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Revision as of 19:45, 29 April 2016

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.^[1] Tumours originating in the ciliary body of the eye are referred to as embryonal medulloepitheliomas,^[1] or diktyomas.^[2]

Historical Perspective

[Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].

Classification

[Disease name] may be classified according to [classification method] into [number] subtypes/groups:

[group1]
[group2]
[group3]

Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].

Pathophysiology

The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Causes

[Disease name] may be caused by either [cause1], [cause2], or [cause3].
[Disease name] is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
There are no established causes for [disease name].

Differentiating [disease name] from other Diseases

[Disease name] 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

The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

Patients of all age groups may develop [disease name].

[Disease name] is more commonly observed among patients aged [age range] years old.
[Disease name] is more commonly observed among [elderly patients/young patients/children].

Gender

[Disease name] affects men and women equally.

[Gender 1] are more commonly affected with [disease name] than [gender 2].
The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

There is no racial predilection for [disease name].

[Disease name] usually affects individuals of the [race 1] race.
[Race 2] individuals are less likely to develop [disease name].

Risk Factors

Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis

The majority of patients with [disease name] remain asymptomatic for [duration/years].
Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
Prognosis is generally [excellent/good/poor], and the [1/5/10year mortality/survival rate] of patients with [disease name] is approximately [#%].

Diagnosis

Diagnostic Criteria

The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:

[criterion 1]
[criterion 2]
[criterion 3]
[criterion 4]

Symptoms

[Disease name] is usually asymptomatic.
Symptoms of [disease name] may include the following:

[symptom 1]
[symptom 2]
[symptom 3]
[symptom 4]
[symptom 5]
[symptom 6]

Physical Examination

Patients with [disease name] 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 [disease name].

A [positive/negative] [test name] is diagnostic of [disease name].
An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Imaging Findings

There are no [imaging study] findings associated with [disease name].

[Imaging study 1] is the imaging modality of choice for [disease name].
On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

[Disease name] may also be diagnosed using [diagnostic study name].
Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

There is no treatment for [disease name]; the mainstay of therapy is supportive care.

The mainstay of therapy for [disease name] 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 [disease name].
[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
[Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention

There are no primary preventive measures available for [disease name].

Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

References

↑ ^1.0 ^1.1 Definition of Medulloepithelioma, from Online Medical Dictionary. Retrieved 7 January 2010.

[mondofacto.com-1] 1.0 ^1.1 Definition of Medulloepithelioma, from Online Medical Dictionary. Retrieved 7 January 2010.

[McGraw-Hill_Concise_Dictionary_of_Modern_Medicine-2] McGraw-Hill Concise Dictionary of Modern Medicine. © 2002 by The McGraw-Hill Companies, Inc.

[1]

[2]

