Pineal gland

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The pineal gland is thingy that is most noted for the fact that it calcifies with age.

Normal histology

See Neurohistology#Pineal gland.
  • Cellular.

Overview

Non-neoplastic:

  • Cysts

Tumours:[1]

  • Primary pineal tumours ~15% of (pineal) tumours - benign to malignant:[2]
    • Pineocytoma.
    • Pineal parenchymal tumor of intermediate differentiation.
    • Pineoblastoma.
  • Germ cell tumours:
  • Others:

Pineal gland cyst

  • Quite common. [3]
  • large cysts can cause CSF obstruction. [4]

  • Pineal gland cyst found at autopsy. (WC/Marvin101)

  • Pineal gland cyst. HE, low mag. (WC/jensflorian)

  • Pineal gland cyst. HE, higher mag. (WC/jensflorian)


Pineocytoma

General

  • Benign tumour of the pineal gland.
    • WHO Grade I.

Microscopic

Features:

  • Cytologically benign cells (uniform size of nuclei, regular nuclear membrane, light chromatin).
  • Pineocytomatous/neurocytic rosette = (irregular) rosette with a large meshwork of fibers (neuropil) at the centre.[5]
    • Similar to Homer-Wright rosette... but:
      1. Neuropil centre is larger in pineocytoma.
      2. Edge of neuropil meshwork irregular.

Notes:

  • Rosette = circular/flower-like arrangement of cells.

Images

  • Pineocytoma - intermed. mag. (WC)

  • Pineocytoma - high mag. (WC)

  • Pineocytoma - very high mag. (WC)

IHC

  • Synaptophysin +ve.
  • Chromogranin A -ve.
  • NSE +ve (cytoplasmic + nuclear).[6]
  • GFAP -ve.
    • +ve in gliomas.
  • PLAP -ve.
    • Usu. +ve in germ cell tumours.
  • Ki-67.

Another ref.:[7]

Pineal parenchymal tumor of intermediate differentiation

General

  • 20% of all pineal tumors.
  • Affects all ages.
  • ICD-O: 9362/3
  • No WHO grade yet, clinical behaviour corresponds to grade II/III[8]

Microscopic

Features:[9]

  • High cellularity.
  • Mild to moderate atypia.
  • Mitoses.
  • Usually no pinecytomatous rosettes.
  • High pleomorphism possible.

Molecular

  • Insertions in KBTBD4.[10]


Images:

  • PPID, Smear with plemorphic cells. (WC/jensflorian)

  • PPID, HE intermed. mag. (WC/jensflorian)

  • PPID, HE higher mag. (WC/jensflorian)

  • PPID, HE, rosette-like growth pattern. (WC/jensflorian)

Papillary tumour of pineal region (PTPR)

General

  • Very rare neuoepithelial tumor of adults.
  • Proposed ICD-O: 9395/3
  • No WHO grade yet, clinical behaviour corresponds to grade II/III[11]


Gross:

  • Well circumscribed.
  • Can be quite large (2-4cm).
  • Macroscopy mimics pineocytoma.

  • PTPR MRI scan (WC/marvin101)

Microscopic

Features:[12]

  • Papillary growth pattern.
  • Dense areas exhibit ependymal features.
    • Clear, vacuolated cytoplasm.
    • Rosettes.
  • Round to oval nuclei.
  • Mitoses (0-10/HPF).
  • Necroses.
  • Hyalinized vessels.

IHC

  • +ve for keratins (KL1, AE1/AE3, Cam5.2, CK18).
  • GFAP and Synaptophysin focally +ve.
  • S100+ve.
  • EMA mostly -ve.
  • NF and Kir 7.1-ve.

DDx: Ependymoma.

Images:

  • PTPR H&E stain (WC/jensflorian)

  • PTPR CK KL1 (WC/marvin101)

  • PTPR CK18 IHC (WC/jensflorian)

  • PTPR EMA IHC (WC/marvin101)

  • PTPR Kir 7.1 IHC (WC/marvin101)

Pineoblastoma

General

  • Rare.
  • Malignant.
  • Males > females.
  • Children & young adults.
  • Corresponds to WHO IV (ICD-O: 9362/3).
  • May be a manifestation of the DICER1 syndrome.[13]

Microscopic

Features:

  • Hypercellular.
  • Mitoses.
  • Nuclear atypia.
  • Homer-wright & Flexner-Winterstein rosettes
  • Fleurettes.

IHC

  • GFAP -ve/+ve.
  • NF+ve.
  • Synaptophysin+ve
  • MIB-1: high.

