de Monléon J V, Simonin G, Pincemaille O, Buttin C, Sarles J
Service de pédiatrie multidisciplinaire, hôpital des enfants de la Timone, Marseille, France.
Arch Pediatr. 1999 Jan;6(1):46-9. doi: 10.1016/s0929-693x(99)80073-6.
hCG secreting tumors are responsible for 21% of precocious puberties in boys. Usual localizations are hepatic, cerebral, mediastinal and gonadic.
A 4-year-old boy developed precocious puberty with rapid evolution. Serum beta hCG suggested germinal etiology, but radiological procedures failed to find any usual localization. Further occurrence of pain in the legs led to carry out a lumbar puncture. The high cerebrospinal fluid/blood gradient of beta hCG suggested the presence of an intramedullar tumor. Medullar magnetic resonance imaging found a large tumor facing L1 and L2.
To our knowledge, this localization is described for only the second time.
分泌人绒毛膜促性腺激素(hCG)的肿瘤是男孩性早熟病例中21%的病因。常见的发病部位是肝脏、脑部、纵隔和性腺。
一名4岁男孩出现快速进展的性早熟。血清β-hCG提示为生殖细胞源性病因,但影像学检查未发现任何常见的发病部位。随后患儿腿部疼痛,遂进行腰椎穿刺。脑脊液与血液中β-hCG的高梯度提示存在髓内肿瘤。脊髓磁共振成像发现一个位于L1和L2椎体水平的大肿瘤。
据我们所知,这种发病部位仅为第二次被描述。