Eberhart Charles G, Cohen Kenneth J, Tihan Tarik, Goldthwaite Patricia T, Burger Peter C
Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA.
J Pediatr Hematol Oncol. 2003 Mar;25(3):198-203. doi: 10.1097/00043426-200303000-00004.
To review the clinical behavior and histopathologic features of medulloblastomas that metastasize outside the central nervous systems (CNS).
The authors studied 23 cases of medulloblastomas that metastasized outside the CNS. The patients included 15 males and 8 females, ages 1 to 40 years at initial diagnosis (median 8.5). Five of the patients were over 20 years of age at diagnosis. The histologic grade of anaplasia was determined for each case.
Extra-CNS metastases were identified at initial presentation in four individuals and up to 11 years later in the remaining cases. Metastatic sites included bone/bone marrow (21 cases), soft tissue/lymph nodes (3 cases), and lung (1 case). In seven cases, multiple extra-CNS metastatic sites were documented. Of the patients with available clinical follow-up after metastasis, 10 died of their disease 1 to 39 months after detection of extra-CNS metastases (median 9 months), while 5 are alive 16 to 120 months after extra-CNS metastasis (median 45 months). Moderate or severe anaplasia was detected in 8 of 20 intracranial specimens (40%) and in 4 of 6 extra-CNS metastases (66%); these frequencies are higher than observed in medulloblastomas overall. Tissue from both the initial resection and subsequent recurrence or metastasis was available in six cases. The anaplasia grade was higher upon recurrence or metastasis in four of these six, consistent with tumor progression.
Metastasis of medulloblastomas outside the CNS can occur after long periods of clinical remission and is associated with anaplasia in some cases. Medulloblastomas can show histologic progression on recurrence or metastasis.
回顾髓母细胞瘤转移至中枢神经系统（CNS）外的临床行为和组织病理学特征。
作者研究了23例转移至CNS外的髓母细胞瘤病例。患者包括15名男性和8名女性，初次诊断时年龄为1至40岁（中位年龄8.5岁）。其中5例患者诊断时年龄超过20岁。确定了每例病例的间变组织学分级。
4例患者在初次就诊时发现CNS外转移，其余病例在长达11年后发现。转移部位包括骨/骨髓（21例）、软组织/淋巴结（3例）和肺（1例）。7例患者记录有多个CNS外转移部位。在转移后有可用临床随访资料的患者中，10例在发现CNS外转移后1至39个月死于疾病（中位时间9个月），而5例在CNS外转移后16至120个月存活（中位时间45个月）。在20例颅内标本中有8例（40%）检测到中度或重度间变，在6例CNS外转移中有4例（66%）检测到；这些频率高于总体髓母细胞瘤的观察频率。6例患者既有初次切除组织，又有随后复发或转移的组织。这6例中有4例在复发或转移时间变分级更高，与肿瘤进展一致。
髓母细胞瘤转移至CNS外可发生在长期临床缓解后，且在某些情况下与间变有关。髓母细胞瘤在复发或转移时可显示组织学进展。
