Sex cord–gonadal stromal tumour

Sex cord–gonadal stromal tumour
Synonyms	Sex cord–stromal tumour
Pronounce
Field	N/A
Symptoms
Complications
Onset
Duration
Types
Causes
Risks
Diagnosis
Differential diagnosis
Prevention
Treatment
Medication
Prognosis
Frequency
Deaths

Sex cord–gonadal stromal tumour is a group of tumors derived from the stromal component of the ovary and testis, which comprises the granulosa, thecal cells and fibrocytes<ref name=":0">Maoz, Asaf,

 Molecular Pathways and Targeted Therapies for Malignant Ovarian Germ Cell Tumors and Sex Cord-Stromal Tumors: A Contemporary Review, 
 Cancers, 
 
 Vol. 12(Issue: 6),
 
 DOI: 10.3390/cancers12061398,
 PMID: 32485873,
 
 
 Full text,</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 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,<ref name="pmid19125206">,

 Sex Cord-Gonadal Stromal Tumor of the Rete Testis, 
 Advances in Urology, 
 2009,
 Vol. 2009,
 pp. 1,
 DOI: 10.1155/2009/624173,
 PMID: 19125206,
 PMC: 2612754,</ref> and slightly less common than ovarian germ cell tumours in women (see Ovarian cancer)<ref name=":0" />.

Types

Ovarian tumors by incidence and risk of ovarian cancer, with sex cord-stromal tumors at right.<ref>- , Spectrum of ovarian tumors in a referral hospital in Nepal, Journal of Pathology of Nepal, 2014, Vol. 4(Issue: 7), pp. 539–543, DOI: 10.3126/jpn.v4i7.10295,
- Minor adjustment for mature cystic teratomas (0.17 to 2% risk of ovarian cancer): , Malignant Transformation in a Mature Teratoma with Metastatic Deposits in the Omentum: A Case Report, Case Reports in Pathology, 2012, Vol. 2012, pp. 1–3, DOI: 10.1155/2012/568062,</ref>

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

• 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, 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 cells, 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 cells, 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. 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">,

 Recurrent Gynandroblastoma of Ovary-A Case Report, 
 International Journal of Gynecological Pathology, 
 2007,
 Vol. 26(Issue: 1),
 pp. 30–3,
 DOI: 10.1097/01.pgp.0000225387.48868.39,
 PMID: 17197894,</ref><ref name="pmid18686520">, 
 Gynandroblastoma. Report of an unusual ovarian tumour and literature review, 
 Pathologica, 
 2008,
 Vol. 100(Issue: 1),
 pp. 13–7,
 
 PMID: 18686520,</ref> but it has been reported with juvenile-type granulosa cells.<ref name="pmid9421080">, 
 Gynandroblastoma with Elements Resembling Juvenile Granulosa Cell Tumor, 
 International Journal of Gynecological Pathology, 
 1997,
 Vol. 16(Issue: 4),
 pp. 387–91,
 DOI: 10.1097/00004347-199710000-00016,
 PMID: 9421080,</ref> It has been reported to occur in the ovary usually, rarely in the testis.<ref name="pmid11943029">, 
 Gynandroblastoma of the testis in an infant: a morphological, immunohistochemical and in-situ hybridization report, 
 Histopathology, 
 2002,
 Vol. 40(Issue: 4),
 pp. 395–7,
 DOI: 10.1046/j.1365-2559.2002.t01-2-01299.x,
 PMID: 11943029,</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">,

 Ovarian sex cord–stromal tumors—a clinicopathological study of 72 cases from the Kiel Pediatric Tumor Registry, 
 Virchows Archiv, 
 2003,
 Vol. 443(Issue: 4),
 pp. 549–60,
 DOI: 10.1007/s00428-003-0869-0,
 PMID: 12910419,</ref>

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.<ref name="pmid17581419">,

 CD56 is a Sensitive and Diagnostically Useful Immunohistochemical Marker of Ovarian Sex Cord-Stromal Tumors, 
 International Journal of Gynecological Pathology, 
 2007,
 Vol. 26(Issue: 3),
 pp. 322–7,
 DOI: 10.1097/01.pgp.0000236947.59463.87,
 PMID: 17581419,</ref>  Calretinin has also been suggested as a marker.<ref name="pmid12808064">, 
 Ovarian Sex Cord-Stromal Tumors: an Immunohistochemical Study Including a Comparison of Calretinin and Inhibin, 
 Modern Pathology, 
 2003,
 Vol. 16(Issue: 6),
 pp. 584–90,
 DOI: 10.1097/01.MP.0000073133.79591.A1,
 PMID: 12808064,</ref>  For diagnosis of granulosa cell tumour, inhibin is under investigation.

^{(March 2009)}

Granulosa cell tumors and Sertoli-Leydig cell tumors 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">,

 Ovarian Fibromatosis, 
 Journal of Computer Assisted Tomography, 
 2008,
 Vol. 32(Issue: 5),
 pp. 776–7,
 DOI: 10.1097/RCT.0b013e318157689a,
 PMID: 18830110,</ref><ref name="pmid18460848">, 
 Usual and Unusual MRI Findings of Ovarian Fibroma: Correlation with Pathologic Findings, 
 Magnetic Resonance in Medical Sciences, 
 2008,
 Vol. 7(Issue: 1),
 pp. 43–8,
 DOI: 10.2463/mrms.7.43,
 PMID: 18460848,</ref>

• 
  Low magnification micrograph of a granulosa cell tumour. H&E stain.
• 
  High magnification micrograph of a thecoma. H&E stain.
• 
  Low magnification micrograph of a thecoma. H&E stain.
• 
  Low magnification micrograph of a Leydig cell tumour. H&E stain.
• 
  Intermediate magnification micrograph of a Leydig cell tumour. H&E stain.
• 
  High magnification micrograph of a Leydig cell tumour. H&E stain.
• 
  Low magnification micrograph of a Sertoli cell tumour. H&E stain.
• 
  High magnification micrograph of a Sertoli cell tumour. H&E stain.

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">,

 Prognostic factors responsible for survival in sex cord stromal tumors of the ovary?A multivariate analysis, 
 Gynecologic Oncology, 
 2005,
 Vol. 96(Issue: 1),
 pp. 204–9,
 DOI: 10.1016/j.ygyno.2004.09.019,
 PMID: 15589602,</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">,

 Ovarian sex cord-stromal tumors in children and adolescents, 
 The Journal of reproductive medicine, 
 2005,
 Vol. 50(Issue: 6),
 pp. 439–46,
 
 PMID: 16050568,</ref> The International Ovarian and Testicular Stromal Tumor Registry is studying these rare tumors and collecting data on them to further research. Targeted treatments are being evaluated for these tumors as well <ref name=":0" />. 

See also

• Sex cord
• Stroma of ovary

External links

   This article is a medical stub. You can help WikiMD by expanding it!