Gemistocytic astrocytoma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]Roukoz A. Karam, M.D.[3]

Synonyms and Keywords: Gemistocytic astrocytomas; Diffuse astrocytoma; Low grade astrocytoma

Overview

Gemistocytic astrocytoma is a histologic subtype of low grade astrocytoma, with a poorer prognosis than other matched WHO grade II astrocytic tumors. Gemistocytic astrocytoma is characterized by a significant gemistocytes population, which are large astrocytes with their cytoplasm filled with eosinophilic material displacing the nucleus eccentrically.

Historical Perspective

  • Gemistocytic astrocyte was first described by Franz Nissl in the 20th century.[1]

Classification

Gemistocytic astrocytoma is a subtype of astrocytoma and is included in the classification of astrocytoma. For more information about the classification of astrocytoma, click here.

Pathophysiology

  • Gray-tan mass
  • Well-defined borders
  • Soft texture
  • Cystic architecture

Differentiating Gemistocytic Astrocytoma from Other Diseases

Epidemiology and Demographics

  • Gemistocytic astrocytoma is a rare disease that tends to affects children and young adults.
  • The mean age at diagnosis is 35 years.
  • Males are more commonly affected than females. The male to female ratio is approximately 1.5 to 1.
  • Gemistocytic astrocytoma makes up approximately 10% of all WHO grade II diffuse astrocytomas. [5]

Risk Factors

Screening

There is insufficient evidence to recommend routine screening for gemcistocytic astrocytoma.

Natural History, Complications, and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

There are no specific physical examination findings associated with gemistocytic astrocytoma.

Laboratory Findings

There are no specific laboratory findings associated with gemistocytic astrocytoma.

Electrocardiogram

There are no ECG findings associated with gemistocytic astrocytoma.

X-ray

There are no x-ray findings associated with gemistocytic astrocytoma.

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with gemistocytic astrocytoma.

CT scan

  • Iso-dense or hypo-dense mass
  • Positive mass effect
  • Wispy enhancement (most low-grade astrocytomas are without any enhancement)
  • In fact, presence of enhancement would suggest more aggressive tumors

MRI

  • Brain MRI is helpful in the diagnosis of gemistocytic astrocytoma.
  • On MRI, gemistocytic astrocytoma is characterized by:
MRI component Findings

T1

T2

  • Hyper-intense compared to white matter
  • Always follows the white matter distribution and causes expansion of the surrounding cortex
  • Cortex may also be involved in late cases, in comparison to the oligodendroglioma, which is a cortical based tumor from the start
  • "Microcystic changes" along the lines of spread of the infiltrative astrocytoma is a very unique behavior for the infiltrative astrocytoma, however, it is only appreciated in a few number of cases
  • Hyper-intense T2 signal is not related to cellularity or cellular atypia, but rather edema, demyelination, and other degenerative changes

T1 with contrast

  • No enhancement
  • Small ill-defined areas of enhancement are not rare; however, when enhancement is seen, it should be considered as a warning sign for progression to a higher grade

Diffusion weighted imaging (DWI)

Other Imaging Findings

Magnetic Resonance Spectroscopy

Magnetic Resonance Perfusion

  • MR perfusion may be helpful in the diagnosis of gemistocytic astrocytoma, which demonstrates no elevation of relative cerebral blood volume (rCBV).

Other Diagnostic Studies

Treatment

Medical Therapy

Chemotherapy is recommended for patients with recurrent or de-differentiated gemistocytic astrocytomas.

Surgery

Primary Prevention

There are no measures for the primary prevention of gemistocytic astrocytoma.

Secondary Prevention

There are no measures for the secondary prevention of gemiistocytic astrocytoma.

References

  1. 1.0 1.1 1.2 Tihan, Tarik; Vohra, Poonam; Berger, Mitchel S.; Keles, G. Evren (2005). "Definition and Diagnostic Implications of Gemistocytic Astrocytomas: A Pathological Perspective". Journal of Neuro-Oncology. 76 (2): 175–183. doi:10.1007/s11060-005-4897-2. ISSN 0167-594X.
  2. Prayson, Richard (2010). Brain tumors. New York: Demos Medical Pub. ISBN 1933864699.
  3. Prayson, Richard (2010). Brain tumors. New York: Demos Medical Pub. ISBN 1933864699.
  4. Tonn, FirstName (2006). Neuro-oncology of CNS tumors. Berlin New York: Springer. ISBN 3540258337.
  5. 5.0 5.1 5.2 5.3 5.4 5.5 Krouwer HG, Davis RL, Silver P, Prados M (1991). "Gemistocytic astrocytomas: a reappraisal". J Neurosurg. 74 (3): 399–406. doi:10.3171/jns.1991.74.3.0399. PMID 1993905.
  6. Raghavan, Derek (2006). Textbook of uncommon cancer. Chichester, England: Wiley. ISBN 0470012021.
  7. Krouwer HG, Davis RL, Silver P, Prados M (1991). "Gemistocytic astrocytomas: a reappraisal". J Neurosurg. 74 (3): 399–406. doi:10.3171/jns.1991.74.3.0399. PMID 1993905.
  8. Pouratian N, Asthagiri A, Jagannathan J, Shaffrey ME, Schiff D (2007). "Surgery Insight: the role of surgery in the management of low-grade gliomas". Nat Clin Pract Neurol. 3 (11): 628–39. doi:10.1038/ncpneuro0634. PMID 17982433.

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