Pediatric Ovarian Tumors Video Podcast

Pediatric Surgery
An interactive discussion between Dr. Ponsky and Dr. Rescorla about the management of ovarian tumors in pediatric patients.

Dr. Frederick Rescorla is surgeon-in-chief at Riley Children's Hospital, Anna Olivia Healey Professor of Pediatric Surgery at Indiana University School of Medicine, and COG germ cell committee member.

Intro track is adapted from "I dunno" by grapes, featuring J Lang, Morusque.
Artist URL: ccmixter.org/files/grapes/16626
License: creativecommons.org/licenses/by/3.0/

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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

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