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Special Feature |

Pathological Case of the Month FREE

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Section Editor: Enid Gilbert-barness, MD

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Arch Pediatr Adolesc Med. 2001;155(4):518. doi:10.1001/archpedi.155.4.517.
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DIAGNOSIS AND DISCUSSION: CYSTIC DESMOPLASTIC MEDULLOBLASTOMA OF INFANCY

Figure 1. Cystic tumor (6 × 5 × 4.5 cm) in the right cerebellum. The largest cyst measures 3 cm in diameter and is filled with greenish fluid.

Figure 2. Undifferentiated areas composed of cells with small hyperchromatic nuclei and inconspicuous cytoplasm. The cells are arranged in cords and trabeculae (hematoxylin-eosin, original magnification ×280).

Figure 3. Marked desmoplasia with connective tissue encircling single cells or groups of cells (reticulin stain, original magnification ×550).

Figure 4. Scattered ganglion cells in a fibrotic background (hematoxylin-eosin, original magnification ×280).

At autopsy, the brain weighed 955 g. The right cerebellum contained a cystic tumor measuring 6 × 5 cm. Medulloblastomas are dense small cell tumors. They are heterogeneous but as a group are known for small hyperchromatic condensed nuclei and inconspicuous cytoplasm. There are several classifications: undifferentiated, neuroblastic differentiation, glial differentiation, mixed glial neuronal differentiation, and divergent differentiation.1 The desmoplastic variant of cerebellar medulloblastoma2 formerly identified by some workers as an arachnoidal cerebellar sarcoma35 is a superficial well-circumscribed form of medulloblastomas that predominantly occurs in young adults. Desmoplastic medulloblastoma has less nodularity compared with the other counterparts and a higher content of reticulin surrounding single cells or groups of cells. Abundance of connective tissue lends firmness to the tumor. The unique features of this case include (1) multiple cysts; (2) marked desmoplasia in a 1-year-old child; and (3) three types of clear-cut areas with abrupt transition: undifferentiated areas, mesenchymal areas with ganglion cells, and transition areas with follicular architecture (ie, pale islands).

Differential diagnosis of this tumor includes other cystic tumors and morphologically, desmoplastic tumors. Other differential diagnoses include (1) desmoplastic cerebral astrocytoma of infancy, (2) desmoplastic infantile ganglioglioma, (3) pilocytic astrocytoma of cerebellum, and (4) hemangioblastoma.

Desmoplastic cerebral astrocytoma of infancy68 is a superficially situated, supratentorial glioma. Grossly, the lesion appears as a large, firm, globular mass, densely adherent to the surface of the brain. A distinctive feature is the intense desmoplasia that imparts a fibrotic appearance. Scattered elongated glial cells, strongly positive for glial fibrillary acid protein, are seen. No ganglion cells are seen. Desmoplastic infantile ganglioglioma9,10 are supratentorial, superficially situated cystic tumors. Microscopically they show collagenous rich tissue of varying cellularity with an appearance of a neurofibroma. Ganglion cells are plump, small to medium sized, and mixed with glial cells or isolated within the desmoplastic tissue. The neuronal nature of these cells is suggested by their roundness, vesicular nuclei, variably prominent nucleoli, and Nissl substance. The spindle cells are strongly positive for glial fibrillary acid protein.

Pilocytic astrocytoma of the cerebellum can be seen as a mural nodule within a cyst. Despite gross circumscription, they permeate the surrounding tissue, particularly the white matter for some distance involving the overlying leptomeninges. They occur in the second decade of life. Microscopically stellate astrocytes in microcystic regions, Rosenthal fibers, and eosinophilic granular bodies are seen.

Hemangioblastomas, another cystic tumor of the cerebellum, are seen in adults and are dark red or yellow. A network of dilated vessels with variably lipidized stromal cells is seen microscopically. This tumor morphologically showed 3 types of clear-cut areas: undifferentiated areas, fibrotic areas with ganglion cells, and transition areas with follicular architecture (ie, pale islands) along with multiple cysts and marked desmoplasia.

Accepted for publication July 7, 2000.

