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gonadotrophic hormone) was done in only 8 patients of suspected teratoma.

Fourteen patients of benign ovarian tumors underwent ovarian cystectomy. One patient of dermoid cyst who had torsion, the cyst was gangrenous and could not be saved. We could not save the ovary of the neonate with the torsion (cyst was diagnosed antenatally) but operated at the age of 15days. The cyst was 7cm × 6cm. The second neonate with simple cyst and Hirschsprung’s disease, only the cyst was excised. Thirty three patients of malignant ovarian tumors underwent unilateral salpingo-oophorectomy. One with bilateral ovarian tumors (non-Hodgkin lymphoma) underwent bilateral salpingo-oophorectomy [Figure 1]. One patient had multiple peritoneal deposits (malignant teratoma intermediate) and two had hemorrhagic ascites. All the specimens were sent for histopathological (HP) examination. All patients with malignant tumors received systemic combination chemotherapy.

INTRODUCTION

Gynecological malignant conditions account for approximately 3% of all types of cancer in children.[1] The incidence of ovarian tumors in pediatric age group account for only 1% of childhood malignancy.[2] Ovarian cysts are from mature follicles and were previously considered to be uncommon in the first year of life.[3] The rarity of these conditions and controversies in the management prompted us to report our experience.

MATERIALS AND METHODS

This is a retrospective study of 49 patients treated at the authors Institutions over 10 years from January 2000 to January 2010. Baseline investigations, ultrasonography of the whole abdomen was done in all cases. CT scan was not available in the initial part and has been done in the last 20 patients. Tumor marker estimation (serum alpha fetoprotein and beta human chorionic

Original Article

ABSTRACT

Aim: Review of the clinical presentation, types (histology), and stages of presentation and overall outcome of ovarian tumors. Materials and Methods: This is a retrospective study. Forty nine girls from 3days to 12 years were included in the study. Results: Fourteen girls had benign and thirty three had malignant ovarian tumors. One girl had bilateral ovarian non-Hodgkin lymphoma. Dysgerminoma (40%) was the commonest malignant tumor followed by malignant teratoma (16.6%). Conclusion: Pain and abdominal lump are the most common modes of presentation. Prognosis depends on the size of the tumor, stage and histology of the tumor. Conservative surgery should be the aim. Multidisciplinary management gives good prognosis.

KEY WORDS: Dysgerminoma, endodermal sinus tumor, germ cell tumors, Ovarian tumors

Ovarian cysts and tumors in infancy and childhood

Madhumita Mukhopadhyay, Ram Mohan Shukla1, Biswanath Mukhopadhyay1, Kartik C. Mandal1, Amit Ray1, Neha Sisodiya1, Mahadev Prasad Patra1

Department of Pathology, Post-Graduate Medical Education and Research, 1Pediatric Surgery, N R S Medical College, Kolkata, India

Address for correspondence: Prof. Biswanath Mukhopadhyay, 7E, Dinobandhu Mukherjee Lane, Sibpur, Howrah - 711 102, West Bengal, India. E-mail: mukhopadhyay_b@yahoo.in

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Source of Support: None, Conflict of Interest: No.

17Journal of Indian Association of Pediatric Surgeons / Jan-Mar 2013 / Vol 18 / Issue 1

RESULTS

The age of presentation ranged from 3 days to 12 years with majority of patients seen between the age of 9 to 12 years. The most common presentation were lower abdominal pain (80%), palpable mobile abdominal lump (73%), urinary symptoms in the form of increased frequency (46%) and dysuria (73%). Lesions in the right ovary were seen in 26 patients; on the left in 22 patients and bilateral in 1 patient.

Interestingly, two patients were admitted with acute pain abdomen and revealed twisted ovarian cyst at laparotomy. One of them presented with acute retention of urine and had twisted dermoid cyst of left ovary. The youngest patient, aged 3 days was admitted with peritonitis. She had perforation of transverse colon due to Hirschsprung’s disease and found to be having a simple cyst in the left ovary [Figure 2]. Another neonate, aged 6 days, presented to us as antenatally diagnosed ovarian cyst in the left side of 6 cm diameter. The baby was crying most of the time. Initially, the parents were not willing for operation. They brought the baby back at the age 15 days. The lower abdomen was tender and distended. Laparotomy revealed twisted ovarian cyst with gangrene of the ovary. The final outcome of HP examination of ovarian tumors with their FIGO staging, outcome of surgery and combination systemic chemotherapy are given in Table 1. The follow up period was 1 to 10 years.

