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 Table of Contents  
ORIGINAL ARTICLE
Year : 2018  |  Volume : 4  |  Issue : 2  |  Page : 24-28

Childhood And Adolescence Ovarian Malignancy : A Study in A Tertiary Care Centre in Bihar


1 Additional Professor, Dept. of Pathology, IGIMS, Patna, India
2 Senior Resident, Dept. of Pediatrics, IGIMS, Patna, India
3 Additional Professor, Dept. of Gynecological Oncology, IGIMS, Patna, India
4 Senior Resident, Dept. of Gynecological Oncology, IGIMS, Patna, India
5 Professor Anesthesiology, IGIMS, Patna, India

Date of Web Publication10-Dec-2020

Correspondence Address:
Jaya Kumari
Additional Professor, Dept. of Gynecological Oncology, IGIMS, Patna, Bihar
India
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Source of Support: None, Conflict of Interest: None


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  Abstract 


Background: Ovarian tumour in children and adolescent girls is uncommon but important part of gynaecological malignancies. They account for 1% of all the childhood malignancies and 8% of all abdominal tumours in children. Incidence is reported to be 2.6 cases per 100,000 girls per year and almost 10-30% of all the ovarian neoplasms occurring in girls up to 17 years of age are malignant We share our experience in childhood ovarian cancers, analysing a series of cases with respect to the clinical profile, treatment and survival.
Methods: All newly diagnosed ovarian tumours in children up to 20 years of, registered in Gynaecological Oncology of Indira Gandhi institute of medical science between 2014 and 2017 were retrospectively reviewed.
Observations: There were 12 patients with newly diagnosed ovarian malignancies. The mean age at presentation was 14 ± 4 years. The most common symptoms at presentation were acute abdominal pain (48.9%) and abdominal mass (40.4%). Histology was germ cell tumours in 11 cases and nongerm cell tumours in one cases. Out of 11 GCTs, 10 patients underwent fertility sparing surgery and one patient lost after neoadjuvant chemotherapy. One patient of mucinous adenocarcinoma underwent debulking surgery. Six patients lost to follow up after surgery and three had taken post-surgery chemotherapy. At a median follow up of 15 month, the 3 year disease?free survival was 46. 62 ± 2.3 % and 3 year overall survival (OS) was 38 ± 3.46 %.
Conclusion: Germ cell tumours are the most common ovarian malignancy in children. With surgery and chemotherapy using BEP, good outcome can be expected in these patients.

Keywords: Childhood ovarian tumours, germ cell tumours, Adenocarcinoma, disease free survival


How to cite this article:
Choudhary V, Kumar R, Pankaj S, Kumari A, Kumari J, Nazneen S, Kumari A, Raghwendra K H. Childhood And Adolescence Ovarian Malignancy : A Study in A Tertiary Care Centre in Bihar. J Indira Gandhi Inst Med Sci 2018;4:24-8

How to cite this URL:
Choudhary V, Kumar R, Pankaj S, Kumari A, Kumari J, Nazneen S, Kumari A, Raghwendra K H. Childhood And Adolescence Ovarian Malignancy : A Study in A Tertiary Care Centre in Bihar. J Indira Gandhi Inst Med Sci [serial online] 2018 [cited 2021 Dec 4];4:24-8. Available from: http://www.jigims.co.in/text.asp?2018/4/2/24/302949




  Introduction: Top


Ovarian tumour in children and adolescent girls is uncommon but important part of gynaecological malignancies. They account for 1% of all the childhood malignancies and 8% of all abdominal tumours in children. Incidence is reported to be 2.6 cases per 100,000 girls per year and almost 10-30% of all the ovarian neoplasms occurring in girls up to 17 years of age are malignant[1],[2].

According to the literature, paediatric ovarian tumours are mostly germ cell tumours in contrast to the tumours occurring in the adult females, which are mostly surface epithelial in origin. Approximately 85 % are germ cell tumours (GCT), 8 % epithelial cell carcinoma, and 5 % sex cord stromal tumours. The overall prognosis of ovarian cancer in children is excellent compared to adults, because of a different histological distribution.[3] There are two important contributing factors to the prognosis of GCT: first, the majority (71%) is detected at stage I and second, they respond well to surgery and chemotherapy leading to a 5-year survival of 95.6% and 73.2% in stage I and advanced stages, respectively.[4] Because of this excellent prognosis of germ cell tutors, overall outcome of ovarian cancer in children is excellent compared to adults.

