Sex cord stromal tumors of the ovary in children: A clinicopathological report from the Italian TREP project

Vyas

Int J Reprod Contracept Obstet Gynecol

2017

Background: Sex cord stromal tumors are a heterogeneous group of rare neoplasms of ovary. These tumors arise from different cells of the ovary and have a fascinating variety of clinical presentations. They are mostly diagnosed on histopathology after surgical removal.Methods: Our study aims at discussing the clinical and histomorphological spectrum of these rare tumors at a tertiary care centre.Results: In our study 158 ovarian sex cord stromal tumors were received over a period of eight years at our institute. Out of these, the most common age group was 30 to 40 years and the chief complaint was abdominal pain and lump in majority of cases. Most common tumor histologically was Adult Granulosa cell tumor (42.4%). There were 8 (5.1%) Juvenile granulosa cell tumors, 31 (19.6%) fibromas, 6 (3.8%) thecomas, 14 (8.9%) fibrothecomas, 24 (15.2%) sertoli leydig cell tumors and 7 (4.4%) sclerosing stromal tumors. We encountered one case of sex cord tumor with annular tubules.Conclusions: Sex cord stromal tumors are uncommon ovarian tumors in Indians but have a wide range of distribution of age, clinical features and histopathological types. Since most of these have a relatively good prognosis, a high index of suspicion and thorough knowledge of clinicopathological findings is important for correct diagnosis and appropriate treatment.

Section: Resultssupporting

confidence: 91%

Ovarian sex cord stromal tumors: an institutional experience

Vyas

Int J Reprod Contracept Obstet Gynecol

2017

Asian Pacific Journal of Cancer Prevention

“…Only one SST and two Fibromas were encountered in premenstrual females. In post pubertal patients, 9 cases each of JGCT and SST were found as depicted in Table 2, while in literature Sertoli Leydig cell tumour are more common in this group Cecchetto et al, 2011).…”

Section: Discussionmentioning

confidence: 99%

“…In most of previous studies reported, fibrothecoma group comprised of one of common neoplasms among SCST in younger age, we also had 10 cases of tumours of this spectrum, and all of these behaved as benign tumours (Cecchetto et al, 2011).…”

Section: Discussionmentioning

confidence: 99%

“…These non germinative tumours are heterogeneous morphologically, being rarer in children in which either benign epithelial tumours or germ cell tumours of ovarian origin are found (Cecchetto et al, 2011;Park et al, 2011). In our centre at Aga Khan University Hospital, the laboratory processes over 50,000 surgical specimens per year and out of these, quite a few are from children and adolescents.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Ovarian Sex Cord Stromal Tumours in Children and Young Girls - A More Than Two Decade Clinicopathological Experience in a Developing Country, Pakistan

Haroon

Idrees²,

Zia³

et al. 2014

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. Objectives: The purpose of our study was to report the clinical and pathological findings in young patients with these tumours in our population. Material and 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.

“…They rarely occur in childhood and adolescence [1,2,3]. In about 40% of cases, they show an autonomous testosterone production which causes virilization [7]. After clinical examination and serum hormone measurement, radiographic imaging plays an important diagnostic role.…”

Section: Discussionmentioning

confidence: 99%

Fast Intraoperative Testosterone Assay Confirms the Location of an Ovarian Virilizing Tumor in a Young Girl

Braun

Peter²,

Warmann³

et al. 2012

Horm Res Paediatr

The detection of testosterone-producing ovarian tumors in childhood and adolescence by imaging techniques only can be difficult because of the tumors' radiological structure and sometimes diminutive size. We describe an 11.5-year-old girl with a 9-month history of voice deepening, mild hirsutism, minor acne, increased growth velocity, weight gain, and clit-oromegaly. Laboratory investigation revealed an extremely elevated serum testosterone level without any additional endocrine abnormalities. Abdominal ultrasound and MRI showed a sparsely noticeable solid mass in the center of the right ovary. At laparotomy, blood was selectively drawn from the right and the left ovarian veins. Rapid testosterone measurement revealed a 70-fold higher testosterone concentration in the right ovarian vein within 45 min. Based on this finding, a right salpingo-oophorectomy was performed. The patient's postoperative testosterone level declined within 24 h. The histopathological diagnosis was Leydig cell tumor. In conclusion, the implementation of a fast intraoperative testosterone assay enabled the localization and curative therapy of a Leydig cell tumor. This technique seems to be a good alternative to preoperative selective venous blood sampling when body imaging does not unveil the tumor's site.