Indian Journal of Pathology and Microbiology
Home About us Instructions Submission Subscribe Advertise Contact e-Alerts Ahead Of Print Login 
Users Online: 3962
Print this page  Email this page Bookmark this page Small font sizeDefault font sizeIncrease font size

  Table of Contents    
Year : 2015  |  Volume : 58  |  Issue : 4  |  Page : 540-542
Pineal parenchymal tumor of intermediate differentiation

Department of Pathology, B.V.D.U. Medical College, Pune, Maharashtra, India

Click here for correspondence address and email

Date of Web Publication4-Nov-2015


The 2007 World Health Organization classification of tumors of the central nervous system identified "pineal parenchymal tumor of intermediate differentiation" (PPTID) as a new pineal parenchymal neoplasm, located between pineocytoma and pineoblastoma as grade II or III. Because of the small number of reported cases, the classification of PPT is still a matter of controversy. We report a case of PPTID. A 25-year-old female patient was admitted to hospital with complaints of a headache, nausea, vomiting since 1-year. Computed tomography/magnetic resonance imaging of the brain showed well-defined, mildly enhancing lesion in the region of the pineal gland with areas of calcification. The tumor was excised. After 3 years, she presented with metastasis in thoracic and lumbosacral spinal region. This is a rare event.

Keywords: Differentiation, intermediate, lumbosacral, metastasis, pineoblastoma, pineocytoma, spine, thoracic

How to cite this article:
Patil M, Karandikar M. Pineal parenchymal tumor of intermediate differentiation. Indian J Pathol Microbiol 2015;58:540-2

How to cite this URL:
Patil M, Karandikar M. Pineal parenchymal tumor of intermediate differentiation. Indian J Pathol Microbiol [serial online] 2015 [cited 2021 Jul 25];58:540-2. Available from: https://www.ijpmonline.org/text.asp?2015/58/4/540/168854

   Introduction Top

Pineal parenchymal tumors (PPTs) represent about 30% of pineal region neoplasms. PPTs are rare tumors, accounting for <0.1% of all brain tumors. They are subdivided into pineocytoma (PC), pineoblastoma (PB), and PPT of intermediate differentiation (PPTID).[1],[2],[3] Of PPT, PCs, and PBs represent approximately 45% each, with PPTID accounting for the remaining 10%.[4] Due to the small number of reported cases, the classification of PPT, especially PPTIDs, remains controversial.[2],[5] Little is known about their clinical behavior, and optimal management of these tumors has not yet been defined.[6],[7]

PPTID was recognized in the 2007 World Health Organization (WHO) classification as a new pineal parenchymal neoplasm intermediate in malignancy (WHO grade II and III) between PC (Gr. I) and PB (Gr. IV).[8] It occurs at all ages, from childhood to adult life, with a peak incidence in early adults.[1]

Histopathologically, PPTID is located between PC and PB. PPTID are composed of diffuse sheets or large lobules of monomorphic, uniform, round cells, and characterized by moderate to high cellularity, mild to moderate nuclear atypia and low to moderate mitotic activity and occasional pineocytomatous rosettes.[1]

In PPTIDs, the expression of neuronal markers is variable. Staining for synaptophysin is mainly diffuse, cytoplasmic, and variable in intensity. Neurofilament protein expression is variable. Chromogranin A can be especially expressed in PPTID, with a pseudostratified architecture. Glial fibrillary acidic protein and S-100 protein staining is only positive in reactive interstitial astrocytes. Ki-67 proliferation index is between 3% and 10%.[9]

   Case Report Top

A 25-year-old woman presented with an intermittent headache, nausea, vomiting, and giddiness since 1-year along with watery discharge from both eyes. Computed tomography/magnetic resonance imaging findings revealed a mass, enhancing in the pineal region with focal areas of calcification and diagnosed as pineal gland tumor. The patient underwent surgery with intraoperative consultation. Frozen sections stained with toluidine blue and a diagnosis of PC was offered [Figure 1] and [Figure 2]. A remaining specimen of the frozen section was sent for routine histopathology; which showed diffuse, highly cellular tumor composed of small, uniform, round cells with mild nuclear atypia. At places, the perivascular arrangement was seen. Few foci of calcification were also noted. There was no evidence of pineocytomatous rosettes or necrosis [Figure 3].
Figure 1: Frozen section : PPTID ( Toluidine Blue, ×400)

Click here to view
Figure 2: Frozen section : PPTID ( Toludine blue, ×400)

Click here to view
Figure 3 : (H.P.) Sheets of small round cells with monomorphic nuclei and moderate amount of cytoplasm (H and E, ×400)

Click here to view

The final diagnosis, according to WHO criteria was PPTID (Gr. II).

After 3 years, she presented with metastasis in thoracic and lumbosacral spinal region [Figure 4] and [Figure 5] and was operated for the same in another hospital.
Figure 4: (a) (left) Sagittal and coronal (b) (right) T1W MR image after IV administration of Gd- DTPA revealed well defined irregular heterogeneously enhancing lesion in the region of the pineal gland effacing the posterior aspect of the third ventricle and compressing the quadrigeminal plate, without causing hydrocephalus

Click here to view
Figure 5: Sagittal gadolinium enhanced T1W MR image of thoracic spine (a) and lumbosacral spine (b) revealed multiple lesions of solid nature with cystic areas of varying sizes dispersed throughout the spinal cord, in the intramedullary and subdural space predominantly involving the thoracic spine. The solid components of these lesions showed moderate enhancement post contrast (c)

