Long-term surgical outcomes in pediatric ovarian neoplasms: 20-year single-center experience.

PURPOSE: The management of pediatric ovarian neoplasms (ON) is based on finding a balance between adequate surgical treatment and future reproductive capacity. We aimed to evaluate long-term results of patients who underwent surgery for ON. METHODS: A retrospective cohort study design was used. Medical records of patients with ON were reviewed. They were invited to participate in a telephone-based survey assessing complaints, menstrual status, and post-surgical recurrence. RESULTS: Eighty-five patients were operated for ON between 1995 and 2015. Median age at surgery was 14.7 years. 62.4% of patients had ovary-sparing surgery (OSS). Median tumor size in oophorectomy group was significantly larger than OSS group (p = 0.029). Median length of follow-up was 5.1 years. Recurrent/metachronous disease was not significantly different between OSS and oophorectomy groups (p = 1.000). In OSS group, irregular menses (p = 0.004) and painful menses (p = 0.002) were significantly higher than oophorectomy group. CONCLUSION: The main goal of treatment in pediatric ON is to find the right balance between adequate and appropriate tumor resection and maximal effort for fertility preservation. Our results showed no difference between oophorectomy and OSS in the terms of recurrence. Although irregular and painful menses were found to be significantly higher in the OSS group, longer follow-up and prospective studies are needed to clarify this issue.

Fiberoptic endoscopic evaluation of swallowing (FEES) study: the first report in children to evaluate the oropharyngeal dysphagia after esophageal atresia repair.

BACKGROUND: The aim of this study was to evaluate the swallowing problems by fiberoptic endoscopic evaluation of swallowing (FEES) study in both short- and long-gap patients after esophageal atresia (EA) repair. METHODS: Hospital records of patients who had undergone surgery for EA were reviewed retrospectively. Patients were divided into two groups as short-gap (SG) group (n:16) and long-gap (LG) group (n:10) to compare the swallowing problems. FEES study was performed, and the results were discussed in detail. RESULTS: There were twenty-six (16 M/10 F) patients with a mean age at evaluation was 7.52 ∓ 3.68 years. Mean follow-up period was 75.35 ∓ 44.48 months. In FEES study, pharyngeal phase abnormalities were detected in 10 patients (38.4%). Pharyngeal phase abnormalities were detected significantly higher in LG group (p:0.015). Laryngeal penetration/aspiration was seen in four patients on FEES study (15.3%). All of them was in LG group (40%). Laryngeal penetration/aspiration was seen significantly higher in LG group (p:0.014). CONCLUSION: This is the first study to conduct FEES study in children after esophageal atresia repair to evaluate their swallowing conditions. Even though our sample is small, swallowing problems are more common than expected in the cases of LG when compared to SG.

Diffusion tensor imaging in anorectal malformations: a pilot study for the evaluation of lumbosacral plexus.

INTRODUCTION: Neurogenic bladder with anorectal malformations (ARM) is a well-known yet understudied topic. Diffusion tensor imaging (DTI) is a special usage of MRI that can evaluate peripheric nerves. The aim of this study is to evaluate the lumbosacral plexuses of patients with ARM using DTI. PATIENTS AND METHODS: Patients with ARM willing to participate were included. Patient files were reviewed, a questionnaire was made, and DTI was performed. Fractional anisotropy (FA) and apparent diffusion coefficient (ADC) were computed for the right and left lumbosacral plexuses. RESULTS: There were 18 patients and 12 controls. Groups were similar in terms of sex (p = 0.232) and age (p = 0.853). There was a significant difference only for ADC of the left plexus. There was visible asymmetry in tractographies and DTI parameters of two patients with severely deformed pelvises (image 1) but also in another patient with a normal sacrum. In addition, despite asymmetry, FA and ADC were similar to controls also in these patients. CONCLUSION: This is the first study presenting quantitative data about the lumbosacral plexus in ARM. We think the most interesting finding of this study was observing the normal values even in the patients with severely malformed sacrum which is yet to be validated with further studies.

Long-term outcome and efficiency of symptom-selective approach to assess gastroesophageal reflux prior to gastrostomy in neurologically impaired children.

