Silent or minute hemorrhages do probably occur even in congenital supratentorial hemangioblastoma cases

Abstract

I read the article by Kim et al with great interest [1].Supratentorial occurrence of hemangioblastoma is anexceedingly rare event. Over almost a century since thefirst report of such a case by Bielschowsky in 1902, atotal of 112 cases appeared in the medical literature [2].Even rarer is the occurrence of a supratentorialhemangioblastoma in infancy. Kim et al cited only twocases from English literature [3, 4] and an additionalcase from Korean neurosurgical literature.I felt obliged to contribute to their data because Irecently had experience with such a case and during anextensive literature search I came to realize that firstinfantile supratentorial hemangioblastoma case waspublished as early as 1948. This is an exceptional casesince there is no record of this citation anywhere in thepediatric neurosurgery literature. In 1948, WallaceLeibner of Brooklyn, NY published a series of six in-fants under the 2 years of age in whom brain tumorswere found at operation or at autopsy [5]. One of thesecases was a 22 months-old-baby-boy who presentedwith left hemiparesis. Positive diagnostic findings werepapiledema, separated sutures on plain x-rays andEEG abnormality from the centro-parietal right cere-bral hemisphere. Surgery was performed on February7, 1947 by Dr Max Tarlov. Surgeon’s description ofoperative findings were as follows(... ‘‘the bone in thisarea was paper thin. A needle introduced to a depth ofabout 0.5 cm from the surface yielded yellow fluid.Following an incision into the brain, a large cysticcavity was disclosed. It contained 60 cc of yellow fluidwhich coagulated spontaneously. Along the anteriorwall of the cavity was a brown, slightly nodular andvery vascular tumor which was extremely tough. Itmeasured about 3–4 cm in diameter. Elsewhere thecavity was lined by slightly yellowish stained tissue thatappeared to be brain. As far could be determined, theentire tumor was removed’’. The child tolerated theprocedure well. When last seen about 8 months afterthe surgery, the boy was cheerful and playful and usedall extremities equally well. I want to emphasize thefact that the surgeon noted xanthochromic cyst fluidwith high protein representing previous or organizedhemorrhage.The case reported by Johnson et al in 2004 was a4 weeks-old-baby-boy who presented with acute onsetof projectile emesis, lethargy and irritability and lefthemiparesis [4]. MR imaging of the brain demon-strated multilobular cysts with enhancing mural nod-ules, displacing the right temporal, parietal and theoccipital lobes. At surgery the multiloculated cystsagain contained xanthochromic fluid under pressure.Several pink-brown tissue masses were identified uponcyst fenestration and tumor biopsy.My patient (e-published in March 2006) was an18 month-old-girl with increasing head size, an openanterior fontanelle and irritability [6]. Although late inreaching her milestones the child was able to stand andbabble at around 15 months of age. Fundoscopy re-vealed elevation of the optic disc. Plain x-rays revealed‘‘beaten copper sign’’. MR scans demonstratedtriventricular hydrocephalus and a cystic mass withinthe left lateral ventricle. There was a 18 · 15 · 13 mmenhancing mural nodule along the medial aspect of thecyst somewhere over the left thalamus. The cystic masswas mainly supratentorial but there was an infraten-