This is a MRI of a brain of a 10 year old girl who admitted to the hospital who attended  puberty 2months before,now complaining of dull and diffuse type headache associated with visual impairement.And growth retardation also noted in this Child.
What is the probable diagnosis in this girl?
What are the complications and possible outcome of this ?
 ANSWER - Craniopharyngioma

Benign, cystic tumours, usually found above the sella turcica thought to arise from remnants of Rathke's pouch.Also known as Rathke pouch tumors or hypophyseal duct tumors.It is common in children.

CLINICAL FEATURES
  1. Very slow growing tumors
  2. Visual disturbance -  bitemporal inferior quadrantinopia progressing to bitemporal hemianopia
  3. Hypothalamic pituitary dysfunction - panhypopituitarism, diabetes insipidus
  4. Hydrocephalus due to obstruction of CSF outflow at the third ventricle - headache, papilloedema and visual impairment
  5. Mild to severe dementia - due to frontal expansion

INVESTIGATIONS
Plain skull radiology - calcification in or above the pituitary fossa is seen in 80% of cases
CT scan
MRI scan
Pituitary stimulation test
Histology-  nesting of squamous epithelium,calcium deposition,microscopic papillary architecture

TREATMENT
  • Drainage of the cystic areas followed  external irradiation
  • Partial removal of tumour followed by radiotherapy
  • Total tumour excision with radiotherapy
  • Subfrontal or transsphenoidal Route

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4 comments

  1. Anonymous Says:
  2. judging of the position this is likely to be a craniopharyngioma, as it lies in the sellar region.Also the age of te girl is highly indicative of sych tumors.Also high in the list of differential diagnosis is adenoma of the pituitary gland,as the growth retardation indicates.

     
  3. Anonymous Says:
  4. What about perinauds's syndrome? it is a tumor of pineal gland that causes loss of pupillary reaction, ataxia, headache due to hydrocephalus and some secrete HCG that causes precocious puberty.

     
  5. linfo Says:
  6. Adenoma ipofisy with a reduction of sexual ormon (puberty) for compression and visual impairment (optical chiasma)and headache for meningeal stretch.

     
  7. Anonymous Says:
  8. Pituitary adenoma? Probably secreting gonadotrophins and leading to precocious puberty. Mass effect leading to compression of optic chiasma (therefore, visual impairment), and headache. Growth hormone secretion suppression.

     

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