Medulloepithelioma
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 tumour thought to stem from cells of the embryonic medullary cavity. Tumours originating in the ciliary body of the eye are referred to as embryonal medulloepitheliomas or diktyomas.[1][2]
Historical Perspective
- Medulloepithelioma was first discovered by Bailey and Cushing, in 1926.
Classification
- Medulloepithelioma may be classified into 3 groups:
- Medulloepithelioma not otherwise specified
- Medulloepithelioma with differentiation into astrocytes, oligodendrocytes; ependymal cells
- Medulloepithelioma with neuronal cells; others (melanin, mesenchymal cells); and mixed cellular elements
Pathophysiology
- The pathogenesis of medulloepithelioma is characterized by [feature1], [feature2], and [feature3].
- The [gene name] gene/Mutation in [gene name] has been associated with the development of medulloepithelioma, involving the [molecular pathway] pathway.
- On gross pathology, characteristic findings of medulloepithelioma, include:
- On microscopic histopathological analysis, characteristic findings of medulloepithelioma, include:
Causes
- Common causes of medulloepithelioma, include:
Differentiating Medulloepithelioma from Other Diseases
- Medulloepithelioma must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
- [Differential dx1]
- [Differential dx2]
- [Differential dx3]
Epidemiology and Demographics
- Medulloepithelioma is a rare disease.
- The prevalence of medulloepithelioma is approximately [number or range] per 100,000 individuals worldwide.
Age
- Medulloepithelioma is more commonly observed among patients aged between 6 months and 5 years old.[3]
- Medulloepithelioma is more commonly observed among children.
Gender
- Medulloepithelioma affects men and women equally.
Race
- There is no racial predilection for medulloepithelioma.
Risk Factors
- Common risk factors in the development of medulloepithelioma, include:
Natural History, Complications and Prognosis
- The majority of patients with medulloepithelioma remain asymptomatic for [duration/years].
- Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
- If left untreated, [#%] of patients with medulloepithelioma may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
- Common complications of medulloepithelioma include [complication 1], [complication 2], and [complication 3].
- Prognosis is generally poor, and the median survival time of patients with medulloepithelioma is approximately 5 months.
Diagnosis
Symptoms
- Medulloepithelioma is usually asymptomatic.
- Symptoms of medulloepithelioma may include the following:
- [symptom 1]
- [symptom 2]
- [symptom 3]
- [symptom 4]
- [symptom 5]
- [symptom 6]
Physical Examination
- Patients with medulloepithelioma usually appear [general appearance].
- Physical examination may be remarkable for:
- [finding 1]
- [finding 2]
- [finding 3]
- [finding 4]
- [finding 5]
- [finding 6]
Laboratory Findings
- There are no specific laboratory findings associated with medulloepithelioma.
Imaging Findings
- On CT, findings of medulloepithelioma, include:
- The lesion is isodense or hypodense with variable heterogeneity and calcification
- On MRI, findings of medulloepithelioma, include:
- The image below demonstrates an example of medulloepithelioma
Treatment
Medical Therapy
- There is no treatment for medulloepithelioma; the mainstay of therapy is supportive care.
- The mainstay of therapy for medulloepithelioma is [medical therapy 1] and [medical therapy 2].
- [Medical therapy 1] acts by [mechanism of action1].
- Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
Surgery
- Surgery is the mainstay of therapy for medulloepithelioma.
- [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of medulloepithelioma.
- [Surgical procedure] can only be performed for patients with [disease stage] medulloepithelioma.
Prevention
- There are no primary preventive measures available for medulloepithelioma.
References
- ↑ McGraw-Hill Concise Dictionary of Modern Medicine. © 2002 by The McGraw-Hill Companies, Inc.
- ↑ Definition of Medulloepithelioma, from Online Medical Dictionary. Retrieved 7 January 2010.
- ↑ Russel DS, Rubinstein LJ. Pathology of tumors the nervous system, 5th ed. Baltimore: Williams & Wilkins 1989; pp. 247-51.