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Lymphocytic Hypophysitis f30774c3-cbd0-4ab3-b3d1-e0574106db1f
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318f80ab-6abb-4067-a809-2ebdaa5a30c9 Kalen Riley, MD, MBA
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8d5254e9-8dda-478b-8f08-bdee97a32c79 Karen L. Salzman, MD, FACR
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Brain
Diagnosis
Anatomy-Based Diagnoses
Sella and Pituitary
Miscellaneous
Lymphocytic Hypophysitis

title: "Lymphocytic Hypophysitis" docid: "f30774c3-cbd0-4ab3-b3d1-e0574106db1f" authors:

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  • "Brain"
  • "Diagnosis"
  • "Anatomy-Based Diagnoses"
  • "Sella and Pituitary"
  • "Miscellaneous"
  • "Lymphocytic Hypophysitis"

KEY FACTS

  • Terminology

    • Lymphocytic hypophysitis (LH)
    • Synonyms: Adenohypophysitis, primary hypophysitis, stalkitis
    • Idiopathic inflammation of pituitary gland &/or stalk

  • Imaging

    • Thick stalk (> 2 mm + loss of normal "top to bottom" tapering)
    • ± enlarged pituitary gland
    • 75% show loss of posterior pituitary "bright spot"
    • Enhances intensely, uniformly
    • May have adjacent dural or sphenoid sinus mucosal thickening

  • Top Differential Diagnoses

    • Macroadenoma
    • Pituitary hyperplasia
    • Adolescent pituitary gland
    • Granulomatous disease
    • Ectopic posterior pituitary gland

  • Clinical Issues

    • Autoimmune, inflammatory disorder; other etiologies: Granulomatous disease, IgG4 disease or drug related
    • Peripartum woman with headache, multiple endocrine deficiencies
    • Middle-aged man with diabetes insipidus (lymphocytic infundibuloneurohypophysitis)
    • Mean in women = 35 years, men = 45 years
    • M:F = 1:8-9
    • Often self-limited
    • Unrecognized, untreated LH can result in death from panhypopituitarism
    • Conservative care (steroids, hormone replacement)

  • Diagnostic Checklist

    • LH can mimic pituitary adenoma

TERMINOLOGY

  • Abbreviations

    • Lymphocytic hypophysitis (LH)

  • Synonyms

    • Adenohypophysitis, primary hypophysitis, stalkitis

  • Definitions

    • Idiopathic inflammation of pituitary gland or stalk

IMAGING

  • General Features

    • Best diagnostic clue

      - Thick, nontapered stalk, ± pituitary mass

    • Location

      - Suprasellar, intrasellar

    • Size

      - Usually < 10 mm but may reach 2-3 cm

    • Morphology

      - Rounded pituitary gland with infundibulum that appears thickened, nontapering, or bulbous

  • MR Findings

    • T1WI

      - Thick stalk (> 2 mm + loss of normal tapering)
- ± enlarged pituitary gland
- 75% show loss of posterior pituitary "bright spot"

    • T2WI

      - Iso-/hypointense

    • T1WI C+

      - Enhances intensely, uniformly
- May see dural or sphenoid sinus mucosal thickening

  • Imaging Recommendations

    • Best imaging tool

      - MR

    • Protocol advice

      - MR: Precontrast thin-section (< 3 mm) sagittal, coronal T1 and T2
- Coronal dynamic T1 C+ (may show delayed pituitary enhancement)

DIFFERENTIAL DIAGNOSIS

  • Macroadenoma

    • Sellar and suprasellar mass
    • Sella turcica enlarged/eroded
    • Diabetes insipidus common in LH, rare with adenoma

  • Pituitary Hyperplasia

    • Stalk usually normal - In young female patients, late pregnancy/peripartum
    • May be seen with hypothyroidism, Addison disease, end-organ failure, neuroendocrine neoplasms

  • Adolescent Pituitary

    • Enlarged gland with uniform enhancement

  • Granulomatous Disease

    • Sarcoid, Langerhans cell histiocytosis (LCH), granulomatosis with polyangiitis; systemic disease often present

  • Pituitary "Dwarf"

    • Stalk may appear short and stubby

  • Ectopic Posterior Pituitary

    • Hyperintense focus at tuber cinereum or truncated stalk

PATHOLOGY

  • General Features

    • Etiology

      - Autoimmune, inflammatory disorder
- Other types of hypophysitis include
        - Granulomatous (sarcoid, LCH, infectious, etc.)
        - IgG4-related hypophysitis
        - Drug-related [cancer immunotherapy (e.g., ipilimumab)]

