Blüher Susann, Schulz Manuela, Bierbach Uta, Meixensberger Jürgen, Tröbs Ralf-Bodo, Hirsch Wolfgang, Schober Ralf, Kiess Wieland, Siekmeyer Werner
Hospital for Children and Adolescents, University of Leipzig, Oststrasse 21-25, 04317, Leipzig, Germany.
Eur J Pediatr. 2008 Apr;167(4):483-5. doi: 10.1007/s00431-007-0507-y. Epub 2007 May 30.
Meningeal tumors are extremely rare in children and are diagnostically as well as therapeutically challenging. Among the least common types of malignancies in childhood is malignant melanoma, counting for less than 1% of pediatric tumors. Due to the rarity and the wide spectrum of appearance, initial clinical features may be misleading. A 3-year-old boy was referred to our hospital with symptoms of hyperventilation, dyspnoea, tachycardia, respiratory alkalosis, inarticulate speech, and fatigue. Measurement of pH in cerebrospinal fluid (CSF) yielded central lactic acidosis despite alkalosis in peripheral blood. Diagnostic imaging procedures as well as histology and immunohistochemistry revealed the diagnosis of a malignant meningeal melanoma. We hypothesize that central lactate production of the tumor nests might have induced central acidification, thus inducing hyperventilation by stimulation of central chemoreceptors. This case is a model example of the key role of central pH as an inducer/suppressor of ventilation in humans and illustrates the critical importance of central pH for regulating both ventilation and acid-base homeostasis. Thus, pH of CSF should be measured whenever a malignant brain tumor is suspected.
脑膜肿瘤在儿童中极为罕见,在诊断和治疗方面都具有挑战性。儿童最不常见的恶性肿瘤类型之一是恶性黑色素瘤,占小儿肿瘤的比例不到1%。由于其罕见性和广泛的表现形式,最初的临床特征可能会产生误导。一名3岁男孩因呼吸急促、呼吸困难、心动过速、呼吸性碱中毒、言语不清和疲劳等症状被转诊至我院。尽管外周血呈碱中毒,但脑脊液(CSF)pH值测量显示存在中枢性乳酸酸中毒。诊断性影像学检查以及组织学和免疫组织化学检查确诊为恶性脑膜黑色素瘤。我们推测肿瘤巢的中枢乳酸生成可能导致中枢酸化,从而通过刺激中枢化学感受器诱导过度通气。该病例是中枢pH作为人体通气诱导剂/抑制剂的关键作用的典型例子,并说明了中枢pH对调节通气和酸碱平衡的至关重要性。因此,每当怀疑患有恶性脑肿瘤时,都应测量脑脊液的pH值。