DISCUSSION

Malignant ovarian tumors are rare, particularly in patients under 5 years of age.[4] Solid ovarian tumors are uncommon in pediatric population but when occurred, they are a major source of anxiety for the patients and their family. Ovarian tumors must be considered in the

differential diagnosis of young girls with abdominal pain, mass or other non-specific symptoms. In a large study, Templeman et al, stated that malignant ovarian tumors in children and adolescents are rare, accounting for 0.9% of all malignancies in this age group.[5] Hassan et al, confirmed that during the first two decade of life ovarian tumors represent the most frequent tumors of female genital tract.[6]

Two-third of malignant tumors in children are germ cell tumors.[7] Most of the malignant germ cell tumors are dysgerminoma,[8] in contrast to adult ovarian tumor where 90% are epithelial cell origin and about 10% are non-epithelial.[9] Ovarian lesions can present with variable signs and symptoms, most often the clinical presentation does not differentiate benign tumor from a malignant tumor as found in our series. Abdominal pain was the most common symptom encountered. Ovarian masses often present with abdominal complaints that can mimic other diseases, in particular, appendicitis.[10] A mobile, palpable abdominal mass was the most frequent physical finding. Bagolan et al,[11] confirmed that patients with echogenic cysts with fluid debris, retracting clot or septation should undergo surgery because such findings were associated with complications.

The treatment of anechoic cysts depends on their size at diagnosis. Those that were less than 5 cm in diameter were unlikely to cause problems. The cysts that were larger than 5 cm in diameter often required surgery for postnatal torsion or other complications as reported in literature[11] Neonatal ovarian cysts are rare. Neonates, influenced by maternal hormones, are likely to have functional cysts.[12] In this report, three cysts had undergone torsion and in none of these could the ovary be preserved.[11] The incidence of ovarian torsion is around 17% and is more common in patients with ovarian cysts and mature cystic teratomas than

Table 1: Details of histology and the management doneNature Histopathology Number FIGO staging Surgery ChemotherapyBenign Simple follicular Cyst 02 Ovariectomy-01

Ovarian cystectomy-01Benign Mature teratoma 12 Ovarian cystectomyBenign Granulosa cell tumor 02 Ovarian cystectomyMalignant: Dysgerminoma 13 Stage I- 4 patients.

Stage II-6 patients.U/L salpingo-oophorectomy VAC-4

BEP-9Immature teratoma 8 Stage I-5 patients

Stage II-2 patientsU/L salpingo-oophorectomy VAC-5

BEP-3Mixed germ cell tumors 5 Stage I-2 patients

Stage II-2 patientsStage III-1 patient

U/L salpingo-oophorectomy VAC-2BEP-3

B/L Non-Hodgkin Lymphoma 1 Stage II B/L salpingo-oophorectomy COMPEndodermal Sinus tumor 6 Stage II-2 patients

Stage III-3 patientsU/L salpingo-oophorectomy VAC-2

BEP-4BEP: B-Bleomycin, E-Etoposide, P-Prednisolone; VAC: V-Vincristine, A-Actinomycin D, C-Cyclophosphamide; COMP: C-Cyclophosphamide, O-Oncovin, M-Methotrexate, P-Prednisolone

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those with malignant tumors.[5] All surgical procedures for ovarian cysts should spare functional ovary as much as is technically possible. Simple cyst may be fenestrated or excised with preservation of the ovary.[13] Laparoscopy has become the approach favored by most pediatric surgeons for the treatment of ovarian cysts.[13] We started doing laparoscopy late and have done laparoscopic ovarian cystectomy in three benign ovarian cysts.

We have treated 49 patients of whom 14 patients had benign (12 dermoid cysts, 2 patients with granulosa cell tumors), 33 malignant ovarian tumors and two neonates with simple cyst. As per FIGO classification, we had 12 patients with Stage I (39%), 13 patients with Stage II (42%) and 7 patients with Stage III tumors (19 %). We treated one patient of bilateral Non-Hodgkin lymphoma of ovaries (stage II). Dysgerminoma was the most common malignant tumor (40%) in our study followed by malignant teratoma (16.6%). Dysgerminomas have a uniform microscopic appearance consisting of large, round cells that have vesicular nuclei and clear to eosinophilic cytoplasm [Figure 3]. These cells resemble

primordial germ cells. Lymphoid infiltrates may be present. 18/31 (58%) presented to us in stage II and stage III conditions; though fortunately none of them had features of distant metastasis (such as liver and lung metastasis), but 2 patients were having ascites and one patient of malignant teratoma intermediate had peritoneal seedlings.

Ovarian malignant tumors are a heterogeneous group including yolk sac tumor [Figure 4], choriocarcinoma, immature and mature teratoma; careful evaluation is important to avoid missing malignant elements in this lesion.[14] Present policy is conservative surgery to preserve fertility, even in patients with advanced disease.[15]

All our patients underwent conservative surgery in the form of ovarian cystectomy and unilateral salpingo-oophorectomy sparing uterus and contralateral ovary and bilateral salpingo-oophorectomy in one patient. All patients with malignant ovarian tumors received systemic chemotherapy in the form of vincristine, actinomycin D and cyclophosphamide (VAC) regimen

Figure 1: Removed right and left ovaries in Non-Hodgkin’s lymphoma Figure 2: Showing perforation of transverse colon and left ovarian cyst

Figure 3: Photomicrograph of dysgerminoma Figure 4: Photomicrograph of yolk sac tumor

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(8 cases) up to 1988 and then all were given bleomycin, etoposide and cyclophosphamide (BEP) regimen (22 cases) and COMP regimen in bilateral NHL in 1 case.

