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8d5254e9-8dda-478b-8f08-bdee97a32c79 Karen L. Salzman, MD, FACR
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Brain
Differential Diagnosis
Sella/Juxtasellar, Pineal Region
Anatomically Based Differentials
Pineal + Suprasellar Lesions

title: "Pineal + Suprasellar Lesions" docid: "b3295383-e7a6-436e-bfcc-801c97aa4501" authors:

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ESSENTIAL INFORMATION

  • Key Differential Diagnosis Issues

    • Age may be helpful differentiating feature
    • Diabetes insipidus common presenting feature of infundibular masses
    • Parinaud syndrome, paralysis of upward gaze common presentation of pineal masses

  • Helpful Clues for Common Diagnoses

    • Germinoma - Most common germ cell tumor - Hug midline near 3rd ventricle: 80-90% - Pineal: 50-65%; suprasellar: 25-35% - Pineal + suprasellar: ~ 10-20% - Hyperdense mass on CT - Homogeneous enhancement; CSF seeding common

  • Helpful Clues for Less Common Diagnoses

    • Lymphoma, Primary CNS - Homogeneous enhancing mass(es) along ependymal surface typical - May involve sellar and pineal regions - DWI/DTI restriction - Typically older adults - Imaging and prognosis varies with immune status
    • Metastases, Intracranial, Other - Enhancing masses at gray-white junctions - May involve pineal and suprasellar regions - Primary tumor often known

  • Helpful Clues for Rare Diagnoses

    • Germ Cell Neoplasms, Malignant NOS - Uncommon, highly malignant tumors: Choriocarcinoma, endodermal sinus tumor, embryonal cell carcinoma, mixed germ cell tumor - Heterogeneously enhancing masses - Imaging cannot reliably differentiate - Characteristic elevation of serum tumor markers - Choriocarcinoma: β-hCG; endodermal sinus tumor: AFP; embryonal cell carcinoma: β-hCG and AFP
    • Neurosarcoid - Multisystem inflammatory disease characterized by noncaseating, epithelioid cell granulomas - Enhancing lesions along dura, leptomeninges, subarachnoid and perivascular spaces - May affect parenchyma, cranial nerves, choroid plexus
    • Pineal Parenchymal Tumor - Pineoblastoma and pineal parenchymal tumor of intermediate differentiation (PPTID) - Markedly enhancing pineal region mass ± CSF spread
    • Retinoblastoma (Quadrilateral) - Bilateral calcified ocular tumors + midline neuroblastic tumors (pineal and suprasellar) - 40% familial; account for most bilateral and multilateral disease - Trilateral disease rare: 5-15% of familial lesions (80% pineal, 20% suprasellar) - Quadrilateral disease extremely rare - Dismal prognosis, < 24 month survival
    • Teratoma - Midline mass with Ca⁺⁺, soft tissue, cysts, and fat - Occasionally huge holocranial mass in newborn or fetus - Often along pineal gland or sellar/suprasellar, rarely both

  • Alternative Differential Approaches

    • Pineal + suprasellar lesions in child: Germinoma, germ cell neoplasms, pineoblastoma, retinoblastoma, teratoma
    • Pineal + suprasellar lesions in adult: Lymphoma, metastases, neurosarcoid

