The Department of Obstetrics & Gynecology, PUMCH is a center for difficult cases from across the country. Its Gynecologic Oncology Center is particularly famous for treating difficult cases. Many rare, difficult and complicated cases of female genital tract tumors are diagnosed and treated here, many of them being first reported in China or in largest number ever reported by a single center in the world. All this won the hospital a good reputation.
Fertility preserved despite young female genital tract sarcoma
Genital tract sarcoma in young women is rare, those in young or very young girls (such as RMS and cervix ASPS) are even rarer. Diagnosis is difficult due to obscure symptoms and the lack of markers; treatment is also difficult considering the rarity of the conditions and lack of medical guidelines, and the necessity of preserving fertility.
After decades of efforts, Obstetrics & Gynecology created its unique diagnostic methods and adopted individualized therapies. According to Professor Cao Dongyan, deputy director of Gynecologic Oncology Center, the vaginal of a little girl is tiny and cannot be checked by conventional devices. Professor Shen Keng and Professor Yang Jiaxin, working together with nurses from operation room, creatively used adult hysteroscope to examine vaginal tumors in small girls and used adult nasal endoscope for open vaginal examinations, and targeted the right part for biopsy or resection. They made surgical and chemotherapy plans according to a patient's age, tumor location, stage, and boosted the cure rate significantly. Without affecting life and prognosis, they preserved the young patients' physiological and reproductive functions, so that they can grow up as healthy persons, to have periods, get married and give birth.
Apart from vaginal sarcoma, the team also treated many other rare genital tract tumors in young and very young girls, and established a databank. These precious information will support follow-up researches and benefit patients of the same conditions.
Working Together on IVL
Intravenous leiomyomatosis (IVL) is a benign smooth muscle tumor of the uterus that grows within the veins but does not invade the surrounding tissue. IVL usually starts in the veins of the uterus and can extend into the inferior vena cava and ultimately into the right side of the heart, resulting in death.
Due to its rare and multi-disciplinary nature, IVL is very often misdiagnosed and reoccurs easily. An operation might involve opening the chest, the abdomen, using deep hypothermic circulatory arrest (DHCA) and has to avoid damaging healthy organs. It is highly traumatic, with a bigger loss of blood, and recovery is slow. Many patients died due to a lack of timely and correct treatment.
Through nearly two decades' clinical research, Obstetrics & Gynecology found out the biological behavior and clinical outcome of this rare tumor, and formulated treatment procedures fitting different patients. Drugs were used to check tumor growth before operation, and after careful assessment, multi-disciplinary consultation decided the operating team; during the operation, doctors from Obstetrics & Gynecology, Urinary Surgery, Vascular Surgery, Liver Surgery, Cardiac Surgery and so on worked together; after operation, pathological conclusions were made, rehabilitative and adjuvant therapies were given to ensure the best outcome.
So far the team has performed more than 200 difficult IVL operations, including 80 that affected the heart. These seriously ill patients not only received complete treatment but were restored to a long, healthy life.
IVL growing to the heart (left) and pelvic (right)
Base Found for Prognosis of Ovarian Sertoli-Leydig Cell Tumor
Ovarian sertoli-leydig cell tumor is a rare sex cord-stromal tumor of the ovary, taking no more than 0.5% of malignant ovarian tumors. It can occur at almost any age. It can be too small to show in image, or big enough to fill the whole pelvic. Symptoms are varied and complicated. They can be masculine features in females, such as the appearing of Adam's apple, beard, leg hairs and enlarged clitoris, casting a huge psychological impact on patients; it can also be "period" in small girls or old ladies, and some patients also have chromosomal abnormalities or genital abnormalities. Often it needs to be distinguished from endocrine diseases and adrenal tumors. Both diagnosis and treatment are complicated, and there lacks clinical guidelines on surgical scale and adjuvant therapies.
Obstetrics & Gynecology, working together with Endocrinology, Pathology and Urinary Surgery, had long-term study of the tumor's structure, clinical symptoms, differentiation and prognosis. It is found that those with no endocrine performance were of worse prognosis, thus establishing the relationship between endocrine differentiation and prognosis. Based on this, individualized operation plans and post-operation adjuvant chemotherapy indicators were formulated. As a result, young patients could preserve their fertility, low-risk patients could avoid unnecessary chemotherapy, patients with genital abnormalities could be clearly diagnosed and correctly treated to avoid tumor on the other side.
Conservative Plan for PSTT Accepted by International Guidelines
Placental site trophoblastic tumor (PSTT) is a rare type of trophoblastic tumor, which shows rather different clinical characters from other trophoblastic tumors. It does not show high HCG, and FIGO's trophoblastic tumor prognosis standards are not very much applicable. Therefore clear diagnosis and prognosis assessment are difficult. Conventional principles included hysterectomy, but the outcome was unsatisfactory and the therapy was disputed.
Since 1998, when the first case of PSTT was diagnosed, the hospital has treated nearly 100 cases. Through improved chemotherapy, III~IV stage patients, after surgery and chemotherapy, reported a complete response rate of over 80%, compared to the around 30% among same stage patients reported by foreign literature.
Obstetrics & Gynecology also tried to preserve the fertility of nearly 20 patients. It proposed the clinical guideline that “for patients who are young, have desire to give birth and the tumor is local, conservative surgery can be used to remove the lesions, assisted by post-surgery chemotherapy." Indications were formulated for preserving fertility, which were accepted into the FIGO guidelines.
The team's achievements lie on the academic teambuilding of Obstetrics & Gynecology, the exploring spirit of doctors and the hospital’s multi-disciplinary support, said Professor Cao.