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Muhammad Ali Sheikh, Jamshed Akhtar, Tayyaba Batool, Rubab Naqvi*, Raees Taqvi, Shazia Jalil, Aqil Soomro, Soofia Ahmed and Farhat Mirza

Objective: To analyze various clinical presentations and the surgical management of ovarian pathologies in pre-menarche girls. Design: Case series. Place and Duration of Study: Surgical Unit B, National Institute of Child Health, Karachi, from September 2002 to August 2004. Patients and Methods: Case records of all the pre-menarche girls child having an ovarian pathology and managed during the study period were reviewed. Age of the patients, presenting symptoms, investigations performed, surgical approaches, operative findings and final histological diagnosis were noted. Ovarian lesions were classified according to WHO criteria. System proposed by the Children’s Cancer Group and the Paediatric Oncology Group was used for staging of tumors. Following resection, patients with malignant tumors were followed-up by oncologist. Results: There were 18 patients with ovarian lesions. Age range of patients was from day 1 to 13 years. Mass in abdomen was the most common presenting symptom (60% patients ) followed by pain (50%). Right ovary was involved in 12 (55%) cases and left in 6 (45%). Six patients had non-tumorous cysts, 3 had benign tumors and 9 were with malignant lesions. Of the non-tumorous cysts, 3 patients had simple follicular cysts and 2 were hemorrhagic cysts. There were 2 patients with benign teratoma and one with dermoid cyst. Four patients had malignant teratoma, 4 dysgerminoma and one yolk sac tumor. Malignant tumors were all of stage IA according to Paediatric Oncology Group staging. Conclusion: Ovarian tumors are rare in paediatric age group especially in pre-menarche girls. Except one, all malignant tumors were found in patients above 7 years of age. Benign (tumorous and non-tumorous) and malignant lesions occurred with equal frequency in pre-menarche girls in this study. KEY WORDS: Ovary. Tumors. Child.

Ovarian lesions are considered rare in paediatric age group.1,2 They range from benign cysts to highly aggressive malignant tumors. Ovarian lesions are divided into non-neoplastic and neoplastic entities according to World Health Organization (WHO) criteria.3 Non-neoplastic lesions include simple cysts, follicular cysts etc. Ovarian cysts are most common during infancy and adolescence, which are hormonally active periods of development. Neoplastic lesions include benign and malignant tumors. Gynecologic malignancies account for approximately 2% of all childhood cancers and 60-70% of which are ovarian in origin.4 Ovarian malignancies are classified on the basis of cell of origin as germ cell tumors, epithelial tumors, sex cord stromal tumors and metastatic tumors. Ovarian malignancies in children differ from adults as epithelial tumors are rare and germ cell tumors occur typically in this age group.5,6 Ovarian tumors make upto 1.5% of all childhood malignancies.7 Gorber reported that the incidence of all ovarian tumors (both cystic and solid) was 17% from birth to 4 years of age, 28% between 5 and 9 years of age and 55% between 10 and 14 years of age. 8 In a review of 91 pediatric patients with ovarian masses, Brown et al. reported 36% incidence of malignancy in solid ovarian masses compared
Department of Paediatric Surgery, National Institute of Child Health, Karachi-75510. *Department of Pathology, National Institute of Child Health, Karachi-75510. Correspondence: Dr. Jamshed Akhtar, Surgical Unit B, National Institute of Child Health, Rafiquee Shaheed Road, Karachi-75510. Email: jamjim88@yahoo.com Received May 02, 2006; accepted January 17, 2007.

with 11% in complex masses and 3% in cystic masses.9 Reviewing the literature, it was found that in almost all of the reported cases, the upper age limit was not the same. Many included adolescent girls were those in whom menarche was already established. No study could be found where only premenarche girls were included. It is important to make this distinction as pre-menarche girls differ from those where menarche starts, with regard to the nature of pathology. Ovarian lesions pose diagnostic and therapeutic challenges because of their rarity and presentation that can mimic other common intraabdominal disorders. Ovarian lesions come to surgical attention in a variety of ways. Patients may present with acute abdominal pain and signs of peritonitis that can be difficult to distinguish from acute appendicitis.10,11 Patient may be referred with an abdominal mass or with endocrine disturbances. Ovarian lesions must be included in the differential diagnosis in any girl who presents for evaluation of abdominal pain or abdominal mass. As ovarian masses are rare, the management is occasionally unclear.12 Specific guidelines for rational management cannot be defined. It thus becomes important that more and more case series are reported so that a meta-analysis could be conducted. The main aim of management in all cases of ovarian tumors is cure while preserving fertility wherever possible. The aim of this paper is to analyze the ovarian pathologies at National Institute of Child Health, Karachi, as found in pre-menarche girls.


