Desmoplastic infantile ganglioglioma: a potentially malignant tumor?

Department of Pathology, University of Leuven, Belgium.
American Journal of Surgical Pathology (Impact Factor: 4.59). 12/2002; 26(11):1515-22.
Source: PubMed

ABSTRACT Desmoplastic infantile ganglioglioma is a rare intracranial tumor of early childhood with a usually excellent prognosis despite malignant features both radiologically and histologically. We present the case of a desmoplastic infantile ganglioglioma with histologically highly anaplastic features and both intracerebral and pial metastases. After partial resection the tumor was rapidly progressive and new metastases appeared. A combination of vincristine and carboplatinum was used according to the Low Grade Glioma Protocol of the International Society of Pediatric Oncology, with a temporary good response. When histologically characterized by highly anaplastic features, it seems the biologic behavior of this tumor remains uncertain. The aggressive behavior and the responsiveness to chemotherapy in this case may challenge the belief in the benign nature of these rare tumors.


Available from: Raf Sciot, Apr 28, 2014
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Bei der Patientin kam es im 7.Lebensmonat nach bisher normal verlaufener Entwicklung zu einer signifikanten Größenzunahme des Kopfumfangs >97. Perzentile, die mit Entwicklungsrückschritten mit Bewegungsasymmetrie einherging. Bei der stationären Aufnahme wegen zunehmender Teilnahmslosigkeit fand sich in der Bildgebung (Ultraschall, Magnetresonanztomographie zur weiteren Abklärung) eine linkshemisphärische Raumforderung. Der Verdacht auf desmoplastisches infantiles Astrozytom bestätigte sich histologisch nach operativer Entfernung des Tumors. Following hitherto normal development, a 7-month-old baby presented with significant enlargement in head circumference >97th percentile, associated with developmental regression and asymmetry of movement. Following inpatient admission due to increasing unresponsiveness, imaging (ultrasound, and magnetic resonance imaging for further classification) demonstrated a tumor in the left hemisphere. The suspicion of desmoplastic infantile astrocytoma was confirmed histologically following surgical removal of the tumor. SchlüsselwörterKopfumfang-Entwicklungsrückschritte-Teilnahmslosigkeit -Raumforderung-Desmoplastisches infantiles Astrozytom KeywordsHead circumference-Developmental regression-Unresponsiveness-Tumor-Desmoplastic infantile astrocytoma
    Monatsschrift Kinderheilkunde 01/2010; 158(9):830-833. DOI:10.1007/s00112-009-1949-2 · 0.28 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: PURPOSE: Desmoplastic infantile gangliogliomas (DIGs) are rare tumors of infancy. Herein, we describe an unusual case of DIG diagnosed by prenatal ultrasound. METHODS: This 5-day-old newborn was delivered after a prenatal ultrasound revealed a large cystic mass in the left cerebral hemisphere along with an echogenic solid component. RESULTS: The tumor revealed a glial and neuronal proliferation in a background of desmoplasia more typical of DIG and a minor component with a more primitive, immature appearance to the glioneuronal elements. A significant component of the tumor was composed of pleomorphic eosinophilic spindle cells in whorls and interlacing fascicles that showed a strong, sharp, and diffuse positivity for desmin, thus mimicking rhabdomyosarcoma. However, the tumor cells were GFAP (+), INI-1 (+), and myogenin (-). Mitoses were seen both in the more spindle cell astroglial areas as well as the more primitive neuroepithelial cells. The MIB-1 proliferation index was brisk, exceeding 15 %, and in areas it was estimated to be as high as 30 %. Such high proliferation index has been described and accepted in the more primitive neuroepithelial areas, but not in the terminally differentiated, spindle cell astroglial areas as in our case. Our patient was incidentally diagnosed prenatally. To our knowledge, this case is the first documented congenital DIG diagnosed prenatally. CONCLUSIONS: This case highlights the pitfalls in diagnosing DIG, which can mimic a rhabdomyosarcoma. Furthermore, it underscores the importance of re-evaluating the grading of these tumors or at least segregating the variants where the prognosis may be more guarded.
    Child s Nervous System 08/2012; DOI:10.1007/s00381-012-1886-6 · 1.16 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Desmoplastic infantile ganglioglioma (DIG) is a rare supratentorial tumor in the central nervous system. Definitive diagnosis of this neoplasm is based on histopathologic analysis evaluating distinctive findings such as the fibroblastic differentiation. Here we present a clinical case of DIG with a long follow-up in an eight-year-old boy with a six-month history of recurrent emesis, psychomotor hyperactivity and generalized tonic-clonic seizures. Computed tomography scan and magnetic resonance imaging (MRI) showed a cystic, heterogeneous, mass on the right temporal uncus. A histopathological diagnosis of late presentation DIG was made. We documented the immunohistochemical expression of a molecular soft tissue / muscle differentiation marker (h-CaD) in addition to a low proliferative index (Ki-67) in this case. After surgical intervention, a control MRI showed changes of right frontal-temporal craniotomy and a persistent mass in the anterior and medial temporal lobe with basal extension. Further surgical intervention was performed, completely removing the tumor, which had the same characteristics. The patient is asymptomatic while receiving anticonvulsant therapy (phenytoin) with no evidence of tumor recurrence on MRI after a follow-up of five years. The low grade and soft tissue appearance in images are correlated with the histopathologic and immunohistochemical profile of this tumor, but the rarity of this tumor makes a presumptive diagnosis by images a challenge. The above-mentioned molecular markers or new ones could be used as molecular targets for molecular imaging studies to increase the probability of a pre-operative diagnosis based on molecular features through images.
    12/2013; 26(6):649-54.