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Some pediatric gynecologic cancer patients have been diagnosed with menstrual abnormalities and other problems. At present, the clinical understanding of pediatric gynecological tumors is still insufficient, and the level of diagnosis and treatment needs to be improved.
Written by | Huang Siyu
Recently, the Chinese Journal of Obstetrics and Gynecology published two special cases reported by the First Affiliated Hospital of Zhengzhou University. Both girls, aged 6 and 7, were diagnosed with clear cell carcinoma of the cervix and eventually underwent wide hysterectomy.
Before being diagnosed with cervical cancer, both girls were on anti-infective therapy and did not go to a higher-level hospital until they found that their symptoms did not improve or that new symptoms appeared. This reflects that in the gynecology departments of many primary hospitals and general hospitals, doctors' understanding and diagnosis and treatment of pediatric gynecological tumors need to be improved.
6-year-old and 7-year-old girls diagnosed with cervical cancer
On February 25 this year, the Chinese Journal of Obstetrics and Gynecology published "Two Cases of Clear Cell Carcinoma of the Cervix in Children", which introduced the clinical manifestations and treatment of two cases of clear cell carcinoma of the cervical in children admitted to the First Affiliated Hospital of Zhengzhou University.
Screenshot source: Chinese Journal of Obstetrics and Gynecology website
The 7-year-old child in case 1 had intermittent irregular vaginal bleeding for half a year, and was given symptomatic treatment such as anti-infection after examination by the local hospital, but the symptoms did not improve significantly, so he went to the First Affiliated Hospital of Zhengzhou University for treatment, and found a cervical mass and resected it by hysteroscopy. After postoperative pathological testing and immunohistochemical testing, the diagnosis of cervical clear cell carcinoma was confirmed. On December 3, 2020, the child underwent laparoscopic radical hysterectomy. The hospital recommended adjuvant chemotherapy after surgery, but the family strongly refused because of the child's young age.
The 6-year-old child in Case 2 presented to the First Affiliated Hospital of Zhengzhou University more than a year later with brown vaginal discharge. The child was previously diagnosed with urinary tract infection by the local hospital, and his symptoms improved after symptomatic treatment, but later he had vaginal bleeding and had to go to the provincial hospital for treatment, and was diagnosed with clear cell carcinoma of the cervix, and went to the First Affiliated Hospital of Zhengzhou University for further treatment. Due to the family's request to preserve fertility, the child first underwent local resection of the cervical tumor. Unfortunately, two years after the end of treatment, the tumor was found to have recurred, and after communicating with the family, the hospital also performed a wide hysterectomy for the child.
Clear cell carcinoma of the cervix is a rare, specific type of adenocarcinoma of the cervix and a malignant tumor. According to data released by the World Health Organization in 2020, clear cell carcinoma of the cervix accounts for 3%-4% of adenocarcinoma of the cervix. Adenocarcinoma of the cervix accounts for 10% to 15% of all types of cervical cancer. Combined, clear cell carcinoma of the cervix accounts for only 0.3%-0.6% of all types of cervical cancer.
In children, the occurrence of clear cell carcinoma of the cervix is even rarer. The authors searched the domestic and foreign literature from 2013 to 2023 and found only 8 patients aged 14 years and younger. The scarcity of clinical cases also leads to the lack of uniform standards for the treatment of patients at present, and the prognosis is even more unknown.
Cervical cancer in children is not necessarily related to HPV infection
"Cervical tumors in children are very rare, and clear cell carcinoma of the cervix is even rarer. The cause of cervical tumors in children is unclear due to the small number of cases available for study. Sun Liying, deputy director of the Department of Pediatric Gynecology at the Children's Hospital of Zhejiang University School of Medicine, told the "medical community".
Sun Liying has been engaged in pediatric and adolescent gynecology for more than 20 years, and before that, she had 12 years of clinical and teaching experience in adult gynecology, but she has not received patients with clear cell carcinoma of the cervix in the Children's Hospital of Zhejiang University. In 2000, the Children's Hospital of Zhejiang University took the lead in opening pediatric and adolescent gynecology outpatient clinics in China, with an annual outpatient volume of nearly 40,000 people, but only 4 cases of cervical tumors have been treated.
However, it is worth noting that the HPV test of these 4 patients with cervical tumors was negative, and 2 of them had no choice but to undergo radical surgery to remove all the uterus because of the high degree of malignancy of the tumor.
The first case was more than a decade ago. An 11-year-old girl who experienced irregular vaginal bleeding after her first period initially thought it was a menstrual problem that predisposes to puberty. However, after 3-4 months of standardized treatment, the girl's vaginal bleeding still does not improve, which seems to be different from the general adolescent menstrual disorder.
