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Child's Nervous System
Published in: Child's Nervous System 12/2009

01-12-2009 | Review Paper

Neurocutaneous melanosis and the Dandy–Walker complex: an uncommon but not so insignificant association

Authors: Dominique Marnet, Matthieu Vinchon, Keyvan Mostofi, Benoit Catteau, Olivier Kerdraon, Patrick Dhellemmes

Published in: Child's Nervous System | Issue 12/2009

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Abstract

Background

Neurocutaneous melanosis represents a rare congenital but nonheritable phakomatosis defined as the association of giant or multiple congenital nonmalignant melanocytic nevi with leptomeningeal melanosis or melanoma of the central nervous system.

Methods

We describe the case of an adolescent with a giant congenital bathing trunk melanocytic nevus who developed progressive intracranial hypertension due to leptomeningeal melanosis confirmed by surgical biopsy. Brain and spine magnetic resonance images showed posterior fossa malformation compatible with the Dandy–Walker complex, hydrocephalus, and extensive enhancement of posterior fossa then spine. Shunt placement, corticotherapy, and chemotherapy were attempted leading to transient relief but the boy died 12 months after the onset of primary neurological symptoms.

Discussion

We discuss diagnosis, pathogenesis, management, and prognosis in the light of data from the recent literature.

Conclusion

Neurocutaneous melanosis is considered to follow from neurulation disorders which could account for associated developmental malformations as the so-called Dandy–Walker complex. Cutaneous lesions are usually recognized at birth whereas neurological manifestations develop later. Numerous neurological symptoms have been reported according to extent of leptomeningeal and parenchymal infiltration. Whether magnetic resonance imaging of the neuroaxis represents the choice radiological exam, definite diagnosis relies upon the histological data obtained by mean of biopsy. Once symptomatic, surgical and medical measures remain palliative since death occurs within 3 years.
Literature
1.
Agero AL, Benvenuto-Andrade C, Dusza SW, Halpern AC, Marghoob AA (2005) Asymptomatic neurocutaneous melanocytosis in patients with large congenital melanocytic nevi: a study of cases from an Internet-based registry. J Am Acad Dermatol 53(6):959–965CrossRefPubMed
2.
Ahmed I, Tope WD, Young TL, Miller DM, Bloom KE (2002) Neurocutaneous melanosis in association with encephalocraniocutaneous lipomatosis. J Am Acad Dermatol 47(2 Suppl):S196–S200CrossRefPubMed
3.
Ahuja SR, Karande S, Kulkarni MV (2003) Multiple giant congenital melanocytic nevi with central nervous system melanosis. Neurol India 51(4):541–543PubMed
4.
Asano T, Hamada H, Takita Y, Watanabe M, Sugano H, Sudoh M et al (2007) Neurocutaneous melanosis with acute disseminated encephalomyelitis. Eur J Pediatr 166(10):1065–1066CrossRefPubMed
5.
Azzoni A, Argentieri R, Raja M (2001) Neurocutaneous melanosis and psychosis: a case report. Psychiatry Clin Neurosci 55(2):93–95CrossRefPubMed
6.
Barkovich AJ, Frieden IJ, Williams ML (1994) MR of neurocutaneous melanosis. AJNR Am J Neuroradiol 15(5):859–867PubMed
7.
Barkovich AJ, Kjos BO, Norman D, Edwards MS (1989) Revised classification of posterior fossa cysts and cystlike malformations based on the results of multiplanar MR imaging. AJR Am J Roentgenol 153(6):1289–1300PubMed
8.
Bett BJ (2005) Large or multiple congenital melanocytic nevi: occurrence of cutaneous melanoma in 1008 persons. J Am Acad Dermatol 52(5):793–797CrossRefPubMed
9.
Bittencourt FV, Marghoob AA, Kopf AW, Koenig KL, Bart RS (2000) Large congenital melanocytic nevi and the risk for development of malignant melanoma and neurocutaneous melanocytosis. Pediatrics 106(4):736–741CrossRefPubMed
