A 8 years boy with headache, supracellar mass, with bilateral hemianopia, showing the following MRI scan of head. What is the diagnosis
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Correct Answer:
Craniopharyngioma
Description:
(A) Craniopharyngioma[?]Craniopharyngioma:Craniopharyngioma are pituitary gland tumor develop from the embryogenic remnants in pituitary gland.Present at any time during childhood, tumor is congenital &d arises from squamous epithelial cell rests of the embryonic Rathke's pouch.The neoplasm is usually cystic & benign.Clinical presentation:Headaches and raised ICPVisual symptoms: 20% of children & 80% adults.Hormonal imbalances:Short stature and delayed puberty in childrenDecreased libido; Amenorrhoea; Diabetes insipidusBehavioural change due to frontal or temporal extension.Growth failure; Signs of increased intracranial pressure; Endocrine abnormalities such as diabetes insipidus and delayed puberty (<10% of cases); Retarded Bone age.X-ray of skull may show calcificationMost preferred treatment is micro surgical excision & transcranial route through craniotomy.Tumor cyst can be aspirated or malignant craniopharyngiomas are treated with radiotherapy or implants.Types of Non Cancerous Brain Tumors seen in ChildrenChordomasMeningiomasCraniopharyngiomaPineocytomasGangliocytomasPituitary AdenomaGlomus JugulareSchwannomasOther Options[?]Pituitary apoplexy:Pituitary apoplexy or pituitary tumor apoplexy is bleeding into or impaired blood supply of the pituitary gland at the base of the brain. This usually occurs in the presence of a tumor of the pituitary, although in 80% of cases this has not been diagnosed previously.The most common initial symptom is a sudden headache, often associated with a rapidly worsening visual field defect or double vision caused by compression of nerves surrounding the gland.This is followed in many cases by acute symptoms caused by lack of secretion of essential hormones, predominantly adrenal insufficiency[?]Nasopharyngeal fibroma:Nasopharyngeal fibroma is a benign tumour but locally invasive and destroys the adjoining structures. It may extend into: Nasal cavity causing nasal obstruction, epistaxis and nasal discharge.Cranial cavity: Middle cranial fossa is the most common.There are two routes of entry:-By erosion of floor of middle cranial fossa, anterior to foramen lacerum. The tumour lies lateral to carotid artery & cavernous sinus.-Through sphenoid sinus, into the sella. Tumour lies medial to carotid artery.Surgical excision is now the treatment of choice. Radiotherapy has been used as a primary mode of treatment.Hormonal therapy as the primary or adjunctive treatment.Recurrent and residual lesions have been treated by chemotherapy.[?]Hypothalamic glioma:Rare tumors cause diencephalic syndrome in infants.Fail to thrive, loss of subcutaneous fat & suffer sleep & respiratory disturbances.Precocious puberty seen in older children.Associated histological types include glioma, pinealoma, teratomas & hamartomas.
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