patient, consider a functional tumor, likely a sex chord stromal tumor.
• This will require an in depth hormonal evaluation of the products of sex chord stroll tumors, such as testosterone and its breakdown levels.
• Workup
• Tumors Marker
• AFP must be checked. HCG is unlikely to be elevated in this age, but you should also check it.
• Not all malignant tumors will have elevated markers, particularly if they are mixed tumors like embryonal.
• Unless there is concern for torsion, it is worth waiting for the tumor markers (particularly AFP) before operating.
• Imaging
• Ultrasound is a good upfront screening tool, but the majority will end up requiring a CT scan to better evaluate the cyst.
• Cyst characteristics will help determine the risk of malignancy.
• For a predominantly cystic mass, the risk of malignancy is 3-4%.
• For a heterogenous mass, the risk of malignancy is 15-20%.
• For a solid mass, the risk of malignancy is over 25%.
• Surgical approach
• If this patient’s tumor markers return normal, this is likely a benign tumor, but you will still need to preserve the principles of an oncologic operation.
• An ovarian preserving procedure should be attempted for this patient.
• An open operation will require a large midline incision given that this is a big cyst, and should avoid this.
• Laparoscopy may be considered.
• Dr. Rescorla performs this surgery using a small lower abdominal incision on the side that the tumor is arising from (described below) [1].
• Surgical technique
• Create a small lower abdominal incision, about 3-4 cm, on the side that the tumor is arising from.
• Once the cystic component is identified, it is drained through a bag to avoid fluid spillage.
• Dry off the cystic component, and then use a surgical glue, such as Dermabon® or Indermil®, to glue a large plastic sheet directly onto the tumor.
• The fluid is then drained through this bag, which prevents spillage of potentially malignant fluid.
• Once the fluid is drained, the tumor can often be delivered out of the abdomen.
• Perform a partial oophorectomy, if it is possible.
• If the tumor is very small, this technique may be difficult as you may not be able to find tumor. Consider laparoscopy in these cases.
• Perform peritoneal washings.
• If there is fluid, send it for cytology.
• If there is not fluid, rinse around some saline and then send it for cytology.
• Inspect the other ovary. Perform a contralateral biopsy if it looks abnormal.
• Remove any omentum that is adhered to the tumor. 
• Inspect the abdominal cavity for any peritoneal implants. Biopsy anything suspicious.
• Evaluate the retroperitoneal lymph nodes.
• Although CT imaging will give a good evaluation of these, it is still recommended to evaluate them intraoperatively.
• This may be performed through the same incision by palpation, by moving your hands up to the aortic bifurcation, and then feeling for both iliac systems.
• Palpation is adequate to evaluate the lymph nodes, and visual inspection is not required.
• If you cannot perform this, then closing the incision and performing a laparoscopic evaluation is an option.
• If a lymph node is enlarged, remove it.
• There is no role for lymph node dissection.
• This differs from adult management, because adults are largely dealing with epithelial tumors, and pediatric germ cell tumors are very chemo-responsive.
• Ovarian Salvage Technique
• Evaluate for the blood supply coming into the ovary and the fallopian tube.
• The tumor is usually off the top of the ovary. Lift up the tumor, and a white rim is usually seen going up on the tumor. This is likely the edge of the tumor and the normal ovary.
• Remove the mass off the ovary using a combination of hold and cold scissors, and make sure to stay on the side of the normal ovary.
• If there is bleeding, close the flat edges of the remaining ovary together.

CASE 2: Ovarian cystic mass in an adolescent

• Case 2: A 13-year-old female presents with vague abdominal discomfort and she has noticed that her abdomen is getting bigger. Ultrasound and then CT A/P identify a large, completely cystic pelvic mass.
• Workup
• This is likely a benign cyst of the ovary, but given the size, recommend checking tumor markers to be sure.
• If the tumors markers are normal, then the risk of malignancy is less than 1%.
• Operative approach
• The likelihood of malignancy is so low that you do not need to use oncologic precautions, and can perform this laparoscopically.
• If the tumor returns as malignant, then the patient will require chemotherapy.
• Given that the risk of malignancy is so low, you should not subject all females to larger abdominal incisions to perform on oncologic resection in these cases. 
•  Surgical Technique
• You can drain the cyst through one of your trocars.
• Use scissors to perform a cystectomy.
• Given that this is unlikely malignant, remove as much of the cyst to minimize its chances of recurring. It is okay to leave a portion of the cysts lining on the ovary. 

