Sowithayasakul Panjarat, Sinlapamongkolkul Phakatip, Treetipsatit Jitsupa, Vathana Nassawee, Narkbunnam Nattee, Sanpakit Kleebsabai, Buaboonnam Jassada
Department of Pediatrics, Faculty of Medicine, Srinakharinwirot University.
Department of Pediatrics, Faculty of Medicine, Thammasat University, PathumThani, Thailand.
J Pediatr Hematol Oncol. 2018 Aug;40(6):450-455. doi: 10.1097/MPH.0000000000001233.
Mediastinal germ cell tumor (MGCT), which accounts for 1% to 3% of extragonadal germ cell tumors, has unique manifestations; it is associated with several types of hematologic malignancy, particularly myeloid neoplasm. The aim of this study was to report the 10-year incidence, clinical characteristics, and outcomes of MGCT at Thailand's national pediatric tertiary referral center. This retrospective study included patients diagnosed with MGCT at the Department of Pediatrics, Siriraj Hospital during 2005 to 2014. Eight patients (all male) were diagnosed with MGCT. Five of 8 patients were found to have hematologic abnormalities. Three patients were diagnosed with acute myeloid leukemia (AML) (one patient with M1, another having M7, and the other with M0). Another patient had mixed MGCT with mediastinal myeloid sarcoma (MMS). The other patient had malignancy-associated hemophagocytic lymphohistiocytosis syndrome (M-HLH). Isochromosome 12p was detected in 3 patients (AML [2], mixed MGCT/MMS [1]). Four of 5 patients with hematologic abnormalities died of hematologic abnormalities or treatment complication (AML [3], M-HLH [1]). One patient with mixed MGCT/MMS survived with chemotherapy. All patients with AML and MMS were nonseminomatous MGCT and the onset of myeloid malignancies were within 1 year after the diagnosis of MGCT. Associated hematologic malignancies should be suspected in MGCT with abnormal blood count or hematologic symptoms. Isochromosome 12p was the most common cytogenetic finding in MGCT-associated myeloid malignancies patients. Those with nonseminomatous MGCT should have their blood count carefully monitored especially during the first year after the diagnosis of MGCT. Better treatment alternatives for MGCT with associated hematologic malignancies are warranted to ameliorate adverse outcomes.
纵隔生殖细胞肿瘤(MGCT)占性腺外生殖细胞肿瘤的1%至3%,具有独特的表现;它与几种血液系统恶性肿瘤相关,尤其是髓系肿瘤。本研究的目的是报告泰国国家儿科三级转诊中心MGCT的10年发病率、临床特征和结局。这项回顾性研究纳入了2005年至2014年期间在诗里拉吉医院儿科被诊断为MGCT的患者。8名患者(均为男性)被诊断为MGCT。8名患者中有5名被发现有血液学异常。3名患者被诊断为急性髓系白血病(AML)(1例为M1型,另1例为M7型,另1例为M0型)。另1例患者为混合性MGCT伴纵隔髓系肉瘤(MMS)。另1例患者患有恶性肿瘤相关噬血细胞性淋巴组织细胞增生症综合征(M-HLH)。3例患者检测到12号染色体短臂等臂染色体(AML[2例],混合性MGCT/MMS[1例])。5例血液学异常患者中有4例死于血液学异常或治疗并发症(AML[3例],M-HLH[1例])。1例混合性MGCT/MMS患者经化疗存活。所有AML和MMS患者均为非精原细胞瘤性MGCT,髓系恶性肿瘤在MGCT诊断后1年内发病。MGCT患者若血常规异常或有血液学症状,应怀疑有相关血液系统恶性肿瘤。12号染色体短臂等臂染色体是MGCT相关髓系恶性肿瘤患者最常见的细胞遗传学发现。非精原细胞瘤性MGCT患者应仔细监测血常规,尤其是在MGCT诊断后的第一年。对于伴有血液系统恶性肿瘤的MGCT,需要更好的治疗方案来改善不良结局。