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title docid authors breadcrumbs category cmeTopicId documentVersionId imageCount lastUpdated pageDescription pageKeywords pageTitle enhancedTitle type references breadcrumbs
Pituicytoma d6e481d5-7742-4354-8dfe-4f04286a709a
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8d5254e9-8dda-478b-8f08-bdee97a32c79 Karen L. Salzman, MD, FACR
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Brain a6bbed04-5608-4dc1-9211-e998fcab6319 8ea88363-a87f-40aa-9884-8274bb644e98 11 08/05/20 Pituicytoma Brain, Diagnosis, Anatomy-Based Diagnoses, Sella and Pituitary, Neoplasms, Pituicytoma Pituicytoma | STATdx Pituicytoma DX true
Brain
Diagnosis
Anatomy-Based Diagnoses
Sella and Pituitary
Neoplasms
Pituicytoma

title: "Pituicytoma" docid: "d6e481d5-7742-4354-8dfe-4f04286a709a" authors:

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  • "Brain"
  • "Diagnosis"
  • "Anatomy-Based Diagnoses"
  • "Sella and Pituitary"
  • "Neoplasms"
  • "Pituicytoma"

KEY FACTS

  • Terminology

    • Rare tumor arising from pituicytes, specialized glial cells in neurohypophysis and infundibulum
    • Part of 2017 WHO spectrum of thyroid transcription factor-1 (TTF-1) expressing pituitary tumors of posterior lobe

  • Imaging

    • Enhancing sellar or suprasellar mass arising from neurohypophysis or infundibulum
    • Location is typically suprasellar (infundibular); pure intrasellar less common
    • MR: Isointense to hypointense solid mass - Posterior pituitary "bright spot" often absent - Variable enhancement, typically strong and uniform
    • Best imaging tool: High-resolution MR of sella  - Sagittal and coronal T1, coronal T2, postcontrast sagittal, and coronal T1 with fat saturation

  • Top Differential Diagnoses

    • Pituitary adenoma
    • Lymphocytic hypophysitis
    • Pituitary hyperplasia
    • Granular cell tumor

  • Pathology

    • WHO grade 1
    • Well-demarcated, round or oval solid mass
    • Hypervascular tumor at surgery

  • Clinical Issues

    • Visual and endocrine dysfunction common symptoms
    • Surgical resection: Primary therapy; transsphenoidal approach difficult given tumor vascularity

  • Diagnostic Checklist

    • If mass is present posteriorly within gland, intrasellar but separate from gland, or involves stalk, consider pituicytoma

TERMINOLOGY

  • Synonyms

    • Less preferred terms include infundibuloma and posterior pituitary astrocytoma

  • Definitions

    • Rare tumor arising from pituicytes, specialized glial cells in neurohypophysis and infundibulum
    • Part of 2017 WHO spectrum of thyroid transcription factor-1 (TTF-1) expressing pituitary tumors of posterior lobe

IMAGING

  • General Features

    • Best diagnostic clue

      - Enhancing sellar or suprasellar mass arising from neurohypophysis or infundibulum

    • Location

      - Typically suprasellar (infundibular); pure intrasellar less common

    • Size

      - Variable (1-2 mm to 4 cm)

    • Morphology

      - Well-demarcated, round or oval solid mass

  • CT Findings

    • NECT

      - Hyperdense sellar &/or suprasellar mass; Ca⁺⁺ rare

    • CECT

      - Homogeneously enhancing

  • MR Findings

    • T1WI

      - Isointense to hypointense solid mass
- Posterior pituitary "bright spot" often absent

    • T2WI

      - Heterogeneously hypo- to isointense

    • T1WI C+

      - Variable enhancement, typically strong and uniform

  • Imaging Recommendations

    • Best imaging tool

      - High-resolution MR of sella (2.5- to 3-mm slice thickness)

    • Protocol advice

      - Sagittal and coronal T1, coronal T2, postcontrast sagittal and coronal T1 with fat saturation through sella

DIFFERENTIAL DIAGNOSIS

  • Pituitary Adenoma

    • May be indistinguishable; typically anterior gland
    • Sellar &/or suprasellar enhancing mass

  • Lymphocytic Hypophysitis

    • May involve gland (anterior > posterior) or stalk
    • Typically pregnant or peripartum females
    • Autoimmune, inflammatory disorder most common
    • May relate to granulomatous disease, IgG4-related or drug-related