@@ Line 1: / Line 1: @@
 __NOTOC__
-{{Infobox disease
+{{SI}}
- | Name            = Medulloepithelioma
+{{CMG}} {{AE}}  {{MV}}
- | Image           = Medulloepithelioma_Histology.jpg
- | Caption         = Histopathology of medulloepithelioma showing characteristic neural tube like strands.
+{{SK}} Synonym 1; Synonym 2; Synonym 3
-}}
-{{CMG}}
-{{SI}}
 ==Overview==
 '''Medulloepithelioma''' is a rare, primitive, fast-growing [[brain tumour]] thought to stem from [[cell (biology)|cell]]s of the [[embryo]]nic [[medullary cavity]].<ref name="mondofacto.com">[http://www.mondofacto.com/facts/dictionary?Medulloepithelioma Definition of Medulloepithelioma], from Online Medical Dictionary. Retrieved 7 January 2010.</ref> Tumours originating in the [[ciliary body]] of the [[human eye|eye]] are referred to as embryonal medulloepitheliomas,<ref name="mondofacto.com"/> or [[diktyoma]]s.<ref name="McGraw-Hill Concise Dictionary of Modern Medicine">McGraw-Hill Concise Dictionary of Modern Medicine. © 2002 by The McGraw-Hill Companies, Inc.</ref>
-A highly [[malignant]] [[Cellular differentiation|undifferentiated]] primitive [[Neuroepithelial cell|neuroepithelial]] tumour of children, medulloepithelioma may contain [[bone]], [[cartilage]], [[skeletal muscle]], and tends to [[Metastasis|metastasize]] extracranially.<ref name="McGraw-Hill Concise Dictionary of Modern Medicine"/>
+==Historical Perspective==
+*[Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
-==Epidemiology==
+*In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
-Medulloepithelioma most commonly affect children between 6 months and 5 years; rarely, this tumour may occur [[Congenital disorder|congenitally]] or beyond this age range.<ref name="Russel">Russel DS, Rubinstein LJ. Pathology of tumors the nervous system, 5th ed. Baltimore: Williams & Wilkins 1989; pp. 247-51.</ref><ref>Sato T, Shimoda A, Takahishi T, et al. Congenital cerebellar neuroepithelial tumor with multiple divergent differentiation. Acta Neuropathol 1980;50:143-6.</ref><ref>Scheithauer BW, Rubinstein LJ. Cerebral medulloepithelioma: Report of a case with multiple divergent neuroepithelial differentiation. Childs Brain 1979;5:62-71.</ref> [[Incidence (epidemiology)|Incidence]] is equal in males and females.<ref name="Molly">Molly PT, Yachnis AT, Rorke LB, et al. Central nervous system medulloepithelioma a series of eight cases including two arising in the pons. J Neurosurg 1996;84:430-6.</ref>
+*In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].
 ==Classification==
-Medulloepithelioma was originally classified as the most primitive neoplasm of the [[Central Nervous System]] (CNS) by Bailey and Cushing in 1926.<ref name="Bailey">Bailey P, Cushing H. A classification of tumors of the glioma group on a histogenetic basis with a correlated study of progress. Philadelphia: JB Lippincott; 1926. pp. 54-6.</ref> Rorke et al.<ref>Rorke LB, Gilles FH, Davis RI, Becker LE. Revision of the World Health Organization classification of brain tumors for child hood brain tumors. Cancer 1985;56:1869-86.</ref> classified this tumour into two subtypes:
+*[Disease name] may be classified according to [classification method] into [number] subtypes/groups:
-:1) medulloepithelioma not otherwise specified
+:*[group1]
-:2) medulloepithelioma with differentiation into [[astrocyte]]s, [[oligodendrocyte]]s; [[ependymal cell]]s; [[Neuron|neuronal cells]]; others ([[melanin]], [[mesenchymal cell]]s); and mixed cellular elements.
+:*[group2]
+:*[group3]
-==Clinical manifestation==
+*Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].
-Medulloepithelioma have been reported to occur in the [[cerebral hemispheres]], [[brainstem]], [[cerebellum]], and [[Peripheral nervous system|peripheral sites]].<ref name="Molly"/><ref name="Bailey"/><ref name="Karch">Karch SB, Urich H. Medulloepithelioma: definition of an entity. J Neuropathol Exp Neurol 1972;31:27-53.</ref><ref name="Sundaram">Sundaram C, Vydehi BV, Reddy JJ, Reddy AK. Medulloepithelioma: A case report. Neurol India 2003;51:546-7.</ref>
+==Pathophysiology==
+*The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
+*The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
+*On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
+*On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
+==Causes==
+* [Disease name] may be caused by either [cause1], [cause2], or [cause3].
+* [Disease name] is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
+* There are no established causes for [disease name].
+==Differentiating [disease name] from other Diseases==
+*[Disease name] 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==
+* The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
+* In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].
+===Age===
+*Patients of all age groups may develop [disease name].
+*[Disease name] is more commonly observed among patients aged [age range] years old.
+*[Disease name] is more commonly observed among [elderly patients/young patients/children].
+===Gender===
+*[Disease name] affects men and women equally.
+*[Gender 1] are more commonly affected with [disease name] than [gender 2].
+* The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.
+===Race===
+*There is no racial predilection for [disease name].
+*[Disease name] usually affects individuals of the [race 1] race.
+*[Race 2] individuals are less likely to develop [disease name].
+==Risk Factors==
+*Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
+== Natural History, Complications and Prognosis==
+*The majority of patients with [disease name] remain asymptomatic for [duration/years].
+*Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
+*If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