Molecular

  • DICER1 mutations.[14]
  • DROSHA deletions. [15]

DDx:

Images:

  • Pineoblastoma H&E (WC/jensflorian)

  • Neurofilament IHC in Pineoblastoma (WC/jensflorian)

  • GFAP IHC in Pineoblastoma (WC/jensflorian)

See also

References

  1. ↑ Gaillard F, Jones J (October 2010). "Masses of the pineal region: clinical presentation and radiographic features". Postgrad Med J 86 (1020): 597–607. doi:10.1136/pgmj.2009.087460. PMID 20971711.
  2. ↑ Smith AB, Rushing EJ, Smirniotopoulos JG (November 2010). "From the archives of the AFIP: lesions of the pineal region: radiologic-pathologic correlation". Radiographics 30 (7): 2001–20. doi:10.1148/rg.307105131. PMID 21057132.
  3. ↑ Pu, Y.; Mahankali, S.; Hou, J.; Li, J.; Lancaster, JL.; Gao, JH.; Appelbaum, DE.; Fox, PT. (Oct 2007). "High prevalence of pineal cysts in healthy adults demonstrated by high-resolution, noncontrast brain MR imaging.". AJNR Am J Neuroradiol 28 (9): 1706-9. doi:10.3174/ajnr.A0656. PMID 17885233.
  4. ↑ Mena, H.; Armonda, RA.; Ribas, JL.; Ondra, SL.; Rushing, EJ. (Oct 1997). "Nonneoplastic pineal cysts: a clinicopathologic study of twenty-one cases.". Ann Diagn Pathol 1 (1): 11-8. PMID 9869821.
  5. ↑ Wippold FJ, Perry A (March 2006). "Neuropathology for the neuroradiologist: rosettes and pseudorosettes". AJNR Am J Neuroradiol 27 (3): 488–92. PMID 16551982.
  6. ↑ URL: http://path.upmc.edu/cases/case157/dx.html. Accessed on: 9 December 2010.
  7. ↑ URL: http://www.springerlink.com/content/k4v88n6h6jknhp2t/fulltext.pdf. Accessed on: 9 December 2010.
  8. ↑ Jouvet, A.; Saint-Pierre, G.; Fauchon, F.; Privat, K.; Bouffet, E.; Ruchoux, MM.; Chauveinc, L.; Fèvre-Montange, M. (Jan 2000). "Pineal parenchymal tumors: a correlation of histological features with prognosis in 66 cases.". Brain Pathol 10 (1): 49-60. PMID 10668895.
  9. ↑ Fèvre-Montange, M.; Szathmari, A.; Champier, J.; Mokhtari, K.; Chrétien, F.; Coulon, A.; Figarella-Branger, D.; Polivka, M. et al. (Jul 2008). "Pineocytoma and pineal parenchymal tumors of intermediate differentiation presenting cytologic pleomorphism: a multicenter study.". Brain Pathol 18 (3): 354-9. doi:10.1111/j.1750-3639.2008.00128.x. PMID 18371183.
  10. ↑ "Molecular subgrouping of primary pineal parenchymal tumors reveals distinct subtypes correlated with clinical parameters and genetic alterations". Acta Neuropathol 139 (2): 243–257. February 2020. doi:10.1007/s00401-019-02101-0. PMID 31768671.
  11. ↑ Fèvre-Montange, M.; Hasselblatt, M.; Figarella-Branger, D.; Chauveinc, L.; Champier, J.; Saint-Pierre, G.; Taillandier, L.; Coulon, A. et al. (Oct 2006). "Prognosis and histopathologic features in papillary tumors of the pineal region: a retrospective multicenter study of 31 cases.". J Neuropathol Exp Neurol 65 (10): 1004-11. doi:10.1097/01.jnen.0000240462.80263.13. PMID 17021405.
  12. ↑ Heim, S.; Beschorner, R.; Mittelbronn, M.; Keyvani, K.; Riemenschneider, MJ.; Vajtai, I.; Hartmann, C.; Acker, T. et al. (Jan 2014). "Increased mitotic and proliferative activity are associated with worse prognosis in papillary tumors of the pineal region.". Am J Surg Pathol 38 (1): 106-10. doi:10.1097/PAS.0b013e31829e492d. PMID 24121176.
  13. ↑ van Engelen, K.; Villani, A.; Wasserman, JD.; Aronoff, L.; Greer, MC.; Tijerin Bueno, M.; Gallinger, B.; Kim, RH. et al. (Jan 2018). "DICER1 syndrome: Approach to testing and management at a large pediatric tertiary care center.". Pediatr Blood Cancer 65 (1). doi:10.1002/pbc.26720. PMID 28960912.
  14. ↑ "Germ-line and somatic DICER1 mutations in pineoblastoma". Acta Neuropathol 128 (4): 583–95. October 2014. doi:10.1007/s00401-014-1318-7. PMC 4381868. PMID 25022261. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4381868/.
  15. ↑ "Recurrent KBTBD4 small in-frame insertions and absence of DROSHA deletion or DICER1 mutation differentiate pineal parenchymal tumor of intermediate differentiation (PPTID) from pineoblastoma". Acta Neuropathol 137 (5): 851–854. May 2019. doi:10.1007/s00401-019-01990-5. PMC 6483828. PMID 30877433. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6483828/.
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