statdx/docs_md/articles/hypothalamus-lesion_0fc29bff-9f20-4e19-a436-04c7791e3972.md

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Hypothalamus Lesion	0fc29bff-9f20-4e19-a436-04c7791e3972	key value 318f80ab-6abb-4067-a809-2ebdaa5a30c9 Kalen Riley, MD, MBA key value f184750a-90b4-47a7-907b-23b05d70357a Chang Yueh Ho, MD key value 5cff4116-3654-4b3a-bb75-5ebe0b8c9850 Anne G. Osborn, MD, FACR	name slug treeNodeId Brain brain 6d8829f1-14d7-45af-8675-255189aa526a name slug treeNodeId Differential Diagnosis differential-diagnosis a7fdd139-664e-4bb8-8d18-400e4733ff60 name slug treeNodeId Sella/Juxtasellar, Pineal Region sellajuxtasellar-pineal-region 5e38b9c1-3137-47e3-aa83-1fc82cb4099a name slug treeNodeId Anatomically Based Differentials anatomically-based-differentials 7a51b2ca-8fee-4c16-aff3-b7189f68ea60 name slug treeNodeId Hypothalamus Lesion hypothalamus-lesion null	Brain	16e05176-4cdf-4ac0-a619-ad1f294263ce	38	03/15/23	Hypothalamus Lesion	Brain, Differential Diagnosis, Sella/Juxtasellar, Pineal Region, Anatomically Based Differentials, Hypothalamus Lesion	Hypothalamus Lesion | STATdx	Hypothalamus Lesion	DDX	true	Brain Differential Diagnosis Sella/Juxtasellar, Pineal Region Anatomically Based Differentials Hypothalamus Lesion

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title: "Hypothalamus Lesion" docid: "0fc29bff-9f20-4e19-a436-04c7791e3972" authors:

• key: "318f80ab-6abb-4067-a809-2ebdaa5a30c9" value: "Kalen Riley, MD, MBA"
• key: "f184750a-90b4-47a7-907b-23b05d70357a" value: "Chang Yueh Ho, MD"
• key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850" value: "Anne G. Osborn, MD, FACR" breadcrumbs:
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• name: "Differential Diagnosis" slug: "differential-diagnosis" treeNodeId: "a7fdd139-664e-4bb8-8d18-400e4733ff60"
• name: "Sella/Juxtasellar, Pineal Region" slug: "sellajuxtasellar-pineal-region" treeNodeId: "5e38b9c1-3137-47e3-aa83-1fc82cb4099a"
• name: "Anatomically Based Differentials" slug: "anatomically-based-differentials" treeNodeId: "7a51b2ca-8fee-4c16-aff3-b7189f68ea60"
• name: "Hypothalamus Lesion" slug: "hypothalamus-lesion" treeNodeId: null category: "Brain" documentVersionId: "16e05176-4cdf-4ac0-a619-ad1f294263ce" imageCount: 38 lastUpdated: "03/15/23" pageDescription: "Hypothalamus Lesion" pageKeywords: "Brain, Differential Diagnosis, Sella/Juxtasellar, Pineal Region, Anatomically Based Differentials, Hypothalamus Lesion" pageTitle: "Hypothalamus Lesion | STATdx" enhancedTitle: "Hypothalamus Lesion" type: "DDX" references: true breadcrumbs:
• "Brain"
• "Differential Diagnosis"
• "Sella/Juxtasellar, Pineal Region"
• "Anatomically Based Differentials"
• "Hypothalamus Lesion"

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ESSENTIAL INFORMATION

• Key Differential Diagnosis Issues

  • Anatomic essentials - Hypothalamus lies below level of anterior, posterior commissures - Inferior hypothalamus formed by - Anterior recesses of 3rd ventricle - Tuber cinereum - Mammillary bodies - Infundibular stalk