At that time, there were only 2 full-time pediatric gynecology doctors in the Children's Hospital of Zhejiang University, and there were only daytime outpatient clinics. Sun Liying instructed the girl's parents to go to the Obstetrics and Gynecology Hospital Affiliated to Zhejiang University School of Medicine to see an emergency department if there was a sudden massive vaginal bleeding at night. One night, the girl was bleeding profusely from the vagina, and her parents immediately took her to the Women's Hospital of Zhejiang University. Upon examination, it was found that the girl had a large mass in her cervix, which was pathologically suggestive of adenocarcinoma of the cervix. The girl eventually underwent surgery and radiotherapy. Fortunately, she is still alive now.
The second patient was only 10 and a half years old, and his menarche lasted for half a month and was still not clean. The attending doctor prescribed an ultrasound examination, but the parents refused to take the examination on the grounds that the queue for the examination was too long and that the leave would delay the study.
A month later, the girl's vaginal bleeding was heavy and smelly, and her parents took her to the children's hospital of Zhejiang University again. Upon examination, it was found that the girl had a large mass in her cervix, most of which had protruded into the vagina. After hysteroscopic surgery, Sun Liying and her team completely removed the tumor and saved the uterus for the girl. Pathology suggests that this is an example of uterine submucosal angioleiomyoma. After the operation, the girl received hormone therapy for 3 years to suppress menstruation and prevent tumor recurrence. Currently, the girl has normal menstruation and is still being followed up.
Two years ago, Sun Liying also treated a 13-and-a-half-year-old patient. The girl was diagnosed with "menstrual disorders" at a local hospital, but the treatment was not effective. In the children's hospital of Zhejiang University, it was found that the girl had a huge mass in the cervix, which completely protruded from the vagina, and the malignant tumor of the cervix was highly suspected before the operation. Pathology of tumor tissue biopsy showed that this was a case of cervical Müllerian adenosarcoma, which was highly malignant and extremely rare in adolescent patients.
According to the literature, even if such patients with cervical sarcoma undergo radical cervical cancer surgery and chemoradiotherapy, the prognosis is still poor and the mortality rate is extremely high, so parents strongly urge to preserve the uterus first. After multidisciplinary consultation inside and outside the hospital, Sun Liying and the tumor team could only perform cervical and tumor resection for her first, and follow up closely after the operation.
Unfortunately, less than 5 months after the operation, the tumor recurred. The girl was later referred to the Department of Obstetrics and Gynecology of Peking Union Medical College Hospital for total hysterectomy + pelvic lymphatic dissection, and is still undergoing chemotherapy follow-up.
"If a child has a 'menstrual disorder' with poor treatment effect after menarche, and abnormal vaginal bleeding accompanied by foul odor in children and adolescence, it must be taken very seriously, which is likely to be a sign of cervical tumor." Sun Liying said. She also stressed that when performing pelvic ultrasound imaging, it is important to pay attention to the local ultrasound examination of the cervix and vagina to avoid missing local lesions of the cervix and vagina.
Although children and adolescents rarely suffer from cervical cancer, Sun Liying stressed that 9-14 years old is still the best age to receive HPV vaccine, and early vaccination can better reduce the probability of cervical cancer in adulthood.
The diagnosis and treatment of pediatric gynecological tumors need to be improved
In recent years, more than 120 patients have been diagnosed with gynecological tumors every year, some of whom have been diagnosed with menstrual abnormalities and other problems in local hospitals. This reflects that the clinical understanding of pediatric gynecological tumors is still insufficient, and the level of diagnosis and treatment needs to be improved.
Director Sun Liying (middle) is the chief surgeon/Photo provided by the interviewee
Taking hysteroscopy as an example, Sun Liying introduced that children's vaginas and uterine cavities are smaller than those of adults, and special "mini" hysteroscopes need to be used. However, most general hospitals have fewer and less experienced gynaecology patients in children, and some hospitals do not have hysteroscopes suitable for pediatric examinations. In addition, because the child's reproductive tract is immature and the patient's degree of cooperation is relatively low, the examination needs to be performed under anesthesia and is completed by a specialist experienced in vaginal and hysteroscopic operations in children. All these put forward higher personnel and equipment requirements for the gynecology department of general hospitals.
In 2008, Sun Liying studied hysteroscopy technology with Professor Xia Enlan, the founder of hysteroscopy diagnosis and treatment medicine in mainland China, and introduced this technology to the Children's Hospital of Zhejiang University. Nowadays, doctors in the Department of Pediatric and Adolescent Gynecology of Zhejiang University Children's Hospital are proficient in using this technology, which can not only be used for the diagnosis and treatment of pediatric gynecological tumors, but also for nearly 100 patients with refractory vaginitis, unexplained vaginal bleeding, and vaginal foreign bodies received by the department every year.