PURPOSE: To evaluate the necessity of preoperative screening for gastroesophageal reflux (GER) prior to gastrostomy in neurologically impaired children. METHODS: Medical records of neurologically impaired children, who have undergone laparoscopic gastrostomy between January, 2004 and June, 2018, were retrospectively reviewed. Before the year of 2014, all patients who required gastrostomy had been routinely screened for GER pre-operatively, but after the year of 2014, only the ones with GER-related symptoms were tested. The characteristics and outcomes of Routine Screening (RS) and Selective Screening (SS) periods were compared. RESULTS: There were 55 and 54 patients in the RS and SS periods, respectively. Demographics, primary pathologies, and mean follow-up durations (> 2 years) were similar. The rate of GER screening was significantly lower in the SS period (29.6% vs. 63.6%). The rate of Laparoscopic Nissen Fundoplication (LNF) combined with gastrostomy was significantly lower in the SS period (14.8% vs. 38.2%). During follow-up, the rates of new-onset GER symptoms (13% vs. 11.7%) and LNF requirement later on (6.5% vs. 8.8%) were statistically similar between the two periods. CONCLUSION: Routine screening for GER is not necessary prior to gastrostomy in neurologically impaired children. Symptom-selective screening algorithm is safe and efficient in the long term.

General complications after surgery for anorectal malformations.

The introduction of posterior sagittal anorectoplasty (PSARP) by deVries and Peña in the early 80s has impacted to the treatment of patients with anorectal malformations (ARM). It gained great recognition worldwide in a very short time, and since then, surgeons dealing with the treatment of this complex malformation could achieve tremendous progress in contemporary management of this anomaly. Despite the growing experience and body of information globally, the treatment of ARMs continues to be a challenge to the pediatric surgeons due to the nature and the variability of the anomaly, and short- and long-term problems continue to exist even after nearly 40 years of the PSARP era. Today, knowing more about it, pediatric surgeons are committed to do more for their ARM patients to have them as physically and socially healthy individuals.

Intracellular localization of beta-catenin expression plays a possible prognostic role on the outcome of hepatoblastoma patients.

PURPOSE: Wnt/Beta-catenin pathway plays an essential role in liver development and regeneration. Abnormal activation in this pathway leads to development of hepatoblastoma (HB). Although its importance has invoked attention, its prognostic role is debatable. We aimed to evaluate the significance of intracellular localization of beta-catenin (BC) expression in the outcome of hepatoblastoma patients. METHODS: Medical records of HB patients between 2004 and 2018 were reviewed. Patients were grouped according to intracellular localization of BC expression by immunohistochemistry as being cytoplasmic or nuclear. Demographics, radiological images, PRETEXT classifications, vascular involvement, risk groups, chemotherapy responses, and survival rates were analyzed and compared between groups. RESULTS: There were 41 patients. Thirteen patients were excluded for unavailability of records in four, negative/unclear BC expressions in seven. Cytoplasmic expression of BC was observed in 17 patients whereas 13 patients displayed nuclear expression. Demographics were similar in both groups. Cytoplasmic BC expression was associated with poor chemotherapy response (p = 0.001) and increased vascular involvement (p = 0.0162) requiring more extensive surgeries (p = 0.039). CONCLUSION: Although the numbers are limited in our series, the intracellular localization of BC expression has been found to be a promising determining factor for hepatoblastoma prognosis. With larger patient series, more reliable results can be achieved.

Pulmonary Metastasectomy of Sarcoma: Is the Ratio of Surgical Margin to Nodule Size a Prognostic Factor?

BACKGROUND: Main prognostic factors of improved survival after pulmonary metastasectomy (PM) for osteogenic and soft tissue sarcomas are suggested as histological type, number and size of pulmonary nodules, and disease-free interval (DFI). METHODS: Sixty-nine patients who underwent PM between January 1999 and December 2017 were evaluated retrospectively. Relations between parameters and prognostic risk factors for overall survival (OS) and disease-free survival (DFS) were evaluated. RESULTS: Osteosarcoma was the most common histologic type (36.2%) and 21 of 25 cases were seen under the age 20 years (p < 0.001). Comparison of patient groups including osteosarcoma and nonosteosarcoma patients showed significant difference according to age (p < 0.001), nodule size (p = 0.033), ratio of surgical margin to nodule size (p = 0.007), and DFI (p = 0.039). Univariate analysis showed that the number of nodules (p = 0.008), ratio of surgical margin to nodule size (p = 0.001), and localization of nodule (p = 0.039) were significant factors associated with DFS. Also, nodule size (p = 0.042), number of nodules (p = 0.003), ratio of surgical margin to nodule size (p < 0.001), and laterality (p = 0.027) were significant prognostic factors associated with OS. Cut-off values of ratio of surgical margin to nodule size for DFS and OS were calculated as 0.94. Logistic regression analysis determined the ratio of surgical margin to nodule size as the common significant risk factor for DFS and OS. CONCLUSIONS: Our study showed that the ratio of surgical margin to nodule size ≥ 1 should be taken as a common risk factor for DFS and OS. Therefore, resection of nodules with the possible widest surgical margin is an important point of PM.

