Schwannomatosis: report of a new case

Schwannomatosis presentación de un nuevo caso

Schwannomatosis presentación de un nuevo caso

Schwannomatosis: report of a new case

Nélida S. Garretto^I; David Monteverde^I; Héctor Giócoli^II; Blanca I. Ravera^I; Hugo A. Molina^III; Ana Perurena Garayalde^IV; Antonia Ruiz León^IV; Roberto E. P. Sica^I

^IDivisión Neurologia. Universidad de Buenos Aires, Hospital José María Ramos Mejia (HRM)

^IINeurocirugía. Universidad de Buenos Aires, Hospital José María Ramos Mejia (HRM)

^IIIPatología. Universidad de Buenos Aires, Hospital José María Ramos Mejia (HRM)

^IVDermatología. Universidad de Buenos Aires, Hospital José María Ramos Mejia (HRM)

RESUMEN

Se describe un paciente de 52 años de edad que presentó 5 tumoraciones subcutaneas adheridas a diferentes nervios periféricos. La anatomía patológica de todas ellas, indicó que se trataba de schwannomas. Una TAC de cerebro demostró una tumoración en lóbulo temporal izquierdo con calcificaciones en su interior, compatible a meningioma. La ausencia de criterios diagnósticos para neurofibromatosis I y II y la característica particular del cuadro, schwannomas múltiples asociados a tumoración endocraneana, sugieren el diagnós tico de schwannomatosis.

Palavras-chave: schwannomatosis, neurinomas, facomatosis.

SUMMARY

Schwannomatosis is a rare disorder, still not quite well defined, seldom described in the literature. In this paper we report the case of male. Patient, 52 years old, who in the last 30 years developed five subcutaneous tumors within his limbs peripheral nerves, which histologically proved to be schwannomas. A brain computed tomography showed a partially calcified tumor in the left temporal lobe which most likely was a meningioma. A thorough clinical examination was unable to find signs of type I or type II neurofibromatosis. The present condition, probably a form of phacomatosis, has to be distinguished from neurofibromatosis and is considered as an independent clinical entity whose origin still awaits further detailed investigations.

Key words: schwannomatosis, neurinomas, phacomatosis.

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Dra. Nélida Susana Garretto — División Neurología, Hospital J. M. Ramos Mejía - Urquiza 609 - 1221 Buenos Aires - Argentina.

• 1. Barker D, Wright E, Nguyen K, Cannon L, Fain P, Goldgar D, Bishop DT, Carey J, Baty B, Kivlin J, Willard H, Waye JS, Greig G, Leinwand L, Nakamura Y, O'Connel P, Leppert M, Lalouel JM, White R, Skolnick M. Gene for Von Recklinghausen neurofibromatosis is in the pericentromeric region of the chromosome 17. Science 1987, 236:1100-1102.
• 2. Garretto NS, Ameriso S, Molina HA, Arberas C, Salvat J, Monteverde D, Sica REP. Type 2 neurofibromatosis with Lisch nodules. Neurofibromatosis 1989, 2:315-321.
• 3. Martuza RL, Eldridge R. Neurofibromatosis 2 (Bilateral acoustic neurofibromatosis). N Engl J Med 1988, 318:684-688.
• 4. Neurofibromatosis Conference Statement. National Institutes of Health Consensus Development Conference. Arch Neurol 1988, 45:575-578.
• 5. Purcell MSM, Dixon MSL. Schwannomatosis: an unusual variant of neurofibromatosis or a distinct clinical entity? Arch Dermatol 1989, 125:390-393.
• 6. Riccardi VM. Neurofibromatosis update. Neurofibromatosis 1989, 2:284-291.
• 7. Rongioletti F, Drago F, Rebora A. Multiple cutaneous plexiform schwannomas whith tumors of the central nervous system. Arch Dermatol 1989, 125:431-432.
• 8. Seizinger BR, Rouleau G, Ozelius LJ, Lane AH, St George Hyslop P, Huson S, Gusella JF, Martuzza RL. Common pathogenetic mechanism for three tumor types in bilateral acoustic neurofibromatosis. Science 1987, 236:317-319.
• 9. Shishiba T, Niimura M, Ohtsuka F, Tsuru N. Multiple cutaneous neurilemmomas as a skin manifestation of neurilemmomatosis. J Am Acad Dermatol 1984, 10:744-754.

Publication Dates

• Publication in this collection
  22 Feb 2011
• Date of issue
  Dec 1992