Sex cord-gonadal stromal tumour

Sex cord-gonadal stromal tumour
Classification and external resources

Micrograph of a granulosa cell tumour, a type of sex-cord stromal tumour. H&E stain.
ICD-10 C56.9, C62
ICD-O: 8590/1, 8600, 8620, 8631
MeSH D018312

Sex cord-gonadal stromal tumour (or sex cord-stromal tumour) is a group of tumours of sex cord-derived tissues of the ovary and testis. In humans, this group accounts for 8% of ovarian cancers and under 5% of testicular cancers. Their diagnosis is histological: only a biopsy of the tumour can make an exact diagnosis. They are often suspected of being malignant prior to operation, being solid ovarian tumours that tend to occur most commonly in post menopausal women.

This group of tumours is significantly less common than testicular germ cell tumours in men,[1] and slightly less common than ovarian germ cell tumours in women (see Ovarian cancer).

Contents

Types

These tumours are of the following types, characterized by their abnormal production of otherwise apparently normal cells or tissues.

Classification of sex cord-gonadal stromal tumours by their histology
Cell/tissue normal location
Ovary (female) Testicle (male) Mixed
Cell/tissue type Sex cord Granulosa cell tumour Sertoli cell tumour Gynandroblastoma
Gonadal stroma Thecoma, Fibroma Leydig cell tumour Gynandroblastoma
Mixed Sertoli-Leydig cell tumour Gynandroblastoma

Although each of the cell and tissue types normally occurs in just one sex (male or female), within a tumour they can occur in the opposite sex. Consequently, depending on the specific histology produced, these tumours can cause virilization in women and feminization in men.

Tumour types in order of prevalence

Diagnosis

Definitive diagnosis of these tumours is based on the histology of tissue obtained in a biopsy or surgical resection. In a retrospective study of 72 cases in children and adolescents, the histology was important to prognosis.[6]

A number of molecules have been proposed as markers for this group of tumours. CD56 may be useful for distinguishing sex cord-stromal tumours from some other types of tumours, although it does not distinguish them from neuroendocrine tumours.[7] Calretinin has also been suggested as a marker.[8] For diagnosis of granulosa cell tumour, inhibin is under investigation.

On magnetic resonance imaging, a fibroma may produce one of several imaging features that might be used in the future to identify this rare tumour prior to surgery.[9][10]

Prognosis

A retrospective study of 83 women with sex cord-stromal tumours (73 with granulosa cell tumour and 10 with Sertoli-Leydig cell tumour), all diagnosed between 1975 and 2003, reported that survival was higher with age under 50, smaller tumour size, and absence of residual disease. The study found no effect of chemotherapy.[11] A retrospective study of 67 children and adolescents reported some benefit of cisplatin-based chemotherapy.[12]

Research

A prospective study of ovarian sex cord-stromal tumours in children and adolescents began enrolling participants in 2005.[12]

Additional images

See also

References

  1. ^ Sajadi, Kamran P.; Dalton, Rory R.; Brown, James A. (2009). "Sex Cord-Gonadal Stromal Tumor of the Rete Testis". Advances in Urology 2009: 1. doi:10.1155/2009/624173. PMC 2612754. PMID 19125206. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2612754. 
  2. ^ Chivukula, Mamatha; Hunt, Jennifer; Carter, Gloria; Kelley, Joseph; Patel, Minita; Kanbour-Shakir, Amal (2007). "Recurrent Gynandroblastoma of Ovary-A Case Report". International Journal of Gynecological Pathology 26 (1): 30–3. doi:10.1097/01.pgp.0000225387.48868.39. PMID 17197894. 
  3. ^ Limaïem, F; Lahmar, A; Ben Fadhel, C; Bouraoui, S; M'zabi-Regaya, S (2008). "Gynandroblastoma. Report of an unusual ovarian tumour and literature review". Pathologica 100 (1): 13–7. PMID 18686520. 
  4. ^ Broshears, John R.; Roth, Lawrence M. (1997). "Gynandroblastoma with Elements Resembling Juvenile Granulosa Cell Tumor". International Journal of Gynecological Pathology 16 (4): 387–91. doi:10.1097/00004347-199710000-00016. PMID 9421080. 
  5. ^ Antunes, L; Ounnoughene-Piet, M; Hennequin, V; Maury, F; Lemelle, J-L; Labouyrie, E; Plenat, F (2002). "Gynandroblastoma of the testis in an infant: a morphological, immunohistochemical and in-situ hybridization report". Histopathology 40 (4): 395–7. doi:10.1046/j.1365-2559.2002.t01-2-01299.x. PMID 11943029. 
  6. ^ Schneider, Dominik T.; Jänig, Ute; Calaminus, Gabriele; Göbel, Ulrich; Harms, Dieter (2003). "Ovarian sex cord–stromal tumors—a clinicopathological study of 72 cases from the Kiel Pediatric Tumor Registry". Virchows Archiv 443 (4): 549–60. doi:10.1007/s00428-003-0869-0. PMID 12910419. 
  7. ^ McCluggage, W. Glenn; McKenna, Michael; McBride, Hilary A. (2007). "CD56 is a Sensitive and Diagnostically Useful Immunohistochemical Marker of Ovarian Sex Cord-Stromal Tumors". International Journal of Gynecological Pathology 26 (3): 322–7. doi:10.1097/01.pgp.0000236947.59463.87. PMID 17581419. 
  8. ^ Deavers, Michael T.; Malpica, Anais; Liu, Jinsong; Broaddus, Russell; Silva, Elvio G. (2003). "Ovarian Sex Cord-Stromal Tumors: an Immunohistochemical Study Including a Comparison of Calretinin and Inhibin". Modern Pathology 16 (6): 584–90. doi:10.1097/01.MP.0000073133.79591.A1. PMID 12808064. 
  9. ^ Takeuchi, Mayumi; Matsuzaki, Kenji; Sano, Nobuya; Furumoto, Hiroyuki; Nishitani, Hiromu (2008). "Ovarian Fibromatosis". Journal of Computer Assisted Tomography 32 (5): 776–7. doi:10.1097/RCT.0b013e318157689a. PMID 18830110. 
  10. ^ Kitajima, Kazuhiro; Kaji, Yasushi; Sugimura, Kazuro (2008). "Usual and Unusual MRI Findings of Ovarian Fibroma: Correlation with Pathologic Findings". Magnetic Resonance in Medical Sciences 7 (1): 43–8. doi:10.2463/mrms.7.43. PMID 18460848. 
  11. ^ Chan, J; Zhang, M; Kaleb, V; Loizzi, V; Benjamin, J; Vasilev, S; Osann, K; Disaia, P (2005). "Prognostic factors responsible for survival in sex cord stromal tumors of the ovary?A multivariate analysis". Gynecologic Oncology 96 (1): 204–9. doi:10.1016/j.ygyno.2004.09.019. PMID 15589602. 
  12. ^ a b Schneider, DT; Calaminus, G; Harms, D; Göbel, U; German Maligne Keimzelltumoren Study Group (2005). "Ovarian sex cord-stromal tumors in children and adolescents". The Journal of reproductive medicine 50 (6): 439–46. PMID 16050568.