• Helpful Clues for Common Diagnoses

  • Astrocytoma - Most common primary neoplasm arising from hypothalamic-optic chiasm structures - Usually low grade (pilocytic astrocytoma WHO grade1) - Age < 5 years - Endocrine dysfunction in 20% - Look for evidence for neurofibromatosis type 1 - 20-50% of patients with pilocytic astrocytoma
  • Craniopharyngioma - Adamantinomatous - Most common suprasellar mass in children - Occurs anywhere from intrasellar to stalk to anteroinferior 3rd ventricle - 90% calcify, 90% have multiple cysts (mixed signal intensity), 90% calcify - Papillary - Occurs along hypothalamic-pituitary axis, often infundibulum and tuber cinereum of 3rd ventricle floor - Often solid, may be cystic and solid

• Helpful Clues for Less Common Diagnoses

  • Germ Cell Tumor - Can be primary in hypothalamus/stalk - M = F (vs. male predominance in pineal gland) - 10% "double" midline lesions (pineal and hypothalamus) - Diabetes insipidus, diencephalic syndrome, precocious puberty common - Thick, enhancing stalk, 3rd floor, absent posterior pituitary bright spot - Pure germinoma demonstrates more homogeneous enhancement with rapid response to chemotherapy - Intermediate and mixed germ cell tumors are more heterogeneous without rapid treatment response - Mature teratomas may have calcification, fat, and other differentiated cell types (hair, teeth, etc.)
  • Neurosarcoid - Adult with stalk, meningeal lesions - Other infectious/inflammatory lesions that can mimic sarcoid - Granulomatosis with polyangiitis (GPA) - Tuberculosis, syphilis
  • Langerhans Cell Histiocytosis - Stalk/hypothalamus lesion in child
  • Lipoma - Lipoma: Sessile T1-hyperintense lesion on subpial surface of hypothalamus - Osteolipoma: Rare; fat-density/signal intensity and calcification
  • LymphocyticHy****pophysitis - Peripartum female common; other etiologies include granulomatous, Ig-G4-related, drug-related, etc. - Can mimic macroadenoma
  • Metastases - Hypothalamic-pituitary axis metastases - 1-25% of systemic cancers at autopsy - Less common at imaging - Breast, lung most common primary tumors - Lymphoma - Pituitary/stalk/hypothalamus uncommon site - Can be primary or metastatic
  • TuberCinereum Hamartoma - Children with gelastic seizures, males with isosexual precocious puberty - Can be pedunculated or sessile - Density/signal intensity typically isointense with cortex - No calcification, enhancement - Sessile lesion may be difficult to distinguish from hypothalamic astrocytoma (no change on follow-up)
  • Ectopic Posterior Pituitary - Failure of neurohypophysis to migrate from hypothalamus - T1-hyperintense lesion anywhere from hypothalamus through stalk - Associated with septo-optic dysplasia
  • Dermoid Cyst - Inclusion of surface ectoderm with neural tube closure - Includes fat: Fat-suppression MR techniques and negative HU on CT - May rupture with leptomeningeal fat deposits - Can cause chemical meningitis - No fat with decreased diffusion = epidermoid
  • Rathke Cleft Cyst - Like craniopharyngioma, arises from squamous cell rests migrating through craniopharyngeal duct, forming sellar/suprasellar mass - Nonenhancing cysts with central protein: Variable T1 hyperintensity and T2 hypointensity - When large, can involve hypothalamus

• Helpful Clues for Rare Diagnoses

  • OtherGliomas - Chordoid g****lioma - Floor of 3rd ventricle - Hyperintense with strong, uniform enhancement - Pilomyxoid a****strocytoma - Infant/young child - H-shaped tumor of hypothalamus; extension into medial temporal lobes common - Often large, bulky ± hemorrhage (rare in pilocytic astrocytoma) - WHO grade1, subtype of pilocytic astrocytoma - Pituicytoma - Stalk, posterior pituitary lobe - Low-grade astrocytoma - Enhances strongly, uniformly - Ganglioglioma - Very rare in hypothalamus/chiasm - Young adult (mean age = 20 years)
  • Wernicke Encephalopathy - Acute: Abnormal hyperintensity/enhancement of mammillary bodies, inferolateral walls of 3rd ventricle, periaqueductal gray matter - Chronic: Mammillary atrophy - Note: Occurs in both alcoholics, nonalcoholics (e.g., longstanding parenteral nutrition, hyperemesis, bariatric surgery)
  • DemyelinatingDisease - Optic chiasm involvement > > hypothalamus - Enhancing, slightly enlarged optic nerves/chiasm seen with both multiple sclerosis, ADEM

