Clear cell adenocarcinoma of the vagina and cervix is rare. The increase and occurrence of this tumor has been linked with the maternal use of Diethylstilbestrol (DES) during pregnancy. Treatment consists of radical surgery with vaginectomy, radical hysterectomy and lymphadenectomy.
Squamous cell carcinoma of the cervix may become more common in younger women as the age of onset of sexual activity decreases.
Carcinoma of the uterus in children is extremely rare.
VAGINAL RHABDOMYOSARCOMAS
These are malignant tumors of connective tissue arising from rhabdomyoblasts which are precursors of striated muscle.
These tumors are less common than neuroblastomas or nephroblastomas.
They are by far the most common malignant female genital tumor of childhood, occurring more frequently than ovarian malignant tumors.
Since striated muscle is not normally found where these tumors occur, it has been suggested that they may originate from the undifferentiated mesenchyme that surrounds the mesonephric duct. These lesions may also occur in the perineum unrelated to either the urinary or genital system. The tumor is often recognized as it prolapses from the introitus with a characteristic gross appearance of grape-like clusters of tumor masses (sarcoma botryoids). The tumor must not be confused with either urethral prolapse or a prolapsed ureterocele. Other associated findings include swelling of the external genitalia, vaginal bleeding or vaginal discharge with or without an abdominal mass. The most common cause for a vaginal discharge or vaginal bleeding in the prepubertal female, however, is a vaginal foreign body.
Lower genital sarcomas arise almost exclusively from the central vagina but can originate in either the periurethral area or adjacent to the cervix. The uterus itself does not appear to be a site of primary origin.
Current treatment for lower genital sarcomas in females probably parallels that for the bladder and the prostate in boys. First, the diagnosis is established through biopsy and the extent of disease is determined endoscopically and radiologically. Treatment should first be initiated with pulsed VAC chemotherapy. Reevaluation should be carried out after each course of chemotherapy. Surgery is reserved for the removal of residual disease or exenteration if the patient does not respond to chemotherapy. Radiation therapy is added in the postoperative period. Operations should be done only in those who have failed to respond to the non-operative approach.
MALIGNANT OVARIAN TUMORS
1. Malignant Teratomas
These teratomas contain all three primitive cell layer derivatives with malignant change in at least one cell line. The average age of these girls is 15 years, and they present with similar symptoms as outlined on the front of this paper.
These tumors frequently are found in conjunction with an elevation of the alpha 1 fetoprotein marker (teratomas with a definite focus of yolk sac carcinoma appear to have a more malignant course).
Surgery alone or surgery plus radiotherapy has not been very effective. Chemotherapy protocols such as VAC are currently being reviewed.
2. Dysgerinomas
These are the second most common malignant ovarian tumors in childhood. Sixty percent of these tumors occur in patients under 20 years of age with symptoms similar to those already outlined.
If the pelvic lymph nodes are clear most will survive after a salpingooophorectomy.
It is important to bivalve the opposite ovary and biopsy it, as there are a significant number of occult dysgerminomas present in the contralateral ovary. If these tumors do recur they are responsive to radiotherapy with or without chemotherapy.
3. Endodermal Sinus (Yolk Sac Tumors)
These are unusual germ cell tumors of the ovary and among the most lethal.
Extensive surgery in the past has been shown to be of little additional benefit apart from a salpingo-oophorectomy if the tumor is unilateral. Adjunctive chemotherapy with or without radiation is important.
4. Choriocarcinoma
This tumor is extremely rare and is the most lethal ovarian tumor in childhood. It is usually resistant to both radiotherapy and chemotherapy.
5. Sex Cord Stromal Tumors
These tumors secrete estrogens and progesterones, thereby causing precocious puberty or menstrual disturbances in adolescent girls. Not all of these tumors are hormone-producing, and rarely virilization may be produced.
A. Granulosa Tumors
They must be considered in the differential diagnosis of precocious puberty. The majority of cases are cured by unilateral salpingo-oophorectomy, as they are usually unilateral.
The most common presentation in girls is with breast enlargement, vaginal bleeding, increased development of the labia minora, some modest development of axillary and pubic hair, advanced somatic development, uterine enlargement, pigmentation of the areolae, the development of feminine habitus and accelerated bone age. An abdominal mass is palpable in most cases. These tumors are rarely malignant.
B. Sertoli-Leydig cell tumors
Most of these tumors produce androgens and result in virilization. Girls can develop facial hirsutism, hypertrophy of the clitoris, deepening of the voice, accelerated linear growth with increased muscle mass, and a masculine habitus. The post menarchial girl will develop abnormalities of menstruation such as amenorrhea, decreased breast size and other indications of virilism.
Most of the virilizing symptoms will regress when the tumor is removed.
These tumors are considered of low-grade malignancy, but some have a rapidly progressive fatal course.