SHH Signaling Pathway Medulloblastoma

SHH Signaling Pathway Medulloblastoma

Article
Brain & Nerve
Kids' Zone
+4
Contributed byKrish Tangella MD, MBADec 09, 2020

The topic SHH Signaling Pathway Medulloblastoma you are seeking is a synonym, or alternative name, or is closely related to the medical condition Medulloblastoma, SHH Activated.

Quick Summary:

  • A medulloblastoma (MB) may be described as a type of brain tumor. A brain tumor may be described as a mass of abnormally growing cells arising from the brain tissue (brain parenchyma and meninges) or the spinal cord (central spine). The brain along-with the spinal cord constitute the central nervous system (CNS) of the body
  • Broadly, brain tumors are classified as benign (non-cancerous) and malignant. Malignant tumors are cancerous and can spread or metastasize to other regions of the body. They generally exhibit rapid growth and, in some cases, may present symptoms during the early stages of development
  • The World Health Organization (WHO) classifies central nervous system tumors according to their grade and histological subtypes. A tumor subtype is determined by a pathologist after examining a tissue biopsy of the tumor under the microscope. This WHO classification system is used by healthcare professionals all over the world in diagnosing and treating these tumors
  • Medulloblastoma is a rapidly-growing aggressive tumor of the central nervous system that predominantly occurs in young children. This high-grade malignancy is chiefly diagnosed at infratentorial locations, typically involving the posterior fossa/cerebellum. Medulloblastoma is a histologic subtype of primitive neuroectodermal tumors (PNETs)
  • According to WHO, medulloblastomas forms part of a group designated “embryonal tumors”, which are all high-grade malignancies. It is a WHO grade IV primary tumor of the CNS. Grade IV tumors are highly-malignant, exhibit aggressive behavior, and grow rapidly. These tumors are known to infiltrate widely and destroy tissues, causing necrosis. They are also known to recur sooner following treatment
  • Medulloblastoma, SHH Activated is a genetically-defined (or molecular) subtype of medulloblastoma that is listed in the recent World Health Organization classification of CNS tumors (2016). There are four main molecular subgroups of medulloblastomas; the other three being, WNT-activated medulloblastoma, group 3 medulloblastoma, and group 4 medulloblastoma. The SHH-Activated Medulloblastoma has a bimodal distribution, and is not observed in adolescence
  • The cause of formation of Medulloblastoma, SHH Activated is not well-established, although several genetic abnormalities are noted. This tumor type is largely defined in children; in adults, such molecular subtyping is not well-defined. The risk factors for tumor development are not well-established but may include certain genetic disorders (such as Gorlin syndrome) and a positive family history of the condition
  • The signs and symptoms of Medulloblastoma, SHH Activated depend on the location and size of the tumor. Individuals may experience headaches, weakness in different parts of the body, convulsions, vision and speech disturbances. If the ventricular system is affected, it may result in obstruction of the cerebrospinal fluid flow leading to hydrocephalus. Large-sized tumors may compress adjacent brain tissue resulting in complications. Metastatic tumors may spread outside the brain and spinal cord
  • The treatment modalities for SHH-Activated Medulloblastoma may include a combination of surgery, radiation therapy, and chemotherapy. The prognosis is determined by a wide variety of factors, such as tumor histology, age of the individual, tumor size, and overall health status. The prognosis of Medulloblastoma, SHH Activated is good in infant children; it is also better than the Wnt-activated medulloblastoma

Please find comprehensive information on Medulloblastoma, SHH Activated regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention, prognosis, and additional useful information HERE.

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Krish Tangella MD, MBA picture
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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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