Clinical feature analysis of medulloblastoma with extraneural metastasis in children / 中华实用儿科临床杂志

Objective:Summarizing the clinical characteristics of extraneural metastasis in childhood medulloblastoma.Methods:A total of 616 cases with medulloblastoma treated in Beijing Shijitan Hospital from April 2010 to April 2019 were analyzed retrospectively, among which 11 cases developed extraneural metastasis.The age of onset, location and time of extraneural metastasis, pathological and molecular typing, treatment and prognosis were descriptively analyzed.The differences of blood biochemical indexes between medulloblastoma cases with and without extraneural metastasis were statistically analyzed by t test. Results:As of February 2020, the median follow-up period was 16 months (ranging from 3 to 69 months). Eleven cases, including 8 males and 3 females, were diagnosed with extraneural metastasis, with the incidence being about 1.8%.The median age of medulloblastoma was 6 years (2-10 years), and the median age at presentation of extraneural metastasis was 7 years (2-12 years). Extraneural metastasis occurred from 0.5 months to 38.0 months after the operation, and the affected location includes bone (6 cases), bone marrow (3 cases), lung (3 cases), pelvis (2 cases) and abdominal cavity (1 case). In these patients, the range of lactic dehydrogenase (LDH) was (2 298.00±1 570.70) U/L and neuron-specific enolase (NSE) was (201.00±68.34) μg/L, which were significantly higher than those in patients without extraneural metastasis [(249.50±46.28) U/L and (22.80±7.12) μg/L, all P<0.05]. Partial patients were treated with chemotherapy, while the majority of them were treated with palliative treatment in the terminal stage, with the survival period mostly less than 10 months. Conclusions:Although there is a low incidence of extraneural metastasis in medulloblastoma pediatric patients, the prognosis of these patients with extraneural metastasis is poor and most of them would die within one year.The most common sites include bone, followed by bone marrow and lungs, which may be related to the spread of cerebrospinal fluid and the increased levels of LDH and NSE.

Systemic dissemination of glioblastoma: literature review

SUMMARY INTRODUCTION: Glioblastoma (GBM) is the most frequent primary malignant tumor from the central nervous system in adults. However, the presence of systemic metastasis is an extremely rare event. The objective of this study was to review the literature, evaluating the possible biological mechanisms related to the occurrence of systemic metastasis in patients diagnosed with GBM. RESULTS: The mechanisms that may be related to GBM systemic dissemination are the blood-brain barrier breach, often seen in GBM cases, by the tumor itself or by surgical procedures, gaining access to blood and lymphatic vessels, associated with the acquisition of mesenchymal features of invasiveness, resistance to the immune mechanisms of defense and hostile environment through quiescence. CONCLUSIONS: Tumor cells must overcome many obstacles until the development of systemic metastasis. The physiologic mechanisms are not completely clear. Although not fully understood, the pathophysiological understanding of the mechanisms that may be associated with the systemic spread is salutary for a global understanding of the disease. In addition, this knowledge may be used as a basis for a therapy to be performed in patients diagnosed with GBM distant metastasis.

RESUMO INTRODUÇÃO: Glioblastoma (GBM) é o tumor maligno mais comum do sistema nervoso central em adultos. Entretanto, metástase a distância de GBM é um evento extremamente raro. O presente estudo teve o objetivo de realizar uma revisão da literatura para avaliar os possíveis mecanismos biológicos relacionados com a ocorrência de metástase a distância de pacientes com diagnóstico de GBM. RESULTADOS: Os mecanismos que podem estar relacionados com a capacidade de disseminação sistêmica do GBM são a quebra de barreira hematoencefálica (BHE) frequentemente vista em GBM, seja pela doença, seja por procedimentos cirúrgicos, dando acesso aos vasos sanguíneos e linfáticos, associada à aquisição de características mesenquimais de invasividade, resistência aos mecanismos de defesa do sistema imunológico e adaptação a hostilidades dos meios distantes por meio de quiescência. CONCLUSÕES: As células tumorais necessitam vencer diversos obstáculos até a formação de uma metástase distante. Apesar de não totalmente esclarecido, o entendimento fisiopatológico dos mecanismos pelos quais podem estar associados à disseminação sistêmica do GBM é salutar para a compreensão global da doença. Além disso, esse conhecimento pode servir de base para a terapia a ser empregada diante do paciente com diagnóstico de GBM com metástase a distância.

An extraneural primary anaplastic ependymoma at the subcutaneous inguinal region: Report of a rare case.

Ependymomas commonly arise in the central nervous system. Extraneural presentation is quite rare. Herein, we describe a primary extraneural ependymoma in a young female. The mass was located in the right inguinal area. The cut surface of the 7.5 mm × 6.5 mm × 4.5 mm sized tumor was brownish‑yellow in color. Histologically, it was hypercellular exhibiting pseudorosette or rosette formations and some papillary structures. Mitosis was counted as high as 10 per 10 high power fields. Neither necrosis nor vascular endothelial proliferation within the tumor was observed. Tumor cells showed strong glial fibrillary acidic protein immunoreactivity. On epithelial membrane antigen, intracytoplasmic dot‑like immunostaining was observed. This is the first report presenting a primary extraneural anaplastic ependymoma arising in the inguinal subcutaneous region.

