Craniopharyngiomas may be resected with a transsphenoidal approach or during craniotomy, depending on the size of the tumor. Some studies show improved prognosis with radiation therapy used in combination with limited tumor removal (38,53).
2.
Germinomas are highly radiosensitive, and surgery is rarely indicated (54).
3.
Prolactinomas and hyperprolactinemia often may respond to dopamine agonists (bromocriptine or cabergoline) (55).
4.
Specific therapies are directed toward malnutrition, malabsorption, weight loss, anorexia nervosa, exercise amenorrhea, neoplasia, and chronic diseases. Logically, it would appear that patients with hypogonadotropic hypogonadism of hypothalamic origin should be treated with long-term administration of pulsatile GnRH. This form of therapy is impractical because it requires the use of an indwelling catheter and a portable pump for prolonged periods and the lack of availability of this equipment in the United States. The primary focus of treatment should be to correct the underlying problem that is causing the menstrual dysfunction (e.g., malnutrition). If a patient is unable to correct the underlying condition, she may be treated with cyclic estrogen and progestogen therapy at least until sexual maturity is achieved. Once sexual maturation is achieved, hormone therapy can be continued to treat hypoestrogenic symptoms until the underlying disorder leading to amenorrhea can be adequately treated.
5.
Patients with Kallmann syndrome, and patients with other etiologies for hypothalamic amenorrhea, can be treated with hormone replacement, as described above. For individuals with anorexia, intensive treatment to achieve weight gain and emotional well-being is preferable to long-term treatment with hormone therapy (56).
6.
If the patient has physiologic delay of puberty, the only management required is reassurance that the anticipated development will occur eventually.
Individuals whose karyotypes contain a Y cell line (45,X/46,XY mosaicism, or pure gonadal dysgenesis 46,XY) are predisposed to gonadal ridge tumors, such as gonadoblastomas, dys germinomas, and yolk sac tumors. The gonads of these individuals should be removed when the condition is diagnosed to prevent malignant transformation. There is some evidence that hirsute individuals without Y chromosomes should undergo gonad removal. One patient with hirsutism and the karyotype 45,X was noted to have a streak gonad; the contralateral gonad was dysgenic and contained developing follicles, well-differentiated seminiferous tubules, and Leydig cells. This patient was found to be HY antigen–positive (57).
Clomiphene citrate is most often ineffective for inducing ovulation in patients with hypogonadism who desire pregnancy because such patients are hypoestrogenic. In patients with hypogonadism, ovulation induction with injectable gonadotropins is generally successful. In patients without ovarian function, oocyte donation may be appropriate. There are reports of deaths in pregnant patients with Turner syndrome resulting from aortic dissection and rupture (58). Careful counseling and investigation should be undertaken in patients with Turner syndrome before treating them with donated oocytes.
Amenorrhea with Secondary Sexual Characteristics and Abnormalities of Pelvic Anatomy
Amenorrhea occurs if there is blockage of the outﬂow tract, if the outﬂow tract is missing, or if there is no functioning uterus (Table 30.2) In order for menses to occur, the endometrium must be functional and there must be patency of the cervix and vagina. Most women with m¨ullerian abnormalities will have normal ovarian function and thus will have normal secondary sexual characteristic development.
Table 30.2 Anatomic Causes of Amenorrhea
Secondary to prior uterine or cervical surgery