2.Survival of children with recurrent medulloblastoma undergoing sequential therapy: an analysis of 101 cases.
Yan-Ling SUN ; Jing-Jing LIU ; Shu-Xu DU ; Wan-Shui WU ; Li-Ming SUN
Chinese Journal of Contemporary Pediatrics 2021;23(2):164-168
OBJECTIVE:
To study the clinical features of children with recurrent medulloblastoma (MB) and treatment regimens.
METHODS:
A retrospective analysis was performed on 101 children with recurrent MB who were admitted to the hospital from August 1, 2011 to July 31, 2017. The children were followed up to July 31, 2020. The Kaplan-Meier method was used for survival analysis. The Cox regression model was used for multivariate regression analysis.
RESULTS:
Of the 101 children, 95 underwent remission induction therapy, among whom 51 had response, resulting in a response rate of 54%. The median overall survival (OS) time after recurrence was 13 months, and the 1-, 3-, and 5-year OS rates were 50.5%±5.0%, 19.8%±4.0%, and 10%±3.3% respectively. There was no significant difference in the 5-year OS rate between the children with different ages (< 3 years or 3-18 years), sexes, pathological types, or Change stages, between the children with or without radiotherapy before recurrence or re-irradiation after recurrence, and between the children with different times to recurrence (< 12 months or ≥ 12 months after surgery) (
CONCLUSIONS
As for the recurrence of MB, although remission induction therapy again can achieve remission, such children still have a short survival time. Only reoperation can significantly prolong survival time, and therefore, early reoperation can be considered to improve the outcome of children with recurrent MB.
Cerebellar Neoplasms/therapy*
;
Child
;
Humans
;
Medulloblastoma/therapy*
;
Neoplasm Recurrence, Local
;
Retrospective Studies
;
Survival Rate
3.Lhermitte-Duclos disease: A case report and literature review.
Zhiqiang LIU ; Yanqing HE ; Jiaxin FU ; Jun WU ; Tao SONG ; Ying WANG ; Tianxiang HUANG
Journal of Central South University(Medical Sciences) 2021;46(2):195-199
Lhermitte-Duclos disease (LDD) is a type of rare brain tumor located in posterior fossa. A patient with LDD located in the left cerebellum and vermis was admitted by the Department of Neurosurgery, Xiangya Hospital, Central South University. MRI scan showed slightly heterogeneous enhancement at the region close to vermis. The patient underwent partial resection on August 11, 2016 without postoperative chemoradiotherapy. The progress free survival was 11 months and the overall survival was 17 months. What the case reveals is that the partial resection is not beneficial to these patients with LDD as the residual lesion probably recurs in a short term after operation. The pathogenesis, diagnosis and treatment of LDD are explored and summarized in combination with relevant literature.
Cerebellar Neoplasms/surgery*
;
Cerebellum
;
Hamartoma Syndrome, Multiple/diagnostic imaging*
;
Humans
;
Magnetic Resonance Imaging
;
Neoplasm Recurrence, Local
4.Long-term results of suppressing thyroid-stimulating hormone during radiotherapy to prevent primary hypothyroidism in medulloblastoma/PNET and Hodgkin lymphoma: a prospective cohort study.
Maura MASSIMINO ; Marta PODDA ; Lorenza GANDOLA ; Emanuele PIGNOLI ; Ettore SEREGNI ; Carlo MOROSI ; Filippo SPREAFICO ; Andrea FERRARI ; Emilia PECORI ; Monica TERENZIANI
Frontiers of Medicine 2021;15(1):101-107
Primary hypothyroidism commonly occurs after radiotherapy (RT), and coincides with increased circulating thyroid-stimulating hormone (TSH) levels.We tested therefore the protective effect of suppressing TSH with L-thyroxine during RT for medulloblastoma/PNET and Hodgkin lymphoma (HL) in a prospective cohort study. From1998 to 2001, a total of 37 euthyroid children with medulloblastoma/PNET plus 14 with HL, scheduled for craniospinal irradiation and mediastinum/neck radiotherapy, respectively, underwent thyroid ultrasound and free triiodothyronine (FT3), free thyroxine (FT4), and TSH evaluation at the beginning and end of craniospinal iiradiation. From 14 days before and up to the end of radiotherapy, patients were administered L-thyroxine checking every 3 days TSH to ensure a value < 0.3 μIU/mL. During follow-up, blood tests and ultrasound were repeated; primary hypothyroidism was considered an increased TSH level greater than normal range. Twenty-two/37 patients with medulloblastoma/PNET and all the 14 patients with HL were alive after a median 231 months from radiotherapy with 7/22 and 8/14 having correctly reached TSH levels < 0.3 μIU/mL and well matched for other variables. Twenty years on, hypothyroidism-free survival rates differed significantly, being 60% ± 15% and 15.6% ± 8.2% in TSH-suppressed vs. not-TSH suppressed patients, respectively (P = 0.001). These findings suggest that hypothyroidism could be durably prevented in two populations at risk of late RT sequelae, but it should be confirmed in a larger cohort.
