Clear cell sarcoma of the kidney in a newborn

✍ Scribed by Mazzoleni, Stefano ;Vecchiato, Luca ;Alaggio, Rita ;Cecchetto, Giovanni ;Zorzi, Carlo ;Carli, Modesto

• Publisher: John Wiley and Sons
• Year: 2003
• Tongue: English
• Weight: 176 KB
• Volume: 41
• Category: Article
• ISSN: 0098-1532
• DOI: 10.1002/mpo.10318

✦ Synopsis

This remains speculative since we did not have the opportunity to measure his melatonin levels.

To date, scoliosis has not been listed as one of the common sequelae of LCH. Experimental and clinical evidence indicates that the pineal gland may impact the development of idiopathic scoliosis [8,11,12]. In animal experiments, such as those in chickens, removal of the pineal gland induced a scoliosis, which was prevented by adequate doses of injected melatonin [8]. However, assay results of actual serum levels of melatonin in adolescents with idiopathic scoliosis were contradictory [11,12]. Regarding the effect of melatonin on the cardiovascular system, the presence of vascular melatoninergic receptors/binding sites was reported functionally linked with vasoconstrictor or vasodilatory effects of melatonin [9]. Along these lines, a link of pineal gland hypoplasia with coronary heart disease and stroke has been hypothesized [13]. In addition, oral melatonin administration to LCH patients with CNS lesions has been proposed. This is because melatonin's neuroprotective effects are expected to benefit CNS-LCH patients who may be at risk for severe neurologic deterioration or death (LCH-CNS2000 protocol for Histiocyte Society, by N. Grois and H. Gadner).

In summary, attentive long-term care of LCH patients with atrophic pineal glands who were heavily treated during childhood must be carried out cautiously. The rare late death and severe scoliosis in this LCH patient might have been related to impaired melatonin production, although the exact cause of death was not established. Future efforts should be made to determine the role of melatonin in LCH.