Thoracoscopic treatment of left-to-right shunt in a child with scimitar syndrome.

Scimitar syndrome represents a rare variant of partial anomalous pulmonary venous connection with right lung hypoplasia, dextrocardia, and concomitant airway-vessel abnormalities. Surgical correction is preferred in symptomatic patients or in patients with increased left-to-right shunt. In this report, the first case of scimitar syndrome with dual arterial supply and venous drainage to be treated with thoracoscopic approach is presented.

Laparoscopic Treatment of Median Arcuate Ligament Syndrome: A Rare Cause of Chronic Severe Abdominal Pain.

Median arcuate ligament syndrome is a rare disorder characterized by chronic postprandial abdominal pain and weight loss caused by compression on celiac artery. A 17-year-old girl with chronic severe abdominal pain and weight loss was referred to our clinic. Other causes of chronic abdominal pain were investigated and excluded. The compression on celiac artery was detected on Doppler ultrasound and diagnosis was confirmed by computed tomography angiography. The patient underwent laparoscopic release of median arcuate ligament. There were no intraoperative complications; however, partial pain response was observed postoperatively that necessitated para-spinal ganglion blockage. The patient is symptom-free in 1-year follow-up period.

Acquired right diaphragmatic hernia following pediatric living donor orthotopic liver transplantation.

ADH following OLT is a rare entity. Herein, we report a case of Alagille syndrome who developed ADH secondary to OLT, and possible etiological causes are discussed in light of the literature.

The Role of Surgery in Antenatal Ovarian Torsion: Retrospective Evaluation of 28 Cases and Review of the Literature.

STUDY OBJECTIVE: Antenatal ovarian torsion (AOT) is rare and requires differentiating from other congenital cystic masses of the abdomen and pelvis in neonates. In this study, it was aimed to evaluate the prenatal characteristics and postnatal outcomes of AOT. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: Hospital records of patients (n = 28) with diagnosis of AOT between 2004 and 2020 were reviewed and their prenatal characteristics, postnatal examination, imaging, operative, and histopathological findings were evaluated. RESULTS: There were 28 patients during the study period. In most of the patients (25/28; 89.3%) AOTs were detected prenatally. All were term babies with a mean birth weight of 3010 ± 466.6 g. Mean maternal age was 25.75 ± 3.65 years. Prenatal history was event-free in all and none had additional anomalies. Physical examination revealed mobile intra-abdominal cystic lesions in 16 (57.1%) patients. AOTs mimicked other pathologies as intestinal duplication cyst or mesenteric cyst (n = 7), complex ovarian cyst (n = 3), mature cystic teratoma (n = 3), simple renal cyst (n = 1), and ectopic kidney (n = 1) in 15 (53.6%) patients in postnatal ultrasonography. Elective laparoscopic surgery was performed in 26 (92.8%) patients. The other 2 patients required emergency open surgery because of acute symptoms. Ovaries were autoamputated in 17 (60.7%) patients. Histopathological examination revealed necrosis and dystrophic calcification in all specimens. There was neoplastic involvement in 2 patients (serous cystadenoma and gonadoblastoma). No complication occurred in the early follow-up period (mean: 73.9 ± 46.8 months). CONCLUSION: AOTs can be detected easily during the antenatal period. Neoplastic involvement with AOTs is rare but possible. Although AOTs might mimic other cystic pathologies, it should be one of the first diagnoses to be considered, in the presence of a palpable intra-abdominal mass and complex cystic lesion in infant girls. It can be efficiently and safely managed using minimally invasive techniques.

Transoral endoscopic thyroidectomy vestibular approach (TOETVA): A novel surgical technique for scarless thyroidectomy in pediatric surgery.