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A study of ovarian lesions in pre-menarche girls

This study was conducted at the Department of Pediatric Surgery, Surgical Unit B, National Institute of Child Health, Karachi, from September 2002 to August 2004. Case records of all pre-menarche girls, who were admitted with diagnosis of ovarian lesion, were reviewed and analyzed for age, presenting signs and symptoms, radiological reports, surgical procedure performed and final histological diagnosis. Lesions were classified according to WHO criteria into non-tumorous cysts, benign and malignant tumors.3 All the patients with malignancy were referred to Oncology department after surgery for further treatment.

ultrasound ovarian cyst was suspected. The echo of the contents was suggestive of hemorrhage. This patient underwent laparoscopy and following cyst aspiration, partial torsion of ovary was found. De-twisting was done successfully. Ovary looked viable and thus not removed. Four patients presented with abdominal mass and vague abdominal pain. Following confirmation on ultrasound, the surgical removal of cyst was done. Fallopian tube was saved in all. In one case, cyst was hemorrhagic. The histopathology revealed simple ovarian cyst. There were 3 patients with benign and 9 with malignant neoplasm. Patients with malignant mass had variable presentation. Two presented in emergency with abdominal distension and pain. They had history of recurrent abdominal pain as well. Other patients were having painless mass in lower abdomen. The ultrasound revealed complex mass. CT scan was performed in all these patients (Figure 1). The size

There were a total of 18 patients with ovarian lesions who were managed during 2 years period. Age of the patients ranged from day 1 to 13 years. There was one baby, who was suspected of ovarian cyst on antenatal ultrasound. Of the remaining 17 patients, one was an infant and 5 were upto 5 years of age (Table I). Abdominal mass was the most common presenting symptoms that was present in more than 60% patients. This was followed by pain in 50% patients. The nature of pain varied from acute pain to vague discomfort (Table I).
Table I: Master table showing various features of the ovarian lesions in pre-menarche girls. Age Distribution
Less than 1 year 1 – 5 year 6 – 10 year More than 10 year Range: Day 1 – 13 years Clinical Presentation Antenatal ultrasound detection Abdominal pain Acute Acute on recurrent Recurrent Vague pain Abdominal mass Surgical Procedures Performed Laparoscopic cyst aspiration and detorsion Oophorectomy Oophorectomy and salpingectomy Ovary Involved Right Left Bilateral Nature of the Tumors Non-tumorous cysts Benign lesions Malignant lesions 6 3 9 55% 45% None 1 4 12 1 1 2 5 1 11 2 5 6 5

Figure 1: A large ovarian tumor of mixed density occupying almost whole of the abdomen..

*One patient underwent non-operative management for antenatally diagnosed simple ovarian cyst; #One patient underwent laparoscopic cyst aspiration and detorsion of ovary for hemorrhagic simple ovarian cyst.

of mass and extent varied. Unilateral salpingo-oophorectomy was performed in all cases. Opposite ovary, liver, spleen, omentum and pelvic lymph nodes were inspected and palpated for any abnormality. Youngest patient, who had malignant tumor, was 2 years of age with yolk sac tumor of right ovary. All other patients with malignant tumors were above 7 years of age. The eldest child was 13 years old, who was also previously operated for high type of imperforate anus. She had malignant teratoma. All the patients with malignant tumor had stage IA disease, according to staging system used by the Children’s Cancer Group and the Paediatric Oncology Group and followed-up with Oncology department. No patient had complication related to surgery. Overall, 9 patients had right sided lesion and 8 left sided ovarian involvement. There was no patient with bilateral pathology.

The newborn, who had an antenatal diagnosis of cystic abdominal mass, on postnatal ultrasound, was found to have unilocular, simple ovarian cyst of 4 cm size in right ovary. This patient was put on regular follow-up and cyst gradually decreased over the period of observation and resolved completely at 14 months of age. There were 5 patients with non-tumorous cysts. One patient presented with acute abdominal pain of sudden onset and on

Ovarian tumors are unusual lesions in the paediatric population. Unlike adults, such neoplasms generally originate from the germ cell line. 2 Ovarian masses often present with abdominal complaints that can mimic other more common abdominal diseases. Pomeranz et al. reported that 38% of

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Muhammad Ali Sheikh, Jamshed Akhtar, Tayyaba Batool, Rubab Naqvi, Raees Taqvi, Shazia Jalil, Aqil Soomro, Soofia Ahmed and Farhat Mirza

