335 lines
30 KiB
Markdown
335 lines
30 KiB
Markdown
---
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title: "Hypothalamus Lesion"
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docid: "0fc29bff-9f20-4e19-a436-04c7791e3972"
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authors:
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- key: "318f80ab-6abb-4067-a809-2ebdaa5a30c9"
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value: "Kalen Riley, MD, MBA"
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- key: "f184750a-90b4-47a7-907b-23b05d70357a"
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value: "Chang Yueh Ho, MD"
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- key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850"
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value: "Anne G. Osborn, MD, FACR"
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breadcrumbs:
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-
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name: "Brain"
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slug: "brain"
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treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a"
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-
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name: "Differential Diagnosis"
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slug: "differential-diagnosis"
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treeNodeId: "a7fdd139-664e-4bb8-8d18-400e4733ff60"
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-
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name: "Sella/Juxtasellar, Pineal Region"
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slug: "sellajuxtasellar-pineal-region"
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treeNodeId: "5e38b9c1-3137-47e3-aa83-1fc82cb4099a"
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-
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name: "Anatomically Based Differentials"
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slug: "anatomically-based-differentials"
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treeNodeId: "7a51b2ca-8fee-4c16-aff3-b7189f68ea60"
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-
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name: "Hypothalamus Lesion"
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slug: "hypothalamus-lesion"
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treeNodeId: null
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category: "Brain"
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documentVersionId: "16e05176-4cdf-4ac0-a619-ad1f294263ce"
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imageCount: 38
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lastUpdated: "03/15/23"
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pageDescription: "Hypothalamus Lesion"
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pageKeywords: "Brain, Differential Diagnosis, Sella/Juxtasellar, Pineal Region, Anatomically Based Differentials, Hypothalamus Lesion"
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pageTitle: "Hypothalamus Lesion | STATdx"
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enhancedTitle: "Hypothalamus Lesion"
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type: "DDX"
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references: true
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breadcrumbs:
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- "Brain"
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- "Differential Diagnosis"
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- "Sella/Juxtasellar, Pineal Region"
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- "Anatomically Based Differentials"
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- "Hypothalamus Lesion"
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---
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# ESSENTIAL INFORMATION
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- ## Key Differential Diagnosis Issues
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- Anatomic essentials
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- Hypothalamus lies below level of anterior, posterior commissures
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- Inferior hypothalamus formed by
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- Anterior recesses of 3rd ventricle
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- Tuber cinereum
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- Mammillary bodies
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- Infundibular stalk
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- ## Helpful Clues for Common Diagnoses
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- **Astrocytoma**
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- Most common primary neoplasm arising from hypothalamic-optic chiasm structures
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- Usually low grade (pilocytic astrocytoma WHO grade1)
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- Age < 5 years
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- Endocrine dysfunction in 20%
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- Look for evidence for neurofibromatosis type 1
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- 20-50% of patients with pilocytic astrocytoma
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- **Craniopharyngioma**
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- **Adamantinomatous**
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- Most common suprasellar mass in children
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- Occurs anywhere from intrasellar to stalk to anteroinferior 3rd ventricle
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- 90% calcify, 90% have multiple cysts (mixed signal intensity), 90% calcify
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- **Papillary**
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- Occurs along hypothalamic-pituitary axis, often infundibulum and tuber cinereum of 3rd ventricle floor
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- Often solid, may be cystic and solid
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- ## Helpful Clues for Less Common Diagnoses
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- **Germ Cell Tumor**
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- Can be primary in hypothalamus/stalk
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- M = F (vs. male predominance in pineal gland)
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- 10% "double" midline lesions (pineal and hypothalamus)
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- Diabetes insipidus, diencephalic syndrome, precocious puberty common
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- Thick, enhancing stalk, 3rd floor, absent posterior pituitary bright spot
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- Pure germinoma demonstrates more homogeneous enhancement with rapid response to chemotherapy
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- Intermediate and mixed germ cell tumors are more heterogeneous without rapid treatment response
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- Mature teratomas may have calcification, fat, and other differentiated cell types (hair, teeth, etc.)