Out of 14 patients of benign ovarian tumors, only one patient had re-operation for adhesive intestinal obstruction. We had two newborn babies with ovarian cyst and both the neonates are doing well. The patient of non-Hodgkin's lymphoma (bilateral) was doing well for seven years but then lost to follow up. Primary ovarian lymphoma is very rare and only isolated case reports are available.[16] We treated two patients of granulosa cell tumors. Granulosa cell tumors of the ovary are prepubertal in 5% of the patients and often present with virilization, abdominal lump, pain abdomen and menstrual irregularities.[17] They are characterized by the presence of small cyst like spaces called Call-Exner bodies [Figure 5]. There was 90% survival in Stage I tumors but the result was not satisfactory in stage II (70%) and stage III tumors (40%).

CONCLUSION

Ovarian tumors are uncommon in girls below the age of 12 years. Pain and abdominal lump are the most common modes of presentation. Dysgerminoma is the most common malignant ovarian tumor in our

experience. Prognosis depends on the size of the tumor, stage and histology of the tumor. Conservative surgery should be the aim. Multidisciplinary management gives good prognosis.

REFERENCES

1. Singhal SR, Rattan KN, Nanda S, Singhal SK. A 9-Year Review of ovarian masses in Children and Adolescents. J Gynecol Surg 2008;24:113-6.

2. Andres MM, Costa E, Canete A, Moreno L, Castel V. Solid ovarian tumors in childhood: A 35-year review in a single institution. Clin Transl Oncol 2010;12:287-91.

3. Chiramonte C, Piscopa A, Cataliotti F. Ovarian cysts in newborn. Pediatr Surg Int 2001;17:171-4.

4. Brookfield KF, Cheung MC, Koniaris LG, Sola JE, Fischer AC. Population based analysis of 1037 Malignant Ovarian Tumors in the Pediatric Population. J Surg Res 2009;156:45-9.

5. Templeman C, Fallat ME, Blinchevsky A, Hertweck S. Non inflammatory ovarian masses in girls and young women. Obstet Gynecol 2000;96:229-33.

6. Hassan E, Creatsas G, Deligeorolgou E, Mihalas S. Ovarian tumours during childhood and adolescence. J Gynaecol Oncol 1999;20:124-6.

7. Lazer EL, Stolar CJ. Evaluation and management of paediatric solid tumours. Sem Paediatr Surg 1998;7:29-34.

8. Akyuz C, Varan A, Buyukpamukcu N, Kutlul T, Büyükpamukçu M. Malignant ovarian tumors in children: 22 years of experience at a single Institution. J Paediatr Haematol Oncol 2000;22: 422-7.

9. Berek JS. Novak’s Gynecology. 13th ed. In: Berek JS, editor. Philadelphia: Lippincott-Williams and Wilkins; 2002. p. 1245-82.

10. Pomeranz AJ, Sabins S. Misdiagnosis of ovarian masses in children and adolescents. Pediatr Emerg Care 2004;20:172-4.

11. Bagolan P, Rivosecchi M, Giorlandino C, Bilancioni E, Nahom A, Zaccara A, et al. Prenatal Diagnosis and clinical outcome of ovarian cysts. J Pediatr Surg 1992;27:879-81.

12. Shauris RN, Gohary E, Cook RC. Ovarian cysts and tumors in infancy and childhood. Ann R Coll Surg Engl 1985;67:17-9.

13. Brandt ML, Helmarth MA. Ovarian cysts in infants and children. Semin Pediatr Surg 2005;14:78-85.

14. Valla JS. Long term outcomes. Pediatric Surgery and Urology. In: Stringer MD, Oldham KT, Mouriquand PD, editors. Cambridge: Cambridge University Press; 2006. p. 708-23.

15. Low JJ, Perrin C, Cradon AJ, Hacker NF. Conservative Surgery to preserve ovarian function in patients with malignant ovarian tumors - A review of 74 cases. Cancer 2000;89:391-8.

16. Turken A, Ci ftci A, Akcoren Z, Köseoglu V, Akata D, Senocak ME. Primary ovarian lymphoma in an infant: Report of a case. Surg Today 2000;30:305-7.

17. Merras-Salimo L, Vettenranta K, Heikinheimo M. Ovarian granulosa cell tumors in childhood. Pediatr Hematol Oncol 2002;19:145-56.

Figure 5: Photomicrograph of granulosa cell tumor with call–exner bodies

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