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: Synonym 1; Synonym 2; Synonym 3
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, [feature1], [feature2], and [feature3] are characteristic findings of medulloepithelioma.
- On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of medulloepithelioma.
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
- There are no [imaging study] findings associated with medulloepithelioma.
- [Imaging study 1] is the imaging modality of choice for medulloepithelioma.
- On MRI, medulloepithelioma is characterized by [finding 1], [finding 2], and [finding 3].
- The image below demonstrates an example of medulloepithelioma.
Imaging studies such as Computerized Tomography (CT) and Magnetic Resonance Imaging (MRI) can aid diagnosis. Medulloepithelioma appears isodense or hypodense with variable heterogeneity and calcification on non-contrast CT scan, and enhances with contrast.[4] This radiographical finding is consistent with a primitive neuroectodermal tumour, especially in children.[5] Blood studies and imaging studies of the abdomen may be used to detect metastases.[5]
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.
- ↑
- ↑ 5.0 5.1