Luteoma
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A luteoma is a tumor that occurs in the
ovaries 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 body. T ...
during pregnancy. It is associated with an increase of sex
hormones A hormone (from the Ancient Greek, Greek participle , "setting in motion") is a class of cell signaling, signaling molecules in multicellular organisms that are sent to distant organs by complex biological processes to regulate physiology and beh ...
, primarily
progesterone Progesterone (P4) is an endogenous steroid and progestogen sex hormone involved in the menstrual cycle, pregnancy, and embryogenesis of humans and other species. It belongs to a group of steroid hormones called the progestogens and is the m ...
and
testosterone Testosterone is the primary sex hormone and anabolic steroid in males. In humans, testosterone plays a key role in the development of Male reproductive system, male reproductive tissues such as testes and prostate, as well as promoting secondar ...
. The size of the tumor can range from 1 to 25 cm in diameter, but is usually 6 to 10 cm in diameter and can grow throughout the duration of the pregnancy. However, luteomas are
benign Malignancy () is the tendency of a medical condition to become progressively worse. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous benign tumor, ''benign'' tumor in that a malign ...
and resolve themselves after delivery. This type of tumor is rare with only about 200 documented cases; many of these cases were detected accidentally, so the actual rate of occurrence may be higher. The most obvious symptom of a luteoma is
masculinization Virilization or masculinization is the biological development of adult male characteristics in young males or females. Most of the changes of virilization are produced by androgens. Virilization is most commonly used in three medical and biology ...
of the mother and the possible masculinization of the
fetus A fetus or foetus (; plural fetuses, feti, foetuses, or foeti) is the unborn offspring that develops from an animal embryo. Following embryonic development the fetal stage of development takes place. In human prenatal development, fetal deve ...
. This occurs because of the release of testosterone by the luteoma. Testosterone is a sex hormone most abundant in men although small amounts are naturally present in women. Testosterone is responsible for the male characteristics such as deepening of the voice, growth of dark hair, and acne. While not life-threatening, the development of male characteristics associated with luteomas can cause visible changes in the mother and can have drastic effects on the formation of the fetus. Luteomas can cause the fetus to be born with an ambiguous sex, which, depending on how the parents prefer to raise the infant, may result in the parents choosing a sex for the fetus. Luteomas can be associated with
disorders of sex development Disorders of sex development (DSDs), also known as differences in sex development, diverse sex development and variations in sex characteristics (VSC), are congenital conditions affecting the reproductive system, in which development of chromoso ...
(formerly known as
pseudohermaphroditism Pseudohermaphroditism is a condition in which an individual has a matching chromosomal and gonadal tissue (ovary or testis) sex, but mismatching external genitalia. Female pseudohermaphroditism refers to an individual with ovaries and external gen ...
).


Signs and symptoms

Luteoma is frequently asymptomatic; only 36% of women actually show signs of masculinization. These signs include acne, the growth of dark hair (especially on the face), deepening of the voice, temporal balding, and
clitoromegaly Clitoromegaly (or macroclitoris) is an abnormal enlargement of the clitoris that is mostly congenital or acquired, though deliberately induced clitoris enlargement as a form of genital body modification is achieved through various uses of anabo ...
. An increase in testosterone levels in the mother does not necessarily mean masculinization will occur. During a normal pregnancy, the testosterone level will increase slightly in the first and second trimester, but doubles in the third trimester. The testosterone level also depends on the sex of the fetus; male fetuses cause a bigger increase in testosterone levels than female fetuses. Male fetuses, when carried by a mother who develops male characteristics from a luteoma, are not highly affected by the increase in testosterone in the mother due to this conditions. However, after birth, the male fetus may have abnormally high levels of testosterone, but this resolves itself. There have not been any ties between luteomas and the male infant producing high amounts of testosterone by itself. Out of the 36% of women who show male characteristics from the luteoma, 75% of female fetuses will also show signs of masculinization. Female fetuses can have a variety of symptoms ranging from severe, requiring surgery, to mild, which resolves itself after birth. The severity of the symptoms a female fetus undergoes depends on when the exposure occurs and the duration of the exposure. If a female fetus is exposed to increased levels of testosterone in the first 7–12 weeks of the pregnancy, labioscrotal fusion and clitoromegaly can occur. These conditions would need correctional surgery if the infant was to be raised female. If testosterone exposure occurs after the first 12 weeks of pregnancy, no fusion will occur but the clitoris could still be enlarged. The enlarged clitoris usually corrects itself after birth and the abnormally high testosterone levels will decrease as the body produces its own hormones.


