Author Information
Desai GS*, Tiruke R*, Panchbudhe S*, Mayadeo NM**.
(* Assistant Professor, ** Professor Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai, India.)
Abstract
Germ cell tumors of the ovary are highly malignant and are prone to metastases to neighboring organs such as the sigmoid colon and uterus. We describe a case in which an ovarian malignant germ cell tumor occurred in a 6 year old girl who presented to our institution with abdominal pain and a palpable abdominal mass arising from the pelvis. Imaging showed a pelvic mass possibly arising from the retroperitoneum with raised serum tumor markers. On staging laparotomy a Stage III ovarian tumor involving the uterus and adherent to the sigmoid colon was seen. Frozen section revealed malignant germ cell tumor of the ovary. Malignant germ cell tumors of the ovary are common in occurrence and should be suspected in the pediatric age group. Surgical removal in tumors involving uterus and bowel requires expertise and precise dissection.
Germ cell tumors of the ovary are highly malignant and are prone to metastases to neighboring organs such as the sigmoid colon and uterus. We describe a case in which an ovarian malignant germ cell tumor occurred in a 6 year old girl who presented to our institution with abdominal pain and a palpable abdominal mass arising from the pelvis. Imaging showed a pelvic mass possibly arising from the retroperitoneum with raised serum tumor markers. On staging laparotomy a Stage III ovarian tumor involving the uterus and adherent to the sigmoid colon was seen. Frozen section revealed malignant germ cell tumor of the ovary. Malignant germ cell tumors of the ovary are common in occurrence and should be suspected in the pediatric age group. Surgical removal in tumors involving uterus and bowel requires expertise and precise dissection.
Introduction
Germ cell tumors of the ovary are frequently seen in children. Malignant germ cell tumors comprise fewer than 5 percent of malignant ovarian cancers and include dysgerminoma, yolk sac tumor and immature teratoma.[1] These tumors present at a younger age, usually in their teens or early 20’s. Subacute abdominal pain is the usual presenting symptom in most patients. Patients with a suspected malignant germ cell tumor should have serum hCG and AFP tumor markers tested. The appearance of malignant germ cell tumors is usually a multilobulated complex ovarian mass. The authors present a case of malignant germ cell tumor in a 6 year old child which metastasized to the uterus and the large bowel. We present the imaging features of this tumor and its intraoperative appearance.
Case Report
A 6 year old girl was admitted in the ward with complaints of pain in abdomen and distension of the abdomen since 2 weeks. She also complained of fever, weight loss and vomiting during this time along with tingling and numbness of the right leg. Per abdominal examination revealed a tense solid and cystic mass arising from the pelvis and extending upto the umbilicus. Her tumor markers were as follows: CA 125 was 209 U/mL, CEA was 66 ng/mL, AFP was 32859 ng/mL, hCG was < 1.0 mIU/L, and LDH was 1183 U/L. Ultrasonographic examination of the pelvis suggested an ovarian tumor. Contrast enhanced CT (CECT) scan done subsequently showed 10x7x5 cm retroperitoneal mass extending into the presacral space displacing the bowel anteriorly and extending upto the umbilicus. Inferiorly the mass extended upto the umbilicus (Figure 1-3) An oncosurgical opinion suggested the mass was clinically operable and she was posted for a staging laparotomy. On staging laparotomy an ovarian tumor involving the uterus and adherent to the sigmoid colon was seen. The tumor was hemorrhagic in appearance and friable in consistency. Contralateral ovary appeared normal. Unilateral salpingo-ooporectomy with hysterectomy was done and sample was sent for histopathology. Frozen section revealed malignant germ cell tumor of the ovary. A diagnosis of stage III malignant germ cell neoplasm was made. Tumor was resected around the sigmoid colon but a colostomy was deferred owing to the poor socioeconomic status of the child. She is doing well postoperatively (Figure 4). She was referred to the medical oncologist for adjuvant chemotherapy.
Germ cell tumors of the ovary are frequently seen in children. Malignant germ cell tumors comprise fewer than 5 percent of malignant ovarian cancers and include dysgerminoma, yolk sac tumor and immature teratoma.[1] These tumors present at a younger age, usually in their teens or early 20’s. Subacute abdominal pain is the usual presenting symptom in most patients. Patients with a suspected malignant germ cell tumor should have serum hCG and AFP tumor markers tested. The appearance of malignant germ cell tumors is usually a multilobulated complex ovarian mass. The authors present a case of malignant germ cell tumor in a 6 year old child which metastasized to the uterus and the large bowel. We present the imaging features of this tumor and its intraoperative appearance.
Case Report
A 6 year old girl was admitted in the ward with complaints of pain in abdomen and distension of the abdomen since 2 weeks. She also complained of fever, weight loss and vomiting during this time along with tingling and numbness of the right leg. Per abdominal examination revealed a tense solid and cystic mass arising from the pelvis and extending upto the umbilicus. Her tumor markers were as follows: CA 125 was 209 U/mL, CEA was 66 ng/mL, AFP was 32859 ng/mL, hCG was < 1.0 mIU/L, and LDH was 1183 U/L. Ultrasonographic examination of the pelvis suggested an ovarian tumor. Contrast enhanced CT (CECT) scan done subsequently showed 10x7x5 cm retroperitoneal mass extending into the presacral space displacing the bowel anteriorly and extending upto the umbilicus. Inferiorly the mass extended upto the umbilicus (Figure 1-3) An oncosurgical opinion suggested the mass was clinically operable and she was posted for a staging laparotomy. On staging laparotomy an ovarian tumor involving the uterus and adherent to the sigmoid colon was seen. The tumor was hemorrhagic in appearance and friable in consistency. Contralateral ovary appeared normal. Unilateral salpingo-ooporectomy with hysterectomy was done and sample was sent for histopathology. Frozen section revealed malignant germ cell tumor of the ovary. A diagnosis of stage III malignant germ cell neoplasm was made. Tumor was resected around the sigmoid colon but a colostomy was deferred owing to the poor socioeconomic status of the child. She is doing well postoperatively (Figure 4). She was referred to the medical oncologist for adjuvant chemotherapy.