10.
Burstein F, Seier H, Hudgins PA, Zapiach L (2005) Neurocutaneous melanosis. J Craniofac Surg 16(5):874–876CrossRefPubMed
11.
Byrd SE, Reyes-Mugica M, Darling CF, Chou P, Tomita T (1995) MR of leptomeningeal melanosis in children. Eur J Radiol 20(2):93–99CrossRefPubMed
12.
Caceres A, Trejos H (2006) Neurocutaneous melanosis with associated Dandy–Walker complex. Childs Nerv Syst 22(1):67–72CrossRefPubMed
13.
Chaloupka JC, Wolf RJ, Varma PK (1996) Neurocutaneous melanosis with the Dandy–Walker malformation: a possible rare pathoetiologic association. Neuroradiology 38(5):486–489CrossRefPubMed
14.
Chu WC, Lee V, Chan YL, Shing MM, Chik KW, Li CK, Ma KC (2003) Neurocutaneous melanomatosis with a rapidly deteriorating course. AJNR Am J Neuroradiol 24(2):287–290PubMed
15.
D'Argenio A, David P, Engohan C, Hennequin Y, Balériaux D, Jissendi P (2007) Neurocutaneous melanosis in a newborn with giant congenital melanocytic nevus. J Neuroradiol 34(4):272–275PubMed
16.
De Andrade DO, Dravet C, Raybaud C, Broglin D, Laguitton V, Girard N (2004) An unusual case of neurocutaneous melanosis. Epileptic Disord 6(3):145–152PubMed
17.
Demirici A, Kawamura Y, Sze G, Duncan C (1995) MR of parenchymal neurocutaneous melanosis. AJNR Am J Neuroradiol 16(3):603–606
18.
Di Rocco F, Sabatino G, Koutzoglou M, Battaglia D, Caldarelli M, Tamburrini G (2004) Neurocutaneous melanosis. Childs Nerv Syst 20(1):23–28CrossRefPubMed
19.
Dupin E, Calloni G, Real C, Gonçalves-Trentin A, Le Douarin NM (2007) Neural crest progenitors and stem cells. C R Biol 330(6–7):521–529CrossRefPubMed
20.
Faillace WJ, Okawara SH, McDonald JV (1984) Neurocutaneous melanosis with extensive intracerebral and spinal cord involvement. Report of two cases. J Neurosurg 61(4):782–785CrossRefPubMed
21.
Foster RD, Williams ML, Barkovich AJ, Hoffman WY, Mathes SJ, Frieden IJ (2001) Giant congenital melanocytic nevi: the significance of neurocutaneous melanosis in neurologically asymptomatic children. Plast Reconstr Surg 107(4):933–941CrossRefPubMed
22.
Fox H (1972) Neurocutaneous melanosis. In: Vinken PJ, Bruyn GW (eds) Handbook of clinical neurology. Elsevier, New York, pp 414–428
23.
Hale EK, Stein J, Ben-Porat L, Panageas KS, Eichenbaum MS, Marghoob AA et al (2005) Association of melanoma and neurocutaneous melanocytosis with large congenital melanocytic naevi—results from the NYU-LCMN registry. Br J Dermatol 152(3):512–517CrossRefPubMed
24.
Hartmann D, Ziegenhagen MW, Sievers J (1998) Meningeal cells stimulate neuronal migration and the formation of radial glial fascicles from the cerebellar external granular layer. Neurosci Lett 244(3):129–132CrossRefPubMed
25.
Hayashi M, Maeda M, Maji T, Matsubara T, Tsukahara H, Takeda K (2004) Diffuse leptomeningeal hyperintensity on fluid-attenuated inversion recovery MR images in neurocutaneous melanosis. AJNR Am J Neuroradiol 25(1):138–141PubMed
26.
Humes RA, Roskamp J, Eisenbrey AB (1984) Melanosis and hydrocephalus. Report of four cases. J Neurosurg 61(2):365–368CrossRefPubMed
27.
Kadonaga JN, Barkovich AJ, Edwards MS, Frieden IJ (1992) Neurocutaneous melanosis in association with the Dandy–Walker complex. Pediatr Dermatol 9(1):37–43CrossRefPubMed
28.
Kadonaga JN, Frieden IJ (1991) Neurocutaneous melanosis: definition and review of the literature. J Am Acad Dermatol 24(5 Pt 1):747–755CrossRefPubMed
29.
Kalayci M, Cağavi F, Bayar U, Gül S, Dursun A, Ermis B et al (2006) Neurocutaneous melanosis associated with Dandy–Walker malformation. Acta Neurochir (Wien) 148(10):1103–1106CrossRef
30.
Khera S, Sarkar R, Jain RK, Saxena AK (2005) Neurocutaneous melanosis: an atypical presentation. J Dermatol 32(7):602–605PubMed
31.
Kiecker F, Hofmann MA, Audring H, Brenner A, Labitzke C, Sterry W, Trefzer U (2007) Large primary meningeal melanoma in an adult patient with neurocutaneous melanosis. Clin Neurol Neurosurg 109(5):448–451CrossRefPubMed