References

Selected References

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
2. 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
3. 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
4. Zhang Y et al: Hypothalamus syndrome in opticospinal multiple sclerosis. AJNR Am J Neuroradiol. 32(8):E153-5, 2011
5. Hamilton BE et al: Anatomic and pathologic spectrum of pituitary infundibulum lesions. AJR Am J Roentgenol. 188(3):W223-32, 2007
6. Saleem SN et al: Lesions of the hypothalamus: MR imaging diagnostic features. Radiographics. 27(4):1087-108, 2007

Images

Selected Images

Astrocytoma Axial T2 MR shows a T2-hyperintense mass centered in the hypothalamus and optic chiasm with associated cysts . This was a pilocytic astrocytoma at resection. Low-grade astrocytomas typically have T2-hyperintense solid components.

Astrocytoma Axial T2 MR shows a T2-hyperintense mass centered in the hypothalamus and optic chiasm with associated cysts . This was a pilocytic astrocytoma at resection. Low-grade astrocytomas typically have T2-hyperintense solid components.

Astrocytoma Sagittal T1 C+ MR shows a lobular, intensely enhancing suprasellar mass involving the hypothalamus and optic chiasm . A nonenhancing cyst is seen anteriorly to the mass. This was a pilocytic astrocytoma, WHO grade 1 at resection.

Craniopharyngioma Sagittal NECT shows a cystic mass with a solid and densely calcified component in the sella of this child. The mass involves the entire hypothalamic pituitary axis and extends into the 3rd ventricle.

Craniopharyngioma Sagittal T1 C+ MR shows rim enhancement of the cystic component of the suprasellar mass with heterogeneous enhancement of the solid component in the sella . The solid portion showed dense calcification on a prior CT scan, consistent with adamantinomatous craniopharyngioma.

Germ Cell Tumor Coronal T1 C+ FS MR shows a lobular, enhancing mass 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.

Neurosarcoid 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 . In addition, there is leptomeningeal enhancement seen in the midline sulci.

Langerhans Cell Histiocytosis Sagittal T1 C+ FS MR shows a heterogeneously enhancing hypothalamic mass 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.

Lipoma Sagittal T1 MR shows an intrinsically T1- hyperintense hypothalamic/suprasellar mass, consistent with lipoma . Fat-saturated images (not shown) demonstrated complete suppression of hyperintense signal.

Lymphocytic Hypophysitis 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 . There is reactive thickening of adjacent meninges .

Metastases Sagittal T1 C+ FS MR shows a newly developed, avidly enhancing hypothalamic mass in a patient with known metastatic genitourinary malignancy. Lung and breast cancer are the most common primary tumors to metastasize to the hypothalamic region.

Tuber Cinereum Hamartoma Sagittal T1 C+ MR shows a nonenhancing mass arising from the tuber cinereum , which follows brain parenchyma in intensity. In a child with gelastic seizures, this is a hypothalamic hamartoma.

Ectopic Posterior Pituitary Coronal T1 MR shows a hyperintense lesion in the hypothalamus in this infant with hormonal abnormality. This is consistent with ectopic posterior pituitary . Note the intact septum pellucidum , which does not suggest septo-optic dysplasia. Ectopic posterior pituitary is often associated with septo-optic dysplasia.

Dermoid Cyst Sagittal T1 MR shows a cystic lesion with T1 hyperintensity involving the sella and stalk . 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.

Rathke Cleft Cyst Axial T2 FS MR shows a unilocular cyst involving the stalk and hypothalamus with a fluid-fluid level . This was a Rathke cleft cyst at surgery. Fluid levels in a Rathke cleft cyst may be from layering protein or recent hemorrhage.

Ganglioglioma Axial T1 C+ MR shows a heterogeneous, enhancing mass involving the chiasm, hypothalamus, and left optic tract . A cyst is seen associated with the mass with effacement of the left temporal horn . This was a ganglioglioma at resection.

