Medulloepithelioma

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Medulloepithelioma
Medulloepithelioma Histology.jpg
Histopathology of medulloepithelioma showing characteristic neural tube like strands.
Specialty Neurosurgery, Neuro-oncology

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]

Contents

A highly malignant undifferentiated primitive neuroepithelial tumour of children, medulloepithelioma may contain bone, cartilage, skeletal muscle, and tends to metastasize extracranially. [2]

Signs and symptoms

Medulloepithelioma have been reported to occur in the cerebral hemispheres, brainstem, cerebellum, and peripheral sites. [3] [4] [5] [6]

Due to rapid growth of the tumour, patients typically present with increased intracranial pressure, seizures, and focal neurologic signs. [7]

Diagnosis

Neuronal differentiation, ranging from neuroblasts to ganglion cells, is seen in some medulloepitheliomas. Medulloepithelioma.jpg
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 diagnosis. Medulloepithelioma appears isodense or hypodense with variable heterogeneity and calcification on non-contrast CT scan, and enhances with contrast. [3] This radiographical finding is consistent with a primitive neuroectodermal tumour, especially in children. [6] Blood studies and imaging studies of the abdomen may be used to detect metastases. [6]

Needle aspiration biopsy can be used to aid diagnosis. [6] Definitive diagnosis requires histopathological examination of surgically excised tumour tissues.[ citation needed ]

Histologically, medulloepithelioma resemble a primitive neural tube and with neuronal, glial and mesenchymal elements. [8] [9] Flexner-Wintersteiner rosettes may also be observed. [10]

Immunohistochemically, neural tube-like structures are vimentin positive in the majority of medulloepitheliomas. [11] Poorly differentiated medulloepitheliomas are vimentin negative.[ citation needed ]

Classification

Medulloepithelioma was originally classified as the most primitive neoplasm of the Central Nervous System (CNS) by Bailey and Cushing in 1926. [4] Rorke et al. [12] classified this tumour into two subtypes:

1) medulloepithelioma not otherwise specified
2) medulloepithelioma with differentiation into astrocytes, oligodendrocytes; ependymal cells; neuronal cells; others (melanin, mesenchymal cells); and mixed cellular elements.

Treatment

Total resection of the tumour, followed by radiation therapy is the standard treatment modality. [3] Medulloepithelioma of the ciliary body may necessitate enucleation of the eye. [13] [14] Radiation therapy alone may prolong survival. [3] Aggressive chemotherapy with autologous bone marrow transplant is used for metastatic medulloepitheliomas. [6]

Prognosis

Medulloepithelioma carries a dismal prognosis with a median survival of 5 months. [3] [15] [16] [17]

Epidemiology

Medulloepithelioma most commonly affect children between 6 months and 5 years; rarely, this tumour may occur congenitally or beyond this age range. [8] [18] [19] Incidence is equal in males and females. [3]

Related Research Articles

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Nervous tissue Main component of the nervous system

Nervous tissue, also called neural tissue, is the main tissue component of the nervous system. The nervous system regulates and controls bodily functions and activity. It consists of two parts: the central nervous system (CNS) comprising the brain and spinal cord, and the peripheral nervous system (PNS) comprising the branching peripheral nerves. It is composed of neurons, also known as nerve cells, which receive and transmit impulses, and neuroglia, also known as glial cells or glia, which assist the propagation of the nerve impulse as well as provide nutrients to the neurons.

Oligodendroglioma Medical condition

Oligodendrogliomas are a type of glioma that are believed to originate from the oligodendrocytes of the brain or from a glial precursor cell. They occur primarily in adults but are also found in children.

Fibrosarcoma Medical condition

Fibrosarcoma is a malignant mesenchymal tumour derived from fibrous connective tissue and characterized by the presence of immature proliferating fibroblasts or undifferentiated anaplastic spindle cells in a storiform pattern. In humans it is usually found in males aged 30 to 40. It originates in fibrous tissues of the bone and invades long or flat bones such as the femur, tibia, and mandible. It also involves the periosteum and overlying muscle.

Desmoplastic small-round-cell tumor Aggressive and rare cancer

Desmoplastic small-round-cell tumor (DSRCT) is an aggressive and rare cancer that primarily occurs as masses in the abdomen. Other areas affected may include the lymph nodes, the lining of the abdomen, diaphragm, spleen, liver, chest wall, skull, spinal cord, large intestine, small intestine, bladder, brain, lungs, testicles, ovaries, and the pelvis. Reported sites of metastatic spread include the liver, lungs, lymph nodes, brain, skull, and bones. It is characterized by the EWS-WT1 fusion protein.