Reprints: R. K. Vasishta, MD, FRCPath, Department of Histopathology, PGIMER, Chandigarh–160012, India.

Burger  PCGrehmann  PCBliestle  AKleihues  P Differentiation in the medulloblastoma: a histological and immunohistochemical study. Acta Neuropathol. 1987;73115- 123
Link to Article
Rubinstein  LJNorthfield  DWC The medulloblastoma and the so-called "arachnoidal cerebellar sarcoma": a critical re-examination of a nosological problem. Brain. 1964;87379- 412
Link to Article
Foester  OGagel  O Das umschriebene arachnoidal Sarcom des Kleinhirns. Z Gesamte Neurol Psychiatr. 1939;164565- 580
Link to Article
Langheim  WKernohan  JWUihlein  A Arachnoidal sarcoma of the cerebellum. Cancer. 1962;15705- 716
Link to Article
Ramsey  HJKernohan  JW Circumscribed sarcoma of the cerebellum: an electron microscopic study. J Neuropathol Exp Neurol. 1964;23706- 718
Link to Article
Taratulo  ALMonges  JLyeyk  PLeiguarda  R Superficial cerebral astrocytoma attached to dura: report of six cases in infants. Cancer. 1984;542502- 2512
de Chadarevian  JPPattisapu  JVFaerber  EN Desmoplastic cerebral astrocytoma of infancy: light microscopy, immunocytochemistry and ultrastructure. Cancer. 1990;66173- 179
Link to Article
Aydin  FGhatak  NRSalvant  JMuizelaar  P Desmoplastic cerebral astrocytoma of infancy: a case report with immunohistochemical, ultrastructural and proliferation studies. Acta Neuropathol (Berl). 1993;86666- 670
Link to Article
Ng  THKFung  CFMa  LT The pathological spectrum of desmoplastic infantile gangliogliomas. Histopathology. 1990;16235- 241
Link to Article
Vanderberg  SRMay  EERubinstein  LJ  et al.  Desmoplastic supratentorial neuroepithelial tumors of infancy with divergent differentiation potential ("desmoplastic infantile gangliomas"). J Neurosurg. 1987;6658- 71
Link to Article

Tables

References

Burger  PCGrehmann  PCBliestle  AKleihues  P Differentiation in the medulloblastoma: a histological and immunohistochemical study. Acta Neuropathol. 1987;73115- 123
Link to Article
Rubinstein  LJNorthfield  DWC The medulloblastoma and the so-called "arachnoidal cerebellar sarcoma": a critical re-examination of a nosological problem. Brain. 1964;87379- 412
Link to Article
Foester  OGagel  O Das umschriebene arachnoidal Sarcom des Kleinhirns. Z Gesamte Neurol Psychiatr. 1939;164565- 580
Link to Article
Langheim  WKernohan  JWUihlein  A Arachnoidal sarcoma of the cerebellum. Cancer. 1962;15705- 716
Link to Article
Ramsey  HJKernohan  JW Circumscribed sarcoma of the cerebellum: an electron microscopic study. J Neuropathol Exp Neurol. 1964;23706- 718
Link to Article
Taratulo  ALMonges  JLyeyk  PLeiguarda  R Superficial cerebral astrocytoma attached to dura: report of six cases in infants. Cancer. 1984;542502- 2512
de Chadarevian  JPPattisapu  JVFaerber  EN Desmoplastic cerebral astrocytoma of infancy: light microscopy, immunocytochemistry and ultrastructure. Cancer. 1990;66173- 179
Link to Article
Aydin  FGhatak  NRSalvant  JMuizelaar  P Desmoplastic cerebral astrocytoma of infancy: a case report with immunohistochemical, ultrastructural and proliferation studies. Acta Neuropathol (Berl). 1993;86666- 670
Link to Article
Ng  THKFung  CFMa  LT The pathological spectrum of desmoplastic infantile gangliogliomas. Histopathology. 1990;16235- 241
Link to Article
Vanderberg  SRMay  EERubinstein  LJ  et al.  Desmoplastic supratentorial neuroepithelial tumors of infancy with divergent differentiation potential ("desmoplastic infantile gangliomas"). J Neurosurg. 1987;6658- 71
Link to Article

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