  • Gross Pathologic & Surgical Features

    • Diffusely enlarged stalk/pituitary gland

  • Microscopic Features

    • Acute - Dense infiltrate of B/T lymphocytes, plasma cells, occasionally eosinophils; ± lymphoid follicles - No granulomas, giant cells, or organisms; no neoplasm
    • Chronic may demonstrate extensive fibrosis

CLINICAL ISSUES

  • Presentation

    • Most common signs/symptoms

      - Headache, visual impairment

    • Clinical profile

      - Peripartum woman with headache, multiple endocrine deficiencies
- Middle-aged man with diabetes insipidus (lymphocytic infundibuloneurohypophysitis)

  • Demographics

    • Age

      - Mean in women = 35 years, men = 45 years

    • Sex

      - M:F = 1:8-9

    • Epidemiology

      - Rare (1-2% of sellar lesions)

  • Natural History & Prognosis

    • Often self-limited
    • Unrecognized, untreated LH can result in death from panhypopituitarism

  • Treatment

    • Conservative (steroids, hormone replacement)

DIAGNOSTIC CHECKLIST

  • Image Interpretation Pearls

    • LH can mimic pituitary adenoma

1c7008d4-15aa-4440-a8e1-29841f3caa42

References

Selected References

  1. Angelousi A et al: Hypophysitis (including IgG4 and immunotherapy). Neuroendocrinology. ePub, 2020
  2. Caranci F et al: Imaging findings in hypophysitis: a review. Radiol Med. 125(3):319-28, 2020
  3. Oguz SH et al: Clinical characteristics, management, and treatment outcomes of primary hypophysitis: a monocentric cohort. Horm Metab Res. 52(4):220-7, 2020
  4. Pal R et al: Co-occurrence of craniopharyngioma and IgG4-related hypophysitis: an epiphenomenon or a mere coincidence? World Neurosurg. 136:193-7, 2020
  5. Goulam-Houssein S et al: IgG4-related intracranial disease. Neuroradiol J. 32(1):29-35, 2019
  6. Mekki A et al: Machine learning defined diagnostic criteria for differentiating pituitary metastasis from autoimmune hypophysitis in patients undergoing immune checkpoint blockade therapy. Eur J Cancer. 119:44-56, 2019
  7. Snyders T et al: Ipilimumab-induced hypophysitis, a single academic center experience. Pituitary. 22(5):488-96, 2019
  8. Zhu Q et al: Clinical features, magnetic resonance imaging, and treatment experience of 20 patients with lymphocytic hypophysitis in a single center. World Neurosurg. 127:e22-9, 2019
  9. Alessandrino F et al: Multimodality imaging of endocrine immune related adverse events: a primer for radiologists. Clin Imaging. 50:96-103, 2018
  10. Faje A: Immunotherapy and hypophysitis: clinical presentation, treatment, and biologic insights. Pituitary. 19(1):82-92, 2016
  11. Imber BS et al: Hypophysitis: a single-center case series. Pituitary. 18(5):630-41, 2015
  12. Tauziede-Espariat A et al: The prevalence of IgG4-positive plasma cells in hypophysitis: a possible relationship to IgG4-related disease. Clin Neuropathol. 34(4):181-92, 2015
  13. Chodakiewitz Y et al: Ipilimumab treatment associated pituitary hypophysitis: clinical presentation and imaging diagnosis. Clin Neurol Neurosurg. 125:125-30, 2014
  14. Hunn BH et al: Idiopathic granulomatous hypophysitis: a systematic review of 82 cases in the literature. Pituitary. 17(4):357-65, 2014
  15. Hindocha A et al: Lymphocytic hypophysitis in males. J Clin Neurosci. 20(5):743-5, 2013
  16. De Bellis A et al: Involvement of hypothalamus autoimmunity in patients with autoimmune hypopituitarism: role of antibodies to hypothalamic cells. J Clin Endocrinol Metab. 97(10):3684-90, 2012
  17. Peruzzotti-Jametti L et al: Bilateral intracavernous carotid artery occlusion caused by invasive lymphocytic hypophysitis. J Stroke Cerebrovasc Dis. 21(8):918, 2012
  18. Mirocha S et al: T regulatory cells distinguish two types of primary hypophysitis. Clin Exp Immunol. 155(3):403-11, 2009
  19. Molitch ME et al: Lymphocytic hypophysitis. Horm Res. 68 Suppl 5:145-50, 2007
  20. Zak IT et al: Imaging of neurologic disorders associated with pregnancy and the postpartum period. Radiographics. 27(1):95-108, 2007
  21. Unlühizarci K et al: Distinct radiological and clinical appearance of lymphocytic hypophysitis. J Clin Endocrinol Metab. 86(5):1861-4, 2001
  22. Sato N et al: Hypophysitis: endocrinologic and dynamic MR findings. AJNR Am J Neuroradiol. 19(3):439-44, 1998
  23. Ahmadi J et al: Lymphocytic adenohypophysitis: contrast-enhanced MR imaging in five cases. Radiology. 195(1):30-4, 1995