Neuroepithelial cells, or neuroectodermal cells, form the wall of the closed neural tube in early embryonic development. The neuroepithelial cells span the thickness of the tube's wall, connecting with the pial surface and with the ventricular or lumenal surface. They are joined at the lumen of the tube by junctional complexes, where they form a pseudostratified layer of epithelium called neuroepithelium.

Primary central nervous system lymphoma Medical condition

A primary central nervous system lymphoma (PCNSL), also termed primary diffuse large B-cell lymphoma of the central nervous system, is a primary intracranial tumor appearing mostly in patients with severe immunodeficiency. It is a subtype and one of the most aggressive of the diffuse large B-cell lymphomas. PCNSLs represent around 20% of all cases of lymphomas in HIV infections.. Primary CNS lymphoma is highly associated with Epstein-Barr virus (EBV) infection in immunodeficient patients, and does not have a predilection for any particular age group. Mean CD4+ count at time of diagnosis is ~50/uL. In immunocompromised patients, prognosis is usually poor. In immunocompetent patients, there is rarely an association with EBV infection or other DNA viruses. In the immunocompetent population, PCNSLs typically appear in older patients in their 50s and 60s. Importantly, the incidence of PCNSL in the immunocompetent population has been reported to have increased more than 10-fold from 2.5 cases to 30 cases per 10 million population. The cause for the increase in incidence of this disease in the immunocompetent population is unknown.

Malignant rhabdoid tumour (MRT) is a very aggressive form of tumour originally described as a variant of Wilms' tumour, which is primarily a kidney tumour that occurs mainly in children.

Limbic encephalitis Inflammation involving the limbic system in the brain

Limbic encephalitis is a form of encephalitis, a disease characterized by inflammation of the brain. Limbic encephalitis is caused by autoimmunity: an abnormal state where the body produces antibodies against itself. Some cases are associated with cancer and some are not. Although the disease is known as "limbic" encephalitis, it is seldom limited to the limbic system and post-mortem studies usually show involvement of other parts of the brain. The disease was first described by Brierley and others in 1960 as a series of three cases. The link to cancer was first noted in 1968 and confirmed by later investigators.

Atypical teratoid rhabdoid tumor Medical condition

An atypical teratoid rhabdoid tumor (AT/RT) is a rare tumor usually diagnosed in childhood. Although usually a brain tumor, AT/RT can occur anywhere in the central nervous system (CNS), including the spinal cord. About 60% will be in the posterior cranial fossa. One review estimated 52% in the posterior fossa, 39% are supratentorial primitive neuroectodermal tumors (sPNET), 5% are in the pineal, 2% are spinal, and 2% are multifocal.

Ectomesenchymoma is a rare, fast-growing tumor of the nervous system or soft tissue that occurs mainly in children, although cases have been reported in patients up to age 60. Ectomesenchymomas may form in the head and neck, abdomen, perineum, scrotum, or limbs. Also called malignant ectomesenchymoma.

Juxtaglomerular cell tumor is an extremely rare kidney tumour of the juxtaglomerular cells, with less than 100 cases reported in literature. This tumor typically secretes renin, hence the former name of reninoma. It often causes severe hypertension that is difficult to control, in adults and children, although among causes of secondary hypertension it is rare. It develops most commonly in young adults, but can be diagnosed much later in life. It is generally considered benign, but its malignant potential is uncertain.

WHO classification of tumours of the central nervous system

The following is a simplified (deprecated) version of the last 2007 WHO classification of the tumours of the central nervous system. Currently, as of 2016, clinicians are using revised WHO grade 4th edition which incorporates recent advance in molecular pathology. The 4th revised edition, as the name implies, will soon be updated to catch up ever growing knowledge of this field.

Diktyoma Type of eye tumor

Diktyoma, or ciliary body medulloepithelioma, or teratoneuroma, is a rare tumor arising from primitive medullary epithelium in the ciliary body of the eye. Almost all diktyomas arise in the ciliary body, although, rarely, they may arise from the optic nerve head or retina.

Large cell lung carcinoma with rhabdoid phenotype (LCLC-RP) is a rare histological form of lung cancer, currently classified as a variant of large cell lung carcinoma (LCLC). In order for a LCLC to be subclassified as the rhabdoid phenotype variant, at least 10% of the malignant tumor cells must contain distinctive structures composed of tangled intermediate filaments that displace the cell nucleus outward toward the cell membrane. The whorled eosinophilic inclusions in LCLC-RP cells give it a microscopic resemblance to malignant cells found in rhabdomyosarcoma (RMS), a rare neoplasm arising from transformed skeletal muscle. Despite their microscopic similarities, LCLC-RP is not associated with rhabdomyosarcoma.