References

Selected References

  1. Chalif EJ et al: Malignant pineal parenchymal tumors in adults: a national cancer database analysis. Neurosurgery. 90(6):807-15, 2022
  2. Dai W et al: Imaging findings and clinical analysis of primary intracranial pure yolk sac tumors in children and adolescents: a retrospective study from China. AJNR Am J Neuroradiol. 43(7):1054-9, 2022
  3. Frappaz D et al: EANO, SNO and Euracan consensus review on the current management and future development of intracranial germ cell tumors in adolescents and young adults. Neuro Oncol. 24(4):516-27, 2022
  4. Vasiljevic A: Histopathology and molecular pathology of pediatric pineal parenchymal tumors. Childs Nerv Syst. ePub, 2022
  5. Liu APY et al: Clinical and molecular heterogeneity of pineal parenchymal tumors: a consensus study. Acta Neuropathol. 141(5):771-85, 2021
  6. Tamrazi B et al: Pineal region masses in pediatric patients. Neuroimaging Clin N Am. 27(1):85-97, 2017
  7. Yoneoka Y et al: Perifocal inflammatory reaction with volume fluctuation caused by diagnostic radiation-induced regression in germinoma makes histological diagnosis difficult despite its disappearance following treatment: a significant pitfall and countermeasures to it. Pediatr Neurosurg. 52(2):87-92, 2017
  8. Akiyama Y et al: Germinoma mimicking tumefactive demyelinating disease in pediatric patients. Pediatr Neurosurg. 51(3):149-53, 2016
  9. Causil LD et al: Adult brain tumors and pseudotumors: interesting (bizarre) cases. Neuroimaging Clin N Am. 26(4):667-89, 2016
  10. Sonabend AM et al: Microsurgical resection of pineal region tumors. J Neurooncol. 130(2):351-66, 2016
  11. Smith AB et al: From the archives of the AFIP: lesions of the pineal region: radiologic-pathologic correlation. Radiographics. 30(7):2001-20, 2010

Images

Selected Images

Axial T2 MR shows a mass in the suprasellar and pineal region in a teenage male patient with visual symptoms. The low signal is related to a high nuclear:cytoplasmic ratio. Germinoma Axial T2 MR shows a mass in the suprasellar and pineal region in a teenage male patient with visual symptoms. The low signal is related to a high nuclear:cytoplasmic ratio.

Axial T2 MR shows a mass in the suprasellar and pineal region in a teenage male patient with visual symptoms. The low signal is related to a high nuclear:cytoplasmic ratio. Germinoma Axial T2 MR shows a mass in the suprasellar and pineal region in a teenage male patient with visual symptoms. The low signal is related to a high nuclear:cytoplasmic ratio.

Axial T1 C+ MR in the same patient shows enhancing masses of the pineal and suprasellar related to CSF spread of germinoma. These masses are hyperdense on CT. The combination of suprasellar and pineal mass is sometimes called double midline germinoma and occurs in ~ 20% of cases. Germinoma Axial T1 C+ MR in the same patient shows enhancing masses of the pineal and suprasellar related to CSF spread of germinoma. These masses are hyperdense on CT. The combination of suprasellar and pineal mass is sometimes called double midline germinoma and occurs in ~ 20% of cases.

Sagittal T1 C+ MR shows an enhancing pineal mass with synchronous involvement of the suprasellar region in this patient who presented with diabetes insipidus. Note additional CSF spread with involvement of the optic recess of the 3rd ventricle. Germinoma Sagittal T1 C+ MR shows an enhancing pineal mass with synchronous involvement of the suprasellar region in this patient who presented with diabetes insipidus. Note additional CSF spread with involvement of the optic recess of the 3rd ventricle.

Sagittal T1 C+ FS MR shows enhancing masses in the suprasellar and pineal regions in a young male patient. Imaging mimics a germinoma. Biopsy revealed an embryonal carcinoma. Elevation of serum markers β-hCG and AFP is characteristic. Germ Cell Neoplasms, Malignant NOS Sagittal T1 C+ FS MR shows enhancing masses in the suprasellar and pineal regions in a young male patient. Imaging mimics a germinoma. Biopsy revealed an embryonal carcinoma. Elevation of serum markers β-hCG and AFP is characteristic.

Axial T1 C+ FS MR shows an enhancing suprasellar mass with additional enhancement in the subarachnoid spaces along the superior cerebellum related to neurosarcoid. Neurosarcoid often mimics lymphoma and other granulomatous processes. Neurosarcoid Axial T1 C+ FS MR shows an enhancing suprasellar mass with additional enhancement in the subarachnoid spaces along the superior cerebellum related to neurosarcoid. Neurosarcoid often mimics lymphoma and other granulomatous processes.

Axial T1 C+ FS MR shows a large enhancing pineal region mass and an enhancing suprasellar mass related to a WHO grade 4 pineoblastoma with CSF dissemination. Note the ventricular enhancement related to ependymal spread. Pineal Parenchymal Tumor Axial T1 C+ FS MR shows a large enhancing pineal region mass and an enhancing suprasellar mass related to a WHO grade 4 pineoblastoma with CSF dissemination. Note the ventricular enhancement related to ependymal spread.

