Medulloepithelioma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and Keywords: Medullary epithelioma

Overview

Medulloepithelioma is a rare, primitive, fast-growing brain tumor thought to stem from cells of the embryonic medullary cavity. Tumors originating in the ciliary body of the eye are referred to as embryonal medulloepitheliomas or diktyomas.[1][2] Medulloepithelioma was first discovered by Bailey and Cushing in 1926. The pathogenesis of medulloepithelioma is characterized by highly malignant undifferentiated primitive neuroepithelial tumor. Tumors generally originate in the ciliary body of the eye. It most commonly manifests in the cerebral hemispheres, brainstem, cerebellum, and in peripheral sites. Medulloepithelioma arises from primitive medullary epithelium, which is normally involved in the embryonic formation of CNS. Medulloepithelioma is more commonly observed among patients between 6 months and 5 years old. On CT, findings of medulloepithelioma, include an isodense or hypodense lesion with variable heterogeneity and calcification. Surgery is the mainstay of therapy for medulloepithelioma. Total resection in conjunction with radiation therapy is the most common approach to the treatment of medulloepithelioma.

Historical Perspective

Medulloepithelioma was first discovered by Bailey and Cushing in 1926.

Classification

Medulloepithelioma may be classified into 3 groups:

Pathophysiology

Pathogenesis

The pathogenesis of medulloepithelioma is characterized by:

Medulloepithelioma arises from primitive medullary epithelium, which is normally involved in the embryonic formation of CNS.

Genetics

The germline DICER1 gene mutation has been associated with the development of medulloepithelioma.

Gross Pathology

There are no characteristic gross pathology findings associated with medulloepithelioma.

Microscopic Pathology

On microscopic histopathological analysis, characteristic findings of medulloepithelioma may include:

  • Small round blue cell tumor
  • Focal differentiation into astrocytic, neuronal or ependymal phenotypes possible
  • May have true rosettes (slit-like/oval)
  • Growth in streams or palisades
  • Vascular endothelial proliferations
  • Fibrillary background in tumors with advanced neuronal maturation
  • Variable mitotic activity

On immunohistopathological analysis, characteristic findings of medulloepithelioma may include:

  • Positive S-100
  • Positive INI1
  • Positive LIN28

Causes

The cause of medulloepithelioma is unknown.

Differentiating Medulloepithelioma from Other Diseases

Medulloepithelioma must be differentiated from other diseases that cause vision loss, ocular mass, and headache such as:

Epidemiology and Demographics

Medulloepithelioma is a rare disease.

Age

Medulloepithelioma is most commonly observed among patients between 6 months and 5 years old.[3]

Gender

Medulloepithelioma affects men and women equally.

Race

There is no racial predilection for medulloepithelioma.

Risk Factors

There are no known associated risk factors in the development of medulloepithelioma.

Natural History, Complications and Prognosis

Natural History

  • Patients with medulloepithelioma may be initially asymptomatic. Early clinical features include vision loss, irritability, and neurological deficit. If left untreated, patients with medulloepithelioma may progress to mortality.

Complications

Common complications of medulloepithelioma include:

Prognosis

The prognosis of medulloepithelioma is generally poor; the median survival time of patients with medulloepithelioma is approximately 5 months.

Diagnosis

History & Symptoms

Medulloepithelioma is usually asymptomatic. Symptoms of medulloepithelioma may include:

Physical Examination

Patients with medulloepithelioma usually are well-appearing. Physical examination may be remarkable for:

Laboratory Findings

There are no specific laboratory findings associated with medulloepithelioma.

Imaging Findings

CT Findings

On CT, findings of medulloepithelioma include isodense or hypodense lesions with variable heterogeneity and calcification.

Treatment

Medical Therapy

There is no medical treatment for medulloepithelioma; the mainstay of therapy is surgery.

Surgery

Surgery is the mainstay of therapy for medulloepithelioma. Total resection in conjunction with radiation therapy is the most common approach to the treatment of medulloepithelioma.

Prevention

There are no primary preventive measures available for medulloepithelioma.

References

  1. McGraw-Hill Concise Dictionary of Modern Medicine. © 2002 by The McGraw-Hill Companies, Inc.
  2. Definition of Medulloepithelioma, from Online Medical Dictionary. Retrieved 7 January 2010.
  3. Russel DS, Rubinstein LJ. Pathology of tumors the nervous system, 5th ed. Baltimore: Williams & Wilkins 1989; pp. 247-51.

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