Guide Express || 2020 ESGO/SIOPE Guide: Management of Non-Cortized Ovarian Cancer in Adolescents and Young People

In July 2020, the European Society of Gynaecological Oncology (ESGO) and the European Society of Paediatric Oncology (SIOPE) jointly published guidelines for the management of non-endottretic ovarian cancer in adolescents and young people, targeting non-endottremic ovarian cancer patients aged 15-25 years, including ovarian malignant reproductive cell tumors (MOGCT), serotonin tumors (SCST) and hypercalcemia ovarian cell carcinoma (SCHT).
The guidelines cover the diagnosis, pathological characteristics, stages, auxiliary examinations, treatments and follow-up of various types of oncology pathology, including early, late, and refractic/recurring diseases, and clarify the principles of pathological assessment and treatment, defined by AYAs as women between the ages of 15 and 25, although somewhat arbitrary.
Follow the five-step process: Multidisciplinary International Development Group nominations -- identification of scientific evidence -- Development guidelines -- External assessment of the guidelines (international review) - Integration of international assessers' opinions ESGO/SIOPE guidelines aims to improve and integrate in a multidisciplinary environment The management of an AYAs for paediatric oncologists, paediatric surgeons, reproductive endocrinologists, psycho-oncologists, psychologists, gynaecologists, general gynecologists, surgeons, radiation oncologists, pathologists, medical and clinical oncologists, radiologists, general practitioners, palliative care teams, and related health professionals.
guidelines do not include an economic analysis of strategies, and clinicians applying or referencing the guidelines should determine individual management measures in the light of the patient's specific circumstances.
to ensure that the content of the guide is based on objective basis, the existing literature has been rigorously reviewed and evaluated.
a systematic literature review of studies published between January 1998 and May 2018.
can be used if there is sufficient high level of evidence to support it and/or if there is broad consensus among experts.
The reliability and quality of the evidence is graded by reference to the Scottish Inter-University Guide Network Scoring System: A has at least one meta-analysis, systematic review or RCT support, with a rating of 1 plus, which is directly applicable to the target population;
B-rated 2-plus-series study evidence, the results are generally consistent, directly applicable to the target population, or the evidence inferred from studies rated 1 plus or 1 plus, C-rated 2 plus study evidence, the results are consistent, directly applicable to the target population, or inferred from studies rated 2 plus, D evidence level 3 or 4, or from studies rated 2 plus, √ based on practical experience, the expert recommendation team recommended evidence.
best practices based on the clinical experience of the Guide Development Group.
1 high-quality meta-analysis, systematic overview of randomized controlled trials, or randomized controlled trials with very low risk of bias1 plus good meta-analysis, systematic review, or randomized controlled trials with a low risk of bias;
2 plus good case control or queue studies with a low risk of mixing or bias and a moderate probability of causation; 3 Non-analytical studies, such as case reports, case series analysis√ General principles of treatment of expert opinion√ Patients should be referred to specialized centres with multidisciplinary backgrounds, including adult and child specialists, as the various non-endal ovary tumors covered by this guide are very rare.
√ treatment should involve experienced oncologists throughout the process.
√ need to be phased in according to the FIGO 2014 phased system.
For patients with suspected ovarian tumors, preoperative diagnosis should include abdominal and pelvic MRI and chest CT examinations, as well as serum AFP, β-HCG, inhibitor B, anti-miaole tube hormone (AMH) and lactic acid dehydrogenase (LDH).
√ blood calcium levels should be measured√ for patients who have completed emergency surgery, the examination should be perfected as soon as possible after surgery, preferably MRI instead of CT, in order to reduce radiation.
preoperative pelvic MRI helps to assess whether the ovaries are suffering (e.g. asexual cell tumors and teratomas) and to understand tumor characteristics.
√ PET-CT is not recommended and the negative prediction is low.
√ should avoid tumor punctures.
√ The surgical plan should refer to the imaging results, the operation preferred ovarian excision, rather than cyst excision /tumor excision, try to avoid tumor rupture, prohibit tumor biopsy, unless there is ovarian spread.
√ the preferred caesarean section, remove the abdominal indicing.