Cerebellar Neoplasms
;
Child
;
Hodgkin Disease/radiotherapy*
;
Humans
;
Hypothyroidism/prevention & control*
;
Medulloblastoma/radiotherapy*
;
Prospective Studies
;
Thyrotropin
5.Risk factors for recurrence within 2 years in children with medulloblastoma.
Jing-Jing LIU ; Yan-Ling SUN ; Shu-Xu DU ; Chun-De LI ; Xiao-Jun GONG ; Miao LI ; Wan-Shui WU ; Li-Ming SUN
Chinese Journal of Contemporary Pediatrics 2019;21(8):761-765
OBJECTIVE:
To investigate the risk factors for recurrence of medulloblastoma (MB) within 2 years and their influence on progression-free survival (PFS).
METHODS:
A retrospective analysis was performed for the clinical data of 123 children with MB who were admitted from January to December, 2017. According to the presence or absence of recurrence, they were divided into recurrence group with 30 children and non-recurrence group with 93 children. The risk factors for recurrence within 2 years were analyzed, and PFS was compared between the children with different risk factors.
RESULTS:
Large-cell/anaplastic type and M stage were risk factors for MB recurrence within 2 years. The risk of recurrence in the children with M+ MB was 3.525 times that in those with M0 MB, and the risk of recurrence in the children with large-cell/anaplastic MB was 3.358 times that in those with classic MB (P<0.05). The survival analysis showed that the median PFS time was 20 months in the children with M+ MB, and the 20-month PFS rate was 50% ± 11% in the children with M+ MB and 81% ± 5% in those with M0 MB (P<0.05). The 20-month PFS rate was 80% ± 5% in the children with classic MB, 65% ± 10% in those with desmoplastic/nodular MB, 86% ± 13% in those with MB with extensible nodularity, and 36% ± 20% in those with large-cell/anaplastic MB (P<0.05).
CONCLUSIONS
Recurrence is an important influencing factor for the prognosis of MB, and M+ stage and large-cell/anaplastic MB are risk factors for recurrence. Children with such risk factors tend to have a low PFS rate.
Cerebellar Neoplasms
;
Child
;
Humans
;
Medulloblastoma
;
Neoplasm Recurrence, Local
;
Prognosis
;
Recurrence
;
Retrospective Studies
;
Risk Factors
6.Down syndrome with asymptomatic neuroglial cyst: A case report and review of the literature.
Seung Do YANG ; Seung Ju LEE ; Dong Hwan LEE ; Yong Hee HONG
Journal of Genetic Medicine 2017;14(2):67-70
Down syndrome (DS, trisomy 21) is associated with neuroanatomical abnormalities, including choroid plexus cysts and various types of brain tumors. Trisomy 21 is associated with oncogenic factor, especially in brain tumor. The brain of DS patients had a smaller volume of gray and white matter and an unbalanced cerebellum volume, indicating a smaller volume overall than normal. We report a case of a DS male patient who had an incidentally discovered neuroglial cyst in left cerebellar vermis. He visited our hospital with gait disturbance and fatigue. But, the neurologic exam was normal. To the best of our knowledge, this is the first reported case of a neuroglial cyst in a trisomy 21 patient. As the developmental mechanisms of a cyst and the choroid plexus are related, more research is needed.
Brain
;
Brain Neoplasms
;
Central Nervous System Cysts
;
Cerebellar Vermis
;
Cerebellum
;
Choroid Plexus
;
Down Syndrome*
;
Fatigue
;
Gait
;
Humans
;
Male
;
Neuroglia
;
Trisomy
;
White Matter
7.A Case of Breast Cancer Brain Metastasis with a 16-Year Time Interval without Evidence of Cancer Recurrence.