There are very few reports of childhood ovarian malignancies from India. The aim of our study is to review the clinical presentation, histological type, tumour marker, treatment, and outcome of children and adolescence with ovarian tumours below the age of 20 years, undergo surgery in our gynaecological oncology department over a period of seven years.


  Material and Methods: Top


The study was conducted in at a tertiary hospital of Bihar. All the cases malignant ovarian tumours up to the age of 20 years from 2014 to 2017 were included for the study. The medical and surgical records of all these patients were reviewed retrospectively. The data collected included the age, presenting symptoms, details of investigations including imaging modality used, tumour markers, preoperative finding, histopathology, the treatment given, and outcome. Imaging studies done were ultrasound, computed tomography (CT) scan or both. The patients with GCTs were staged according to the Children's Oncology Group (COG) staging. Statistical analysis was performed using Epi Info Version 6.00.


  Result: Top


We received a total of 220 cases of malignant ovarian tumours over the study period, of which 12 cases were seen in girls up to 20 years of age. This constituted 12/220 (5.45%) of all the ovarian tumours [Table 1]. Three patient files were missing required data so they were excluded from study the rest 9 have been reported here.
Table 1: prevalence of childhood and ovarian malignancy

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The mean age of the patients was 14 ± 4 years. A majority of patients (n = 8) were more than 10 years old [Figure 1]. The most common presenting complaint was lump abdomen (37.5 %) followed by pain abdomen (25%) and abdominal distension (25%). There was 1 patient who were incidentally diagnosed by the clinician and confirmed by ultrasonography. [Figure 2]. All 9 patients had GCT(Germ cell tumor) and were staged according to the COG staging [Table 3]. 6 of the patients 9 (66.67%) presented in stage III,1 (11.11 %) was in stage II and 2 (22.22%) were diagnosed in stage I.
Figure 1: Age distribution

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Figure 2: Presenting complain

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Table 3: Patients characteristics

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Table 4: Follow up

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Tumour marker which was done in all these cases were CA 125, CEA, CA19-9, LDH, beta HCG and alphafeto protein (AFP). Tumour markerreports were available in eight patients. Out of these, AFP were elevated in 4 patients (3 patients with yolk sac tumour and one patient with immature teratoma). Three patients had elevated LDH and histopathology report of all of them showed dysgerminoma. One patient was of mature cystic teratoma and her tumour markers were within normal range. Five patients had normal CA 125 while in three cases it was elevated and out of three, two had yolk sac tumour and one had dysgerminoma on histopathology. [Table 3]

We categorised the cases into size, laterality, gross finding and cut surface of the masses, as shown in [Table 2]. The size was further subdivided into 3 categories of less than 15 cm, 15-20 cm and >20 cms. It was noted that majority of cases (58.33%) had tumor size measuring more than 20 cm. Laterality of each tumour was also recorded and three case had bilateral ovarian involvement the and rest 9 were unilateral. Ascites and omental involvement was present in 66.66% (6/9) of cases. All the tumours were multilocular on cut section. [Table 2]
Table 2: Per- operative findings

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One case of stage III disease underwent total abdominal hysterectomy with bilateral salpingectomy, total omentectomy and ureteroneostomy. Two cases of stage III disease underwent bilateral salpingo oophorectomy with total omentectomy and five cases underwent unilateral salpingo oophorectomy and omentectomy. The two patients of stage I disease underwent unilateral salpingo oophorectomy. The patient with stage II disease had dense adhesions and the tumor could only be removed piecemeal. [Table 3]

Out of the 9 patients 1 patient was advised neoadjuvant chemotherapy. She underwent ultrasound guided biopsy which proved dysgerminoma and the patient was lost after 3 cycles chemotherapy (BEP regimen). The rest 8 patients underwent primary cytoreduction. 3 patients had yolk sac tumor, 3 had dysgerminoma, 1 patient had mature teratoma, 1 had immature teratoma and 1 had mucinious cystadenocarcinoma.