Papillary tumors of the pineal region

Papillary tumors of the pineal region (PTPR) were first described by A. Jouvet et al. in 2003 and were introduced in the World Health Organization (WHO) classification of Central Nervous System (CNS) in 2007. Papillary Tumors of the Pineal Region are located on the pineal gland which is located in the center of the brain. The pineal gland is located on roof of the diencephalon. It is a cone shaped structure dorsal to the midbrain tectum. The tumor appears to be derived from the specialized ependymal cells of the subcommissural organ. Papillary tumors of the central nervous system and particularly of the pineal region are very rare and so diagnosing them is extremely difficult.

Plexiform angiomyxoid myofibroblastic tumor (PAMT), also called plexiform angiomyxoma, plexiform angiomyxoid tumor, or myxofibroma, is an extremely rare benign mesenchymal myxoid tumor along the gastrointestinal tract. Most of PAMTs occur in the gastric antral region, but they can be situated anywhere in the stomach. There is one recorded case of PAMT located in duodenum.

Central nervous system primitive neuroectodermal tumor Medical condition

A central nervous system primitive neuroectodermal tumor, often abbreviated as PNET, supratentorial PNET, or CNS-PNET, is one of the 3 types of embryonal central nervous system tumors defined by the World Health Organization. It is considered an embryonal tumor because it arises from cells partially differentiated or still undifferentiated from birth. Those cells are usually neuroepithelial cells, stem cells destined to turn into glia or neurons. It can occur anywhere within the spinal cord and cerebrum and can have multiple sites of origins, with a high probability of metastasis through cerebrospinal fluid (CSF).

Palisade (pathology) Single layer of relatively long cells arranged loosely perpendicular to a perpendicular to a surface and parallel to each other

In histopathology, a palisade is a single layer of relatively long cells, arranged loosely perpendicular to a surface and parallel to each other. A rosette is a palisade in a halo or spoke-and-wheel arrangement, surrounding a central core or hub. A pseudorosette is a perivascular radial arrangement of neoplastic cells around a small blood vessel.

Anaplastic oligodendroglioma Human brain tumor

Anaplastic oligodendroglioma is a neuroepithelial tumor which is believed to originate from oligodendrocytes, a cell type of the glia. In the World Health Organization (WHO) classification of brain tumors, anaplastic oligodendrogliomas are classified as grade III. In the course of the disease, they can degenerate into WHO grade IV glioblastoma. The vast majority of oligodendrogliomas occur sporadically, without a confirmed cause and without inheritance within a family.

References

  1. 1 2 Definition of Medulloepithelioma Archived 2015-12-25 at the Wayback Machine , from Online Medical Dictionary. Retrieved 7 January 2010.
  2. 1 2 McGraw-Hill Concise Dictionary of Modern Medicine. © 2002 by The McGraw-Hill Companies, Inc.
  3. 1 2 3 4 5 6 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.
  4. 1 2 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.
  5. ↑ Karch SB, Urich H. Medulloepithelioma: definition of an entity. J Neuropathol Exp Neurol 1972;31:27-53.
  6. 1 2 3 4 5 Sundaram C, Vydehi BV, Reddy JJ, Reddy AK. Medulloepithelioma: A case report. Neurol India 2003;51:546-7.
  7. ↑ Uncommon brain tumors, from UpToDate Online 17.3. Last updated 21 July 2009, Retrieved 7 January 2010.
  8. 1 2 Russel DS, Rubinstein LJ. Pathology of tumors the nervous system, 5th ed. Baltimore: Williams & Wilkins 1989; pp. 247-51.
  9. ↑ Deck JHN. Cerebral medulloepithelioma with maturation into ependymal cells and ganglion cells. J Neuropathol Exp Neurol 1969;28:442-54.
  10. ↑ 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.
  11. ↑ Troost D, Jansen GH, Dingemans KP. Cerebral medulloepithelioma - electron microscopy and immunohistochemistry. Acta Neuropathol 1990;80:103-7.
  12. ↑ 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.
  13. ↑ 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.
  14. ↑ 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.
  15. ↑ 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.
  16. ↑ 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.
  17. ↑ Depper, MH, Hart, BL. Pediatric Brain Tumors. In: Neuroimaging, Orrison, WW (ed), WB Saunders, Philadelphia, 2000. p. 1625.
  18. ↑ Sato T, Shimoda A, Takahishi T, et al. Congenital cerebellar neuroepithelial tumor with multiple divergent differentiation. Acta Neuropathol 1980;50:143-6.
  19. ↑ Scheithauer BW, Rubinstein LJ. Cerebral medulloepithelioma: Report of a case with multiple divergent neuroepithelial differentiation. Childs Brain 1979;5:62-71.
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