Only minimally invascopic surgery can be selected: surgeons have received special training in laparoscopic tumor surgery to ensure that the tumor does not rupture, complete removal into the specimen bag to remove, prohibit shredding to fully explore the abdominal cavity√
√ Comprehensive phased surgery also includes peritina surface examination, including diaphragm, colon side groin, pelvic peritina biopsy, pelvic cavity and aorta side lymph node examination and contact diagnosis, should be removed swollen lymph nodes, large retina detection and biopsy, should be removed adhesion or abnormal large mesh, check the side ovaries, the abnormal site biopsy.
procedures (FSS) that retain reproductive function (retention of the uterus and at least part of the ovarian tissue) should be preferred until the conventional pathology is clear.
√ for ovarian lesions that are visible to the naked eye, the location and size should be described in detail and biopsies taken.
√ treatment should consider the acute and late side effects of chemotherapy drugs and avoid steroids.
√ all AYA patients should be provided with oncology fertility counselling before treatment.
from initial treatment to follow-up, oncology psychological support should be provided to all AYA patients and their relatives.
√ if the tumor markers rise in diagnosis, after surgery, before auxiliary treatment should be tested, before each chemotherapy should be detected tumor markers, the decline should be in line with the half-life.
√ AYAs patients with MOGCT, SCST or SCCOHT should be included in clinical trials to ensure safety on the basis of improved efficacy for "high-risk" patients and to maintain efficacy while reducing toxicity in "low- and medium-risk" patients.
if there is no standard treatment, clinical trials should be added to assess the effectiveness of the new approach;
General Principles of Pathology Assessment√ Tumors should be classified according to the WHO Classification Act 2014 In view of the rarity of these tumors and the significant risk of misdiagnosis, it is highly recommended that they be confirmed by an experienced gynaecologist or paediatric pathologist, and pathology consultations are highly recommended.
immunology and molecular testing are usually necessary for diagnosis, but are lacking in many pathology laboratories.
For certain types of tumors, familial tumor syndrome should be considered and genetic counselling, including psychological counseling and gene analysis of genus mutations, in particular two-sided GCT, one-sided GCT companion glands or adolescent stunting, ovarian testoblastoma (SLCT) and SCCOH, should be considered.
Ovarian malignant reproductive cell tumor diagnosis, pathology/phased and examination√ Clinical stages follow the recommendations of management principles, MOGCT includes asexual cell tumors, ythroid cystoma (YST), immature teratomas, embryonic cancer and non-pregnancy velvet cancer.
√ should also report the presence of lymphatic tube gaps.
Immune histification markers have some reference value due to diagnostic difficulties: SALL4: usually all MOGCT positive, including immature neurothyracisal octelectotyte 3/4, PLAP, D2-40, NANOG and CD117: asexual cell tumors Positive PLAP: YST positive AFP and Glypican-3:YST positive OCT3/4, CD30, NANOG and SOX10: Embryonic cancer often positive β-HCG and inhibitor: non-pregnancy velvet cancer usually positive.
√ because of overlapping interference and dys staining anomalies between various immunoglomeration markers, it is preferable to use a set of markers rather than a single marker for staining.
there are many chromosomal and genetic abnormalities with unknown characteristics in MOGCT, and its clinical significance needs to be evaluated.
most common chromosomal distortion is chromosome 12 p-arm gain, abbreviated as i (12p).
because MOGCTs often occur in patients with adenode dysplatic disorders (e.g. Swyer syndrome), a genetic assessment of sexual chromosomal aberrations is recommended.
treatment of early patients should be supplemented with MRI if an ultrasound reveals that the ovarian tumor is real or partially real.
if MRI finds a real ingredient, surgical treatment is required and is the initial treatment of early MOGCT.
√ should carefully evaluate the surgical method to avoid in-operative rupture.
for double-sided ovarian tumors, two-sided fallopian ovary excision (BSO) should not be used to retain at least part of the ovaries.
for one-sided ovarian solid tumors, ovarian excision should be selected, and if the tumor is cystic, cyst excision should be avoided.
FSS is now considered the standard surgical treatment for young MOGCT patients.
if the side ovaries look normal and imaging negative, no ovarian biopsy is required.
e.g. bi-sided ovaries are affected (mainly asexual germ cell tumors), it is recommended to retain at least one healthy part of the ovaries (one-sided fallopian ovary excision and lateral partial ovary excision) and the uterus, unless genetic analysis reveals abnormal gland development, the ermostic condition recommends the removal of the remaining ovaries.
√ should receive genetic counseling and psychological support for double-sided ovarian tumors.
BSO can be performed due to the risk of developing adenoblastoma and asexual cytoma in cases of adenode dysplus.
√ According to the final pathology report, if the initial surgical cyst excision/tumor excision, there are no signs of ancillary treatment, and the serum tumor markers are normal, secondary surgery is required to remove the remaining ovaries to reduce the risk of recurrence.
there is no need for secondary surgery when complementary treatment is required.
√ risk of both asexual cytomas and teratomas, it is recommended to follow up the retained ovaries closely after surgery.
C if the tumor is suspected to be malignant (secretion, cystic enumeration, ovarian diffusion), such as the choice of laparoscopic stage surgery, should minimize the risk of tumor rupture.
√ for small or uncertain malignant lumps, a minimally invasitable path (laparoscopic or robotic) can be selected.
√ if diagnosed after surgery, you should avoid full phased surgery and lymph node excision again.
lymph node excision is only called if an in-serum is detected and/or an MRI or CT indicates an abnormal lymph node.
for completely removed stage IA tumors, postoperative tumor markers normal or negative, the preferred strict follow-up.
√ for patients with IA phase YST and poor compliance, complementary chemotherapy (up to 2 cycles) is an option.
√ treatment of stage IB tumors is complex, the histological types of double ovary tumors should be weighed.
√ IC1 MOGCT, chemotherapy (up to 2 cycles) or close monitoring.
all types of IC2 and IC3 MOGCT patients should receive complementary chemotherapy (up to 3 cycles).
of chemotherapy in patients with advanced stage of the disease, FSS should be considered even in late stages.
√ initial treatment should avoid tumor cell reduction.
√ only a small number of lesions remaining after chemotherapy (perina, remaining ovaries, and/or lymph nodes) will require surgical removal.
√ patients with asexual cytomas with small lesions in post-treatment imaging should be closely followed, and these residual lesions are usually not viable. Standard chemotherapy for
Iii-IV diseases is used in adult programmes, with bolemycin, etoposide and platinum (BEP) performing 3 to 4 cycles and removing bolemycin after cycle 3 (maximum cumulative dose of 270 IU) to avoid pulmonary toxicity.
For adolescent patients, options are available for PEI (cisplatin, etoposide and isocycline phosphamide), PEB (cisplatin, etoposide and bolemycin) or JEB (carptonine, etoposide and bolemycin) for 3-4 cycles of chemotherapy.
√ the tumor marker remained positive after 4 courses of chemotherapy, the treatment failed.
√ tumor markers did not show a half-life decline after the second course of chemotherapy and should be considered high risk and require intensive treatment.
√ If immature teratomas are accompanied by ovarian diseases (e.g., peritaniform gliomas, which are in the form of benign glial tissue and do not contain immature components), peritina lesions do not need to be completely removed, more biopsies should be required to ensure that all tissues are mature glial components.
treatment of incurable/recurring diseases √ must be discussed by a multidisciplinary team to determine whether treatment is required and specific treatment measures are required.
√ If it is an immature teratoma or a hybrid tumor containing the components of immature teratoma, relapse after chemotherapy but the serum markers are normal, consideration should be given to growth teratoma syndrome (a fully mature component of the peritarean membrane and/or lymph nodes after chemotherapy).
this case, if all tissue pathology is mature tissue, it can only be surgically removed and the operation is conservative, preserving the uterus and part of the ovarian tissue.
√ histology before the start of treatment confirms that relapse is essential.
√ all specimens should be examined in histology to confirm or exclude only mature tissues.
there is no clear recommendation for patients who relapse after chemotherapy, chemotherapy options should refer to past treatment options and no recurrence period, for asexual cell tumors, also consider radiotherapy.
consider intensive chemotherapy with stem cell support.
meaning of palliative surgery is not yet clear.
Follow-up√ Monitoring programs in MOGCT management are based on clinical examination, tumor markers, and imaging, as follows: Clinical examination of chest X-rays/low-dose CT abdomens/pelvic √ #26666√ #2 MRI/CT, which report patient outcomes for the first 6 months of surgery only for other treatments #2. every #6-12 months every 2 months every 2 months every 2 months every 6 months every 3 months every 3 months every 6 months√ 3 years 3-6 months 3-6 months 3-6 months 4-5 years every 6 months √6-10 years per year√