Gonadoblastoma
Encyclopedia
A gonadoblastoma is a complex neoplasm composed of a mixture of gonad
al elements, such as large primordial germ cell
s, immature Sertoli cell
s or granulosa cell
s of the sex cord
, and gonadal stromal cell
s.
, gonadal dysgenesis
, and the presence of a Y chromosome
in over 90% of cases.
It has been found in association with Turner's syndrome.
Formerly, gonadoblastoma was sometimes regarded as a subset of dysgerminoma
. In modern literature, it is sometimes considered to progress to dysgerminoma.
. Laparoscopy
may be an option if the surgeon is particularly skilled in removing ovarian neoplasms via laparoscopy intact. If the diagnosis of gonadoblastoma is certain, a bilateral salpingo-oopherectomy (BSO) should be performed to remove both the primary tumor and the dysgenic contralateral ovary. If uninvolved, the uterus
should be left intact. Modern reproductive endocrinology technology allows patients post BSO to achieve pregnancy via in-vitro fertilization (IVF) with a donor egg.
Gonad
The gonad is the organ that makes gametes. The gonads in males are the testes and the gonads in females are the ovaries. The product, gametes, are haploid germ cells. For example, spermatozoon and egg cells are gametes...
al elements, such as large primordial germ cell
Germ cell
A germ cell is any biological cell that gives rise to the gametes of an organism that reproduces sexually. In many animals, the germ cells originate near the gut of an embryo and migrate to the developing gonads. There, they undergo cell division of two types, mitosis and meiosis, followed by...
s, immature Sertoli cell
Sertoli cell
A Sertoli cell is a 'nurse' cell of the testes that is part of a seminiferous tubule.It is activated by follicle-stimulating hormone and has FSH-receptor on its membranes.-Functions:...
s or granulosa cell
Granulosa cell
A granulosa cell or follicular cell is a somatic cell of the sex cord that is closely associated with the developing female gamete in the ovary of mammals.-Anatomy and function:...
s of the sex cord
Sex cord
In animal embryology, the gonadal cords or sex cords are structures that develop from the gonadal ridge. After sexual differentiation, in males the sex cords become the testis cords, which help develop and nourish the Sertoli cells, while in females they become the cortical cords.-External...
, and gonadal stromal cell
Stromal cell
In cell biology, stromal cells are connective tissue cells of any organ, for example in the uterine mucosa , prostate, bone marrow, and the ovary. They are cells that support the function of the parenchymal cells of that organ...
s.
Associations
Gonadoblastoma is most often associated with abnormal chromosomal karyotypeKaryotype
A karyotype is the number and appearance of chromosomes in the nucleus of an eukaryotic cell. The term is also used for the complete set of chromosomes in a species, or an individual organism.p28...
, gonadal dysgenesis
Dysgenesis
Dysgenesis may refer to:* Dysgenesis , which indicates abnormal organ development during embryonic growth and development* Anterior segment dysgenesis, a failure of the normal development of the tissues of the anterior segment of the eye...
, and the presence of a Y chromosome
Y chromosome
The Y chromosome is one of the two sex-determining chromosomes in most mammals, including humans. In mammals, it contains the gene SRY, which triggers testis development if present. The human Y chromosome is composed of about 60 million base pairs...
in over 90% of cases.
It has been found in association with Turner's syndrome.
Classification
Gonadoblastomas can contain elements of both germ cells and gonadal stroma.Formerly, gonadoblastoma was sometimes regarded as a subset of dysgerminoma
Dysgerminoma
A dysgerminoma is a type of germ cell tumor; it usually is malignant and usually occurs in the ovary.A tumor of the identical histology but not occurring in the ovary may be described by an alternate name: seminoma in the testis or germinoma in the central nervous system or other parts of the...
. In modern literature, it is sometimes considered to progress to dysgerminoma.
Treatment
Standard treatment would include surgical exploration via laparotomyLaparotomy
A laparotomy is a surgical procedure involving a large incision through the abdominal wall to gain access into the abdominal cavity. It is also known as coeliotomy.- Terminology :...
. Laparoscopy
Laparoscopy
Laparoscopy is an operation performed in the abdomen or pelvis through small incisions with the aid of a camera...
may be an option if the surgeon is particularly skilled in removing ovarian neoplasms via laparoscopy intact. If the diagnosis of gonadoblastoma is certain, a bilateral salpingo-oopherectomy (BSO) should be performed to remove both the primary tumor and the dysgenic contralateral ovary. If uninvolved, the uterus
Uterus
The uterus or womb is a major female hormone-responsive reproductive sex organ of most mammals including humans. One end, the cervix, opens into the vagina, while the other is connected to one or both fallopian tubes, depending on the species...
should be left intact. Modern reproductive endocrinology technology allows patients post BSO to achieve pregnancy via in-vitro fertilization (IVF) with a donor egg.