View source for Sex cord–gonadal stromal tumour

{{Infobox medical condition (new)
| name           = Sex cord–gonadal stromal tumour
| synonyms        = Sex cord–stromal tumour
| image          = Granulosa cell tumour2.jpg
| caption        = [[Micrograph]] of a [[granulosa cell tumour]], a type of sex-cord–gonadal stromal tumour. [[H&E stain]].
| pronounce       =
| specialty       = [[Oncology]], [[gynecology]]
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'''Sex cord–gonadal stromal tumour''' is a group of [[tumour]]s derived from the stromal component of the [[ovary]] and [[testis]], which comprises the [[granulosa cell|granulosa]], [[thecal cell]]s and [[fibrocyte]]s.<ref name=":0">{{Cite journal|last1=Maoz|first1=Asaf|last2=Matsuo|first2=Koji|last3=Ciccone|first3=Marcia A.|last4=Matsuzaki|first4=Shinya|last5=Klar|first5=Maximilian|last6=Roman|first6=Lynda D.|last7=Sood|first7=Anil K.|last8=Gershenson|first8=David M.|date=2020-05-29|title=Molecular Pathways and Targeted Therapies for Malignant Ovarian Germ Cell Tumors and Sex Cord-Stromal Tumors: A Contemporary Review|journal=Cancers|volume=12|issue=6|page=1398|doi=10.3390/cancers12061398|issn=2072-6694|pmid=32485873|pmc=7353025|doi-access=free}}</ref> In contrast, the epithelial cells originate from the outer epithelial lining surrounding the gonad while the germ cell tumors arise from the precursor cells of the gametes, hence the name germ cell.<ref name=":0" />  In humans, this group accounts for 8% of [[ovarian cancer]]s and under 5% of [[testicular cancer]]s. 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 tumor|germ cell tumour]]s in men,<ref name="pmid19125206">{{cite journal |last1=Sajadi |first1=Kamran P. |last2=Dalton |first2=Rory R. |last3=Brown |first3=James A. |title=Sex Cord-Gonadal Stromal Tumor of the Rete Testis |journal=Advances in Urology |volume=2009 |pages=1–3 |year=2009 |pmid=19125206 |pmc=2612754 |doi=10.1155/2009/624173|doi-access=free }}</ref> and slightly less common than ovarian germ cell tumours in women (see [[Ovarian cancer]]).<ref name=":0" />
==Types==
[[File:Ovarian tumors by incidence and cancer risk.png|thumb|300px|[[Ovarian tumour]]s by [[Incidence (epidemiology)|incidence]] and risk of [[ovarian cancer]], with sex cord-stromal tumours at right.<ref>- {{cite journal|last1=Vaidya|first1=SA|last2=Kc|first2=S|last3=Sharma|first3=P|last4=Vaidya|first4=S|title=Spectrum of ovarian tumors in a referral hospital in Nepal|journal=Journal of Pathology of Nepal|volume=4|issue=7|year=2014|pages=539–543|issn=2091-0908|doi=10.3126/jpn.v4i7.10295|doi-access=free}}<br>- Minor adjustment for mature cystic teratomas (0.17 to 2% risk of ovarian cancer): {{cite journal|last1=Mandal|first1=Shramana|last2=Badhe|first2=Bhawana A.|title=Malignant Transformation in a Mature Teratoma with Metastatic Deposits in the Omentum: A Case Report|journal=Case Reports in Pathology|volume=2012|year=2012|pages=1–3|issn=2090-6781|doi=10.1155/2012/568062|pmid=23082264|pmc=3469088|doi-access=free}}</ref>]]
{| class="wikitable"
! colspan="5" | Classification of sex cord–gonadal stromal tumours by their histology
|-
! rowspan="2" colspan="2" |
! colspan="3" | Cell/tissue normal location
|-
! [[Ovary]] (female)
! [[Testicle]] (male)
! Mixed
|-
| rowspan=3 | Cell/tissue type
| [[Sex cord]]
| [[Granulosa cell tumour]]
| [[Sertoli cell tumour]]
| Gynandroblastoma
|-
| [[stromal cell|Gonadal stroma]]
| [[Thecoma]], [[Fibroma]]
| [[Leydig cell tumour]]
| Gynandroblastoma
|-
| Mixed
| colspan=2 | [[Sertoli–Leydig cell tumour]]
| Gynandroblastoma
|-
|}
===Tumour types in order of prevalence===
*[[Granulosa cell tumour]].  This tumour produces [[granulosa cells]], which normally are found in the ovary.  It is malignant in 20% of women diagnosed with it. It tends to present in women in the 50-55yo age group with post menopausal vaginal bleeding.  Uncommonly, a similar but possibly distinct tumour, [[granulosa cell tumour|juvenile granulosa cell tumour]], presents in pre-pubertal girls with precocious puberty.  In both groups, the vaginal bleeding is due to oestrogen secreted by the tumour.  In older women, treatment is total abdominal [[hysterectomy]] and removal of both ovaries.<ref name=":0" />  In young girls, fertility sparing treatment is the mainstay for non-metastatic disease.<ref name=":0" />
* [[Sertoli cell tumour]].  This tumour produces [[Sertoli cell]]s, which normally are found in the [[testicle]]. This tumour occurs in both men and women.