Images

Selected Images

Sagittal graphic shows lymphocytic hypophysitis (LH). Note thickening of the infundibulum with loss of the normal "top to bottom" tapering as well as infiltration into the anterior lobe of the pituitary gland . Sagittal graphic shows lymphocytic hypophysitis (LH). Note thickening of the infundibulum with loss of the normal "top to bottom" tapering as well as infiltration into the anterior lobe of the pituitary gland .

Sagittal graphic shows lymphocytic hypophysitis (LH). Note thickening of the infundibulum with loss of the normal "top to bottom" tapering as well as infiltration into the anterior lobe of the pituitary gland . Sagittal graphic shows lymphocytic hypophysitis (LH). Note thickening of the infundibulum with loss of the normal "top to bottom" tapering as well as infiltration into the anterior lobe of the pituitary gland .

Sagittal graphic shows lymphocytic hypophysitis (LH). Note thickening of the infundibulum with loss of the normal "top to bottom" tapering as well as infiltration into the anterior lobe of the pituitary gland . Sagittal graphic shows lymphocytic hypophysitis (LH). Note thickening of the infundibulum with loss of the normal "top to bottom" tapering as well as infiltration into the anterior lobe of the pituitary gland .

Sagittal T1 C+ MR shows a prominent pituitary gland in a 37-year-old man with panhypopituitarism during treatment with ipilimumab for metastatic melanoma. The gland had doubled in size since MR 6 weeks prior. This drug-induced LH was related to a cancer immunotherapy. Sagittal T1 C+ MR shows a prominent pituitary gland in a 37-year-old man with panhypopituitarism during treatment with ipilimumab for metastatic melanoma. The gland had doubled in size since MR 6 weeks prior. This drug-induced LH was related to a cancer immunotherapy.

Sagittal T1 C+ MR shows a prominent pituitary gland in a 37-year-old man with panhypopituitarism during treatment with ipilimumab for metastatic melanoma. The gland had doubled in size since MR 6 weeks prior. This drug-induced LH was related to a cancer immunotherapy. Sagittal T1 C+ MR shows a prominent pituitary gland in a 37-year-old man with panhypopituitarism during treatment with ipilimumab for metastatic melanoma. The gland had doubled in size since MR 6 weeks prior. This drug-induced LH was related to a cancer immunotherapy.

Sagittal T1 C+ MR in a 36-year-old woman with diabetes insipidus shows marked thickening and enhancement of the pituitary stalk related to autoimmune LH. Absence of the posterior pituitary bright spot was also present. Sagittal T1 C+ MR in a 36-year-old woman with diabetes insipidus shows marked thickening and enhancement of the pituitary stalk related to autoimmune LH. Absence of the posterior pituitary bright spot was also present.

Sagittal T1 C+ MR in a 36-year-old woman with diabetes insipidus shows marked thickening and enhancement of the pituitary stalk related to autoimmune LH. Absence of the posterior pituitary bright spot was also present. Sagittal T1 C+ MR in a 36-year-old woman with diabetes insipidus shows marked thickening and enhancement of the pituitary stalk related to autoimmune LH. Absence of the posterior pituitary bright spot was also present.

Coronal T2 MR in a pregnant woman with visual changes and endocrine abnormalities shows a hyperintense sellar/suprasellar mass related to LH with superior displacement of the optic chiasm . Imaging mimics pituitary macroadenoma. Coronal T2 MR in a pregnant woman with visual changes and endocrine abnormalities shows a hyperintense sellar/suprasellar mass related to LH with superior displacement of the optic chiasm . Imaging mimics pituitary macroadenoma.