Vaginal ultrasound is a commonly used auxiliary examination method for gynecological tumors, but its application in pediatric patients is limited, and transanopelvic ultrasound will cause strong discomfort to pediatric patients, and the willingness of children and parents to accept cooperation is relatively low. To this end, the ultrasound department of the Children's Hospital of Zhejiang University adopts the method of pelvic + perineal ultrasound combination, which can comprehensively understand the condition of the child's reproductive tract. Long-term clinical practice has proved that this examination method can be a good way to detect gynecological problems in children and adolescents.
In addition, vaginal smears and pap smears can be used to perform non-invasive sampling of gynecologic tumors to determine the type and stage of the tumor. However, Sun Liying pointed out that because the child's reproductive system is not yet developed, children and adolescents cannot use speculum to expose the cervix, and these tests are still not possible and cannot be carried out in children. In addition, there are no specific biomarkers for pediatric gynecologic tumors. To a certain extent, these factors affect the early detection and accurate diagnosis of pediatric gynecologic tumors.
Sun Liying (right) and her team performing surgery / Photo courtesy of the interviewee
There is still a lack of guidelines for pediatric and adolescent gynecology in China
In Sun Liying's view, another major reason why clinicians have insufficient understanding of pediatric gynecological tumors and the level of diagnosis and treatment needs to be improved is that there is still a lack of relevant guidelines and expert consensus in the field of pediatric and adolescent gynecology in China for doctors to learn. From gynecological tumors to genital tract infections, the vast majority of the experience of the current guidelines comes from adult gynecology, but the structure of the reproductive system and vaginal flora of children and adolescents are very different from those of adults, and the experience of adult gynecology cannot be directly applied to pediatric and adolescent gynecology.
"Taking bacterial vaginosis as an example, adult patients are usually infected with pathogens such as Gardnerella, trichomoniasis, and Candida, while in childhood, patients may have weak resistance to the vaginal mucosa, respiratory pathogens, etc., which are caused by the spread of blood, and adolescent patients may be infected with Candida." Sun Liying said.
In order to gain experience, Sun and other doctors in the department often consult foreign literature, but they found that the experience of Western populations is not entirely applicable to Chinese children and adolescents.
Domestic guidelines usually need to be led by authoritative societies and associations such as the Chinese Medical Association, but there are still no special pediatric and adolescent gynecology societies and groups in China. Sun Liying hopes that the relevant guidelines for pediatric and adolescent gynecology can be formulated in an expert-led mode, or the opinions of pediatric gynecology experts can be included in the formulation of guidelines for other gynecological diseases, so as to provide gynecologists with diagnosis and treatment experience, and also enable gynecologists and pediatricians to be more confident and give more scientific and standardized diagnosis and treatment opinions when encountering pediatric and adolescent gynecological patients.
"Pediatric adolescent gynecology is a marginal, interdisciplinary and unpopular discipline, and domestic pediatric and adolescent gynecology started late and developed slowly. Over the years, we have been looking for and accumulating experience, and if there are Chinese guidelines and expert consensus, it will be of great help and contribution to the development of pediatric and adolescent gynecology in China. Sun Liying said.
Expert Profile
Sun Liying
Deputy Director and Deputy Chief Physician of the Department of Pediatric Gynecology of Zhejiang University Children's Hospital, specializing in the diagnosis and treatment of various pediatric and adolescent gynecological diseases. In particular, he has rich clinical experience in the diagnosis and treatment of precocious puberty in children, Turner syndrome, ovarian tumors, genital malformations, vulvar itching, vulvar lichenification, polycystic ovary syndrome, abnormal uterine bleeding during puberty, vulvovaginitis in young girls, vaginal foreign bodies and abnormal sexual development. In 2000, it took the lead in opening pediatric and adolescent gynecology clinics in China. He is a visiting scholar in the Department of Paediatrics and Adolescent Gynecology at the Royal Australian Children's Hospital, a member of the International Federation of Infantile and Juvenile Gynecology (FIGIJ), and a member of the Asia Oceania Society of Pediatric and Adolescent Gynecology, AOSPAG). He has published more than 30 papers in core journals at home and abroad, and 5 of the first author's papers have been included in SCI. Associate editor-in-chief and co-editor of 5 related professional works such as "Pediatrics and Adolescent Gynecology". He has undertaken a number of projects such as the Provincial Natural Science Foundation, the Provincial Department of Health, the Department of Education, and the Family Planning Commission. It has 1 practical technology and invention patent. His research interests include the etiology and pathogenesis of vulvovaginitis in children, the treatment of polycystic ovary syndrome in adolescents, HPV infection and vaginal microecology in adolescents.
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Bibliography:
[1]https://rs.yiigle.com/cmaid/1492368
Source of this article: Responsible editor of the medical community: Moon
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