5 patients were lost to follow up after surgery. The patient with cystadenocarcinoma did not take chemotherapy after surgery and died 6 months following surgery due to disease. 1 patient of yolk sac tumor died on post operative day 2 due to iatrogenic cause related to anaesthesia procedure. Patient with mature teratoma did not require any adjuvant chemotherapy and is alive and on follow up. One case of immature teratoma after receiving 4 cycles of chemotherapy developed recurrence and died of disease 8 months following surgery. One patient of yolk sac tumor is on post operative day 10 and is planned for chemotherapy. One case of dysgerminoma received 3 cycles of chemotherapy and is alive and well 8 months following surgery.

Survival analysis was done for all these cases. At a median follow up of 15 month, the 3 year disease free survival was 46. 62 ± 2.3 % [Figure 3] and 3 year overall survival (OS) was 38 ± 3.46 % [Figure 4]
Figure 3

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Figure 4

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  Discussion: Top


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.[5] Another comparable series of Ovarian tumors in children with the clinical profile and survival analysis for 10 year follow-up study by Rajeswari B, et al. with 47 patients was done.[6] 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.[7] In our study the prevalence of ovarian malignancy in children and adolescent was 6.66% and this is lower than other study because we include only those cases who were fit for surgery. The mean age at presentation was 14 years, which is comparable to most of the reported pediatric series.

Although the symptoms at presentation are usually nonspecific like abdominal pain, the presence of an abdominal mass or abdominal distension, 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.[8] Endocrine symptoms were reported in 4 of 66 patients in De Backer series. Isosexual precocious puberty in two, precocious pseudo puberty in one, and hypogonadotropic hypogonadism with a delay of puberty in one.[8] In our study no patients was presented with endocrine symptoms.

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.[9]

The total 12 patients, 11 were GCTs and 1 was an epithelial ovarian tumor. Of the GCTs, 4 patients had yolk sac tumor, 4 had dysgerminoma, 1 patient had mature teratoma, 2 had immature teratoma and 1 had mucinious cystadenocarcinoma. The majority of epithelial ovarian cancers in children reported in literature are mucinous ovarian cancers or low-grade serous ovarian cancers.[10] In the series by Biswajit et al. In the literature most common histopathology is mixed GCT followed by dysgerminoma and endodermal sinus tumors.[5] In our study, the most common histology was endodermal sinus tumor or yolk sac tumour and dysgerminomas followed by mixed GCTs. Dysgerminoma was most common in the studies conducted by Mukhopadhyay et al from India.[11] Patients were presented at an advanced stage of disease in our study. This may be due to the less health awareness, because of at our institute patients visited mostly from rural area and low socioeconomic strata leading to the delayed medical attention in our population. Studies have shown that the stage and tumor markers are the most important prognostic markers in malignant GCTs.[12]

Generally, childhood tumours turned fatal due to lack of postoperative intensive care and hesitation in using cytotoxic drugs in infants and children specially in Indian settng. Fertility sparing surgery is preferable now days with postoperative chemotherapy. Some reports advocate advantages of surgery alone and reserve chemotherapy for relapse. Gradually, BEP results showed its efficacy in achieving long remission periods and fairly adequate drug tolerance. A review of 47 patients with fertility-sparing surgery and cisplatin regimen had good prognosis, documented by Japanese study in last two decades of twentieth century.[13] Children (median age 18 years) with OYSTs, undergoing conservative surgery and cumulative high-dose combination chemotherapy (BEP), had 90 % survival at 6 years, gave birth to children, and showed no adverse effect of chemotherapy on ovarian function.[14] de La Motte Rouge et al.[15] reported 84 cases without ascites having 84 % five-year survival with favorable serum AFP decline rate, with fertility-conserving surgery and not radical surgery. Some other studies also noted same.[16],[17] We had done fertility sparing surgery in our most of the patients.

Standard chemotherapy for advanced or incompletely resected GCT is BEP (Bleomycine, Etopiside, Cisplatinum), alternatively JEB can be used in young children (Carboplatin replacing Cisplatin) or PEI (Cisplatin, Etoposide, Ifosfamide). Cisplatin-based BEP regimen provides excellent disease 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.[6] The first-line chemotherapy used in our patients was the BEP regimen, administered once in 3 weeks for 4-6 cycles. Second line chemotherapy was given to one patient of immature teratoma for recurrence.

Survival analysis was done for all these cases. At a median follow up of 15 month, the 3 year disease free survival was 46. 62 ± 2.3 % and 3 year overall survival (OS) was 38 ± 3.46 %. In Rajeswari et al, at a median follow?up of 80 months, DFS and OS rates for malignant GCTs were 80.8% and 92.7% respectively while in 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 5. Disease free survival and overall survival is less in our study because our patients were presented in advanced stage and they did not follow up properly.