Coronal T2 MR in a pregnant woman with visual changes and endocrine abnormalities shows a hyperintense sellar/suprasellar mass related to LH with superior displacement of the optic chiasm . Imaging mimics pituitary macroadenoma. Coronal T2 MR in a pregnant woman with visual changes and endocrine abnormalities shows a hyperintense sellar/suprasellar mass related to LH with superior displacement of the optic chiasm . Imaging mimics pituitary macroadenoma.

Additional Images

Coronal T1 MR in a 19-year-old pregnant woman who developed onset of vision problems in the late 3rd trimester shows a large "figure of 8," intra- and suprasellar mass that elevates the optic chiasm . Coronal T1 MR in a 19-year-old pregnant woman who developed onset of vision problems in the late 3rd trimester shows a large "figure of 8," intra- and suprasellar mass that elevates the optic chiasm .

Coronal T1 MR in a 19-year-old pregnant woman who developed onset of vision problems in the late 3rd trimester shows a large "figure of 8," intra- and suprasellar mass that elevates the optic chiasm . Coronal T1 MR in a 19-year-old pregnant woman who developed onset of vision problems in the late 3rd trimester shows a large "figure of 8," intra- and suprasellar mass that elevates the optic chiasm .

Coronal T1 C+ FS MR in the same patient shows intense enhancement of the mass . Preoperative diagnosis was macroadenoma. LH was found at surgery. Coronal T1 C+ FS MR in the same patient shows intense enhancement of the mass . Preoperative diagnosis was macroadenoma. LH was found at surgery.

Coronal T1 C+ MR shows an enlarged, uniformly enhancing infundibulum in a peripartum woman with visual complaints and pituitary dysfunction. These are classic symptoms and findings of LH. Coronal T1 C+ MR shows an enlarged, uniformly enhancing infundibulum in a peripartum woman with visual complaints and pituitary dysfunction. These are classic symptoms and findings of LH.

Coronal T1 MR shows a sellar/suprasellar mass in a 42-year-old man with diabetes insipidus. Note the "figure of 8" configuration identical to macroadenoma. Biopsy proved LH. Coronal T1 MR shows a sellar/suprasellar mass in a 42-year-old man with diabetes insipidus. Note the "figure of 8" configuration identical to macroadenoma. Biopsy proved LH.

Coronal T1 C+ MR shows LH. Note that the mass enhances strongly and uniformly. The imaging appearance is indistinguishable from that of macroadenoma. Coronal T1 C+ MR shows LH. Note that the mass enhances strongly and uniformly. The imaging appearance is indistinguishable from that of macroadenoma.

Sagittal T1 C+ FS MR shows marked enlargement of the pituitary infundibulum related to infundibuloneurohypophysitis. Sagittal T1 C+ FS MR shows marked enlargement of the pituitary infundibulum related to infundibuloneurohypophysitis.

Sagittal T1 C+ FS MR in a 41-year-old peripartum woman with headache and multiple endocrine deficiencies demonstrates enlargement of the pituitary gland and infundibular stalk, classic MR findings for LH. The lesion resolved with corticosteroids and endocrine replacement. Sagittal T1 C+ FS MR in a 41-year-old peripartum woman with headache and multiple endocrine deficiencies demonstrates enlargement of the pituitary gland and infundibular stalk, classic MR findings for LH. The lesion resolved with corticosteroids and endocrine replacement.

Sagittal T1 C+ FS MR in a middle-aged man who presented with diabetes insipidus shows a slightly thickened infundibular stalk and an infiltrating enhancing lesion in the hypothalamus. Imaging diagnosis was sarcoidosis vs. LH. Biopsy confirmed LH. Sagittal T1 C+ FS MR in a middle-aged man who presented with diabetes insipidus shows a slightly thickened infundibular stalk and an infiltrating enhancing lesion in the hypothalamus. Imaging diagnosis was sarcoidosis vs. LH. Biopsy confirmed LH.

Sagittal T1 C+ FS MR in a 50-year-old patient with diabetes insipidus shows focal enlargement of the pituitary infundibulum related to lymphocytic infundibuloneurohypophysitis. Sagittal T1 C+ FS MR in a 50-year-old patient with diabetes insipidus shows focal enlargement of the pituitary infundibulum related to lymphocytic infundibuloneurohypophysitis.

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