their patients of ovarian lesions had preliminary diagnosis of appendicitis.6 Pain was one of the common presentations in this series (50% of the patients), which is consistent with other studies.2,6 Pain is acute when there is haemorrhage or torsion of tumor containing ovary. It is chronic due to slowly growing tumor causing capsular distension. One patient had partial ovarian torsion due to a cyst. Mass abdomen was the presenting symptom in 60% of our patients, which was also noted in other series. 9 None of our patients presented with endocrine disturbances. Many factors like patient’s age, menarche status etc. should be kept in mind when managing patient with ovarian tumors. As ovaries are abdominal organ in children so it should be a part of differential diagnosis of pain and abdominal mass. There are two peak periods where ovarian cysts are commonly found, one in the first year of life and second around the time of menarche. The second peak is due to gonadotrophin release from pituitary. In-utero, follicular cysts develop under the influence of maternal, placental, and fetal hormones. Follicular cyst have been detected through antenatal ultrasonography as early as the 28th week of gestation. After birth, the infant is removed from the increased hormonal stimulus and regression of these cysts is expected. Follicular cysts continue to develop throughout childhood. In a study of 1,818 girls between birth and 8 years of age, an incidence of 2 – 5% has been reported. 13 After birth most ovarian cysts will regress owing to lack of hormonal stimulation. Until this occurs, the adnexa has an increased risk for complications, primarily torsion. It is, therefore, suggested that a cyst, which is unilocular, and echoic and less than 5 cm, should be observed. The one, larger than 5cm, can be aspirated. If it recurs then it should be explored. The complex and hemorrhagic cyst should always be explored. 14 One of the patients had antenatal diagnosis of ovarian cyst, which resolved spontaneously over a period of 14 months. It is important that this mode of treatment was reserved for only those cases where good follow-up was ensured and parents could be sensitized about possible complications during the period of observation. For treatment of pediatric ovarian cysts, which do not resolve by appropriate observation, intervention is required. There should always be an attempt to spare ovary.15 Laparoscopy is helpful in this regard. In this series only one patient had laparoscopic cyst aspiration.16 Germ cell tumors are the most common tumor of pediatric age group. They arise from primordial germ cell that become malignant and if it remains undifferentiated, it develops into dysgerminoma. If it follows a pathway of embryonal transformation, it may result in mature or immature teratoma and if follows, extra embryonic differentiation may result in yolk sac tumor. Among benign tumors, teratomas are the commonest variety as reported by De Silva et al.13 There were 7 cases of benign and mature teratomas in this series. Only one of the patients with malignancy was under 2 years of age. The rest were more than 7 years old. The risk of malignancy increases with increasing age, as reported by other authors. 17 Brown et al. reported that risk of malignancy was 3% in the children upto the age of 8 years and risk increased to 33% for children older than 8 years. So, early diagnosis of ovarian tumors carries good prognosis. All of the tumors in this series were germ cell tumors. No tumor of epithelial origin was found.9 Dysgerminoma has been reported to be the most common germ cell tumor. 15

According to Cass et al. ovarian neoplasms are estimated to occur at a rate of approximately 2.6 cases per 100,000 girls per year. In a study of 16 years, 102 patients upto 20 years of age were identified having ovarian pathology. Only 10 patients were having malignant neoplasm, of which only 4 were dysgerminoma.1 In the present study of 18 patients, there were also 4 patients with dysgerminoma. There seems to be higher percentage (22% in comparison with 4%). In a series reported by Gribbon et al. in a period of 43 years, 38 patients with malignant ovarian tumors were managed. It included 4 bilateral tumors. The ages of patients included were upto 16 years. There were 8 dysgerminomas (21%) and 4 teratomas. In this study, there was no patient with bilateral tumor. Dysgerminoma is most primitive of germ cell tumors, a counterpart of seminoma in females and is reported to be the most common malignant tumor in children. It is extremely chemo- and radio sensitive. No serum markers are produced by this tumor. It is bilateral in 10 -15% of cases. Selective biopsy and frozen section is advised of any suspicion area of opposite ovary. 17 In this study, dysgerminoma and malignant teratoma were found in equal number.

Though ovarian lesions are rare in paediatric population, yet, they should be considered in differential diagnosis of girls presenting with abdominal pain and / or mass. Ultrasound is a good screening investigation but it is highly operator dependent. Laparoscopy plays an important role not only in establishing diagnosis but also in the treatment. A multidisciplinary approach, with the involvement of paediatric oncologist, is mandatory for the management of these lesions. The patients, in whom ovary is removed, should always have this information available to them as an adult, because it may have bearing on their future reproductive potential.

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adolescents. Pediatr Emerg Care 2004; 20: 172-4. 11. Nour S, MacKinnon AE, Dickson JA. Ovarian cysts and tumors in childhood. J R Coll Surg Edinb 1992; 37: 39-41. 12. Majhi AK, Bhattacharya D, Sarkar K, Mondal T, Sengupta P. Ovarian neoplasms in adolescence and childhood: an analysis of twenty cases. J Indian Med Assoc 2005; 103: 422-4. 13. De Silva KS, Kanumakala S, Grover SR, Chow CW, Warne GL. Ovarian lesions in children and adolescents: an 11-year review. J Pediatr Endocrinol Metab 2004; 17: 951-7. 14. Hayes-Jordan A. Surgical management of the incidentally identified ovarian mass. Semin Pediatr Surg 2005; 14: 106-10.

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