Shoko Merrit YAMADA ; Yusuke TOMITA ; Soichiro SHIBUI ; Takashi KUROKAWA ; Yasuhisa BABA
Journal of Breast Cancer 2017;20(2):212-216
The median time of brain metastasis from the diagnosis of breast cancer is approximately 3 years. In this case report, a 69-year-old woman demonstrated cerebellar ataxia. Brain magnetic resonance imaging revealed enhanced lesions in bilateral cerebellar hemispheres. She had undergone surgery, radiation, and chemotherapy for uterine and breast cancer 24 years prior and 16 years prior, respectively. Although she had not received any anticancer treatment for 10 years, no recurrences were identified using whole body scans. A partial tumor resection was performed and the histological diagnosis was an adenocarcinoma from breast cancer. As no extracranial lesions were found, gamma-knife irradiation was performed, without additional systemic chemotherapy. One month posttreatment, the tumors dramatically reduced in size and the patient completely recovered from cerebellar ataxia. Systemic chemotherapy is not always required for brain metastasis from breast cancer with a long interval period, as long as no evidence of extracranial recurrence is detected.
Adenocarcinoma
;
Aged
;
Brain*
;
Breast Neoplasms*
;
Breast*
;
Cerebellar Ataxia
;
Diagnosis
;
Drug Therapy
;
Female
;
Humans
;
Magnetic Resonance Imaging
;
Neoplasm Metastasis*
;
Neoplasm Recurrence, Local
;
Prognosis
;
Recurrence*
;
Whole Body Imaging
8.A Case of Nongerminomatous Germ Cell Tumor with Fulminant Course Concomitant Leptomeningeal Metastasis.
Youn Beom JEONG ; Kyu Chang WANG ; Ji Hoon PHI ; Ji Yeoun LEE ; Jung Eun CHEON ; Hyoung Jin KANG ; Il Han KIM ; Seung Ki KIM
Brain Tumor Research and Treatment 2016;4(1):21-25
We present the case of a 9-year-old boy with a non-germinomatous germ cell tumor (NGGCT) in the pineal gland that exhibited a fulminant course following chemo- and radiotherapy. After the detection of the tiny cerebellar enhancing nodule at the end of chemo- and radiotherapy, tumor seeding progressed rapidly into the entire cisternal space. We herein report a rare case of NGGCT with fulminant clinical course of concomitant cerebellar seeding, with review of literature.
Cerebellar Neoplasms
;
Cerebrospinal Fluid
;
Child
;
Germ Cells*
;
Humans
;
Male
;
Neoplasm Metastasis*
;
Neoplasms, Germ Cell and Embryonal*
;
Pineal Gland
;
Pinealoma
;
Radiotherapy
;
Biomarkers, Tumor
9.A Case of Nongerminomatous Germ Cell Tumor with Fulminant Course Concomitant Leptomeningeal Metastasis.
Youn Beom JEONG ; Kyu Chang WANG ; Ji Hoon PHI ; Ji Yeoun LEE ; Jung Eun CHEON ; Hyoung Jin KANG ; Il Han KIM ; Seung Ki KIM
Brain Tumor Research and Treatment 2016;4(1):21-25
We present the case of a 9-year-old boy with a non-germinomatous germ cell tumor (NGGCT) in the pineal gland that exhibited a fulminant course following chemo- and radiotherapy. After the detection of the tiny cerebellar enhancing nodule at the end of chemo- and radiotherapy, tumor seeding progressed rapidly into the entire cisternal space. We herein report a rare case of NGGCT with fulminant clinical course of concomitant cerebellar seeding, with review of literature.
Cerebellar Neoplasms
;
Cerebrospinal Fluid
;
Child
;
Germ Cells*
;
Humans
;
Male
;
Neoplasm Metastasis*
;
Neoplasms, Germ Cell and Embryonal*
;
Pineal Gland
;
Pinealoma
;
Radiotherapy
;
Biomarkers, Tumor
10.Ultrastructural characteristics of medulloblastoma.
Qi ZHANG ; Pinan LIU ; Fu ZHAO ; Yilin SUN
Chinese Journal of Pathology 2016;45(2):115-116

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