*[[Thecoma]]. This tumour produces [[theca of follicle]], a tissue normally found in the [[ovarian follicle]].  The tumour is almost exclusively benign and unilateral.  It typically secretes estrogen, and as a result women with this tumour often present with postmenopausal bleeding.
*[[Leydig cell tumour]].  This tumour produces [[Leydig cell]]s, which normally are found in the testicle and tend to secrete androgens.
*[[Sertoli–Leydig cell tumour]]. This tumour produces both Sertoli and Leydig cells. Although both cell types normally occur in the testicle, this tumour can occur in the ovary.<ref name=":0" />
*'''Gynandroblastoma'''.<!-- [[Gynandroblastoma]] redirects here -->   A very rare tumour producing both ovarian (granulosa and/or theca) and testicular (Sertoli and/or Leydig) cells or tissues.  Typically it consists of adult-type granulosa cells and Sertoli cells,<ref name="pmid17197894">{{cite journal |last1=Chivukula |first1=Mamatha |last2=Hunt |first2=Jennifer |last3=Carter |first3=Gloria |last4=Kelley |first4=Joseph |last5=Patel |first5=Minita |last6=Kanbour-Shakir |first6=Amal |title=Recurrent Gynandroblastoma of Ovary-A Case Report |journal=International Journal of Gynecological Pathology |volume=26 |issue=1 |pages=30–3 |year=2007 |pmid=17197894 |doi=10.1097/01.pgp.0000225387.48868.39}}</ref><ref name="pmid18686520">{{cite journal |last1=Limaïem |first1=F |last2=Lahmar |first2=A |last3=Ben Fadhel |first3=C |last4=Bouraoui |first4=S |last5=M'zabi-Regaya |first5=S |title=Gynandroblastoma. Report of an unusual ovarian tumour and literature review |journal=Pathologica |volume=100 |issue=1 |pages=13–7 |year=2008 |pmid=18686520}}</ref> but it has been reported with juvenile-type granulosa cells.<ref name="pmid9421080">{{cite journal |last1=Broshears |first1=John R. |last2=Roth |first2=Lawrence M. |title=Gynandroblastoma with Elements Resembling Juvenile Granulosa Cell Tumor |journal=International Journal of Gynecological Pathology |volume=16 |issue=4 |pages=387–91 |year=1997 |pmid=9421080 |doi=10.1097/00004347-199710000-00016}}</ref> It has been reported to occur in the ovary usually, rarely in the testis.<ref name="pmid11943029">{{cite journal |last1=Antunes |first1=L |last2=Ounnoughene-Piet |first2=M |last3=Hennequin |first3=V |last4=Maury |first4=F |last5=Lemelle |first5=J-L |last6=Labouyrie |first6=E |last7=Plenat |first7=F |title=Gynandroblastoma of the testis in an infant: a morphological, immunohistochemical and in-situ hybridization report |journal=Histopathology |volume=40 |issue=4 |pages=395–7 |year=2002 |pmid=11943029 |doi=10.1046/j.1365-2559.2002.t01-2-01299.x|s2cid=35602891 }}</ref> Due to its rarity, the malignant potential of this tumour is unclear;  there is one case report of late metastasis.
*'''[[Sex cord tumour with annular tubules]]''', abbreviated '''SCTAT'''. These are rare tumours that may be sporadic or associated with [[Peutz–Jeghers syndrome]].
