|Year : 2016 | Volume
| Issue : 2 | Page : 292-295
Ovarian tumors in children: 10-year experience from a tertiary care center in South India
B Rajeswari, M Nair, A Ninan, K Parukuttyamma
Division of Pediatric Oncology, Regional Cancer Centre, Thiruvananthapuram, Kerala, India
|Date of Web Publication||6-Jan-2017|
Division of Pediatric Oncology, Regional Cancer Centre, Thiruvananthapuram, Kerala
Source of Support: None, Conflict of Interest: None
Background: Ovarian tumors are uncommon in childhood and constitute around 1% of childhood malignancies. Two thirds of pediatric ovarian tumors are germ cell tumors. Epithelial ovarian tumors and stromal tumors are less frequent. We share our experience in childhood ovarian cancers, analyzing a series of cases with respect to the clinical profile, treatment and survival. Methods: All newly diagnosed ovarian tumors in children up to 14 years of age, registered in our Pediatric Oncology Division between January 2000 and December 2009 were retrospectively reviewed. Observations: There were 47 patients with newly diagnosed ovarian tumors. The mean age at presentation was 10.0 ± 3.4 years. The most common symptoms at presentation were acute abdominal pain (48.9%) and abdominal mass (40.4%). Precocious puberty was uncommon (6.3%). Histology was germ cell tumors in 44 cases and nongerm cell tumors in three cases. The benign teratomas (mature and immature grade 1 and 2; n=9) underwent complete surgical resection alone; none had recurrence on follow up. Of the remaining 35 GCTs, 31 patients were given chemotherapy and 4 refused treatment.26 out of the 31 patients completed chemotherapy with BEP (bleomycin, etoposide, cisplatin) regimen with acceptable toxicities. 5 children i.e.; (19.2%) developed recurrence. At a median follow up of 80 months, 10 year disease free survival was 80.8 ± 7.7% and 10 year overall survival was 92.7 ± 4.9%. Conclusion: Germ cell tumors are the most common ovarian malignancy in children. With surgery and chemotherapy using BEP, good outcome can be expected in these patients.
Keywords: Bleomycin-etoposide-cisplatin regimen, childhood ovarian tumors, germ cell tumors
|How to cite this article:|
Rajeswari B, Nair M, Ninan A, Parukuttyamma K. Ovarian tumors in children: 10-year experience from a tertiary care center in South India. Indian J Cancer 2016;53:292-5
|How to cite this URL:|
Rajeswari B, Nair M, Ninan A, Parukuttyamma K. Ovarian tumors in children: 10-year experience from a tertiary care center in South India. Indian J Cancer [serial online] 2016 [cited 2019 Aug 24];53:292-5. Available from: http://www.indianjcancer.com/text.asp?2016/53/2/292/197726
| » Introduction|| |
Ovarian tumors constitute around 1% of childhood malignancies. Incidence is reported to be 2.6 cases per 100,000 girls per year. Around one-third of childhood ovarian neoplasms are thought to be malignant. In contrast to adults in whom epithelial ovarian tumors are more frequent, two-thirds of pediatric ovarian tumors are germ cell tumors (GCTs). There are very few reports of childhood ovarian tumors from India. The aim of our study is to review the clinical presentation, treatment, and outcome of children with ovarian tumors below the age of 14 years, treated in our institution, over a 10-year period.
| » Materials And Methods|| |
All newly diagnosed ovarian tumors in children up to 14 years of age, registered in our Pediatric Oncology Division between January 2000 and December 2009, were included. The medical records of all these patients were reviewed retrospectively. The data collected included the age, presenting symptoms, details of investigations including imaging modality used, tumor markers and histopathology, the treatment given, and outcome. The patients with GCTs were staged according to the Children's Oncology Group (COG) staging.
| » Results|| |
There were 47 patients with newly diagnosed ovarian tumors during the study period.
The mean age at presentation was 10.0 ± 3.4 years. A majority of patients (n = 32) were between the ages of 10 and 14 years. Nine patients presented between 5 and 10 years of age and six patients between 1 and 5 years of age. No cases were reported in infancy. All cases of mature teratoma were in the 10–15-year age group.