Transoral endoscopic thyroidectomy vestibular approach (TOETVA) is a novel surgical technique in thyroid surgery that provides a feasible opportunity to avoid a visible neck scar. In recent years, TOETVA became popular in the thyroid surgery of selected adult patients with cosmetic concerns. We conducted a right lobectomy with the TOETVA technique in a 17-year-old adolescent with a suspicious thyroid nodule the present report aimed to describe the details of the operative technique step by step. Level of evidence: LEVEL IV, case series with no comparison group.

Comparison of the multi-epitope recombinant antigen DIPOL and hydatid fluid for the diagnosis of patients with cystic echinococcosis.

The use of serological tests containing multiple immunodominant antigens rather than single antigens have the potential to improve the diagnostic performance in Cystic Echinococcoses (CE) as a complement tool to clear the inconclusive imaging data. Here, we comparatively evaluated the diagnostic value of Hydatid Fluid (HF) and the recently described recombinant multi-epitope antigen DIPOL in IgG-ELISA in a clinically defined cohort of CE patients. The serum samples from 149 CE patients were collected just before surgical or Percutaneous- Aspiration- Injection- Reaspiration (PAIR) procedures. Additionally, serum samples of patients with other parasitic infections (n=49) and healthy individuals (n=21) were also included in the study as controls. To investigate the association between the genotype of the parasite and DIPOL, cyst materials from 20 CE patients were sequenced. In terms of overall sensitivity, HF was higher than DIPOL (82.55%,78.52%, respectively). However, while the sensitivity of HF was higher than DIPOL in patients with active and transitional cysts (83.3%, 75.4%, respectively), sensitivity of DIPOL in inactive cysts was higher compared to HF (95.6%, 78.3%, respectively). The sensitivity of DIPOL depending on cyst stage was statistically significant (P= 0.041). In terms of specificity, DIPOL was found to be better than HF (97.71%, 91.43%, respectively). By genotyping, the majority of 20 patients showed G1 genotype (80%). All patients harboring G3 and G1/G3 cyst genotypes were positive with both antigens, while 87.5% of patients with G1 genotype were seropositive with HF and 75% with DIPOL. The overall sensitivity and high specificity of DIPOL suggest that this recombinant protein containing immunodominant epitopes is a potential substitute for the HF by serological tests for the diagnosis of CE.

Single-incision laparoscopy-assisted gastric transposition combined with thoracoscopic esophagectomy for esophageal replacement.

INTRODUCTION: We present a novel approach to single-incision laparoscopy-assisted gastric transposition combined with thoracoscopic esophagectomy in a child with long-segment corrosive esophageal stricture (CES). MATERIALS AND SURGICAL TECHNIQUE: A 2.5-year-old boy with a history of caustic ingestion underwent periodic esophageal dilatations at 3-week intervals, four sessions of topical mitomycin C application, and a strict antacid/H2 antagonist therapy for 8 months. The esophageal replacement was indicated because of persistent corrosive esophageal stricture. First, thoracoscopic native esophagus dissection was performed. After gastric mobilization with single-incision laparoscopy, esophagectomy and esophagogastric anastomosis were performed through the cervical incision. There was no complication, and the patient had gained weight by the end of the 12-month follow-up. DISCUSSION: Single-incision laparoscopy-assisted gastric transposition combined with thoracoscopic esophagectomy is feasible, safe, and effective in patients with corrosive esophageal stricture.

Laparoscopic adrenalectomy in children: A 25-case series and review of the literature.

BACKGROUND: Laparoscopic adrenalectomy (LA) is the gold standard and is widely performed in adults, but its use in children is relatively new. We aim to present our experience in twenty-five children with diverse adrenal pathologies and to discuss an extensive review of pediatric LA in English literature. METHODS: Medical records of children with adrenal tumors admitted to a tertiary center and treated with LA were reviewed. Characteristics and outcome of patients were compared with results of a systematic Pubmed/Medline literature review. RESULTS: Transperitoneal LAs were performed for 12 malignant and 14 benign adrenal masses (size range: 2-8cm) in 25 children (median age:63months). Fourteen lesions were on the right side, and there was one bilateral case. There were no conversion and no complication within 36months of follow-up. A literature review revealed 437 pediatric LAs with left side predomination (51.4%). The indication was a malignant lesion in 60.2% of the cases (sizes range: 1-10cm). The transperitoneal route was preferred in 94.2% of the procedures. Conversion and complication rates were 7.5% and 3.1%, respectively. CONCLUSION: LA should be preferred in selected children with adrenal pathologies. The transperitoneal route seems to be the standard approach for pediatric surgeons. Preoperative planning and surgical expertise are the keys to success. TYPE OF THE STUDY: Case series with systematic literature review. LEVEL OF EVIDENCE: IV.

