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Pineal Tumors

 

 

Classification

There are a wide range of tumors that can occur in this location - each having its own course, prognosis, and treatment.  

 

Pineal tumors are classified according to embryonal origin as well as histological cell type:

Classification

Subtypes

Pineal Parenchyma
  • Pineoblastoma
  • Pineocytoma

 

Germ cell origin

 Germinoma (also called Dysgerminoma)

 

Non Germinomatous Germ Cell Tumors (NGGCTs):

Also called Non Seminomatous Germ Cell Tumors (NSGCTs):

  • Embryonal
  • Teratoma
  • Endodermal sinus (= yolk sac)
  • Choriocarcinoma

 

Glial
  • Astrocytoma
  • Glioblastoma
  • Oligodendroglioma
  • Ependymoma

 

Other
  • Meningioma
  • Chemodectoma
  • Craniopharyngioma
  • Metastases

 

Non-neoplastic
  • Cysts
  • Dermoid cyst
  • Arachnoid cyst
  • Lipoma

 

 

Proportion of pineal tumors accounted for by each type: 

Benign lesions

(teratoma, pineocytoma, meningioma)

30-40%

Germ cell tumors

60%

Glial tumor

25%

Ependymal tumor

15%

 

Classification of CNS Non Germinomatous Germ Cell Tumors (NGGCTs):

Prognosis

Subtypes

Good
  • Mature teratoma

 

Intermediate
  • Immature teratoma
  • Germinoma with mature or immature teratoma

 

Poor
  • Teratoma with malignant transformation
  • Embryonal carcinoma
  • Yolk sac tumour
  • Choriocarcinoma
  • Mixed (any of above)

 

 

Each type of pineal tumor has its own set of characteristics:

 

Germinoma:

  • 40% of intracranial germ cell tumors
  • Male predominance
  • Similar to testicular germinoma
  • Rapid progression if left untreated
  • Very sensitive to RT and chemotherapy

 

Embryonal carcinoma:

  •  Rare to be the major component of tumor
  • Can secrete Beta-HCG and alpha-fetoprotein

 

Teratomas:

  • Can be mature (benign) or immature (malignant behaviour)
  • 15% of intracranial germ cell tumors

 

Choriocarcinoma:

  • Very rare
  • Malignant behaviour
  • Associated with increased gonadotropin levels in CSF and serum
  • Poor prognosis

 

Pineoblastoma:

  • Supratentorial primitive neuroectodermal tumor (supratentorial PNET)
  • Least differentiated of pineal parenchymal neoplasms
  • Generally treated with combined craniospinal RT and chemotherapy

 

Pineocytoma:

  • Generally benign and treated with surgery alone

 

Tumors of glial cell origin:

  • 25% of pineal tumors

 

      

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