Symptoms at presentation
There were varied symptoms at presentation ranging from acute abdominal pain to asymptomatic presentation. The most common symptoms at presentation were acute abdominal pain (48.9%) and abdominal mass (40.4%). Precocious puberty was uncommon (6.3%). All the three patients who presented with precocious puberty had heterosexual precocity. Less frequently reported symptoms include vomiting, dysuria, fever, loss of appetite, amenorrhea, and bleeding per vagina. The tumor was unilateral in most patients (n = 46). The only case of bilateral tumor was that of a 12-year-old girl who had bilateral mature teratoma. She underwent right ovariectomy and excision of the ovarian cyst on the left side. She is now on regular follow-up with no evidence of disease. Forty-three patients presented to us after a surgical procedure for the tumor. Residual tumor was present in two patients. Only four patients presented preoperatively.
Diagnostic imaging studies
Imaging studies done were ultrasound, computed tomography (CT) scan or both. The diagnosis was established in 14 patients with an ultrasound only and in 11 patients with a CT scan only. Both ultrasound and CT scan were done for diagnosis in two patients. Twenty patients had undergone exploratory laparotomy before any imaging studies.
Data regarding the preoperative levels of serum alpha-fetoprotein (AFP) were available in 22 patients, of which the values were elevated in 16 patients (8 patients with yolk sac tumor, 3 patients each with mixed GCT and immature teratoma Grade 3, and 2 patients with immature teratoma Grade 2). Postoperative values of serum AFP are available in 40 patients, of which 17 patients had elevated values. This included the two patients with residual tumor after surgery.
Of the 47 ovarian tumors, 44 were GCTs and three were non-GCTs. [Table 1] shows the distribution of tumors according to histopathology. Thirty-three tumors (70.2%) were of malignant or borderline histology.
Staging of germ cell tumors
The 44 patients with GCT were staged according to the COG staging [Table 2]. The patient with stage 4 disease presented with bilateral pleural effusion and the pleural fluid cytology was positive for malignant cells.
|Table 2: Staging of germ cell tumors according to Children's Oncology Group staging|
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The patients with benign teratomas (mature and immature Grade 1 and 2; n = 9) underwent complete surgical resection alone; none had a recurrence on follow-up. Of the 30 malignant GCTs and five immature teratoma Grade 3 patients, 31 patients were given chemotherapy and four refused treatment. Five out of 31 patients, who were initiated on chemotherapy, did not complete chemotherapy and were lost to follow-up. Twenty-six out of the 31 patients completed chemotherapy with bleomycin, etoposide, and cisplatin (BEP) regimen with acceptable toxicities. The major toxicities noted were pulmonary toxicity, dyselectrolytemias, and febrile neutropenia. Patients with sex cord stromal tumors and epithelial mucinous tumor underwent surgical resection alone.
Five patients developed recurrence, i.e., 19.2%. Time to recurrence ranged from five months to 4 years. Of the recurrences, four had an initial pathological diagnosis of immature teratoma Grade 3. All patients underwent repeat surgical excision. Histopathology was mature teratoma in three patients and did not require any further chemotherapy while the other two with malignant elements received second-line chemotherapy. Three of the patients are on regular follow-up with no evidence of disease and two were lost to follow-up. [Table 3] shows the details of recurrence, time to recurrence, treatment given, and outcome.
