Summary
Three cases of tuberous sclerosis in neonates were found to have focal, frequently perivascular, collections of large cells with abundant eosinophilic cytoplasm. These cells resembled those found in brain lesions of tuberous sclerosis but did not stain for acidic protein. Ultrastructurally, they were characterized by many membrane bound cytoplasmic bodies, 90 to 270 nm in diameter, with amorphous contents. Filaments were not demonstrated. Their appearance is considered most consistent with histiocytic origin.
Large cells with a histiocytic appearance and a superficial resemblance to those seen in the brain in tuberous sclerosis, but a different ultrastructure and reaction to GFAP staining, may be found in the spleen of neonates with this disease.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Arseni C, Alexianu M, Horvat L, Alexianu D, Petrovici A (1972) Fine structure of atypical cells in tuberous sclerosis. Acta Neuropathol (Berl) 21:185–193
Bender BL, Yunis EJ (1980) The central nervous system pathology of tuberous sclerosis in children. Ultrastruct Pathol 1:287–299
Bhawan J, Edelstein L (1977) Angiofibromas in tuberous sclerosis: A light and electron microscopic study. J Cutan Pathol 4:300–307
Darden JW, Teeslink R. Parrish RA (1975) Hamartoma of the spleen: A manifestation of tuberous sclerosis. Am J Surg 41:564–566
de Chadarévian J, Hollenberg RD (1979) Subependymal giant cell tumor of tuberose sclerosis. J Neuropathol Exp Neurol 38:419–434
Donegani G, Grattarola FR, Wildi E (1972) Tuberous sclerosis: Bourneville disease. In: PJ Vinken, GW Bruyn (eds) Handbook of clinical neurology, the phakomatoses, vol 14. American Elsevier, New York, pp 340–389
Eng LF, Rubinstein LJ (1978) Contribution of immunohistochemistry to diagnostic problems of human cerebral tumors. J Histochem Cytochem 26:513–522
Inglis K (1954) The relation of the renal lesions to the cerebral lesions in the tuberous sclerosis complex. Am J Pathol 30:739–755
Morales JB (1961) Congenital rhabdomyoma, tuberous sclerosis, and splenic histiocytosis. Arch Pathol 71:485–493
Mori M, Ikeda T, Onoe T (1971) Blastic Schwann cells in renal tumor of tuberous sclerosis complex. Acta Pathol Jap 21:121–129
Östör AG, Fortune DW (1978) Tuberous sclerosis initially seen as hydrops fetalis: Report of a case and review of the literature. Arch Pathol Lab Med 102:34–39
Ribadeau Dumas JL, Poirer J, Escourolle R (1973) Etude ultrastructurale des lésions cérébrales de la sclérose tubéreuse de Bourneville. Acta Neuropathol (Berl) 25:259–270
Tsakraklides V, Burke B, Mastri A, Runge W, Roe E, Anderson R (1974) Rhabdomyomas of heart: A report of four cases. Am J Dis Child 128:639–646
Van Heerden JA, Longo MF, Cardoza F, Farrow GM (1967) The abdominal mass in the patient with tuberous sclerosis. Arch Surg 95:317–319
Velasco ME, Dahl D, Roessman U, Gambetti P (1980) Immunohistochemical localization of glial fibrillary acidic protein in human glial neoplasms. Cancer 45:484–494
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Bender, B.L., Yunis, E.J. Splenic involvement in tuberous sclerosis. Virchows Arch. A Path. Anat. and Histol. 391, 363–369 (1981). https://doi.org/10.1007/BF00709169
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00709169