Risk factors

Several conditions predispose a woman to forming a luteoma during pregnancy.
Polycystic ovary syndrome Polycystic ovary syndrome, or PCOS, is the most common endocrine disorder in women of reproductive age. The syndrome is named after the characteristic cysts which may form on the ovaries, though it is important to note that this is a sign and no ...
(PCOS) is one such condition. This syndrome is associated with high hormone levels and the failure of the ovaries to release an egg during the
menstrual cycle The menstrual cycle is a series of natural changes in hormone production and the structures of the uterus and ovaries of the female reproductive system that make pregnancy possible. The ovarian cycle controls the production and release of eggs a ...
, a symptom more often associated with
menopause Menopause, also known as the climacteric, is the time in women's lives when menstrual periods stop permanently, and they are no longer able to bear children. Menopause usually occurs between the age of 47 and 54. Medical professionals often d ...
. The high levels of hormones in polycystic ovary syndrome seem to predispose women to forming a luteoma during pregnancy. A characteristic of luteomas is that they grow better in the presence of high levels of hormones that function in normal growth, sexual development, and reproductive function. PCOS causes an excess of hormones in the body including some of the hormones related to these functions. Women who have already had a luteoma during a previous pregnancy have a higher high risk of having another luteoma. In this situation, women can be counseled on the risks of another pregnancy and their alternatives. Other risk factors associated with luteomas are multiple pregnancies, advanced maternal age, and Afro-Caribbean ethnicity.


Diagnosis

Luteomas are not often detected before delivery. Most luteomas are found during surgery if a
caesarean section Caesarean section, also known as C-section or caesarean delivery, is the surgical procedure by which one or more babies are delivered through an incision in the mother's abdomen, often performed because vaginal delivery would put the baby or mo ...
is performed or when some other surgery is performed. Pre-delivery detection is not effective for many reasons. Some tests, that can be performed pre-delivery, measure the amount of testosterone in the blood; however, this is not a very useful detection method since normal pregnancies have increased amounts of testosterone. Another method that would be useful to determine if a fetus is being exposed to testosterone is to test the
placenta The placenta is a temporary embryonic and later fetal organ that begins developing from the blastocyst shortly after implantation. It plays critical roles in facilitating nutrient, gas and waste exchange between the physically separate mater ...
and
umbilical cord In placental mammals, the umbilical cord (also called the navel string, birth cord or ''funiculus umbilicalis'') is a conduit between the developing embryo or fetus and the placenta. During prenatal development, the umbilical cord is physiologic ...
for testosterone. The placenta has a mechanism for converting hormones from the mother into hormones that the fetus needs. If the amount of testosterone in the umbilical cord is higher than normal, the
gene In biology, the word gene (from , ; "...Wilhelm Johannsen coined the word gene to describe the Mendelian units of heredity..." meaning ''generation'' or ''birth'' or ''gender'') can have several different meanings. The Mendelian gene is a ba ...
type of the fetus should be determined to see if the fetus is male or female. If the fetus is female then the high levels of testosterone in the umbilical cord could be an indicator that a luteoma is present. This procedure cannot be safely performed until after the fetus has undergone differentiation (when the sex of the fetus becomes apparent). But by this time the damage has already been done.


Treatment

No treatments for luteomas are currently available. The luteomas can be detected through
ultrasound Ultrasound is sound waves with frequency, frequencies higher than the upper audible limit of human hearing range, hearing. Ultrasound is not different from "normal" (audible) sound in its physical properties, except that humans cannot hea ...
if masculinization is apparent in the mother. The fetus can be tested for gene type and if the fetus is female and the umbilical cord tests high for testosterone levels then the risks of masculinization of the fetus can be considered. Interventions cannot be made to change the outcomes, but the potential risks can be analyzed in order to make preparations. After the fetus is delivered the luteoma regresses on its own and only monitoring of the mother is needed after delivery. Depending on the sex of the fetus, exposure time and duration, the parents may need to decide if they will raise the child as male or female. Surgery may be necessary depending on what sex the child is going to be raised.


References


External links


Histology at esynopsis.uchc.edu
{{Breast cancer/urogenital neoplasia Ovarian cancer Intersex and medicine Human pregnancy Women's health