Successful treatment of rare disease case first recorded in Vietnam

On May 30, Cho Ray Hospital (HCMC) announced that it had successfully treated a case of hidden testicular cancer in an intersex patient. This is considered the first case in Vietnam of intersex with a mosaic chromosome set of 46, XX/46, XY in an elderly patient with testicular cancer that was discovered and treated with surgery and chemotherapy.

The patient is A. (40 years old, living in Dong Nai ), with a female appearance. Family members said that when A. was 3 years old, they discovered that he had a left testicle in the inguinal canal and went to a children's hospital for examination. Since then, A. has not had any further examination or intervention and has never had a period.

Rare disease in the world just recorded in Vietnam

10 days before being admitted to the hospital, A. noticed that her left groin area began to grow larger and become more painful. A. went to a local hospital for examination before being transferred to Cho Ray Hospital.

Examination results showed that the patient's breasts were not developed; the clitoris was enlarged, with labia majora on both sides; there was no vagina; no menstruation, and the external urethral opening was normal. The mass in the left groin area was about 5x8 cm, firm, immobile, and slightly painful to palpation.

Assessing this as an extremely rare case, the medical team held a multidisciplinary consultation and decided to perform surgery to remove the left testicular tumor. During the surgery, the team also discovered that the patient had a right ovary and no uterus.

The pathological result was seminoma (a type of germ cell cancer in the testicles, commonly found in young men). The patient received 6 cycles of adjuvant chemotherapy and responded well to all of them.

According to Dr. Tran Trong Tri, Department of Urology - Cho Ray Hospital, mosaic hermaphroditism is a very rare abnormality, due to a disorder of sex differentiation characterized by the simultaneous presence of male and female genitalia in the same individual with a rate of 1/100,000 people. The first case was recorded in 1962 and to date, the world has only had about 50 such cases.

Source: https://nld.com.vn/suc-khoe/dieu-tri-thanh-cong-ca-benh-hiem-gap-lan-dau-ghi-nhan-tai-viet-nam-20230530134025413.htm