title: Ovarian sex cord stromal tumours in children and young girls - a more than two decade clinicopathological experience in a developing country, Pakistan.
abstract: BACKGROUND
  Ovarian sex-cord stromal tumours (SCST) are rare, and relatively infrequent in children. These have to be distinguished from more common germ cell tumors in children and also from benign epithelial neoplasms.
  
  
  OBJECTIVE
  The purpose of our study was to report the clinical and pathological findings in young patients with these tumours in our population.
  
  
  METHODS
  The present observational cross-sectional study included all subjects <21 years of age diagnosed with ovarian SCST, in Aga Khan University Hospital Histopathology Laboratory, Karachi, Pakistan, from January 1992 till July 2013.
  
  
  RESULTS
  Of the total of 513 SCSTs presented during the study period, 39 fulfilled inclusion criteria and were assessed. The age range was 4-250 months. Most of the tumours presented at stage-1 and an abdominal mass was the most common presenting symptom, along with menstrual disturbance. The left side ovary was slightly more affected (53.5%). Of the total, 15 were juvenile granulosa cell tumours (JGCT), 11 sclerosing stromal tumours (SST), 10 of the fibrothecomas spectrum, 2 Sertoli leydig cell tumours (SLCT) and one a sex cord tumour with annular tubules (SCTAT). Detailed immunohistochemical analyses were performed in 33 cases. Recurrence/metastasis was noted in 4/21 cases with follow-up data.
  
  
  CONCLUSIONS
  Ovarian sex cord stromal tumours are very rare in young age in our population, and usually present at an early stage. Most common among these are juvenile granulosa cell tumours, although surprisingly sclerosing stromal tumours were also common. Clinical symptoms due to hormone secretion in premenstrual girls and menstrual disturbance in menstruating girls are common presenting features.
authors.affiliation: Histopathology section, Dept of Pathology and Microbiology, The Aga Khan University, Karachi, Pakistan E-mail : saroonakm@yahoo.com.
authors.forename: Saroona
authors.initials: S
authors.lastname: Haroon
authors.forename: Romana
authors.initials: R
authors.lastname: Idrees
authors.forename: Aleena
authors.initials: A
authors.lastname: Zia
authors.forename: Aisha
authors.initials: A
authors.lastname: Memon
authors.forename: Saira
authors.initials: S
authors.lastname: Fatima
authors.forename: Naila
authors.initials: N
authors.lastname: Kayani
mesh_terms: D000293:Adolescent; D000328:Adult; D014408:Biomarkers, Tumor; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D036821:Endometrial Stromal Tumors / Q000473:pathology; D005260:Female; D006106:Granulosa Cell Tumor / Q000473:pathology; D006801:Humans; D007223:Infant; D008599:Menstruation Disturbances; D009362:Neoplasm Metastasis / Q000473:pathology; D009364:Neoplasm Recurrence, Local / Q000473:pathology; D010051:Ovarian Neoplasms / Q000473:pathology*; D010154:Pakistan / Q000453:epidemiology; D018310:Sertoli-Leydig Cell Tumor / Q000473:pathology; D018312:Sex Cord-Gonadal Stromal Tumors / Q000453:epidemiology* / Q000473:pathology*; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor