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Frontiers in Public Health 2022Testicular yolk sac tumor (TYST) is a rare malignant germ cell tumor that mainly occurs in young men. Due to the low incidence of yolk sac tumors, there is a lack of... (Randomized Controlled Trial)
Randomized Controlled Trial
PURPOSE
Testicular yolk sac tumor (TYST) is a rare malignant germ cell tumor that mainly occurs in young men. Due to the low incidence of yolk sac tumors, there is a lack of prospective cohort studies with large samples. We aimed to develop a nomogram to predict cancer-specific survival (CSS) in patients with TYST.
MATERIALS AND METHODS
Patient information was downloaded from the Surveillance, Epidemiology and End Results (SEER) database. We enrolled all patients with TYST from 2000 to 2018, and all patients were randomly divided into a training set and a validation set. Univariate and multivariate Cox proportional hazards regression models were used to identify independent risk factors for patients. We constructed a nomogram based on the multivariate Cox regression model to predict 1-, 3-, and 5-year CSS in patients with TYST. We used a series of validation methods to test the accuracy and reliability of the model, including the concordance index (C-index), calibration curve and the area under the receiver operating characteristic curve (AUC).
RESULTS
619 patients with TYST were enrolled in the study. Univariate and multivariate Cox regression analysis showed that age, T stage, M stage and chemotherapy were independent risk factors for CSS. A nomogram was constructed to predict the patient's CSS. The C-index of the training set and the validation set were 0.901 (95%CI: 0.859-0.847) and 0.855 (95%CI: 0.865-0.845), respectively, indicating that the model had excellent discrimination. The AUC showed the same results. The calibration curve also indicated that the model had good accuracy.
CONCLUSIONS
In this study, we constructed the nomogram for the first time to predict the CSS of patients with TYST, which has good accuracy and reliability and can help doctors and patients make clinical decisions.
Topics: Male; Humans; Nomograms; Endodermal Sinus Tumor; SEER Program; Neoplasm Staging; Reproducibility of Results
PubMed: 36324443
DOI: 10.3389/fpubh.2022.1038502 -
BMC Pediatrics Oct 2022The objective of the study was to analyze the clinical features, treatment, and outcomes of primary vaginal endodermal sinus tumor (EST) in infants and children treated...
BACKGROUND
The objective of the study was to analyze the clinical features, treatment, and outcomes of primary vaginal endodermal sinus tumor (EST) in infants and children treated in a tertiary center.
METHODS
Clinical data of patients with pathologically confirmed primary vaginal EST in our hospital from January 1997 to December 2017 were retrospectively reviewed and analyzed.
RESULTS
A total of 21 patients were included in this study. The median age at diagnosis was 11 months (range, 4-44 months). The most common manifestations were abnormal vaginal bleeding, and a polypoid mass protruding from the vagina. Chemotherapy based on PEB (cisplatin, etoposide, bleomycin) regimen was given, and serum alpha-fetoprotein (AFP) levels dropped to normal levels after 2 to 4 cycles of chemotherapy (median, 2 cycles). After 3 to 13 cycles of chemotherapy, with a median of 5 cycles, 20 patients achieved complete remission (95.2%). The median follow-up time was 80 months (range, 4-281months). At the time of the last follow-up, 19 cases were alive without disease, and the survival rate was 90.5%.
CONCLUSION
Vaginal EST is a very rare malignant germ cell tumor and is sensitive to chemotherapy. Conservative surgery combined with PEB chemotherapy is an effective way of treatment. Serum AFP and imaging examinations can monitor the treatment response and recurrence.
Topics: Antineoplastic Combined Chemotherapy Protocols; Bleomycin; Child, Preschool; Cisplatin; Endodermal Sinus Tumor; Etoposide; Female; Humans; Infant; Retrospective Studies; Vaginal Neoplasms; alpha-Fetoproteins
PubMed: 36207682
DOI: 10.1186/s12887-022-03634-2 -
Archives of Pathology & Laboratory... Jun 2023The interpretation of postchemotherapy resections of anterior mediastinal germ cell tumors plays a critical role in determining future patient management and prognosis....
CONTEXT.—
The interpretation of postchemotherapy resections of anterior mediastinal germ cell tumors plays a critical role in determining future patient management and prognosis. Treatment-related changes in the thymus may mimic residual teratoma or microcystic-pattern yolk sac tumor. There is limited extant information concerning therapy-related pseudoneoplastic thymic alterations.
OBJECTIVE.—
To provide diagnostic assistance to distinguish nonneoplastic thymic abnormalities secondary to chemotherapy from residual germ cell tumor.
DESIGN.—
We retrospectively reviewed 91 resections of primary anterior mediastinal germ cell tumors with recognizable thymic gland following cisplatin-based chemotherapy.
RESULTS.—
The cohort included 90 men and 1 woman (median age, 29 years). A spectrum of thymic epithelial alterations occurred, including cystic change (macrocysts [n = 21] or microcysts [n = 20]); hyperplasia with reactive atypia (n = 8); ciliated, mucinous, or columnar cell metaplasia (n = 3); and mature squamous metaplasia (n = 2). These changes were similar to so-called acquired multilocular thymic cysts, were often contiguous with and adjacent to normal thymic epithelium, and lacked the neoplastic-type atypia seen in teratomatous elements in this setting. In 1 case, confluent microcysts closely mimicked the appearance of yolk sac tumor but lacked other distinctive features of that neoplasm and its characteristic immunoreactivity.
CONCLUSIONS.—
Recognition of therapy-induced thymic changes is important to avoid misinterpretation as residual teratoma or yolk sac tumor. Continuity with and proximity to benign thymic epithelium, absence of neoplastic-type atypia, and awareness of this phenomenon are important in avoiding this pitfall.
Topics: Male; Female; Humans; Adult; Endodermal Sinus Tumor; Retrospective Studies; Neoplasms, Germ Cell and Embryonal; Mediastinal Neoplasms; Teratoma
PubMed: 36136298
DOI: 10.5858/arpa.2021-0631-OA -
Internal Medicine (Tokyo, Japan) Mar 2023Yolk sac tumor (YST) is a rare primary brain tumor that occurs almost exclusively in patients under 30 years old. Intracranial germ cell tumors are most frequently... (Review)
Review
Yolk sac tumor (YST) is a rare primary brain tumor that occurs almost exclusively in patients under 30 years old. Intracranial germ cell tumors are most frequently located in the pineal and suprasellar region. Medulla oblongata YSTs are particularly rare. Extragonadal YSTs may be difficult to diagnose because of their characteristics, such as the rarity and variety of growth patterns. Furthermore, they are known to have a very poor prognosis. We herein report a case of YST of the medulla oblongata in a 50-year-old woman. She was followed up for 18 months without any tumor recurrence.
Topics: Female; Humans; Middle Aged; Adult; Endodermal Sinus Tumor; Neoplasm Recurrence, Local; Neoplasms, Germ Cell and Embryonal; Medulla Oblongata; Skull
PubMed: 35989277
DOI: 10.2169/internalmedicine.9447-22 -
Medicine Jul 2022Testicular neoplasms are not commonly found in children and are a formidable threat if treated inappropriately. However, there is no consensus regarding its management....
Testicular neoplasms are not commonly found in children and are a formidable threat if treated inappropriately. However, there is no consensus regarding its management. This study aimed to create a holistic picture of the interprofessional team in the management of malignant testicular tumors. Seventeen patients had mixed germ cell tumors, 15 had pure yolk sac tumors, 2 had immature teratomas, 2 had teratocarcinomas, and 1 had a sex cord stromal tumor. Five lesions were diagnosed as nongerm cell tumors: 2 embryonal rhabdomyosarcomas, 2 lymphomas, and 1 acute myeloid leukemia. At initial presentation, retroperitoneal (n = 2), bone marrow (n =1), and mediastinal (n = 1) metastases were identified in 4 (10%) patients. The operative interventions performed included radical inguinal orchiectomy (n = 5), scrotal orchiectomy (n = 31), and testicular biopsy or testis-sparing enucleation of the tumor (n = 6). Postoperatively, 18 patients received either adjuvant chemotherapy (n = 14) or chemoradiation (n = 5). Five patients with mixed germ cell tumors (n = 2), group IV paratesticular rhabdomyosarcoma (n = 2), and acute myeloid leukemia with myeloid sarcoma (n =1) died of disease progression. Thirty-six patients remained alive and disease-free at the last visit. Malignant testicular tumors in children deserve proper diagnostic support from a therapeutic perspective. Any concern or suspicion of a testicular tumor warrants an inguinal approach to avoid scrotal violation.
Topics: Child; Endodermal Sinus Tumor; Humans; Male; Neoplasms, Germ Cell and Embryonal; Orchiectomy; Teratoma; Testicular Neoplasms
PubMed: 35866814
DOI: 10.1097/MD.0000000000029735 -
Medicine Jul 2022Ovarian yolk sac tumor (YST) is a very rare malignant tumor in young women. This study aimed to explore the clinicopathological prognostic characteristics and... (Review)
Review
Clinicopathological features, prognosis, and fertility outcomes in Chinese Han women treated for ovarian yolk sac tumor: A retrospective case series study from two tertiary-care academic medical centers.
OBJECTIVE
Ovarian yolk sac tumor (YST) is a very rare malignant tumor in young women. This study aimed to explore the clinicopathological prognostic characteristics and reproductive outcomes of Chinese Han patients.
METHODS
To describe a case series study, we reviewed the clinicopathological data of 50 YST patients treated from 2 tertiary medical academic medical centers from January 2009 to December 2019. The Akaike information criterion was used to select variables. The influence of relevant characteristics on prognosis factors was analyzed by the Cox proportional hazard model.
RESULTS
The median follow-up time was 64.5 months (range from 3 to 124 months). The median age was 22.7 years (3 to 34 years). Abdominal pain (54.0%) or mass (42.0%) were the most common clinical symptoms in the early stage of diagnosis. The tumors were located bilaterally in 4 cases. 27 patients, 7 patients, 13 patients, and 3 patients were in stage I, II, III, and IV, respectively. Twenty-one stage I patients and 12 stage II to IV patients underwent fertility-preserving surgery. Of the 50 patients who received postoperative chemotherapy, 49 received the BEP regimen. At the last follow-up, 92% of the patients were still alive. The overall survival rate and disease-free survival rate were 91.6% and 90.6%, respectively. Recurrence occurred in 7 (14%) patients with a median survival time of 16.7 months (range from 3 to 50 months). Six patients had recurrence in the abdominal space. The percentage of Ki67 (P = .01) and tumor size (P = .03) were 2 important prognostic factors in multivariate analysis. In terms of survival outcomes, fertility-preserving surgery can be equivalent to radical surgery. Sixteen patients tried to conceive, and 6 patients with advanced-stage succeeded in 10 pregnancies. Of these, 6 patients successfully gave birth to 6 healthy babies.
CONCLUSIONS
The diagnosis of YST of childbearing age is very rare. Because the failure of primary treatment is related to the residual disease after salvage surgery, the fertility and survival results of patients undergoing fertility-preserving surgery are promising.
Topics: Academic Medical Centers; Adult; China; Endodermal Sinus Tumor; Female; Fertility; Humans; Neoplasm Staging; Neoplasms, Germ Cell and Embryonal; Ovarian Neoplasms; Pregnancy; Prognosis; Retrospective Studies; Young Adult
PubMed: 35866762
DOI: 10.1097/MD.0000000000029868 -
Arquivos Brasileiros de Cardiologia Jul 2022
Topics: Endodermal Sinus Tumor; Humans; Magnetic Resonance Imaging
PubMed: 35830114
DOI: 10.36660/abc.20210335 -
AJNR. American Journal of Neuroradiology Jul 2022Primary intracranial pure yolk sac tumor is very rare. Our aim was to summarize the characteristics of primary intracranial pure yolk sac tumors from the clinical and...
BACKGROUND AND PURPOSE
Primary intracranial pure yolk sac tumor is very rare. Our aim was to summarize the characteristics of primary intracranial pure yolk sac tumors from the clinical and imaging aspects in a retrospective study.
MATERIALS AND METHODS
We studied 5 patients with primary intracranial pure yolk sac tumors in Guangzhou Women and Children's Medical Center from January 2015 to June 2021. A comprehensive literature search was performed on the electronic database of the China National Knowledge Infrastructure (1990 to June 2021). Clinical data based on age, sex, treatment, CT, and MR imaging findings were collected and analyzed.
RESULTS
A total of 25 patients were included in the study, 21 boys and 4 girls. Twenty-one patients underwent plain MR imaging and an enhanced examination, 9 patients underwent DWI, and 12 patients underwent plain CT and/or an enhanced examination. The tumors were posterior fossa in 9 cases and supratentorial in 16 cases. All tumors showed marked enhancement after enhanced scanning by MR imaging or CT. The signal on DWI was similar to that of the cerebral cortex, and the ADC map was similar to or slightly higher than that of the cerebral cortex. Among the cases, 13 were followed up from 2 months to 5 years. There was no recurrence or metastasis in 9 patients with postoperative chemotherapy or chemoradiotherapy followed up for 1.5-5 years. Four patients died 2 months to 1.5 years after only an operation, or chemoradiotherapy but no operation.
CONCLUSIONS
There are some relatively specific imaging findings of primary intracranial yolk sac tumors that could assist in their diagnosis. Surgery combined with radiation therapy and/or chemotherapy can achieve a better prognosis.
Topics: Adolescent; Child; China; Endodermal Sinus Tumor; Female; Humans; Male; Prognosis; Retrospective Studies
PubMed: 35798388
DOI: 10.3174/ajnr.A7556 -
Cancer Biology & Therapy Dec 2022Mediastinal yolk sac tumors (YSTs) are highly aggressive germ cell tumors with an extremely poor prognosis. Radiotherapy plays an important role in the treatment of...
Mediastinal yolk sac tumors (YSTs) are highly aggressive germ cell tumors with an extremely poor prognosis. Radiotherapy plays an important role in the treatment of mediastinal YSTs. To maximize benefit from radiotherapy in patients with mediastinal YSTs, exploring functionally relevant biomarkers is essential. Previous studies have demonstrated that mutations in DNA-damage repair (DDR) genes, including , potentially enhance sensitivity to radiotherapy in solid tumors. However, DDR-gene mutations, as possible predictive biomarkers for radiotherapy in primary mediastinal YSTs, have not yet been reported. Herein, we report a 29-year-old male patient with a refractory metastatic primary YST involving a germline frameshift mutation in the gene (NM_000059.3: exon11: c.4563_4564delAT: L1522fs). During treatment alternation, the patient was found to respond poorly to chemotherapy with or without an immune checkpoint inhibitor but well to radiotherapy. Finally, the patient achieved approximately 17 months of overall survival. To the best of our knowledge, this case report is the first to describe a remarkable response to local radiotherapy in a patient with a refractory metastatic mediastinal YST involving a DDR-gene mutation (germline frameshift variation). This case report provides insightful clues for precision radiotherapy in clinical practice.
Topics: Adult; BRCA2 Protein; Biomarkers; Endodermal Sinus Tumor; Frameshift Mutation; Germ-Line Mutation; Humans; Male; Mediastinal Neoplasms
PubMed: 35576916
DOI: 10.1080/15384047.2022.2072635 -
Chirurgia (Bucharest, Romania : 1990) Apr 2022Malignant extramucosal esophageal tumors are rare. We publish a case of an extramucosal esophageal tumor which turned out to be a yolk sac tumor. This type of tumor is...
Malignant extramucosal esophageal tumors are rare. We publish a case of an extramucosal esophageal tumor which turned out to be a yolk sac tumor. This type of tumor is extremely rare. We present the initial and also late management of this case (from the diagnostic to the management of the late complications). The patient was admitted for dysphagia. Contrast thoraco-abdominal CT scan revealed an important retrocardiac mass; echoendoscopy with biopsy confirmed one malignant extra-mucosal esophageal tumor. We performed an esophagectomy through triple approaches (McKeown) â?" right thoracotomy, laparotomy, and left cervicotomy for anastomosis. The pathological report raised the suspicion of a mediastinal tumor with germ cells or a poorly differentiated carcinoma. The immunohistochemistry tests confirmed a yolk sac tumor. The patient was referred to the oncologist and was treated with etoposide and cisplatin chemotherapy. Subsequently, the patient developed respiratory failure secondary to giant hiatal hernia which was surgically treated via laparoscopy.
Topics: Cisplatin; Endodermal Sinus Tumor; Esophageal Neoplasms; Humans; Mediastinal Neoplasms; Treatment Outcome
PubMed: 35535785
DOI: 10.21614/chirurgia.2718