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Abstract

We investigated why surgery alone provides for a benign clinical course in patients with desmoplastic infantile ganglioglioma and astrocytoma (DIG/A). The clinical course of 4, less than six-month-old girls, surgically treated at our institutions, was evaluated retrospectively. All presented with the clinical symptom of increasing head circumference. CT and MRI scans revealed a solid tumor attached to the dura that was surrounded by large, multiple cysts, in fronto-temporo-parietal lobe. Gross total removal succeeded in all 4 cases because the solid components of the tumor were very firm in contrast to the soft adjacent brain tissue. Microscopically, the surgical specimens consisted almost entirely of dense fibrous connective tissue containing generally elongate cells with inconspicuous cytoplasm. Most of these cells were immunopositive for GFAP. There was no evidence of tumor cells in the cyst wall. In 3 cases, some small neurons were positive for neurofilament immunostain. A high proportion of undifferentiated small cells in a less demoplastic area far from the dura were immunopositive for MIB-1. All of the 4 patients have been free of recurrence for more than five years. In patients with DIG/A, there are 5 reasons for a good clinical course. [1] At surgery, the tumor margin is clearly discernible because of the difference between the solid tumor and the soft adjacent brain tissue. [2] The tumor is located in the superficial cerebral hemisphere. [3] Large, multiple cysts surround the tumor. [4] The growth point appears to be adjacent to the cysts. [5] The cyst walls are free of invading tumor cells.

Perinatal (fetal and neonatal) astrocytoma: a review

“…Desmoplastic cerebral astrocytoma/desmoplastic cerebral ganglioglioma of infancy ( DIAGA ) occurs most often during the first year of life than at any other time [2, 4, 20, 25, 30, 45, 47, 62, 71, 81, 83, 86]. The diagnosis was made in this review in nine neonates and in one fetus (Table 6).…”

“…Perinatal astrocytomas occur most often outside the posterior cranial fossa and above the tentorium. Cerebral hemisphere is the main location where they tend to be large, involve more than one lobe, and displace the lateral and/or third ventricles [24, 30, 35, 36, 55, 56, 81, 82]. Intracranial mass, macrocephaly, hydrocephalus, and hemorrhage are the most common initial imaging findings [23, 27, 30, 32, 38, 51] (Tables 2, 3, 4, 5, 6, 7, and 8).…”

“…Intracranial mass, macrocephaly, hydrocephalus, and hemorrhage are the most common initial imaging findings [23, 27, 30, 32, 38, 51] (Tables 2, 3, 4, 5, 6, 7, and 8). However, the leading presentation is an intracranial mass detected on routine prenatal sonograms [23, 27, 30, 32, 81]. Differential diagnosis of a brain tumor includes vascular malformation, infarction, or hemorrhage [21, 47, 83, 84].…”

Novel suprasellar location of desmoplastic infantile astrocytoma and ganglioglioma: a single institution’s experience

“…17 Generally, DIA/DIGs are considered benign and are often cured by resection alone. 22,30 There have been reports of DIA/DIGs spontaneously regressing after subtotal resection without further surgical intervention or subsequent cytotoxic therapy. 31 …”

“…12 The mainstay of treatment for most hemispheric DIA/DIGs and pilocytic astrocytomas of the cortex and cerebellum is gross-total resection. 11,30 The initial approach for pilocytic astrocytomas of the optochiasmatic/suprasellar/hypothalamic region is frequent imaging and chemotherapy for progressive lesions, rather than surgery, in order to prevent potentially serious surgical complications, including hypothalamic dysfunction, blindness, and injury to vascular structures. Surgery is typically reserved for patients with tumors that have extended into the third ventricle, causing obstructive hydrocephalus or lesions that are not responsive to therapy.…”