Figure 1. CECT scan of the abdomen (cross sectional view) showing the mass extending into the presacral area.
Figure 2. CECT scan of the abdomen and pelvis (lateral view) shows the round tumor with heterogenous appearance suggesting its malignant nature.
Figure 3. CECT scan of the abdomen and pelvis showing pelvic tumor extending upto the umbilicus superiorly and the acetabulum inferiorly.
Figure 4. Intraoperative image shows the hemorrhagic appearance and friable nature of the mass.
Discussion
Once a pelvic mass is detected, especially in younger female patients, the standard workup for suspected germ cell tumors (GCTs) requires lactate dehydrogenase (LDH), alpha-fetoprotein (AFP), and beta-human chorionic gonadotropin (beta-hCG) levels. If any levels are elevated, they may assist in diagnosis and/ or follow-up of women diagnosed with malignant ovarian GCTs.[2]
Cytoreductive surgery is generally recommended for malignant ovarian germ cell tumors. Debulking to a level of minimal residual disease improves response to chemotherapy. For those who have completed childbearing, hysterectomy with bilateral salpingo-oophorectomy (BSO) is appropriate. Preservation of the contralateral ovary leads to “recurrent” dysgerminoma in 5 to 10 percent of retained gonads during the next 2 years.
Chemotherapy has replaced radiation as the preferred adjuvant treatment for all types of malignant ovarian germ cell tumors. Stage IA dysgerminomas and stage IA grade 1 immature teratomas do not require additional chemotherapy. More advanced malignant ovarian germ cell tumors have been treated with combination chemotherapy.[3] The standard regimen is a course of bleomycin, etoposide, and cisplatin (BEP) for five days given every 3 weeks. Variations of this do exist in practice.
Conclusion
Malignant germ cell tumors of the ovary are common in occurrence and should be suspected in the pediatric age group. Surgical removal in tumors involving uterus and bowel requires expertise and precise dissection. Knowledge of pediatric anatomy is important in tackling this histological type of ovarian tumors.
Acknowledgment
Dr Chetan Kantharia MS, Head of Department of GI Surgery, Seth G S Medical College and KEM Hospital.
References
Discussion
Once a pelvic mass is detected, especially in younger female patients, the standard workup for suspected germ cell tumors (GCTs) requires lactate dehydrogenase (LDH), alpha-fetoprotein (AFP), and beta-human chorionic gonadotropin (beta-hCG) levels. If any levels are elevated, they may assist in diagnosis and/ or follow-up of women diagnosed with malignant ovarian GCTs.[2]
Cytoreductive surgery is generally recommended for malignant ovarian germ cell tumors. Debulking to a level of minimal residual disease improves response to chemotherapy. For those who have completed childbearing, hysterectomy with bilateral salpingo-oophorectomy (BSO) is appropriate. Preservation of the contralateral ovary leads to “recurrent” dysgerminoma in 5 to 10 percent of retained gonads during the next 2 years.
Chemotherapy has replaced radiation as the preferred adjuvant treatment for all types of malignant ovarian germ cell tumors. Stage IA dysgerminomas and stage IA grade 1 immature teratomas do not require additional chemotherapy. More advanced malignant ovarian germ cell tumors have been treated with combination chemotherapy.[3] The standard regimen is a course of bleomycin, etoposide, and cisplatin (BEP) for five days given every 3 weeks. Variations of this do exist in practice.
Conclusion
Malignant germ cell tumors of the ovary are common in occurrence and should be suspected in the pediatric age group. Surgical removal in tumors involving uterus and bowel requires expertise and precise dissection. Knowledge of pediatric anatomy is important in tackling this histological type of ovarian tumors.
Acknowledgment
Dr Chetan Kantharia MS, Head of Department of GI Surgery, Seth G S Medical College and KEM Hospital.
References
- Crum CP. The Female Genital Tract. In Kumar V, Abbas AK, Fausto N editors Robbins and Cotran. Pathologic basis of disease.7th ed.Philadelphia, Pa.: Elsevier Saunders. 2005;pp.1059-1119.
- Schorge JO. Ovarian Germ Cell and Sex Cord- Stromal Tumors. In Hoffman BL, Schorge JO, Schaffer JI, Halvorson LM, Bradshaw KD, Cunningham FG editors. Williams Gynecology, 2nd ed. New York: McGraw-Hill 2012.
- Billmire D1, Vinocur C, Rescorla F, Cushing B, London W, Schlatter M, 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):424-9.
Citation
Desai GS, Tiruke R, Panchbudhe S, Mayadeo NM. Metastatic Malignant Ovarian Germ Cell Tumor In A 6 Year Old Girl. JPGO 2018. Volume 5 No.1. Available from:http://www.jpgo.org/2018/01/metastatic-malignant-ovarian-germ-cell.html
Desai GS, Tiruke R, Panchbudhe S, Mayadeo NM. Metastatic Malignant Ovarian Germ Cell Tumor In A 6 Year Old Girl. JPGO 2018. Volume 5 No.1. Available from:http://www.jpgo.org/2018/01/metastatic-malignant-ovarian-germ-cell.html