Abstract
Desmoplastic infantile gangliogliomas (DIG) are rare intracranial tumors that typically occur in infants and involve the cerebral cortex and the leptomeninges. They are usually very large in size and partially cystic. Total resection, if possible, is the treatment of choice, without the need for further adjuvant therapy. We report 2 cases of DIG. In both cases, computed tomography and magnetic resonance imaging revealed supratentorial mixed cystic and solid tumors, which presented as large cystic components with intense contrast enhancement of a mural nodule. The tumors were completely removed by surgery. The histological diagnosis was DIG. On follow-up examinations no tumor recurrence was noted.
References
1.
VandenBerg SF, May EE, Rubinstein LJ, Herman MM, Perentes E, Vinores SA, Collins VP, Park TS: Desmoplastic supratentorial neuroepithelial tumors of infancy with divergent differentiation potential (‘desmoplastic infantile gangliogliomas’). Report on 11 cases of a distinctive embryonal tumor with favorable prognosis. J Neurosurg 1987;66:58–71.
2.
Taratuto AL, Monges J, Lylyk P, Leiguarda R: Superficial cerebral astrocyoma attached to dura. Report of six cases in infants. Cancer 1984;54:2505–2512.
3.
Cerda-Nicolas M, Lopez-Gines C, Gil-Benso R, Donat J, Fernandez-Delgado R, Pellin A, Lopez-Guerrero JA, Roldan P, Barbera J: Desmoplastic infantile ganglioglioma: morphological, immunohistochemical and genetic features. Histopathology 2006;48:617–621.
4.
Kuchelmeister K, Bergmann M, von Wild K, Hochreuther D, Busch G, Gullota F: Desmoplastic ganglioglioma: report of two non-infantile cases. Acta Neuropathol (Berl) 1993;85:199–204.
5.
Lonnrot K, Terho M, Kahara V, Haapasalo H, Helen P: Desmoplastic infantile ganglioglioma: novel aspects in clinical presentation and genetics. Surg Neurol 2007;68:304–308.
6.
Fan X, Larson TC, Jennings MT, Tulipan NB, Ton SA, Johnson MD: December 2000: 6 month old boy with 2 week history of progressive lethargy. Brain Pathol 2001;11:265–266.
7.
Khaddage A, Chambonniere ML, Morrison AL, Allard D, Dumollard JM: Desmoplastic infantile ganglioglioma: a rare tumor with an unusual presentation. Ann Diagn Pathol 2004;8:280–283.
8.
Duffner PK, Burger PC, Cohen ME, Sanford RA, Crischer JP: Desmoplastic infantile gangliogliomas: an approach to therapy. Neurosurgery 1994;34:583–589.
9.
Sperner J, Gottschalk J, Neumann K, Schörner W, Lanksch WR, Scheffner D: Clinical, radiological and histological findings in desmoplastic infantile ganglioglioma. Childs Nerv Syst 1994;10:458–462.
10.
Bächli H, Avoledo P, Gratzl O, Tolnay M: Therapeutic strategies and management of desmoplastic infantile ganglioglioma: two case reports and literature overview. Childs Nerv Syst 2003;19:359–366.
11.
Shao L, Tihan T, Burger PC: Desmoplastic infantile ganglioglioma: a clinical and pathological review of eight cases. J Neuropathol Exp Neurol 2002;61:466.
12.
Khaddage A, Chambonniere ML, Morrison AL, Allard D, Dumollard JM, Pasquier B, Péoc’h M: Desmoplastic infantile ganglioglioma: a rare tumor with an unusual presentation. Ann Diagn Pathol 2004;8:280–283.
13.
Takeshima H, Kawahara Y, Hirano H, Obara S, Niiro M, Kuratsu J: Postoperative regression of desmoplastic infantile gangliogliomas: report of two cases. Neurosurgery 2003;53:979–983.
14.
Darwish B, Arbuckle S, Kellie S, Besser M, Chaseling R: Desmoplastic infantile ganglioglioma/astrocytoma with cerebrospinal metastasis. J Clin Neurosci 2007;14:498–501.
15.
De Munnynck K, Van Gool S, Van Calenbergh F, Demaerel P, Uyttebroeck A, Buyse G, Sciot R: Desmoplastic infantile ganglioglioma: a potentially malignant tumor? Am J Surg Pathol 2002;26:1515–1522.
© 2008 S. Karger AG, Basel
2008
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