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- **Neurosarcoid**
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- Adult with stalk, meningeal lesions
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- Other infectious/inflammatory lesions that can mimic sarcoid
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- Granulomatosis with polyangiitis (GPA)
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- Tuberculosis, syphilis
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- **Langerhans Cell Histiocytosis**
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- Stalk/hypothalamus lesion in child
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- **Lipoma**
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- Lipoma: Sessile T1-hyperintense lesion on subpial surface of hypothalamus
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- Osteolipoma: Rare; fat-density/signal intensity and calcification
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- **Lymphocytic****H****y****pophysitis**
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- Peripartum female common; other etiologies include granulomatous, Ig-G4-related, drug-related, etc.
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- Can mimic macroadenoma
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- **Metastases**
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- **Hypothalamic-pituitary axis metastases**
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- 1-25% of systemic cancers at autopsy
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- Less common at imaging
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- Breast, lung most common primary tumors
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- **Lymphoma**
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- Pituitary/stalk/hypothalamus uncommon site
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- Can be primary or metastatic
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- **Tuber****C****inereum Hamartoma**
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- Children with gelastic seizures, males with isosexual precocious puberty
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- Can be pedunculated or sessile
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- Density/signal intensity typically isointense with cortex
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- No calcification, enhancement
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- Sessile lesion may be difficult to distinguish from hypothalamic astrocytoma (no change on follow-up)
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- **Ectopic Posterior Pituitary**
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- Failure of neurohypophysis to migrate from hypothalamus
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- T1-hyperintense lesion anywhere from hypothalamus through stalk
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- Associated with septo-optic dysplasia
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- **Dermoid Cyst**
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- Inclusion of surface ectoderm with neural tube closure
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- Includes fat: Fat-suppression MR techniques and negative HU on CT
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- May rupture with leptomeningeal fat deposits
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- Can cause chemical meningitis
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- No fat with decreased diffusion = epidermoid
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- **Rathke Cleft Cyst**
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- Like craniopharyngioma, arises from squamous cell rests migrating through craniopharyngeal duct, forming sellar/suprasellar mass
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- Nonenhancing cysts with central protein: Variable T1 hyperintensity and T2 hypointensity
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- When large, can involve hypothalamus
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- ## Helpful Clues for Rare Diagnoses
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- **Other****G****liomas**
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- **Chordoid g****lioma**
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- Floor of 3rd ventricle
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- Hyperintense with strong, uniform enhancement
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- **Pilomyxoid a****strocytoma**
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- Infant/young child
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- H-shaped tumor of hypothalamus; extension into medial temporal lobes common
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- Often large, bulky ± hemorrhage (rare in pilocytic astrocytoma)
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- WHO grade1, subtype of pilocytic astrocytoma
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- **Pituicytoma**
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- Stalk, posterior pituitary lobe
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- Low-grade astrocytoma
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- Enhances strongly, uniformly
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- **Ganglioglioma**
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- Very rare in hypothalamus/chiasm
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- Young adult (mean age = 20 years)
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- **Wernicke Encephalopathy**