  • Pituitary Hyperplasia

    • Typically diffuse gland enlargement
    • May relate to hypothyroidism, Addison disease, end-organ failure, neuroendocrine neoplasms

  • Metastasis

    • Primary tumor often known; multiple lesions common

  • Granular Cell Tumor

    • Glial neoplasm originating in neurohypophysis or infundibulum
    • May be indistinguishable

  • Spindle Cell Oncocytoma

    • Rare nonneuroendocrine tumor of pituitary gland
    • Sellar and suprasellar mass
    • May be indistinguishable

PATHOLOGY

  • General Features

    • Etiology

      - Arise from pituicytes in neurohypophysis or infundibulum
- Part of 2017 WHO spectrum of TTF-1 expressing posterior lobe pituitary tumors
- Low-grade, nonneuroendocrine tumors of sella
- Hypothesis: These nonneuroendocrine pituitary tumors would originate from specific morphological variants of pituicytes 
        - Pituicytomas from light/major variant, granular cell tumors from granular variant, spindle cell oncocytomas from oncocytic variant, sellar ependymomas from ependymal pituicytes

  • Staging, Grading, & Classification

    • WHO grade 1

  • Gross Pathologic & Surgical Features

    • Well-circumscribed, soft to firm mass - Necrosis and cystic degeneration are uncommon - Rarely, tumor infiltrates surrounding structures, including optic chiasm and cavernous sinus
    • Hypervascular tumor at surgery

  • Microscopic Features

    • Elongate, bipolar spindle cells with abundant cytoplasm
    • Perivascular lymphocytic aggregates common
    • Vimentin (+), S100(+), GFAP(+), TTF-1(+)

CLINICAL ISSUES

  • Presentation

    • Most common signs/symptoms

      - Visual and endocrine dysfunction common
        - Headache, amenorrhea, galactorrhea, decreased libido, infertility, diabetes insipidus, hypopituitarism
- May be asymptomatic

  • Demographics

    • Age

      - Peak incidence: 5th decade
- No cases reported in patients < 20 years old

    • Sex

      - M:F = 1.6:1

    • Epidemiology

      - Rare (~ 135 symptomatic cases reported)

  • Natural History & Prognosis

    • Benign, slow-growing tumor
    • Regrowth after subtotal resection not uncommon

  • Treatment

    • Surgical resection: Primary therapy; transsphenoidal approach difficult given tumor vascularity
    • Inconsistent data regarding radiation therapy
    • Preoperative embolization may be helpful

DIAGNOSTIC CHECKLIST

  • Consider

    • If mass is present posteriorly within gland, intrasellar but separate from gland, or involves stalk, consider pituicytoma