CASE 3: Adolescent with a solid ovarian mass

• Case 3: A 14-year-old female presents with a 3-month history of lower abdominal pain and she has noticed her abdomen is enlarging. On imaging, she was found to have a large solid pelvic mass. Tumors markers were checked and her AFP returned as 44,000.
• Preoperative Workup
• This is going to be malignant, given that it is large, solid and the AFP is elevated [2].
• First decision that needs to be made is if this can be resected up front.
• Imaging
• CT chest/abdomen/pelvis will help determine stage and evaluate if the patient is a candidate for an upfront resection.  
• CT is used to evaluate the retroperitoneal lymph nodes, contralateral ovary, and evidence of metastases.
• CT chest should be performed at the same time as the abdominal CT.
• Distant metastases do not require biopsy, and can be followed with imaging.
• Surgical considerations
• If the tumor is confined to the tumor, it is ideal to perform an upfront resection.
• If there is evidence of disease outside of the ovary, the patient will get neoadjuvant chemotherapy.
• If there is evidence of bilateral ovarian involvement, then consider neoadjuvant chemotherapy and delayed resection.
• Chemotherapy
• It is a platinum-based regimen, with bleomycin and etoposide.
• There is no role for radiation therapy.
• Surgical approach
• If it is confined to the ovary, perform an open operation through a pfannenstiel incision.
• This is different than in adults, who perform midline incisions.
• There is no role for laparoscopy in these patients.
• Perform a complete oophorectomy, making sure to leave the tumor intact.
• Performing a salpingooophorectomy is optional. There is no oncologic reason to remove the fallopian tube, so if it is not encased or adhered to the tumor, it is okay to preserve it.
• Evaluate the retroperitoneal lymph nodes and remove any enlarged lymph nodes.
• Only perform omental and peritoneal biopsies if they are suspicious looking. If the omentum is adhered to the tumor, remove it with the tumor.
• Perform peritoneal washings.
• Peritoneal washings are the main thing that can upstage her in the OR.
• Billmire et al. found that 5 patients were Stage 3 just based on peritoneal washings alone  [3].
• If the peritoneal washings are positive, then she will need chemotherapy.
• Cryopreservation
• This is currently not being done in young patients, but if chemotherapy is being given, then it should be considered.

CASE 4: Ovarian torsion

• Case 4:  A 13-year-old female presents with 24 hours of right lower quadrant abdominal pain. An ultrasound and CT are obtained to evaluate for appendicitis, and there is a 6 cm ovary with heterogenous fluid. The radiologist cannot tell for sure if this is a hemorrhagic ovarian cyst with torsion, but they do see blood flow.
• Workup
• This patient requires an urgent operation.
• Consider sending tumor markers, but they will not be back before you go to the OR.
• Surgical approach
• Detorse the ovary.
• If there is a clear cystic component, then it is ok to decompress the ovarian cyst, and remove part of the cyst wall to prevent it from recurring.
• If it is a mass, it is ok to detorse the ovary, close the abdomen and return at a later date once you have performed additional workup.
• Returning in 1 week or so will not make a difference oncologically.
• This approach will also allow for the inflammation to decrease, and allow for a potential ovarian preserving operation.
• Oophoropexy
• Some controversy about the benefits of this.
• Adults will often just perform detorsion without oophoropexy.
• Smorgick et al. found that premenarchal patients have a higher risk of torsion and an oophoropexy should be considered in these patients [4].
• Technical options are stitching the ovary to the lateral side wall or shortening the utero-ovarian ligament.
• Followup
• Recommend a repeat ultrasound in 4-6 weeks to evaluate the ovarian function.

SURVIVAL OUTCOMES

• Germ cell tumors
• For stage 1 (no evidence of malignancy outside of the ovary), the overall survival is 96%.
• Managing them with surgery alone without chemotherapy is an option.
• There is a 50% relapse rate of those treated this way but salvage chemotherapy is successful in most patients [5].
• For stage 2 and 3 (with lymph node and peritoneal disease), survival is 97% with chemotherapy.
• For stage 4 cancers (metastases), overall survival is 80%, but this is dependent on the patient's age.
• If less than 11 years of age, survival is 92%.
• If greater than 11 years of age, survival is 60%.
• These patients are considered high-risk patients and require additional chemotherapy.

REFERENCES

[1] Ehrlich PF, Teitelbaum DH, Hirschl RB, Rescorla F. Excision of large cystic ovarian tumors: combining minimal invasive surgery techniques and cancer surgery—the best of both worlds. J Pediatr Surg. 2007;42(5):890-893. doi:10.1016/j.jpedsurg.2006.12.069

[2] Papic JC, Finnell SME, Slaven JE, Billmire DF, Rescorla FJ, Leys CM. Predictors of ovarian malignancy in children: Overcoming clinical barriers of ovarian preservation. J Pediatr Surg. 2014;49(1):144-148. doi:10.1016/j.jpedsurg.2013.09.068

[3] Billmire D, Vinocur C, Rescorla F, et al. Outcome and staging evaluation in malignant germ cell tumors of the ovary in children and adolescents: an intergroup study. J Pediatr Surg. 2004;39(3):424-429. doi:10.1016/j.jpedsurg.2003.11.027

[4]  Smorgick N, Melcer Y, Sarig-Meth T, Maymon R, Vaknin Z, Pansky M. High risk of recurrent torsion in premenarchal girls with torsion of normal adnexa. Fertil Steril. 2016;105(6):1561-1565.e3. doi:10.1016/j.fertnstert.2016.02.010

[5] Billmire DF, Cullen JW, Rescorla FJ, et al. Surveillance After Initial Surgery for Pediatric and Adolescent Girls With Stage I Ovarian Germ Cell Tumors: Report From the Children’s Oncology Group. J Clin Oncol. 2014;32(5):465-470. doi:10.1200/JCO.2013.51.1006