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- Acute: Abnormal hyperintensity/enhancement of mammillary bodies, inferolateral walls of 3rd ventricle, periaqueductal gray matter
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- Chronic: Mammillary atrophy
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- Note: Occurs in both alcoholics, nonalcoholics (e.g., longstanding parenteral nutrition, hyperemesis, bariatric surgery)
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- **Demyelinating****D****isease**
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- Optic chiasm involvement > > hypothalamus
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- Enhancing, slightly enlarged optic nerves/chiasm seen with both multiple sclerosis, ADEM
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## References
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# Selected References
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1. [Pascual JM et al: Duct-like recess in the infundibular portion of third ventricle craniopharyngiomas: an MRI sign identifying the papillary type. AJNR Am J Neuroradiol. 43(9):1333-40, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35953277%5Bpmid%5D)
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1. [Tierney TS et al: Initial experience with magnetic resonance-guided focused ultrasound stereotactic surgery for central brain lesions in young adults. J Neurosurg. 1-8, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35171812%5Bpmid%5D)
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1. [Shields R et al: Magnetic resonance imaging of sellar and juxtasellar abnormalities in the paediatric population: an imaging review. Insights Imaging. 6(2):241-60, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25794595%5Bpmid%5D)
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1. [Zhang Y et al: Hypothalamus syndrome in opticospinal multiple sclerosis. AJNR Am J Neuroradiol. 32(8):E153-5, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21051517%5Bpmid%5D)
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1. [Hamilton BE et al: Anatomic and pathologic spectrum of pituitary infundibulum lesions. AJR Am J Roentgenol. 188(3):W223-32, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17312027%5Bpmid%5D)
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1. [Saleem SN et al: Lesions of the hypothalamus: MR imaging diagnostic features. Radiographics. 27(4):1087-108, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17620469%5Bpmid%5D)
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## Images
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### Selected Images
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**Astrocytoma**
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*Axial T2 MR shows a T2-hyperintense mass <img src='img/arrows/CS.png'/> centered in the hypothalamus and optic chiasm with associated cysts <img src='img/arrows/CO.png'/>. This was a pilocytic astrocytoma at resection. Low-grade astrocytomas typically have T2-hyperintense solid components.*
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**Astrocytoma**
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*Axial T2 MR shows a T2-hyperintense mass <img src='img/arrows/CS.png'/> centered in the hypothalamus and optic chiasm with associated cysts <img src='img/arrows/CO.png'/>. This was a pilocytic astrocytoma at resection. Low-grade astrocytomas typically have T2-hyperintense solid components.*
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**Astrocytoma**
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*Sagittal T1 C+ MR shows a lobular, intensely enhancing suprasellar mass involving the hypothalamus and optic chiasm <img src='img/arrows/CO.png'/>. A nonenhancing cyst <img src='img/arrows/CS.png'/> is seen anteriorly to the mass. This was a pilocytic astrocytoma, WHO grade 1 at resection.*
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**Craniopharyngioma**
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*Sagittal NECT shows a cystic mass <img src='img/arrows/CS.png'/> with a solid and densely calcified component <img src='img/arrows/CO.png'/> in the sella of this child. The mass involves the entire hypothalamic pituitary axis and extends into the 3rd ventricle.*
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**Craniopharyngioma**
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*Sagittal T1 C+ MR shows rim enhancement <img src='img/arrows/CS.png'/> of the cystic component of the suprasellar mass with heterogeneous enhancement of the solid component in the sella <img src='img/arrows/CO.png'/>. The solid portion showed dense calcification on a prior CT scan, consistent with adamantinomatous craniopharyngioma.*
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**Germ Cell Tumor**
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*Coronal T1 C+ FS MR shows a lobular, enhancing mass <img src='img/arrows/CS.png'/> centered in the hypothalamus and pituitary stalk. This was a germinoma at surgery. Suprasellar and pineal midline locations are the most common location for intracranial germinomas.*
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**Neurosarcoid**
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*Sagittal T1 C+ FS MR shows heterogeneous pial and parenchymal enhancement of the hypothalamus, anterior 3rd ventricle, lateral ventricles, and rostrum of the corpus callosum <img src='img/arrows/CS.png'/>. In addition, there is leptomeningeal enhancement <img src='img/arrows/CO.png'/> seen in the midline sulci.*
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**Langerhans Cell Histiocytosis**