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References

Selected References

  1. Borg A et al: Tumors of the neurohypophysis: one unit's experience and literature review. World Neurosurg. 134:e968-78, 2020
  2. McNamara KJ et al: A recurrent case of pituicytoma 16 years later. Ann R Coll Surg Engl. 1-3, 2020
  3. Cole TS et al: Rare Thyroid Transcription Factor 1-Positive Tumors of the Sellar Region: Barrow Neurological Institute Retrospective Case Series. World Neurosurg. 129:e294-e302, 2019
  4. Gezer E et al: The coexistence of infundibular pituicytoma and Cushing's disease due to pituitary adenoma: A case report. Endocr Regul. 53(4):263-267, 2019
  5. Guerrero-Pérez F et al: Primary tumors of the posterior pituitary: A systematic review. Rev Endocr Metab Disord. 20(2):219-238, 2019
  6. Guerrero-Pérez F et al: Posterior pituitary tumours: the spectrum of a unique entity. A clinical and histological study of a large case series. Endocrine. 63(1):36-43, 2019
  7. Marco Del Pont F et al: Pituicytoma Associated with Acromegaly and Cushing Disease. World Neurosurg. 136:78-82, 2019
  8. Lefevre E et al: Management of pituicytomas: a multicenter series of eight cases. Pituitary. ePub, 2018
  9. Shibuya M: Welcoming the new WHO classification of pituitary tumors 2017: revolution in TTF-1-positive posterior pituitary tumors. Brain Tumor Pathol. 35(2):62-70, 2018
  10. Lopes MBS: The 2017 World Health Organization classification of tumors of the pituitary gland: a summary. Acta Neuropathol. 134(4):521-535, 2017
  11. Mete O et al: Overview of the 2017 WHO Classification of Pituitary Tumors. Endocr Pathol. 21(5): 507-14, 2018
  12. Zygourakis CC et al: Pituicytomas and spindle cell oncocytomas: modern case series from the University of California, San Francisco. Pituitary. 18(1):150-8, 2015
  13. Feng M et al: Surgical management of pituicytomas: case series and comprehensive literature review. Pituitary. 17(5):399-413, 2014
  14. Teti C et al: Pituitary image: pituicytoma. Pituitary. ePub, 2014
  15. Kleinschmidt-DeMasters BK et al: Update on hypophysitis and TTF-1 expressing sellar region masses. Brain Pathol. 23(5):495-514, 2013
  16. Secci F et al: Pituicytomas: radiological findings, clinical behavior and surgical management. Acta Neurochir (Wien). 154(4):649-57; discussion 657, 2012
  17. Covington MF et al: Pituicytoma, spindle cell oncocytoma, and granular cell tumor: clarification and meta-analysis of the world literature since 1893. AJNR Am J Neuroradiol. 32(11):2067-72, 2011
  18. Brat DJ et al: Newly codified glial neoplasms of the 2007 WHO Classification of Tumours of the Central Nervous System: angiocentric glioma, pilomyxoid astrocytoma and pituicytoma. Brain Pathol. 17(3):319-24, 2007
  19. Louis DN et al: The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol. 2007 Aug;114(2):97-109. Epub 2007 Jul 6. Review. Erratum in: Acta Neuropathol. 114(5):547, 2007
  20. Wesseling et al: Pituicytoma. In Louis DN et al (eds): WHO Classification of Tumours of the Central Nervous System. Lyon, France: IARC Press. 243-4, 2007
  21. Benveniste RJ et al: Pituicytoma presenting with spontaneous hemorrhage. Pituitary. 9(1):53-8, 2006
  22. Gibbs WN et al: Pituicytoma: diagnostic features on selective carotid angiography and MR imaging. AJNR Am J Neuroradiol. 27(8):1639-42, 2006
  23. Takei H et al: Pituicytoma incidentally found at autopsy. Pathol Int. 55(11):745-9, 2005
  24. Ulm AJ et al: Pituicytoma: report of two cases and clues regarding histogenesis. Neurosurgery. 2004 Mar;54(3):753-7; discussion 757-8. Erratum in: Neurosurgery. 54(5):1252, 2004
  25. Katsuta T et al: Distinctions between pituicytoma and ordinary pilocytic astrocytoma. Case report. J Neurosurg. 98(2):404-6, 2003
  26. Figarella-Branger D et al: Pituicytomas, a mis-diagnosed benign tumor of the neurohypophysis: report of three cases. Acta Neuropathol. 104(3):313-9, 2002
  27. Brat DJ et al: Pituicytoma: a distinctive low-grade glioma of the neurohypophysis. Am J Surg Pathol. 24(3):362-8, 2000
  28. WHO classification of tumours of the central nervous system, revised WK Cavenee, S Leung, C Hawkins, EG Van Meir… - World Health Organization, 2016

Images

Selected Images

Sagittal T1 C+ MR shows a diffusely enhancing infundibular mass in this 68-year-old woman with hypopituitarism. Pituicytomas are WHO grade I tumors with nuclear expression of TTF-1 that arise in the neurohypophysis or infundibulum. Sagittal T1 C+ MR shows a diffusely enhancing infundibular mass in this 68-year-old woman with hypopituitarism. Pituicytomas are WHO grade I tumors with nuclear expression of TTF-1 that arise in the neurohypophysis or infundibulum.

Sagittal T1 C+ MR shows a diffusely enhancing infundibular mass in this 68-year-old woman with hypopituitarism. Pituicytomas are WHO grade I tumors with nuclear expression of TTF-1 that arise in the neurohypophysis or infundibulum. Sagittal T1 C+ MR shows a diffusely enhancing infundibular mass in this 68-year-old woman with hypopituitarism. Pituicytomas are WHO grade I tumors with nuclear expression of TTF-1 that arise in the neurohypophysis or infundibulum.

Sagittal graphic shows a pituicytoma involving the infundibular stalk and neurohypophysis. A lobular suprasellar mass without significant compression of the adjacent structures is typical for this rare, low-grade, spindle cell glial neoplasm. Sagittal graphic shows a pituicytoma involving the infundibular stalk and neurohypophysis. A lobular suprasellar mass without significant compression of the adjacent structures is typical for this rare, low-grade, spindle cell glial neoplasm.