Retrospective Multivariate Analysis of Data from Children with Suspected Appendicitis: A New Tool for Diagnosis.

BACKGROUND: Decision-making for management may sometimes be difficult in acute appendicitis (AA). Various diagnostic scoring systems exist, but their sensitivity and specificity rates are far from ideal. In this study, the determination of the predictors and the effect of radiological data and developing a new scoring system were aimed. METHODS: Medical records of patients who were hospitalized for AA between February 2012 and October 2016 were retrospectively reviewed. All data were compared between patients with and without appendicitis. The multivariate analysis was performed to define significant variables and to examine the sensitivity and specificity of each group of predictors including radiological data. A new scoring system (NSS) was formed and was compared with two existing scoring systems: pediatric appendicitis score (PAS) and Alvarado scoring system (ASS) by using reclassification method. RESULTS: Negative appendectomy rate was 11.3%. Statistical analysis identified 21 independently significant variables. The heel drop test had the highest odds ratio. Sensitivity and specificity rates of clinical predictors were 84.6% and 94.8%, respectively. Radiological predictors increased the sensitivity rate to 86.9%. Sensitivity and specificity rates for PAS, ASS, and NSS were 86.8% and 83.9%, 84.7% and 81.6%, and 96.8% and 95.6%, respectively. The "re-assessed negative appendectomy rate" was 6.2% and false positive results were remarkably more common in patients with duration of symptoms less than 24 hours. CONCLUSION: Radiological data improves the accuracy of diagnosis. Containing detailed clinical and radiological data, NSS performs superiorly to PAS and ASS, regarding sensitivity and specificity without any age limitation. The efficiency of NSS may be enhanced by determining different predictors for different phases of the inflammatory process.

Radiological nodule behavior: A critical parameter in the surgical management of pediatric pulmonary metastases.

Divarci E, Arslan S, Dökümcü Z, Kantar M, Demirag B, Öniz H, Ertan Y, Alper H, Erdener A, Özcan C. Radiological nodule behavior: A critical parameter in the surgical management of pediatric pulmonary metastases. Turk J Pediatr 2018; 60: 372-379. Radiological nodule behavior (RNB) means the course of nodule appearance on consecutive CT scans. In this study, we aimed to discuss the effects of RNB on prognosis of patients with pulmonary metastasis. Retrospective analysis of patients who underwent pulmonary metastasectomy (PM) between 2005-2015 was performed. RNBs were grouped as stable, enlarging or new rising nodules. The effects of RNBs were analyzed on recurrence, 1- and 3-year event free survival (EFS) and mortality. Twenty-seven patients (18 male) underwent PM with a median age of 15 years (3-18 years). The diagnoses were listed as osteosarcoma (13), Wilms` tumor (7), Ewing`s sarcoma (3), synovial sarcoma (2), rabdomyosarcoma (1) and mixed germ cell tumor of testis (1). RNBs were new rising in 15 patients (55%), enlarging in seven patients (26%) and stable in five patients (19%). Vital tumor metastasis was detected in all of the patients with enlarging nodules (100%); in 10 of the 15 patients with new rising nodules (66%) and none of the patients with stable nodules. None of the patients with stable nodules developed recurrence or died after PM (p˂0.05). In patients with enlarging nodules, metastases recurred and they died in the postoperative period. These findings were similar in different types of tumors. RNB could be used as a critical parameter in deciding surgical management strategies of pulmonary metastases. Stabile nodules should be observed by close follow-up with serial CT scans without surgery. All of the suspected new rising nodules should undergo surgical sampling to avoid unnecessary chemotherapy. Nodule progression under chemotherapy is a poor prognostic criteria for overall survival.

Topical Mitomycin C Application Is Effective Even in Esophageal Strictures Resistant to Dilatation Therapy in Children.