32.
Kim YJ, Won YD, Kim KT, Chang ED, Huh PW (2006) Parenchymal neurocutaneous melanosis in association with intraventricular dermoid and Dandy–Walker variant: a case report. Korean J Radiol 7(2):145–148CrossRefPubMed
33.
Krengel S, Hauschild A, Schäfer T (2006) Melanoma risk in congenital melanocytic naevi: a systematic review. Br J Dermatol 155(1):1–8CrossRefPubMed
34.
35.
Marghoob AA, Dusza S, Oliveria S, Halpern AC (2004) Number of satellite nevi as a correlate for neurocutaneous melanocytosis in patients with large congenital melanocytic nevi. Arch Dermatol 140(2):171–175CrossRefPubMed
36.
Mateos González ME, Nieto del Rincón N, Torres Valdivieso MJ, López-Laso E, López Pérez J, Simón de las Heras R et al (2000) Neurocutaneous melanosis. Case report and review of the literature. An Esp Pediatr 52(6):573–576PubMed
37.
Mena-Cedillos CA, Valencia-Herrera AM, Arroyo-Pineda AI, Salgado-Jiménez MA, Espinoza-Montero R, Martínez-Avalos AB et al (2002) Neurocutaneous melanosis in association with the Dandy–Walker complex, complicated by melanoma: report of a case and literature review. Pediatr Dermatol 19(3):237–242CrossRefPubMed
38.
Patel S, Barkovich AJ (2002) Analysis and classification of cerebellar malformations. AJNR Am J Neuroradiol 23(7):1074–1087PubMed
39.
Peretti-Viton P, Gorincour G, Feuillet L, Lambot K, Brunel H, Raybaud C et al (2002) Neurocutaneous melanosis: radiological–pathological correlation. Eur Radiol 12(6):1349–1353CrossRefPubMed
40.
Peters R, Jansen G, Engelbrecht V (2000) Neurocutaneous melanosis with hydrocephalus, intraspinal arachnoid collections and syringomyelia: case report and literature review. Pediatr Radiol 30(4):284–288CrossRefPubMed
41.
Rhodes RE, Friedman HS, Hatten HP Jr, Hockenberger B, Oakes WJ, Tomita T (1991) Contrast-enhanced MR imaging of neurocutaneous melanosis. AJNR Am J Neuroradiol 12(2):380–382PubMed
42.
von Rokitansky K (1861) Ein ausgezeichneter Fall von Pigment-Mal mit. ausgebreiteter Pigmentierung der inneren Hirn-und Rückenmarkshäute. Allg Wien Med Z 6:113
43.
Schaffer JV, McNiff JM, Bolognia JL (2001) Cerebral mass due to neurocutaneous melanosis: eight years later. Pediatr Dermatol 18(5):369–377CrossRefPubMed
44.
Shinno K, Nagahiro S, Uno M, Kannuki S, Nakaiso M, Sano N, Horiguchi H (2003) Neurocutaneous melanosis associated with malignant leptomeningeal melanoma in an adult: clinical significance of 5-S-cysteinyldopa in the cerebrospinal fluid-case report. Neurol Med Chir (Tokyo) 43(12):619–625CrossRef
45.
Takayama H, Nagashima Y, Hara M, Takagi H, Mori M, Merlino G et al (2001) Immunohistochemical detection of the c-met proto-oncogene product in the congenital melanocytic nevus of an infant with neurocutaneous melanosis. J Am Acad Dermatol 44(3):538–540CrossRefPubMed
46.
Van Bogaert L (1948) La mélanose neurocutanée diffuse hérédofamiliale. Bull Acad R Med Belg 3:397–427
47.
Vanzieleghem BD, Lemmerling MM, Van Coster RN (1999) Neurocutaneous melanosis presenting with intracranial amelanotic melanoma. AJNR Am J Neuroradiol 20(3):457–460PubMed
48.
Zhang W, Miao J, Li Q, Liu R, Li Z (2008) Neurocutaneous melanosis in an adult patient with diffuse leptomeningeal melanosis and a rapidly deteriorating course: case report and review of the literature. Clin Neurol Neurosurg 110(6):609–613CrossRefPubMed
Metadata
Title
Neurocutaneous melanosis and the Dandy–Walker complex: an uncommon but not so insignificant association
Authors
Dominique Marnet
Matthieu Vinchon
Keyvan Mostofi
Benoit Catteau
Olivier Kerdraon
Patrick Dhellemmes
Publication date
01-12-2009
Publisher
Springer-Verlag
Published in
Child's Nervous System / Issue 12/2009
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-009-0976-6

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