Intracranial Hemangiopericytomas : A Retrospective Study of 15 Patients with a Special Review of Recurrence

OBJECTIVE: Although surgical resection is used to treat meningeal hemangiopericytoma (MHPC), there is a high risk of subsequent recurrence. This study investigated factors associated with treatment outcomes and recurrence in patients who had undergone surgical resection of intracranial MHPC. METHODS: Fifteen patients underwent surgical treatments performed by one senior neurosurgeon between 1997 and 2013. Clinical data, radiologic images, surgical outcomes, recurrence, and other relevant characteristics were reviewed and analyzed. RESULTS: Fifteen patients were included in the analysis, 12 (80%) of whom had tumors in the supratentorial region, and 3 (20%) of whom had tumors in the infratentorial region. Complete resection was achieved in all 15 patients, and 3 (20%) patients were administered radiosurgery and conventional radiotherapy after surgery as adjuvant radiotherapy. Three patients developed recurrence, 2 of whom had not received adjuvant radiotherapy. In 1 of the patients who had not received adjuvant radiotherapy, recurrence developed at the original tumor site, 81 months after surgery. The other 2 recurrences occurred at other sites, 78 and 41 months after surgery. The 5- and 10-year overall survival rates were 88.3%, while the 5- and 10-year recurrence-free survival rates were 83% and 52%, respectively. Additionally the mean Ki-67 index differed significantly between patients who did and did not develop recurrence (43% vs. 14%; p=0.001). CONCLUSION: Because of the high risk of MHPC recurrence, MHPC tumors should be completely resected, whenever feasible. However, even when complete resection is achieved, adjuvant radiotherapy might be necessary to prevent recurrence.

Extraneural Metastasis of Glioblastoma Multiforme Presenting as an Unusual Neck Mass

Glioblastoma multiforme (GBM) is the most aggressive intracranial tumor and it commonly spreads by direct extension and infiltration into the adjacent brain tissue and along the white matter tract. The metastatic spread of GBM outside of the central nervous system (CNS) is rare. The possible mechanisms of extraneural metastasis of the GBM have been suggested. They include the lymphatic spread, the venous invasion and the direct invasion through dura and bone. We experienced a 46-year-old man who had extraneural metastasis of the GBM on his left neck. The patient was treated with surgery for 5 times, radiotherapy and chemotherapy. He had survived 6 years since first diagnosed. Although the exact mechanism of the extraneural metastasis is not well understood, this present case shows the possibility of extraneural metastasis of the GBM, especially in patients with long survival.

Extraneural metastases in medulloblastoma / Mestástases extraneurais em moduloblastoma

Medulloblastoma is the most common childhood malignant tumor of central nervous system, but it may also occur in adults. It presents high invasive growth with spreading of tumor cells into the leptomeningeal space along the neuroaxis early in the course of the disease. Extraneural metastases are rare but frequently lethal, occurring only in 1 to 5 percent of patients, and are related, in the most of cases, to the presence of ventriculoperitoneal shunt. Here we characterize the clinical profile of five cases of medulloblastoma with systemic spreading of tumor cells, also comparing them to cases already described in the literature.

O meduloblastoma é o tumor maligno mais frequente do sistema nervoso central na infância, mas também pode ocorrer em adultos. Ele apresenta crescimento altamente invasivo com disseminação de células tumorais ao longo do neuroeixo precocemente no curso da doença. Metástases extraneurais são raras mas frequentemente letais, ocorrendo apenas em 1 a 5 por cento dos pacientes, e estão relacionadas, na maioria dos casos, a presença de derivação ventriculperitoneal. Neste artigo ,apresentamos o perfil de cinco casos de meduloblastoma com disseminção sistêmica das células tumorais, comparando-os com os casos já descritos na literatura.

Recurrence and Extraneural Metastasis in 31 Meningeal Hemangiopericytomas

Purpose: Meningeal hemangiopericytoma(M-HPC), characterized by a high local recurrency and metastatic potential, is a rare neoplasm arising from perivascular pericytes. A retrospective study was performed to identify the recurrence and extraneural metastasis in M-HPC. MATERIALS AND METHODS: We reviewed the records of 31 M-HPC patients treated from 1982 through 1999 at our institution. The time to recurrence and the various parameters affecting recurrence were determined. Extreneural metastasis was also analyzed. RESULTS: The rate of local recurrency was 38.7%(12/31). The overall average recurrence-free period(RFP) before the first recurrence was 104 months, with overall recurrence-free rates(RFRs) at 5 and 10 years after first surgery of 59.2% and 33.6%, respectively. Of the 12 patients who experienced local recurrence, 4 had recurrences 5 years later after the first surgery. Complete excision at the first operation significantly extended the average time before first recurrence from 43 to 111 months. The 5-year RFRs for the groups of complete excision and incomplete excision were 72.7% and 20.8%, respectively(p=0.0060). Although there was no statistical significance, complete excision followed by adjuvant radiotherapy of more than 50Gy extended the RFP. The 5-year RFRs for the groups of complete excision and complete excision with adjuvant radiotherapy were 70.3% and 100%, respectively(p=0.3359). Four patients(12.9%) presented one or more extraneural metastases that were developed at an average of 107 months after the first operation with the 5- and 10-year metastasis rates of 4.4% and 24.9%, respectively. Conclusions: M-HPC has a propensity to recur either locally or at distant sites after surgical resection. Complete excision is the most important factor to reduce recurrence. However, even with complete excision, adjuvant radiotherapy of more than 50Gy significantly reduces the risk of recurrence. Local and distant recurrences may occur after a prolonged disease-free interval, emphasizing the need for long-term follow-up.