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*Sagittal T1 C+ FS MR shows a heterogeneously enhancing hypothalamic mass <img src='img/arrows/CS.png'/> in a child with known Langerhans cell histiocytosis (LCH). This significantly decreased in size after treatment. An absent posterior pituitary "bright spot" is common in LCH.*
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**Lipoma**
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*Sagittal T1 MR shows an intrinsically T1- hyperintense hypothalamic/suprasellar mass, consistent with lipoma <img src='img/arrows/CS.png'/>. Fat-saturated images (not shown) demonstrated complete suppression of hyperintense signal.*
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**Lymphocytic Hypophysitis**
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*Sagittal T1 C+ FS MR in a postpartum female with lymphocytic hypophysitis shows an enhancing, heterogeneous lesion involving the pituitary infundibulum and anterior pituitary gland <img src='img/arrows/CS.png'/>. There is reactive thickening of adjacent meninges <img src='img/arrows/CO.png'/>.*
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**Metastases**
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*Sagittal T1 C+ FS MR shows a newly developed, avidly enhancing hypothalamic mass <img src='img/arrows/CS.png'/> in a patient with known metastatic genitourinary malignancy. Lung and breast cancer are the most common primary tumors to metastasize to the hypothalamic region.*
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**Tuber Cinereum Hamartoma**
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*Sagittal T1 C+ MR shows a nonenhancing mass arising from the tuber cinereum <img src='img/arrows/CS.png'/>, which follows brain parenchyma in intensity. In a child with gelastic seizures, this is a hypothalamic hamartoma.*
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**Ectopic Posterior Pituitary**
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*Coronal T1 MR shows a hyperintense lesion in the hypothalamus in this infant with hormonal abnormality. This is consistent with ectopic posterior pituitary <img src='img/arrows/CS.png'/>. Note the intact septum pellucidum <img src='img/arrows/CO.png'/>, which does not suggest septo-optic dysplasia. Ectopic posterior pituitary is often associated with septo-optic dysplasia.*
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**Dermoid Cyst**
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*Sagittal T1 MR shows a cystic lesion with T1 hyperintensity involving the sella and stalk <img src='img/arrows/CS.png'/>. This demonstrates loss of signal on fat saturation technique (not shown) and is consistent with a dermoid cyst confirmed at surgery. No leptomeningeal fatty deposits were seen to suggest rupture.*
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**Rathke Cleft Cyst**
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*Axial T2 FS MR shows a unilocular cyst involving the stalk and hypothalamus with a fluid-fluid level <img src='img/arrows/CS.png'/>. This was a Rathke cleft cyst at surgery. Fluid levels in a Rathke cleft cyst may be from layering protein or recent hemorrhage.*
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**Ganglioglioma**
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*Axial T1 C+ MR shows a heterogeneous, enhancing mass involving the chiasm, hypothalamus, and left optic tract <img src='img/arrows/CS.png'/>. A cyst <img src='img/arrows/CO.png'/> is seen associated with the mass with effacement of the left temporal horn <img src='img/arrows/CC.png'/>. This was a ganglioglioma at resection.*
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**Demyelinating Disease**
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*Sagittal FLAIR MR shows T2 hyperintensity and swelling involving the ependymal margins at the foramen of Monro <img src='img/arrows/CS.png'/> and hypothalamus/optic chiasm <img src='img/arrows/CO.png'/>. The patient later developed myelitis and was diagnosed with neuromyelitis optica.*
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### Additional Images
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**Metastases**
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*Sagittal T1 C+ MR shows a newly developed enhancing solid and cystic suprasellar and hypothalamic mass <img src='img/arrows/CS.png'/> in a patient with known metastatic lung cancer.*
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**Metastases**
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*Sagittal T1 C+ MR shows an enhancing, thickened infundibular stalk <img src='img/arrows/WS.png'/> in patient with known systemic cancer. This was the only intracranial lesion identified.*
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**Lipoma**
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*Sagittal T1 MR shows a lobulated, hyperintense lesion <img src='img/arrows/WS.png'/> extending posteriorly along the hypothalamus from the tuber cinereum to the mammillary bodies.*
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**Ectopic Posterior Pituitary**
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*Sagittal T1 C+ MR shows small pituitary fossa <img src='img/arrows/BO.png'/> with absent posterior pituitary "bright spot" and bulbous enlargement of infundibulum <img src='img/arrows/WC.png'/> at its origin from the hypothalamus.*
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**Demyelinating Disease**
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*Axial FLAIR MR shows multiple subcortical white matter hyperintensities <img src='img/arrows/WS.png'/> with hyperintense hypothalamus and optic tracts <img src='img/arrows/WO.png'/> in a child with ADEM.*
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**Astrocytoma**
