Answer for BIR CoW 03 Sep 2023
CRANIOPHARYNGIOMA
Findings
*Well defined cystic lesion with peripheral rim of calcification noted in sella and suprasellar region . * The lesion causes mass effect over thalamus,hypothalamus and 3rd ventricle. *The lesion causes obstructive hydrocephalous . IMPRESSION: Suggestive of Adamantinomatous Craniopharyngioma.
Discussion
Craniopharyngiomas are a specific kind of brain tumor originating from leftover cells of the craniopharyngeal duct or Rathke's pouch. Although they are generally categorized as benign and fall under the World Health Organization's Grade 1 classification, they have a clinically aggressive nature, marked by their proclivity for recurrence.
These tumors predominantly appear in the suprasellar region of the brain and may also affect surrounding structures like the hypothalamus, optic chiasm, cranial nerves, third ventricles, and major blood vessels.
Both children and adults can develop craniopharyngiomas. In terms of malignancy, these tumors rarely undergo malignant transformation but when they do, the outlook is generally bleak.
Types of Craniopharyngioma
There are two primary subtypes: papillary and adamantinomatous.
CT Scans
- The tumor often displays a mix of cystic and solid elements, with variable density.
- About 90% of these lesions feature peripheral calcifications.
- Changes to the skull base, such as an enlarged sella turcica or eroded dorsum sellae, may be observable.
MRI Findings
- T1-weighted scans often reveal cystic parts ranging from isointense to hyperintense compared to brain tissue.
- Hyperintense regions are generally indicative of high protein, cholesterol granules, and methemoglobin content.
- Post-contrast images often show strong enhancement of solid parts and a more subtle rim-like enhancement for cystic areas.
- T2-weighted scans generally reveal mixed hyperintensity.
- Magnetic Resonance Angiography (MRA) can be particularly useful for assessing the tumor's relationship to the internal carotid artery and nearby structures.
Subtypes
Adamantinomatous (90%)
- Primarily affects children.
- Most common in ages 5-14 and 50-74.
- Characterized by cystic structure, calcification, and rim enhancement.
Papillary (10%)
- Mostly found in adults, predominantly in their 50s.
- More likely to be a solid mass with minor cystic components.
- Minimal enhancement is typical.
Differential Diagnoses
- Giant Craniopharyngioma (adamantinomatous subtype), exceeding 5-6 cm, often accompanied by obstructive hydrocephalus.
- Germinoma.
- Dermoid cyst.
- Pituitary macroadenoma, especially those with cystic degeneration or necrosis.
- Teratoma.
REFERENCES:
*Ahmadi J, Destian S, Apuzzo ML, Segall HD, Zee CS. Cystic fluid in craniopharyngiomas : MR imaging and quantitative analysis. Radiology. 1992;182:783–785
*. Curran JG, O'Connor E. Imaging of craniopharyngioma. Childs Nerv Syst. 2005;21:635–639.
*. Eldevik OP, Blaivas M, Gabrielsen TO, Hald JK, Chandler WF. Craniopharyngioma : radiologic and histologic findings and recurrence. AJNR Am J Neuroradiol. 1996;17:1427–2439.
* Sartoretti-Schefer S, Wichmann W, Aguzzi A, Valavanis A. MR differentiation of adamantinous and squamous-papillary craniopharyngiomas. AJNR Am J Neuroradiol. 1997;18:77–87.
Findings
*Well defined cystic lesion with peripheral rim of calcification noted in sella and suprasellar region . * The lesion causes mass effect over thalamus,hypothalamus and 3rd ventricle. *The lesion causes obstructive hydrocephalous . IMPRESSION: Suggestive of Adamantinomatous Craniopharyngioma.
Discussion
Craniopharyngiomas are a specific kind of brain tumor originating from leftover cells of the craniopharyngeal duct or Rathke's pouch. Although they are generally categorized as benign and fall under the World Health Organization's Grade 1 classification, they have a clinically aggressive nature, marked by their proclivity for recurrence.
These tumors predominantly appear in the suprasellar region of the brain and may also affect surrounding structures like the hypothalamus, optic chiasm, cranial nerves, third ventricles, and major blood vessels.
Both children and adults can develop craniopharyngiomas. In terms of malignancy, these tumors rarely undergo malignant transformation but when they do, the outlook is generally bleak.
Types of Craniopharyngioma
There are two primary subtypes: papillary and adamantinomatous.
CT Scans
- The tumor often displays a mix of cystic and solid elements, with variable density.
- About 90% of these lesions feature peripheral calcifications.
- Changes to the skull base, such as an enlarged sella turcica or eroded dorsum sellae, may be observable.
MRI Findings
- T1-weighted scans often reveal cystic parts ranging from isointense to hyperintense compared to brain tissue.
- Hyperintense regions are generally indicative of high protein, cholesterol granules, and methemoglobin content.
- Post-contrast images often show strong enhancement of solid parts and a more subtle rim-like enhancement for cystic areas.
- T2-weighted scans generally reveal mixed hyperintensity.
- Magnetic Resonance Angiography (MRA) can be particularly useful for assessing the tumor's relationship to the internal carotid artery and nearby structures.
Subtypes
Adamantinomatous (90%)
- Primarily affects children.
- Most common in ages 5-14 and 50-74.
- Characterized by cystic structure, calcification, and rim enhancement.
Papillary (10%)
- Mostly found in adults, predominantly in their 50s.
- More likely to be a solid mass with minor cystic components.
- Minimal enhancement is typical.
Differential Diagnoses
- Giant Craniopharyngioma (adamantinomatous subtype), exceeding 5-6 cm, often accompanied by obstructive hydrocephalus.
- Germinoma.
- Dermoid cyst.
- Pituitary macroadenoma, especially those with cystic degeneration or necrosis.
- Teratoma.
REFERENCES:
*Ahmadi J, Destian S, Apuzzo ML, Segall HD, Zee CS. Cystic fluid in craniopharyngiomas : MR imaging and quantitative analysis. Radiology. 1992;182:783–785
*. Curran JG, O'Connor E. Imaging of craniopharyngioma. Childs Nerv Syst. 2005;21:635–639.
*. Eldevik OP, Blaivas M, Gabrielsen TO, Hald JK, Chandler WF. Craniopharyngioma : radiologic and histologic findings and recurrence. AJNR Am J Neuroradiol. 1996;17:1427–2439.
* Sartoretti-Schefer S, Wichmann W, Aguzzi A, Valavanis A. MR differentiation of adamantinous and squamous-papillary craniopharyngiomas. AJNR Am J Neuroradiol. 1997;18:77–87.
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!