Demyelinating Disease Sagittal FLAIR MR shows T2 hyperintensity and swelling involving the ependymal margins at the foramen of Monro and hypothalamus/optic chiasm . The patient later developed myelitis and was diagnosed with neuromyelitis optica.

Additional Images

Metastases Sagittal T1 C+ MR shows a newly developed enhancing solid and cystic suprasellar and hypothalamic mass in a patient with known metastatic lung cancer.

Metastases Sagittal T1 C+ MR shows an enhancing, thickened infundibular stalk in patient with known systemic cancer. This was the only intracranial lesion identified.

Lipoma Sagittal T1 MR shows a lobulated, hyperintense lesion extending posteriorly along the hypothalamus from the tuber cinereum to the mammillary bodies.

Ectopic Posterior Pituitary Sagittal T1 C+ MR shows small pituitary fossa with absent posterior pituitary "bright spot" and bulbous enlargement of infundibulum at its origin from the hypothalamus.

Demyelinating Disease Axial FLAIR MR shows multiple subcortical white matter hyperintensities with hyperintense hypothalamus and optic tracts in a child with ADEM.

Astrocytoma Sagittal T2 MR shows classic pilocytic astrocytoma originating from hypothalamus and optic chiasm. (Courtesy P. Rodriguez, MD).

Astrocytoma Sagittal T1 C+ MR shows an inhomogeneously enhancing mass in the anterior 3rd ventricle, hypothalamus .

Craniopharyngioma Sagittal T1 MR shows large, hyperintense craniopharyngioma originating from the 3rd ventricle and hypothalamus. Note sparing of the suprasellar cistern .

Germ Cell Tumor Sagittal T1 C+ MR in 13-year-old boy with central diabetes insipidus shows an enhancing mass in the anterior 3rd ventricle/hypothalamus displacing the pituitary stalk anteriorly.

Neurosarcoid Sagittal T1 C+ MR shows an enhancing mass infiltrating the hypothalamus and infundibular stalk. The patient is an adult who presented with diabetes insipidus (DI).

Langerhans Cell Histiocytosis Sagittal T1 C+ MR in child with DI shows enhancing mass infiltrating the hypothalamus, tuber cinereum, infundibular stalk, and pituitary gland.

Lymphocytic Hypophysitis Sagittal T1 C+ MR shows enhancing mass in anterior 3rd ventricle, hypothalamus . The pituitary stalk is slightly thickened.

Lymphoma Sagittal T1 C+ FS MR shows pituitary , hypothalamic masses in this patient with proven B-cell lymphoma.

Tuber Cinereum Hamartoma Sagittal T2WI MR in a 12-year-old child with gelastic seizures shows a sessile hypothalamic mass with a cyst . No enhancement was seen on T1 C+ MR Variant cases may mimic astrocytoma.

Chordoid Glioma Sagittal T1 MR in this 65-year-old patient shows an isointense hypothalamic/3rd ventricular mass displacing and compressing optic chiasm . Intense homogeneous enhancement was seen on T1 C+ study.

Pilomyxoid Astrocytoma 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 .

Pituicytoma Sagittal T1 C+ MR in a 22-year-old woman with hypopituitarism shows a large, enhancing hypothalamic/infundibular stalk mass .

Demyelinating Disease Sagittal FLAIR MR shows multifocal hyperintensities along the callososeptal interface and in the hemispheric white matter as well as optic chiasm/hypothalamus . This is a known patient with multiple sclerosis.

Wernicke Encephalopathy Axial FLAIR MR in a patient with longstanding hyperalimentation shows hyperintensity in mammillary bodies as well as periaqueductal gray matter .

Lipoma Axial T2 FS MR with fat saturation shows a hypointense hypothalamic mass , which was hyperintense on noncontrast T1 images (not shown). Loss of signal with fat saturation is compatible with fatty composition in this hypothalamic lipoma.

Metastases 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 . There is edema and midline shift from another metastatic mass (not shown).

Langerhans Cell Histiocytosis Sagittal T1 C+ FS MR shows enhancing mass of the stalk and pituitary gland extending to the hypothalamus . In a child with diabetes insipidus, this is consistent with Langerhans cell histiocytosis. There is an incidental pineal cyst .