Sagittal T1 MR shows a well-delineated suprasellar mass extending into the posterior sella. Note the lack of a normal pituitary "bright spot," a common feature of pituicytoma. Sagittal T1 MR shows a well-delineated suprasellar mass extending into the posterior sella. Note the lack of a normal pituitary "bright spot," a common feature of pituicytoma.

Coronal T1 C+ MR in the same patient shows strong uniform enhancement of this large pituicytoma. Such enhancement is typical of these rare tumors of the neurohypophysis or infundibulum. These vascular tumors are WHO grade 1. (Courtesy A.V. Hasso, MD.) Coronal T1 C+ MR in the same patient shows strong uniform enhancement of this large pituicytoma. Such enhancement is typical of these rare tumors of the neurohypophysis or infundibulum. These vascular tumors are WHO grade 1. (Courtesy A.V. Hasso, MD.)

Additional Images

Axial T1 C+ MR in the same patient shows strong, uniform enhancement of the pituicytoma, which is common in these tumors. True pituicytomas are rare tumors that arise from pituicytes in the posterior pituitary gland or infundibular stalk. Pituicytes are specialized glial cells of the posterior lobe and stalk. Axial T1 C+ MR in the same patient shows strong, uniform enhancement of the pituicytoma, which is common in these tumors. True pituicytomas are rare tumors that arise from pituicytes in the posterior pituitary gland or infundibular stalk. Pituicytes are specialized glial cells of the posterior lobe and stalk.

Sagittal T1 C+ MR shows a diffusely enhancing infundibular mass in this 22-year-old woman with delayed growth and hypopituitarism. After stable imaging over 5 years, this was presumed to be a pituicytoma. Sagittal T1 C+ MR shows a diffusely enhancing infundibular mass in this 22-year-old woman with delayed growth and hypopituitarism. After stable imaging over 5 years, this was presumed to be a pituicytoma.

Sagittal T1 MR shows a suprasellar mass in a 35 year old with visual changes. The hyperintensity is atypical. Note the lack of a normal posterior "bright spot." Sagittal T1 MR shows a suprasellar mass in a 35 year old with visual changes. The hyperintensity is atypical. Note the lack of a normal posterior "bright spot."

Sagittal T1 C+ MR in the same patient shows diffuse enhancement of the suprasellar mass, similar to the adjacent pituitary gland. Biopsy proved pituicytoma. (Courtesy B. Chong, MD.) Sagittal T1 C+ MR in the same patient shows diffuse enhancement of the suprasellar mass, similar to the adjacent pituitary gland. Biopsy proved pituicytoma. (Courtesy B. Chong, MD.)

H&E histopathology shows the typical benign features of a pituicytoma with bipolar spindle cells with abundant eosinophilic cytoplasm. Nuclear atypia and mitoses are absent. GFAP (not shown) showed strong staining, indicating the astrocytic origin of the tumor. H&E histopathology shows the typical benign features of a pituicytoma with bipolar spindle cells with abundant eosinophilic cytoplasm. Nuclear atypia and mitoses are absent. GFAP (not shown) showed strong staining, indicating the astrocytic origin of the tumor.

Coronal T1 C+ MR shows a diffusely enhancing infundibular mass in this 68-year-old woman with hypopituitarism. Pituicytomas are WHO grade I tumors with nuclear expression of TTF1 that arise in the neurohypophysis or infundibulum. Coronal T1 C+ MR shows a diffusely enhancing infundibular mass in this 68-year-old woman with hypopituitarism. Pituicytomas are WHO grade I tumors with nuclear expression of TTF1 that arise in the neurohypophysis or infundibulum.

Axial T1 C+ MR in the same patient shows a diffusely enhancing infundibular mass . Imaging mimics lymphocytic hypophysitis of the stalk (infundibuloneurohypophysitis). However, lymphocytic hypophysitis usually occurs in postpartum women or in patients with other endocrine abnormalities, commonly pituitary insufficiency or diabetes insipidus. Axial T1 C+ MR in the same patient shows a diffusely enhancing infundibular mass . Imaging mimics lymphocytic hypophysitis of the stalk (infundibuloneurohypophysitis). However, lymphocytic hypophysitis usually occurs in postpartum women or in patients with other endocrine abnormalities, commonly pituitary insufficiency or diabetes insipidus.

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