Survival analysis was done for the GCTs. At a median follow-up of 80 months, the 10-year disease-free survival was 80.8 ± 7.7% and 10-year overall survival (OS) was 92.7 ± 4.9% [Figure 1] and [Figure 2].
| » Discussion|| |
Although there are many series on ovarian tumors in children, there are only few reports from India. A comparable series analyzing the clinical profile and survival from India is a 7-year follow-up study by Biswajit et al. with 40 patients. Ovarian tumors in children are mainly GCTs. Nearly two-thirds of ovarian tumors in children are GCTs in contrast to adults in whom a majority of tumors are of epithelial origin. In our series also, a majority of patients are of germ cell origin. The rates of malignancy differ among various analyses ranging from 16% to 41.4%., One of the largest series by Liu et al. analyzed 203 children and adolescents with ovarian masses and reported that 13.6% were of borderline or malignant histology. The higher rate of malignancy of 70.2% in our report is probably a referral bias as we are a referral center for oncology patients. The median age at presentation was 10 years, which is comparable to most of the reported pediatric series. De Backer et al. had reported a median age at the presentation of 9 years.
Although the symptoms at presentation are usually nonspecific like abdominal pain, the presence of an abdominal mass or precocious puberty, especially in the presence of elevated tumor markers may suggest an ovarian malignancy. The main symptoms at presentation were abdominal pain and abdominal mass in ours as well as most other series. Endocrine symptoms were reported in 4 of 66 patients in De Backer series - isosexual precocious puberty in two, precocious pseudopuberty in one, and hypogonadotropic hypogonadism with a delay of puberty in one. Three of our 47 patients presented with heterosexual precocious puberty.
Several imaging modalities such as ultrasound and CT scan are done as an initial imaging modality in children with ovarian masses. A transabdominal ultrasound is usually the first imaging of choice and will help to distinguish between solid and cystic masses. CT scan will help to assess the nature and extent of tumor better and may also aid in predicting the chance of malignancy. Malignant tumors tend to be larger and more solid or heterogeneous than benign tumors. In contrast to most other studies, around half of our patients underwent exploratory laparotomy before any other imaging studies. Imaging studies before laparotomy were done in 27 patients.
Of the total 47 patients, 44 were GCTs, 2 were sex cord stromal tumors, and 1 was an epithelial ovarian tumor. Of the GCTs, 9 were benign teratomas (mature and immature Grades 1 and 2); 5 were immature teratomas Grade 3; the remaining were malignant GCTs. In the series by Biswajit et al., the most common histopathology is mixed GCT followed by dysgerminoma and endodermal sinus tumors. In our study, the most common histology was endodermal sinus tumor followed by dysgerminomas and mixed GCTs. There were equal numbers of early stage and advanced stage diseases in our series in contrast to above-mentioned study, in which patients presented at an advanced stage of disease. This may be due to the greater health awareness leading to the early seeking of medical attention in our population. Studies have shown that the stage and tumor markers are the most important prognostic markers in malignant GCTs.
Cisplatin-based BEP regimen is the most widely used chemotherapy for ovarian GCTs. This regimen provides excellent event-free survival (EFS) and OS rates with minimal toxicity. A randomized comparison of high-dose cisplatin (HDPEB) with standard-dose cisplatin in high-risk malignant GCTs showed improved EFS with HDPEB but the risk of significant toxicity limits its use., The first-line chemotherapy used in our patients was the BEP regimen, administered once in 3 weeks for 4–6 cycles. Second-line chemotherapy using vincristine, Adriamycin, cyclophosphamide and vinblastine, ifosfamide, carboplatin was given to two patients with metastatic malignant recurrence. The chemotherapy was generally well tolerated with acceptable toxicities. We had five recurrences, of which four had a primary histology of immature teratoma. Similar results are also observed in the series by De Backer et al., in which all the three patients who developed recurrence had immature teratoma. However, in the study by Schultz et al., recurrence was seen with dysgerminoma and mixed GCT. It is difficult to explain why immature teratomas were associated with higher recurrence rates.
At a median follow-up of 80 months, our EFS and OS rates for malignant GCTs were 80.8% and 92.7% which are comparable to most of the recently published series. In the study by Biswajit et al. from India, relapses were seen in 25% of the patients and the 5-year relapse-free survival and OS rates were 72.8% and 94.9%, respectively.
| » Conclusion|| |
GCTs are the most common ovarian malignancy in children. With surgery and chemotherapy using BEP, a good outcome can be expected in majority of the patients.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]
[Table 1], [Table 2], [Table 3]