==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.<ref name="pmid12910419">{{cite journal |last1=Schneider |first1=Dominik T. |last2=Jänig |first2=Ute |last3=Calaminus |first3=Gabriele |last4=Göbel |first4=Ulrich |last5=Harms |first5=Dieter |title=Ovarian sex cord–stromal tumors—a clinicopathological study of 72 cases from the Kiel Pediatric Tumor Registry  |journal=Virchows Archiv |volume=443 |issue=4 |pages=549–60 |year=2003 |pmid=12910419 |doi=10.1007/s00428-003-0869-0|s2cid=25768728 }}</ref>
A number of molecules have been proposed as [[tumour marker|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.<ref name="pmid17581419">{{cite journal |last1=McCluggage |first1=W. Glenn |last2=McKenna |first2=Michael |last3=McBride |first3=Hilary A. |title=CD56 is a Sensitive and Diagnostically Useful Immunohistochemical Marker of Ovarian Sex Cord-Stromal Tumors |journal=International Journal of Gynecological Pathology |volume=26 |issue=3 |pages=322–7 |year=2007 |pmid=17581419 |doi=10.1097/01.pgp.0000236947.59463.87|s2cid=34551261 }}</ref>  [[Calretinin]] has also been suggested as a marker.<ref name="pmid12808064">{{cite journal |last1=Deavers |first1=Michael T. |last2=Malpica |first2=Anais |last3=Liu |first3=Jinsong |last4=Broaddus |first4=Russell |last5=Silva |first5=Elvio G. |title=Ovarian Sex Cord-Stromal Tumors: an Immunohistochemical Study Including a Comparison of Calretinin and Inhibin |journal=Modern Pathology |volume=16 |issue=6 |pages=584–90 |year=2003 |pmid=12808064 |doi=10.1097/01.MP.0000073133.79591.A1|doi-access=free }}</ref>  For diagnosis of granulosa cell tumour, [[inhibin]] is under investigation.{{Citation needed|date=March 2009}} Granulosa cell tumours and Sertoli-Leydig cell tumours have specific genetic mutations that are characteristic and can help support the diagnosis.<ref name=":0" />
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.<ref name="pmid18830110">{{cite journal |last1=Takeuchi |first1=Mayumi |last2=Matsuzaki |first2=Kenji |last3=Sano |first3=Nobuya |last4=Furumoto |first4=Hiroyuki |last5=Nishitani |first5=Hiromu |title=Ovarian Fibromatosis |journal=Journal of Computer Assisted Tomography |volume=32 |issue=5 |pages=776–7 |year=2008 |pmid=18830110 |doi=10.1097/RCT.0b013e318157689a}}</ref><ref name="pmid18460848">{{cite journal |last1=Kitajima |first1=Kazuhiro |last2=Kaji |first2=Yasushi |last3=Sugimura |first3=Kazuro |title=Usual and Unusual MRI Findings of Ovarian Fibroma: Correlation with Pathologic Findings |journal=Magnetic Resonance in Medical Sciences |volume=7 |issue=1 |pages=43–8 |year=2008 |pmid=18460848 |doi=10.2463/mrms.7.43|doi-access=free }}</ref>
<gallery>
Image:Granulosa cell tumour1.jpg|Low magnification [[micrograph]] of a [[granulosa cell tumour]]. [[H&E stain]].
Image:Thecoma high mag.jpg|High magnification [[micrograph]] of a [[thecoma]]. [[H&E stain]].
Image:Thecoma low mag.jpg|Low magnification [[micrograph]] of a [[thecoma]]. [[H&E stain]].
Image:Leydig cell tumour1.jpg|Low magnification [[micrograph]] of a [[Leydig cell tumour]]. [[H&E stain]].
Image:Leydig cell tumour2.jpg|Intermediate magnification [[micrograph]] of a [[Leydig cell tumour]]. [[H&E stain]].
Image:Leydig cell tumour3.jpg|High magnification [[micrograph]] of a [[Leydig cell tumour]]. [[H&E stain]].
Image: Sertoli cell tumour low mag.jpg|Low magnification [[micrograph]] of a [[Sertoli cell tumour]]. [[H&E stain]].
Image: Sertoli cell tumour high mag.jpg|High magnification [[micrograph]] of a [[Sertoli cell tumour]]. [[H&E stain]].
</gallery>
==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.<ref name="pmid15589602">{{cite journal |last1=Chan |first1=J |last2=Zhang |first2=M |last3=Kaleb |first3=V |last4=Loizzi |first4=V |last5=Benjamin |first5=J |last6=Vasilev |first6=S |last7=Osann |first7=K |last8=Disaia |first8=P |title=Prognostic factors responsible for survival in sex cord stromal tumors of the ovary?A multivariate analysis |journal=Gynecologic Oncology |volume=96 |issue=1 |pages=204–9 |year=2005 |pmid=15589602 |doi=10.1016/j.ygyno.2004.09.019}}</ref> A retrospective study of 67 children and adolescents reported some benefit of cisplatin-based chemotherapy.<ref name="pmid16050568"/>
==Research==
A prospective study of ovarian sex cord–stromal tumours in children and adolescents began enrolling participants in 2005.<ref name="pmid16050568">{{cite journal |last1=Schneider |first1=DT |last2=Calaminus |first2=G |last3=Harms |first3=D |last4=Göbel |first4=U |author5=German Maligne Keimzelltumoren Study Group |title=Ovarian sex cord-stromal tumors in children and adolescents |journal=The Journal of Reproductive Medicine |volume=50 |issue=6 |pages=439–46 |year=2005 |pmid=16050568}}</ref> The International Ovarian and Testicular Stromal Tumor Registry is studying these rare tumours and collecting data on them to further research. Targeted treatments are being evaluated for these tumours as well.<ref name=":0" />
==See also==
* [[Sex cord]]
* [[Stroma of ovary]]
==References==
{{reflist}}
==External links==
*{{Commons category-inline|Sex cord–gonadal stromal tumors}}
{{Medical resources
| DiseasesDB     =
| ICD10          = C56.9, C62
| ICD9           =
| ICDO           = 8590/1, 8600, 8620, 8631
| OMIM           =
| MedlinePlus    =
| eMedicineSubj  =
| eMedicineTopic =
| MeshID         = D018312
}}
{{Female genital neoplasia}}
{{Male genital neoplasia}}
{{DEFAULTSORT:Sex Cord-Gonadal Stromal Tumour}}
[[Category:Gynaecological cancer]]
[[Category:Male genital neoplasia]]
[[Category:Ovarian cancer]]