198 lines
18 KiB
Markdown
198 lines
18 KiB
Markdown
---
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title: "Pineal + Suprasellar Lesions"
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docid: "b3295383-e7a6-436e-bfcc-801c97aa4501"
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authors:
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- key: "8d5254e9-8dda-478b-8f08-bdee97a32c79"
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value: "Karen L. Salzman, 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: "Pineal + Suprasellar Lesions"
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slug: "pineal--suprasellar-lesions"
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treeNodeId: null
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category: "Brain"
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cmeTopicId: "7ea7701e-a2e5-4772-8aaa-e112d5c7fb5b"
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documentVersionId: "0c8952a3-b074-4ace-9d11-5b06a280a183"
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imageCount: 15
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lastUpdated: "01/26/23"
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pageDescription: "Pineal + Suprasellar Lesions"
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pageKeywords: "Brain, Differential Diagnosis, Sella/Juxtasellar, Pineal Region, Anatomically Based Differentials, Pineal + Suprasellar Lesions"
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pageTitle: "Pineal + Suprasellar Lesions | STATdx"
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enhancedTitle: "Pineal + Suprasellar Lesions"
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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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- "Pineal + Suprasellar Lesions"
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---
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# ESSENTIAL INFORMATION
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- ## Key Differential Diagnosis Issues
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- Age may be helpful differentiating feature
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- Diabetes insipidus common presenting feature of infundibular masses
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- Parinaud syndrome, paralysis of upward gaze common presentation of pineal masses
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- ## Helpful Clues for Common Diagnoses
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- **Germinoma**
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- Most common germ cell tumor
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- Hug midline near 3rd ventricle: 80-90%
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- Pineal: 50-65%; suprasellar: 25-35%
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- Pineal + suprasellar: ~ 10-20%
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- Hyperdense mass on CT
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- Homogeneous enhancement; CSF seeding common
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- ## Helpful Clues for Less Common Diagnoses
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- **Lymphoma, Primary CNS**
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- Homogeneous enhancing mass(es) along ependymal surface typical
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- May involve sellar and pineal regions
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- DWI/DTI restriction
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- Typically older adults
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- Imaging and prognosis varies with immune status
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- **Metastases, Intracranial, Other**
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- Enhancing masses at gray-white junctions
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- May involve pineal and suprasellar regions
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- Primary tumor often known
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- ## Helpful Clues for Rare Diagnoses
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- **Germ Cell Neoplasms, Malignant NOS**
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- Uncommon, highly malignant tumors: Choriocarcinoma, endodermal sinus tumor, embryonal cell carcinoma, mixed germ cell tumor
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- Heterogeneously enhancing masses
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- Imaging cannot reliably differentiate
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- Characteristic elevation of serum tumor markers
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- Choriocarcinoma: β-hCG; endodermal sinus tumor: AFP; embryonal cell carcinoma: β-hCG and AFP
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- **Neurosarcoid**
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- Multisystem inflammatory disease characterized by noncaseating, epithelioid cell granulomas
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- Enhancing lesions along dura, leptomeninges, subarachnoid and perivascular spaces
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- May affect parenchyma, cranial nerves, choroid plexus
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- **Pineal Parenchymal Tumor**
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- Pineoblastoma and pineal parenchymal tumor of intermediate differentiation (PPTID)
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- Markedly enhancing pineal region mass ± CSF spread
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- **Retinoblastoma (Quadrilateral)**
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- Bilateral calcified ocular tumors + midline neuroblastic tumors (pineal and suprasellar)
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- 40% familial; account for most bilateral and multilateral disease
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- Trilateral disease rare: 5-15% of familial lesions (80% pineal, 20% suprasellar)
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- Quadrilateral disease extremely rare
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- Dismal prognosis, < 24 month survival
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- **Teratoma**
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- Midline mass with Ca⁺⁺, soft tissue, cysts, and fat
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- Occasionally huge holocranial mass in newborn or fetus
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- Often along pineal gland or sellar/suprasellar, rarely both
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- ## Alternative Differential Approaches
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- Pineal + suprasellar lesions in **child**: Germinoma, germ cell neoplasms, pineoblastoma, retinoblastoma, teratoma
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- Pineal + suprasellar lesions in **adult**: Lymphoma, metastases, neurosarcoid
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## References
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# Selected References
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1. [Chalif EJ et al: Malignant pineal parenchymal tumors in adults: a national cancer database analysis. Neurosurgery. 90(6):807-15, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35311743%5Bpmid%5D)
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1. [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](http://www.ncbi.nlm.nih.gov/pubmed/?term=35798388%5Bpmid%5D)
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1. [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](http://www.ncbi.nlm.nih.gov/pubmed/?term=34724065%5Bpmid%5D)
