Primary Melanoblastosis of the Leptomeninges and Brain

Farnell, Frederic J.; Globus, Joseph H.

doi:10.1001/archneurpsyc.1931.02230040137006

Cited by 43 publications

(7 citation statements)

References 4 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Baader (1935) found such cells most prominently in the sulcus between pons and medulla, over the cerebral peduncles and over the base of the brain. Farnell and Globus (1931) have detected such cells in the meninges of children and infants and have also found them in a 5-month foetus. There seems little doubt that these cells are the source ofmelanosis of the meninges and of any supervening tumours (Willis, 1960).…”

Section: Discausionmentioning

confidence: 86%

“…The dura has been normal in all cases. Macroscopic pigmentation of the cerebral cortex has been seen in only 1 case (Wilcox, 1939), but in 7 cases (Berblinger, 1915: Farnell andGlobus, 1931;Lecouturier, Ley, Titeca, and van Bogaert, 1939;Maclachlan, 1914;Grahl, 1906;Russell, 1949;Jiitte, 1939) there was macroscopic pigmentation of the cerebellum and pons. The cerebellar pigmentation was in all cases most marked in the cortex, but striking pigmentation of the dentate nucleus on one or both sides was noted in 3 cases (Berblinger, 1915;Maclachlan, 1914: Lecouturier et al, 1939.…”

Section: Discausionmentioning

confidence: 99%

“…The malignant change may be diffuse or localized, and the criteria by which malignancy has been assessed vary. Thus infiltration of melanoblasts into the cerebral tissue has been taken to indicate malignant change in 7 cases (Wilcox, 1939;Grahl, 1906;Russell, 1949: Farnell andGlobus, 1931;Berblinger, 1915;Lecouturier et al, 1939;Lua, 1914), while in one (Schlesinger and Schopper, 1912) there was infiltration of the optic chiasma. Evidence of cerebral infiltration was also seen in our Case 1 and has been taken together with cellular irregularity and mitotic activity as clear evidence of malignant change.…”

Section: Discausionmentioning

confidence: 99%

See 2 more Smart Citations

Neuro-cutaneous Melanosis

Fox¹,

Emery²,

Goodbody³

et al. 1964

Archives of Disease in Childhood

View full text Add to dashboard Cite

Combined congenital abnormalities of the skin and central nervous system are well recognized-such conditions as Sturge-Weber syndrome, tuberous sclerosis, and neurofibromatosis. A very much rarer and less well-known member of this group is neurocutaneous melanosis. posterior fossa decompression was performed, and it was noted at operation that the meninges overlying the cerebellum were thickened and pigmented. The underlying cerebellar cortex also appeared to be pigmented. After operation the child renained well for eight weeks, but it was then noted that he was ataxic and though he was talking well he was emitting a shrill high-pitched cry at frequent intervals. The posterior fossa decompression was tense and bulging, and a Pudenz valve was therefore inserted into the right lateral ventricle draining into the right internal jugular vein. The child then remained fairly well, apart from some degree of ataxia, for a further period of four months, when he was readmitted with increasing restlessness, ataxia, and rigidity. The posterior fossa decompression was again tense and bulging, and at operation the Pudenz valve was found to be blocked by a protein coagulum, and it was replaced. Three days after the operation the child's conscious level began to decline:he developed left-sided Jacksonian epileptiform attacks and, despite repeated ventricular taps, died at 25 months.NECROPSY. There were no abnormal pathological findings outside of the skin and the central nervous system. In particular, no evidence of tumour was found in any other organ. The skin showed numerous large pigmented hairy naevi on the skin of the limbs, chest, trunk, back, and scalp. These varied in size, but ranged up to a maximum diameter of 6 cm.The dura appeared normal and showed no evidence of pigmentation, but the pia-arachnoid showed a diffusely. grey coloration most marked over the right cerebral hemisphere. In addition there were numerous scattered FiG.

