Sex cord–gonadal stromal tumour is a group of

tumour A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...

s derived from the stromal component of the

ovary The ovary is an organ in the female reproductive system that produces an ovum. When released, this travels down the fallopian tube into the uterus, where it may become fertilized by a sperm. There is an ovary () found on each side of the bod ...

and

testis A testicle or testis (plural testes) is the male reproductive gland or gonad in all bilaterians, including humans. It is homologous to the female ovary. The functions of the testes are to produce both sperm and androgens, primarily testoster ...

, which comprises the granulosa, thecal cells and fibrocytes. 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. In humans, this group accounts for 8% of

ovarian cancer Ovarian cancer is a cancerous tumor of an ovary. It may originate from the ovary itself or more commonly from communicating nearby structures such as fallopian tubes or the inner lining of the abdomen. The ovary is made up of three different ...

s and under 5% of

testicular cancer Testicular cancer is cancer that develops in the testicles, a part of the male reproductive system. Symptoms may include a lump in the testicle, or swelling or pain in the scrotum. Treatment may result in infertility. Risk factors include an ...

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 tumours in men, and slightly less common than ovarian germ cell tumours in women (see

Ovarian cancer Ovarian cancer is a cancerous tumor of an ovary. It may originate from the ovary itself or more commonly from communicating nearby structures such as fallopian tubes or the inner lining of the abdomen. The ovary is made up of three different ...

Types

Ovarian tumors by incidence and cancer risk

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 Hysterectomy is the surgical removal of the uterus. It may also involve removal of the cervix, ovaries ( oophorectomy), Fallopian tubes ( salpingectomy), and other surrounding structures. Usually performed by a gynecologist, a hysterectomy may ...

and removal of both ovaries. In young girls, fertility sparing treatment is the mainstay for non-metastatic disease. *

Sertoli cell tumour A Sertoli cell tumour, also Sertoli cell tumor (US spelling), is a sex cord-gonadal stromal tumor of Sertoli cells. They can occur in the testis or ovary. They are very rare and generally peak between the ages of 35 and 50. They are typically wel ...

. 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 The theca folliculi comprise a layer of the ovarian follicles. They appear as the follicles become secondary follicles. The theca are divided into two layers, the theca interna and the theca externa. Theca cells are a group of endocrine cel ...

, a tissue normally found in the

ovarian follicle An ovarian follicle is a roughly spheroid cellular aggregation set found in the ovaries. It secretes hormones that influence stages of the menstrual cycle. At the time of puberty, women have approximately 200,000 to 300,000 follicles, each with t ...

. 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 Sertoli–Leydig cell tumour is a group of tumors composed of variable proportions of Sertoli cells, Leydig cells, and in the case of intermediate and poorly differentiated neoplasms, primitive gonadal stroma and sometimes heterologous elements.W ...

. This tumour produces both Sertoli and Leydig cells. Although both cell types normally occur in the testicle, this tumour can occur in the ovary. *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, but it has been reported with juvenile-type granulosa cells. It has been reported to occur in the ovary usually, rarely in the testis. 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 Peutz–Jeghers syndrome (often abbreviated PJS) is an autosomal dominant genetic disorder characterized by the development of benign hamartomatous polyps in the gastrointestinal tract and hyperpigmented macules on the lips and oral mucosa ( ...

Diagnosis

Definitive diagnosis of these tumours is based on the

histology Histology, also known as microscopic anatomy or microanatomy, is the branch of biology which studies the microscopic anatomy of biological tissues. Histology is the microscopic counterpart to gross anatomy, which looks at larger structures vi ...

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. A number of molecules have been proposed as

markers The term Marker may refer to: Common uses * Marker (linguistics), a morpheme that indicates some grammatical function * Marker (telecommunications), a special-purpose computer * Boundary marker, an object that identifies a land boundary * Marke ...

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.

Calretinin Calretinin, also known as calbindin 2 (formerly 29 kDa calbindin), is a calcium-binding protein involved in calcium signaling. In humans, the calretinin protein is encoded by the ''CALB2'' gene. Function This gene encodes an intracellular ...

has also been suggested as a marker. For diagnosis of granulosa cell tumour, inhibin is under investigation. Granulosa cell tumours and Sertoli-Leydig cell tumours have specific genetic mutations that are characteristic and can help support the diagnosis. 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. Image:Granulosa cell tumour1.jpg, Low magnification

micrograph A micrograph or photomicrograph is a photograph or digital image taken through a microscope or similar device to show a magnify, magnified image of an object. This is opposed to a macrograph or photomacrograph, an image which is also taken ...

of a granulosa cell tumour.

H&E stain Hematoxylin and eosin stain ( or haematoxylin and eosin stain or hematoxylin-eosin stain; often abbreviated as H&E stain or HE stain) is one of the principal tissue stains used in histology. It is the most widely used stain in medical diagnos ...

. Image:Thecoma high mag.jpg, High magnification

of a thecoma.

. Image:Thecoma low mag.jpg, Low magnification

of a thecoma.

. Image:Leydig cell tumour1.jpg, Low magnification

of a Leydig cell tumour.

. Image:Leydig cell tumour2.jpg, Intermediate magnification

of a Leydig cell tumour.

. Image:Leydig cell tumour3.jpg, High magnification

of a Leydig cell tumour.

. Image: Sertoli cell tumour low mag.jpg, Low magnification

of a

. Image: Sertoli cell tumour high mag.jpg, High magnification

of a

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. A retrospective study of 67 children and adolescents reported some benefit of cisplatin-based chemotherapy.

Research

A prospective study of ovarian sex cord–stromal tumours in children and adolescents began enrolling participants in 2005. 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.

References

External links

* {{DEFAULTSORT:Sex Cord-Gonadal Stromal Tumour Gynaecological cancer Male genital neoplasia Ovarian cancer

Types

Tumour types in order of prevalence

Diagnosis

Prognosis

Research

See also

References

External links