  Conclusion: Top


Childhood ovarian malignancy is as such rare, but it poses a clinical challenge due to its varied histologic nature and fatal outcome. 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.



 
  References Top

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Alobaid AS. Mucinous cystadenoma of ovary in a 12-yearold girl. Soudi Med J.2008;29:126-8.  Back to cited text no. 1
    
2.
Bhattacharyya NK, De A, Bera P, Mongal S, Chakraborty S, Bandopadhyay R. Ovarian tumors in pediatric age group- A clinicopathologic study of 10 years' cases in West Bengal, India. Ind J Med Paed Oncol. 2010;31(2):54-7.  Back to cited text no. 2
    
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E. Van Nieuwenhuysen, S. Lambrechts, D. Lambrechts, K. Leunen, F. Amant, I. Vergote, Genetic changes in nonepithelial ovarian cancer, Expert Rev. Anticancer Ther. 13 (2013) 871-882.  Back to cited text no. 3
    
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G. Mangili, C. Sigismondi, A. Gadducci, G. Cormio, P. Scollo, S. Tateo, et al., Outcome and risk factors for recurrence in malignant ovarian germ cell tumors: a MITO-9 retrospective study., Int. J. Gynecol. Cancer. 21 (2011) 1414-21  Back to cited text no. 4
    
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Biswajit D, Patil CN, Sagar TG. Clinical presentation and outcome of pediatric ovarian germ cell tumor: A study of 40 patients. J Pediatr Hematol Oncol 2010;32:e54?6  Back to cited text no. 5
    
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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.  Back to cited text no. 6
[PUBMED]  [Full text]  
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Liu H, Wang X, Lu D, Liu Z, Shi G. Ovarian masses in children and adolescents in China: Analysis of 203 cases. J Ovarian Res 2013;6:47.  Back to cited text no. 7
    
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Ruchi Rathore1*, Sonal Sharma2, Deepshikha Arora. Spectrum of Childhood and Adolescent Ovarian Tumors in India: 25 Years Experience at a Single Institution pen Access Macedonian Journal of Medical Sciences. 2016 Dec 15; 4(4):551 555.  Back to cited text no. 8
    
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De Backer A, Madern GC, Oosterhuis JW, Hakvoort?Cammel FG, Hazebroek FW. Ovarian germ cell tumors in children: A clinical study of 66 patients. Pediatr Blood Cancer 2006;46:459?64  Back to cited text no. 9
    
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Heo SH, Kim JW, Shin SS, Jeong SI, Lim HS, Choi YD, et al. Review of ovarian tumors in children and adolescents: Radiologic?pathologic correlation. Radiographics 2014;34:2039?55  Back to cited text no. 10
    
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T. Baert1,2, N. Storme1, E. Van Nieuwenhuysen1,2, A. Uyttebroeck3, N. Van Damme4, I. Vergote1,2, A. Coosemans. Ovarian cancer in children and adolescents: a rare disease that needs more attention. Maturitas.2016 jun 88 :3-8  Back to cited text no. 11
    
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Mukhopadhyay M, Shukla RM, Mukhopadhyay B, et al. Ovarian cysts and tumors in infancy and childhood. Journal of Indian Association of Pediatric Surgeons. 2013;18(1):16-19.  Back to cited text no. 12
    
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Metwalley KA, Elsers DA, Farghaly HS, et al. Precocious puberty secondary to a mixed germ cell-sex cord-stromal tumor associated with an ovarian yolk sac tumor: a case report. J Med Case Rep. 2012;6(1):162. 1  Back to cited text no. 13
    
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Kang H, Kim TJ, Kim WY, et al. Outcome and reproductive function after cumulative high-dose combination chemotherapy with bleomycin, etoposide and cisplatin (BEP) for patients with ovarian endodermal sinus tumor. Gynecol Oncol. 2008;111(1): 106-10  Back to cited text no. 14
    
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De La Motte Rouge T, Pautier P, Rey A. Prognostic factors in women treated for ovarian yolk sac tumour: a retrospective analysis of 84 cases. Eur J Cancer. 2011;47(2):175-8  Back to cited text no. 15
    
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    Figures

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    Tables

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