show abstract

Section: Discausionmentioning

confidence: 86%

Section: Discausionmentioning

confidence: 99%

Section: Discausionmentioning

confidence: 99%

See 1 more Smart Citation

Neuro-cutaneous Melanosis

Fox¹,

Emery²,

Goodbody³

et al. 1964

Archives of Disease in Childhood

View full text Add to dashboard Cite

show abstract

“…An association of neurocutaneous melanosis with the Dandy–Walker malformation, as in our patient, is unusual. The oldest reference pertaining to this association dates from 1931 (13) and at least nine more cases have been reported since then. Their features are shown in Table 1. Ours is the second‐oldest patient reported to have this association.…”

Section: Discussionmentioning

confidence: 99%

Neurocutaneous Melanosis in Association with the Dandy–Walker Complex, Complicated by Melanoma: Report of a Case and Literature Review

Mena-Cedillos

Valencia

Arroyo-Pineda³

et al. 2002

Pediatric Dermatology

View full text Add to dashboard Cite

Neurocutaneous melanosis is a rare congenital neurocutaneous syndrome in which benign and malignant melanocytic tumors of the leptomeninges with large or numerous congenital melanocytic nevi develop. The Dandy-Walker malformation occurs as a broad posterior fossa with high insertion of the tentorium, hypoplasia or aplasia of the cerebellar vermis, and cystic dilation of the fourth ventricle communicating with the posterior fossa. Association of these entities is very unusual and only 10 previous reports were found in the literature. Our patient had multiple, medium-size to small melanocytic nevi present since birth. At 5 years of age the patient has intracranial pressure secondary to hydrocephalus. A diagnosis of Dandy-Walker malformation and suspected neurocutaneous melanosis was established after a skull computed tomography (CT) scan. Three months later the patient developed a right frontal tumor shown on the CT scan. The histologic finding was nevomelanocytic infiltration with strong pleomorphism. The tumor grew rapidly, producing neurogenic shock and death. The postmortem report indicated malignant melanoma.

show abstract

“…The Dandy-Walker malformation is considered one of several related developmental cystic and cystlike anomalies of the posterior fossa that include the Dandy-Walker variant and the megacistema magna; collectively, this continuum is called the Dandy-Walker complex (DWC) (12). Posterior fossa cysts and cystlike malformations were reported in at least four patients with NCM (13)(14)(15)(16). A Dandy-Walker malformation was reported in one other patient with a giant congenital nevus, mental retardation, and seizures (17).…”

Section: Discussionmentioning

confidence: 99%

Neurocutaneous Melanosis in Association with the Dandy‐Walker Complex

Kadonaga

Barkovich²,

Edwards³

et al. 1992

Pediatric Dermatology

103

View full text Add to dashboard Cite

An infant had a giant congenital nevus, neurocutaneous melanosis (NCM), and a Dandy-Walker malformation of the brain. The diagnosis of NCM was suspected at 6 weeks of age when macrocephaly was noted, resulting in the discovery of hydrocephalus and a Dandy-Walker malformation. Serial magnetic resonance imaging scans demonstrated so-called T1 shortening in the pia or subarachnoid spaces surrounding the cerebellar vermis and in the temporal lobes anterior to the temporal horns. Eventually, a biopsy-proved melanoma developed in the anterior temporal lobe, in an area previously noted to have T1 shortening. Since meningeal cells have been shown experimentally to play a critical role in cerebellar development, we hypothesize that the association of NCM with a Dandy-Walker malformation may be due to meningeal melanosis disrupting the normal development of the cerebellum and fourth ventricle.

show abstract

Primary Melanoblastosis of the Leptomeninges and Brain

Cited by 43 publications

References 4 publications

Neuro-cutaneous Melanosis

Neuro-cutaneous Melanosis

Neurocutaneous Melanosis in Association with the Dandy–Walker Complex, Complicated by Melanoma: Report of a Case and Literature Review

Neurocutaneous Melanosis in Association with the Dandy‐Walker Complex

Contact Info

Product

Resources

About