PURPOSE: Several treatment techniques may be used in the treatment of esophageal strictures. The purpose of this study was to present the effects of topical mitomycin C (TMC) as an useful adjunct to dilatation therapy in esophageal strictures. MATERIALS AND METHODS: A retrospective analysis of patients who underwent TMC between February 2015 and July 2016 was performed. Dysphagia score, periodic dilatation index, and number of dilatations were compared before and after intervention to investigate the efficacy of TMC. RESULTS: TMC was performed on 20 patients with a median age of 3.5 years (2 to 17 y). The diagnosis was corrosive esophageal strictures in 14 patients, anastomotic strictures in 5 patients, and congenital esophageal stricture in 1 patient. The length of the stricture was long in 10 patients (50%). The median dysphagia score decreased from 2 (1 to 3) to 0 (0 to 2) after application (P<0.001). The median number of dilatation sessions decreased from 5 (1 to 41) to 1 (0 to 11) after intervention (P<0.001). The median periodic dilatation index decreased from 1 (0.66 to 1.34) to 0 (0 to 1.33) after TMC (P<0.001). Regular esophageal dilatation was not necessary in 16 patients after application (80%). The length of the stricture did not affect the efficacy of TMC. The success of treatment was lower in patients with a long treatment period before TMC (>3 y) (50%). No complications were seen in a median follow-up period of 16 months (7 to 22 mo). CONCLUSIONS: TMC application has a significant positive effect as an adjunct to dilatation therapy in most of the patients with different types of esophageal strictures. It should be performed as a safe and efficient treatment option even in patients who were resistant to dilatation therapy.

Thoracoscopic Treatment of Pulmonary Hydatid Cysts May Have a High Morbidity Risk in Children: Retrospective Analysis.

OBJECTIVE: Thoracoscopic treatment of pulmonary hydatid cyst (PHC) has been considered to be a good treatment option in both children and adults for nearly 25 years. However, there have been very few pediatric studies published during this period. Our goal is to review our results and evaluate the efficiency of thorascopy in pediatric patients. MATERIALS AND METHODS: The medical records of patients with PHC who were surgically treated between 2005 and 2015 were reviewed. As all cysts larger than 5 cm in diameter were surgically removed, cysts less than 5 cm in diameter were medically treated and were not included in the study. Demographics, cyst characteristics, and operative/postoperative data were compared between patients who underwent thorascopy (thoracoscopy group) and patients who underwent thoracotomy (thoracotomy group). Chi-square and t-test were used for statistical analysis where appropriate. RESULTS: There were 26 consecutive children (14 girls, 12 boys; mean age 9.4±2.7) included in the study. Except for 2 incidentally diagnosed patients, all were symptomatic, 4 had multifocal lesions, and multiorgan involvement was detected in 11 patients. Thoracoscopy was performed in 10 patients, and conversion was necessary in 2 patients due to unsuccessful fistula ligation attempts. The thoracoscopy group included 8 thoracoscopically treated patients, and remaining patients constituted the thoracotomy group (n=18). Comparison of preoperative characteristics of the groups was insignificant, whereas the overall complication rate (residual bronchial fistula, prolonged air leak, pneumothorax, and localized air cyst) and median hospital stay were significantly higher in the thoracoscopy group. There was no mortality and no recurrence at the postoperative follow-up after 37.4 months. CONCLUSION: The thoracoscopic approach to PHC may have a high risk of conversion and postoperative complication rate. Thoracotomy in children still seems to be the approach of choice for PHC larger than 5 cm. Routine thoracoscopic hydatid cyst treatment is yet far from being the gold standard, whereas thoracoscopy may be preferred in selected patients.

Primary Ovarian Fibrosarcoma-An Unusual Tumor in an Adolescent: Case Report and Review of the Pediatric Literature.

BACKGROUND: Primary ovarian fibrosarcomas are rare and usually observed in perimenopausal and postmenopausal women. To our knowledge, to date, there are only 3 reports of ovarian fibrosarcoma in childhood and adolescence in the English-language literature. In this report, we aimed to present to our knowledge, the first pediatric case with advanced stage primary ovarian fibrosarcoma and to compare with previous cases. CASE: A 14-year-old teenage girl was admitted because of a giant abdominal mass. Imaging techniques revealed a giant heterogeneous and vascular ovarian mass. Salpingo-oophorectomy along with staging procedures were performed. Pathologic examination confirmed grade III ovarian fibrosarcoma with severe nuclear atypia and high mitotic count. Because of pulmonary metastasis, she received 4 courses of adjuvant combination chemotherapy (Ewing protocol). The patient withstood the treatment well and is disease-free at 2 years of follow-up. CONCLUSION: Fibrosarcoma of the ovary is an uncommon neoplasm with poor prognosis. To our knowledge, the presented patient is the first pediatric case with advanced staged primary ovarian fibrosarcoma to be treated with surgery and adjuvant chemotherapy.