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1. [Vasiljevic A: Histopathology and molecular pathology of pediatric pineal parenchymal tumors. Childs Nerv Syst. ePub, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35972537%5Bpmid%5D)
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1. [Liu APY et al: Clinical and molecular heterogeneity of pineal parenchymal tumors: a consensus study. Acta Neuropathol. 141(5):771-85, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33619588%5Bpmid%5D)
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1. [Tamrazi B et al: Pineal region masses in pediatric patients. Neuroimaging Clin N Am. 27(1):85-97, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27889025%5Bpmid%5D)
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1. [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](http://www.ncbi.nlm.nih.gov/pubmed/?term=27832656%5Bpmid%5D)
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1. [Akiyama Y et al: Germinoma mimicking tumefactive demyelinating disease in pediatric patients. Pediatr Neurosurg. 51(3):149-53, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26811911%5Bpmid%5D)
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1. [Causil LD et al: Adult brain tumors and pseudotumors: interesting (bizarre) cases. Neuroimaging Clin N Am. 26(4):667-89, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27712799%5Bpmid%5D)
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1. [Sonabend AM et al: Microsurgical resection of pineal region tumors. J Neurooncol. 130(2):351-66, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27193692%5Bpmid%5D)
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1. [Smith AB et al: From the archives of the AFIP: lesions of the pineal region: radiologic-pathologic correlation. Radiographics. 30(7):2001-20, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=21057132%5Bpmid%5D)
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## Images
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### Selected Images
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**Germinoma**
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*Axial T2 MR shows a mass in the suprasellar <img src='img/arrows/CO.png'/> and pineal <img src='img/arrows/CC.png'/> region in a teenage male patient with visual symptoms. The low signal is related to a high nuclear:cytoplasmic ratio.*
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**Germinoma**
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*Axial T2 MR shows a mass in the suprasellar <img src='img/arrows/CO.png'/> and pineal <img src='img/arrows/CC.png'/> region in a teenage male patient with visual symptoms. The low signal is related to a high nuclear:cytoplasmic ratio.*
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**Germinoma**
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*Axial T1 C+ MR in the same patient shows enhancing masses of the pineal <img src='img/arrows/CC.png'/> and suprasellar <img src='img/arrows/CO.png'/> 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.*
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**Germinoma**
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*Sagittal T1 C+ MR shows an enhancing pineal mass <img src='img/arrows/CS.png'/> with synchronous involvement of the suprasellar region <img src='img/arrows/CO.png'/> in this patient who presented with diabetes insipidus. Note additional CSF spread with involvement of the optic recess <img src='img/arrows/WS.png'/> of the 3rd ventricle.*
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**Germ Cell Neoplasms, Malignant NOS**
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*Sagittal T1 C+ FS MR shows enhancing masses in the suprasellar <img src='img/arrows/CS.png'/> and pineal <img src='img/arrows/CO.png'/> 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.*
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**Neurosarcoid**
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*Axial T1 C+ FS MR shows an enhancing suprasellar mass <img src='img/arrows/CS.png'/> with additional enhancement in the subarachnoid spaces along the superior cerebellum <img src='img/arrows/CO.png'/> related to neurosarcoid. Neurosarcoid often mimics lymphoma and other granulomatous processes.*
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**Pineal Parenchymal Tumor**
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*Axial T1 C+ FS MR shows a large enhancing pineal region mass <img src='img/arrows/CS.png'/> and an enhancing suprasellar mass <img src='img/arrows/CO.png'/> related to a WHO grade 4 pineoblastoma with CSF dissemination. Note the ventricular enhancement <img src='img/arrows/WO.png'/> related to ependymal spread.*
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**Retinoblastoma (Quadrilateral)**
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*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.*
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**Teratoma**
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*Axial T1 MR shows a heterogeneous, lobular mass in the pineal region with areas of fat signal related to a mature teratoma <img src='img/arrows/CS.png'/>. Note the associated hydrocephalus. Teratomas may be mature (most common), immature, or have malignant transformation.*
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### Additional Images
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**Lymphoma, Primary CNS**
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*Sagittal T1 C+ MR shows an enhancing mass in the suprasellar region that extends into the pituitary infundibulum <img src='img/arrows/CO.png'/> as well as along the dura of the posterior clivus <img src='img/arrows/CC.png'/>. Primary lymphoma was found at biopsy. Note prominent enhancement of the pineal gland <img src='img/arrows/WS.png'/>.*
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**Germinoma**
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*Sagittal T1 C+ MR shows a mildly enhancing suprasellar mass <img src='img/arrows/WC.png'/> and a small, synchronous pineal mass <img src='img/arrows/WO.png'/> 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.*
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**Germinoma**
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*Axial T1 C+ MR shows enhancing masses of the pineal <img src='img/arrows/WC.png'/> and suprasellar regions in this patient with CSF spread of germinoma. An enhancing tumor infiltrates the ependyma of the frontal horns <img src='img/arrows/WS.png'/>.*
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**Germinoma**
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*Axial T1 C+ MR shows a lobular enhancing mass in the suprasellar region <img src='img/arrows/BO.png'/> and the pineal region <img src='img/arrows/BS.png'/> related to germinoma. Germinoma is the most common of the germ cell tumors.*
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**Retinoblastoma (Quadrilateral)**
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*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.*
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**Retinoblastoma (Quadrilateral)**
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*Axial CECT shows a large, enhancing suprasellar mass and dilatation of the temporal horn <img src='img/arrows/WS.png'/> in this patient with bilateral retinoblastoma. The familial hereditary form accounts for essentially all multilateral disease.*
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**Pineal Parenchymal Tumor**
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*Sagittal T2 SPACE MR shows a large pineoblastoma <img src='img/arrows/CS.png'/> with CSF dissemination with involvement of the suprasellar region <img src='img/arrows/CC.png'/>. These malignant WHO grade 4 tumors often present with signs of hydrocephalus and require urgent shunt placement.*
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