A 10 year-old girl with a temporal cystic mass.

Kar-Ming Fung, M.D., Ph.D. 1 i, Kalliopi Petropoulou M.D. 2

1 Department of Pathology and 2 Department of Radiology, University of Oklahoma, Oklahoma City, OK. Last update: June 30, 2005.


Clinical Information: The patient was a 9 year-old, right-handed white female. She had a history of headache for an unknown period of time and her headache had been worsening recently. She was admitted to the hospital because of slurring of and difficulties with speech for one day accompanied by severe headache. The past medical history was unremarkable. A left temporal cystic lesion was identified on magnetic resonance (MR) imaging and it was surgically removed. The followings are representative MR and histopathologic images.

               Click thumbnails to see regular sized picture.

Imaging:

    There is a mass involving cortex and subcortical white matter in the left middle and superior temporal lobes which comprises a solid component with almost isointense to gray matter on T1-wighted (Figures A and B) and mixed but primarily increased intensity signal on T2-weighted sequences (Figures D). There is a cystic component in the dorsal aspect of the mass. On the post contrast images the solid component demonstrates inhomogeneous enhancement (Figure C). In addition, there is leptomeningeal enhancement along the lateral aspect of the middle and superior left temporal gyri with extension along the left sylvian fissure and left frontal operculum. Medial to the tumor and lateral to the left lateral ventricle there is a large cyst the lateral inferior aspect of which abuts the medial surface of the solid enhancing component of the mass. The cyst itself does not reveal any peripheral enhancement and likely is separate from the tumor.

    Given the location and the appearance of the mass main differential consideration would be ganglioglioma. The leptomeningeal enhancement is an unusual finding in ganglioglioma with the exception of the desmoplastic type.       

Pathology:

    The lesion is a desmoplastic, cellular tumor that has a tendency to involve the leptomeninges and infiltrate along the Vichow-Robin space (Figure E). On higer magnification, islands of cells that are well demarcated from the desmoplastic components are present. Large, dysplastic ganglion cells are often found in these islands (Figure F and G). The desmoplastic changes are best evaluated by Masson's trichrome stain (Figure H and I). A reticulin stain also demonstrated deposition of reticulin substance around individual cells (Figure J). Many of the large ganglionic cells are immunoreactive for synaptophysin (Figure K). Some of the ganglion cells are also positive for neurofilament (Figure L). The glial component is best demonstrated by immunohistochemistry for glial fibrillary acidic protein (GFAP) (Figure M).

Diagnosis: Desmoplastic ganglioglioma.

Discussion:

    Desmoplastic infantile ganglioglioma (DIG) is a histologic grade I tumor as per the year 2000 Classification of the World Health Organization (WHO). Several features, as illustrated in this case, distinguishes it from other primary glial and glial-neuronal tumor. DIGs are uncommon tumors and occur most often in infants and children. Tumors with the very similar macroscopic and histologic features can also occur in older children. It is more appropriate to drop the word "infantile" in the diagnosis of these tumors.

    Due to their large size, the most common presentations include increase in cranial circumference, manifestations of hydrocephalus. They can also cause intractable seizure. Macroscopically, DIG can be very large and are often cystic. Most DIGs occur as superficially located, supratentorial  dural attached, contrast enhancing mass with unilocular or multiloculated cysts. However, the cystic component is not always present. Histologically, they are featured by neoplastic glial and neuronal cells in a desmoplastic background that can be well demonstrated by Masson's trichrome stain. There is also reticulin deposition among individual cells as illustrated in this case. In addition, medulloblastoma-like primitive neuroepithelial cell components may also be present and should not be mistaken as medulloblastoma. DIG also has a tendency to spread along the pia and extend alond the Vichow-Robin space. When no neuronal component and no medulloblastoma-like primitive cell components are present, these tumor can be diagnosed as desmoplastic infantile astrocytoma. The glial and neuronal components can be demonstrated respectively by immunohistochemistry for glial and neuronal markers.

    The desmoplastic component may suggest mesenchymal or sarcoma in intraoperative consultation. The diagnosis, particularly with the assistance of immunohistochemistry, is not that difficult on permanent sections.

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Further reading:

  1. VandenBerg SR, 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. Taratulo AL, VandenBerg SR, Rorke LB. Desmoplastic infantile astrocytoma and ganglioma. World Health Organization Classification of Tumours- Tumours of the Nervous System. Kleihues P and Cavenee WK.  IARC Press, 2000. Page 99-102.

  3. de Chadarévian JP, Pattisapu JV, Faerber EN. Desmoplastic cerebral astrocytoma of infancy. Light microscopy, immunocytochemistry, and ultrastructure. Cancer. 1990; 66:173-9.

  4. Louis DN, von Deimling A, Dickersin GR, Dooling EC, Seizinger BR. Desmoplastic cerebral astrocytomas of infancy: a histopathologic, immunohistochemical, ultrastructural, and molecular genetic study.
    Hum Pathol. 1992; 23:1402-9.


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