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*Sagittal T2 MR shows classic pilocytic astrocytoma <img src='img/arrows/WS.png'/> originating from hypothalamus and optic chiasm. (Courtesy P. Rodriguez, MD).*
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**Astrocytoma**
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*Sagittal T1 C+ MR shows an inhomogeneously enhancing mass in the anterior 3rd ventricle, hypothalamus <img src='img/arrows/WS.png'/>.*
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**Craniopharyngioma**
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*Sagittal T1 MR shows large, hyperintense craniopharyngioma originating from the 3rd ventricle <img src='img/arrows/BS.png'/> and hypothalamus. Note sparing of the suprasellar cistern <img src='img/arrows/BO.png'/>.*
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**Germ Cell Tumor**
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*Sagittal T1 C+ MR in 13-year-old boy with central diabetes insipidus shows an enhancing mass in the anterior 3rd ventricle/hypothalamus <img src='img/arrows/WS.png'/> displacing the pituitary stalk <img src='img/arrows/WC.png'/> anteriorly.*
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**Neurosarcoid**
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*Sagittal T1 C+ MR shows an enhancing mass infiltrating the hypothalamus <img src='img/arrows/WS.png'/> and infundibular stalk. The patient is an adult who presented with diabetes insipidus (DI).*
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**Langerhans Cell Histiocytosis**
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*Sagittal T1 C+ MR in child with DI shows enhancing mass <img src='img/arrows/WS.png'/> infiltrating the hypothalamus, tuber cinereum, infundibular stalk, and pituitary gland.*
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**Lymphocytic Hypophysitis**
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*Sagittal T1 C+ MR shows enhancing mass in anterior 3rd ventricle, hypothalamus <img src='img/arrows/WS.png'/>. The pituitary stalk <img src='img/arrows/WO.png'/> is slightly thickened.*
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**Lymphoma**
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*Sagittal T1 C+ FS MR shows pituitary <img src='img/arrows/WS.png'/>, hypothalamic <img src='img/arrows/WO.png'/> masses in this patient with proven B-cell lymphoma.*
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**Tuber Cinereum Hamartoma**
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*Sagittal T2WI MR in a 12-year-old child with gelastic seizures shows a sessile hypothalamic mass <img src='img/arrows/WS.png'/> with a cyst <img src='img/arrows/WO.png'/>. No enhancement was seen on T1 C+ MR Variant cases may mimic astrocytoma.*
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**Chordoid Glioma**
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*Sagittal T1 MR in this 65-year-old patient shows an isointense hypothalamic/3rd ventricular mass <img src='img/arrows/WS.png'/> displacing and compressing optic chiasm <img src='img/arrows/WC.png'/>. Intense homogeneous enhancement was seen on T1 C+ study.*
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**Pilomyxoid Astrocytoma**
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*Sagittal T2 MR in a 3-year-old child with neurofibromatosis type 1 and diencephalic syndrome shows a large, hyperintense hypothalamic mass bulging into the anterior 3rd ventricle <img src='img/arrows/BS.png'/>.*
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**Pituicytoma**
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*Sagittal T1 C+ MR in a 22-year-old woman with hypopituitarism shows a large, enhancing hypothalamic/infundibular stalk mass <img src='img/arrows/WC.png'/>.*
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**Demyelinating Disease**
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*Sagittal FLAIR MR shows multifocal hyperintensities along the callososeptal interface and in the hemispheric white matter as well as optic chiasm/hypothalamus <img src='img/arrows/BO.png'/>. This is a known patient with multiple sclerosis.*
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**Wernicke Encephalopathy**
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*Axial FLAIR MR in a patient with longstanding hyperalimentation shows hyperintensity in mammillary bodies as well as periaqueductal gray matter <img src='img/arrows/WS.png'/>.*
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**Lipoma**
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*Axial T2 FS MR with fat saturation shows a hypointense hypothalamic mass <img src='img/arrows/CS.png'/>, which was hyperintense on noncontrast T1 images (not shown). Loss of signal with fat saturation is compatible with fatty composition in this hypothalamic lipoma.*
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**Metastases**
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*Coronal T1 C+ FS MR shows multiple heterogeneous masses, consistent with metastases in a patient with known lung cancer. A specific metastatic lesion involves the hypothalamus <img src='img/arrows/CS.png'/>. There is edema and midline shift <img src='img/arrows/CO.png'/> from another metastatic mass (not shown).*
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**Langerhans Cell Histiocytosis**
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*Sagittal T1 C+ FS MR shows enhancing mass of the stalk and pituitary gland extending to the hypothalamus <img src='img/arrows/CS.png'/>. In a child with diabetes insipidus, this is consistent with Langerhans cell histiocytosis. There is an incidental pineal cyst <img src='img/arrows/CO.png'/>.*
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