+*Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
+*Prognosis is generally [excellent/good/poor], and the [1/5/10year mortality/survival rate] of patients with [disease name] is approximately [#%].
+== Diagnosis ==
+===Diagnostic Criteria===
+*The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
+:*[criterion 1]
+:*[criterion 2]
+:*[criterion 3]
+:*[criterion 4]
+=== Symptoms ===
+*[Disease name] is usually asymptomatic.
+*Symptoms of [disease name] may include the following:
+:*[symptom 1]
+:*[symptom 2]
+:*[symptom 3]
+:*[symptom 4]
+:*[symptom 5]
+:*[symptom 6]
+=== Physical Examination ===
+*Patients with [disease name] 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 [disease name].
-Due to rapid growth of the tumour, patients typically present with [[Intracranial pressure|increased intracranial pressure]], [[seizure]]s, and [[focal neurologic signs]].<ref>[http://www.uptodate.com/online/content/topic.do?topicKey=brain_ca/17771&selectedTitle=2%7E3&source=search_result#H16 Uncommon brain tumors], from UpToDate Online 17.3. Last updated 21 July 2009, Retrieved 7 January 2010.</ref>
+*A  [positive/negative] [test name] is diagnostic of [disease name].
+*An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
-==Diagnosis==
+*Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
-[[Image:Medulloepithelioma.jpg|thumb|Neuronal differentiation, ranging from neuroblasts to ganglion cells, is seen in some medulloepitheliomas.]]Imaging studies such as [[Computerized Tomography]] (CT) and [[Magnetic Resonance Imaging]] (MRI) can aid [[Medical diagnosis|diagnosis]]. Medulloepithelioma appears isodense or hypodense with variable heterogeneity and [[calcification]] on non-contrast CT scan, and enhances with contrast.<ref name="Molly"/> This radiographical finding is consistent with a primitive neuroectodermal tumour, especially in children.<ref name="Sundaram"/> Blood studies and imaging studies of the abdomen may be used to detect metastases.<ref name="Sundaram"/>
+===Imaging Findings===
-[[Needle aspiration biopsy]] can be used to aid diagnosis.<ref name="Sundaram"/> Definitive diagnosis requires histopathological examination of surgically excised tumour tissues.
+*There are no [imaging study] findings associated with [disease name].
-Histologically, medulloepithelioma resemble a primitive neural tube and with neuronal, glial and mesenchymal elements.<ref name="Russel"/><ref>Deck JHN. Cerebral medulloepithelioma with maturation into ependymal cells and ganglion cells. J Neuropathol Exp Neurol 1969;28:442-54.</ref> [[Flexner-Wintersteiner rosette]]s may also be observed.<ref>McLean IW, Burnier MN, Zimmerman LE, et al. Tumors of the retina. In: Atlas of tumor pathology: tumors of the eye and ocular adnexa. Washington, DC: Armed Forces Institute of Pathology; 1994:97–154.</ref>
+*[Imaging study 1] is the imaging modality of choice for [disease name].
+*On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
-[[Immunohistochemistry|Immunohistochemically]], neural tube-like structures are [[vimentin]] positive in the majority of medulloepitheliomas.<ref>Troost D, Jansen GH, Dingemans KP. Cerebral medulloepithelioma - electron microscopy and immunohistochemistry. Acta Neuropathol 1990;80:103-7.</ref> Poorly differentiated medulloepitheliomas are vimentin negative.
+*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].
-== Treatment==
+=== Other Diagnostic Studies ===
-[[Segmental resection|Total resection]] of the tumour, followed by [[radiation therapy]] is the standard treatment modality.<ref name="Molly"/> Medulloepithelioma of the ciliary body may necessitate enucleation of the eye.<ref>al-Torbak A, Abboud EB, al-Sharif A, el-Okda MO. Medulloepithelioma of the ciliary body. Indian J Ophthalmol. 2002 Jun;50(2):138-40.</ref><ref>Vajaranant, TS, Mafee, MF, Kapur, R, et al. Medulloepithelioma of the ciliary body and optic nerve: clinicopathologic, CT, and MR imaging features. Neuroimaging Clin N Am 2005; 15:69.</ref> Radiation therapy alone may prolong survival.<ref name="Molly"/> Aggressive [[chemotherapy]] with autologous [[Hematopoietic stem cell transplantation|bone marrow transplant]] is used for metastatic medulloepitheliomas.<ref name="Sundaram"/>
+*[Disease name] may also be diagnosed using [diagnostic study name].
+*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].
-==Prognosis==
-Medulloepithelioma carries a dismal [[prognosis]] with a median survival of 5 months.<ref name="Molly"/><ref>Sharma MC; Mahapatra AK; Gaikwad S; Jain AK; Sarkar C. Pigmented medulloepithelioma: report of a case and review of the literature. Childs Nerv Syst. 1998 Jan-Feb;14(1-2):74-8.</ref><ref>Chidambaram B; Santosh V; Balasubramaniam V. Medulloepithelioma of the optic nerve with intradural extension--report of two cases and a review of the literature. Childs Nerv Syst. 2000 Jun;16(6):329-33.</ref><ref>Depper, MH, Hart, BL. Pediatric Brain Tumors. In: Neuroimaging, Orrison, WW (ed), WB Saunders, Philadelphia, 2000. p. 1625.</ref>
+== Treatment ==
+=== Medical Therapy ===
+*There is no treatment for [disease name]; the mainstay of therapy is supportive care.
+*The mainstay of therapy for [disease name] 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 [disease name].
+*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
+*[Surgical procedure] can only be performed for patients with [disease stage] [disease name].
+=== Prevention ===
+*There are no primary preventive measures available for [disease name].
+*Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
+*Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].
 ==References==
 {{Reflist|2}}
-[[Category:Oncology]]
+[[Category: Oncology]]