Axial CECT shows bilateral calcified masses. Bilateral orbital masses occur in 25-30% of patients with retinoblastoma. Brain imaging is important to search for trilateral or quadrilateral disease. Prognosis is poor in these young patients. Retinoblastoma (Quadrilateral) Axial CECT shows bilateral calcified masses. Bilateral orbital masses occur in 25-30% of patients with retinoblastoma. Brain imaging is important to search for trilateral or quadrilateral disease. Prognosis is poor in these young patients.

Axial T1 MR shows a heterogeneous, lobular mass in the pineal region with areas of fat signal related to a mature teratoma . Note the associated hydrocephalus. Teratomas may be mature (most common), immature, or have malignant transformation. Teratoma Axial T1 MR shows a heterogeneous, lobular mass in the pineal region with areas of fat signal related to a mature teratoma . Note the associated hydrocephalus. Teratomas may be mature (most common), immature, or have malignant transformation.

Additional Images

Sagittal T1 C+ MR shows an enhancing mass in the suprasellar region that extends into the pituitary infundibulum as well as along the dura of the posterior clivus . Primary lymphoma was found at biopsy. Note prominent enhancement of the pineal gland . Lymphoma, Primary CNS Sagittal T1 C+ MR shows an enhancing mass in the suprasellar region that extends into the pituitary infundibulum as well as along the dura of the posterior clivus . Primary lymphoma was found at biopsy. Note prominent enhancement of the pineal gland .

Sagittal T1 C+ MR shows a mildly enhancing suprasellar mass and a small, synchronous pineal mass in this patient who presented with diabetes insipidus. The combination of a suprasellar and pineal mass is sometimes called double midline germinoma and occurs in ~ 20% of cases. Germinoma Sagittal T1 C+ MR shows a mildly enhancing suprasellar mass and a small, synchronous pineal mass in this patient who presented with diabetes insipidus. The combination of a suprasellar and pineal mass is sometimes called double midline germinoma and occurs in ~ 20% of cases.

Axial T1 C+ MR shows enhancing masses of the pineal and suprasellar regions in this patient with CSF spread of germinoma. An enhancing tumor infiltrates the ependyma of the frontal horns . Germinoma Axial T1 C+ MR shows enhancing masses of the pineal and suprasellar regions in this patient with CSF spread of germinoma. An enhancing tumor infiltrates the ependyma of the frontal horns .

Axial T1 C+ MR shows a lobular enhancing mass in the suprasellar region and the pineal region related to germinoma. Germinoma is the most common of the germ cell tumors. Germinoma Axial T1 C+ MR shows a lobular enhancing mass in the suprasellar region and the pineal region related to germinoma. Germinoma is the most common of the germ cell tumors.

Axial T1 MR shows an enhancing pineal mass with hydrocephalus in this patient with bilateral retinoblastoma. Imaging represents trilateral disease with a pineal tumor, the most common location in trilateral disease. Retinoblastoma (Quadrilateral) Axial T1 MR shows an enhancing pineal mass with hydrocephalus in this patient with bilateral retinoblastoma. Imaging represents trilateral disease with a pineal tumor, the most common location in trilateral disease.

Axial CECT shows a large, enhancing suprasellar mass and dilatation of the temporal horn in this patient with bilateral retinoblastoma. The familial hereditary form accounts for essentially all multilateral disease. Retinoblastoma (Quadrilateral) Axial CECT shows a large, enhancing suprasellar mass and dilatation of the temporal horn in this patient with bilateral retinoblastoma. The familial hereditary form accounts for essentially all multilateral disease.

Sagittal T2 SPACE MR shows a large pineoblastoma with CSF dissemination with involvement of the suprasellar region . These malignant WHO grade 4 tumors often present with signs of hydrocephalus and require urgent shunt placement. Pineal Parenchymal Tumor Sagittal T2 SPACE MR shows a large pineoblastoma with CSF dissemination with involvement of the suprasellar region . These malignant WHO grade 4 tumors often present with signs of hydrocephalus and require urgent shunt placement.

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