Chariot P, Monnet I, Gaulard P, Abd-Alsamad I, Ruffié P, De Cremoux H
Department of Pathology, Hôpital Henri Mondor, Créteil, France.
Hum Pathol. 1993 Jan;24(1):111-2. doi: 10.1016/0046-8177(93)90071-n.
A 24-year-old man had a mediastinal embryonal carcinoma containing yolk sac foci. Combination chemotherapy with cisplatin, bleomycin, etoposide, and vinblastine was given, and the residual mass was then resected. Histology showed only necrotic cells. No other treatment was given. Two years later the patient presented with episodes of flushing and syncopes related to a systemicmastocytosis. Bone marrow examination showed a diffuse infiltration with large, atypical mast cells often with multilobulated nuclei. The patient suffered several episodes of cardiovascular collapse and died during one of these episodes, 8 months after the diagnosis of systemic mastocytosis and 40 months after the diagnosis of mediastinal tumor. Autopsy findings included the absence of mediastinal tumor and a diffuse liver and spleen mast cell infiltration. This was the second case with the similar clinicopathologic picture of two rare diseases being associated. This fact supports the hypothesis of a distinct entity, part of the mediastinal germ cell tumor/hematologic malignancy syndrome. The hypothesis of a cytokine secretion induced by mediastinal germ cell tumor supporting mast cell proliferation may be considered.
一名24岁男性患有纵隔胚胎癌，其中包含卵黄囊灶。给予顺铂、博来霉素、依托泊苷和长春碱联合化疗，随后切除残留肿块。组织学检查仅显示坏死细胞。未给予其他治疗。两年后，患者出现与系统性肥大细胞增多症相关的潮红和晕厥发作。骨髓检查显示有大量非典型肥大细胞弥漫浸润，这些肥大细胞的细胞核常呈多叶状。患者经历了几次心血管衰竭发作，并在其中一次发作中死亡，此时距系统性肥大细胞增多症诊断8个月，距纵隔肿瘤诊断40个月。尸检结果包括纵隔肿瘤消失以及肝脏和脾脏弥漫性肥大细胞浸润。这是第二例两种罕见疾病具有相似临床病理表现相关联的病例。这一事实支持了一种独特实体的假说，即纵隔生殖细胞肿瘤/血液系统恶性肿瘤综合征的一部分。可以考虑纵隔生殖细胞肿瘤诱导细胞因子分泌支持肥大细胞增殖的假说。