Click here to view

   Discussion Top

A significant number of PPT do not fit accurately into the categories of PB and PC and may exhibit features intermediate between these two distinct tumors. In a recent series of cases classified according to the new WHO guidelines, PPTID accounted for 56% of the cases. This category includes tumors with histologic features, intermediate between PC and PB or tumors that have mixed areas of both PC and PB. PPTID are more aggressive than PCs and commonly present with local infiltration and distant cerebrospinal fluid (CSF) dissemination.[10]

The morphologic features are intermediate between those of PC and PB, with a particular tendency to show lobulated grouping of unipolar tumor cells demarcated by and often aligned along a delicate vascular stroma. When the lobulated pattern occurs in the absence of pineocytomatous rosettes, these tumors are frequently associated with local invasion and dissemination along CSF pathway.[10]

It has been suggested that a grading system based on mitotic activity and neurofilament protein immunoreactivity can distinguish low from high-grade PPTID.[2] Recently, Jouvet et al.[2] proposed a new prognostic grading comprising four grades: Grade I - for PC; grade IV - for PB and grade II and III for PPTID, with grade II being defined as having <6 mitotic figures/10 hpf, positive immunolabeling of neurofilament and grade III being defined as having 6 or more mitotic activity per 10 hpf or fewer than 6 mitosis but with immunolabeling for neurofilament. According to that our case would correspond to a grade II.[2]

The rarity of PPT emphasises the importance of distinguishing these neoplasms from other neuroepithelial tumors that may impinge upon the pineal region including central neurocytoma, ependymoma, and oligodendroglioma. In contrast to central neurocytoma, PCs, and intermediate tumors demonstrated cells with better-defined cytoplasmic borders and much greater cellular pleomorphism. The intercellular matrix is less prominent, and there is a more conspicuous relationship of the cells to the fibrovascular stroma. Ependymal and oligodendroglial tumors are discerned more readily on the basis of their glial differentiation. Analysis of photoreceptor gene expression, particularly interphotoreceptor retinoid-binding protein and retinal S-antigen, may also provide an important information.[10]

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Nakazato Y, Jouvet A, Scheithauer BW. Pineal parenchymal tumor and intermediate differentiation. In: Louis DN, editor. World Health Organization Classification of Tumors of the Central Nervous System. 4th ed., Vol. 7. WHO; 2007. p. 124-5.  Back to cited text no. 1
Jouvet A, Saint-Pierre G, Fauchon F, Privat K, Bouffet E, Ruchoux MM, et al. Pineal parenchymal tumors: a correlation of histological features with prognosis in 66 cases. Brain Pathol 2000;10:49-60.  Back to cited text no. 2
Nakazato Y, Jouvet A, Scheithauer BW. Pineocytoma. In: Louis DN, Ohgati H, Wieslier OD, Cavence WK, editors. World Health Organization Classification of Tumours of the Central Nervous System. Ch. 7. Lyon: IARC; 2007. p. 122-3.  Back to cited text no. 3
Mena H, Nakazato Y, Jouvet A, Scheithauer BW. Pineoblastoma. Pineocytoma. Pineal parenchymal tumors of intermediate differentiation. In: Kleihues P, Cavence WK, editors. Tumours of the Nervous System. 2nd ed. Lyon: IARC; 2000. p. 116-21.  Back to cited text no. 4
Fauchon F, Jouvet A, Paquis P, Saint-Pierre G, Mottolese C, Ben Hassel M, et al. Parenchymal pineal tumors: a clinicopathological study of 76 cases. Int J Radiat Oncol Biol Phys 2000;46:959-68.  Back to cited text no. 5
Pusztaszeri M, Pica A, Janzer R. Pineal parenchymal tumors of intermediate differentiation in adults: case report and literature review. Neuropathology 2006;26:153-7.  Back to cited text no. 6
Schild SE, Scheithauer BW, Schomberg PJ, Hook CC, Kelly PJ, Frick L, et al. Pineal parenchymal tumors. Clinical, pathologic, and therapeutic aspects. Cancer 1993;72:870-80.  Back to cited text no. 7
Mena H, Nakazato Y, Jouvet A, Kleiheus P, Cavenee WK, et al. editors. Pineal Parenchymal Tumors. In: The WHO Classification of Tumors of the Central Nervous System. Lyon: IARC Press:2002. p. 115-22.  Back to cited text no. 8
Rickert CH, Simon R, Bergmann M, Dockhorn-Dworniczak B, Paulus W. Comparative genomic hybridization in pineal parenchymal tumors. Genes Chromosomes Cancer 2001;30:99-104.  Back to cited text no. 9
Silverberg GS, Debellis AR, Frable JW, Livolsi AV, Wick RM. The central nervous system. In: Silverberg's Principles and Practice of Surgical Pathology and Cytopathology. 4th ed., Vol. 2. Philadelphia: Churchil Livingstone; 2006. p. 2329-31.  Back to cited text no. 10

Correspondence Address:
Dr. Meena Patil
Flat No. 8, Surya Appt., Kohinoor Colony, Sahakar Nagar No. 2, Pune - 411 009, Maharashtra
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.168854

Rights and Permissions


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

This article has been cited by
1 Patterns of care and survival outcomes in patients with pineal parenchymal tumor of intermediate differentiation: An individual patient data analysis
Supriya Mallick,Rony Benson,G.K. Rath
Radiotherapy and Oncology. 2016; 121(2): 204
[Pubmed] | [DOI]


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  

   Case Report
    Article Figures

 Article Access Statistics
    PDF Downloaded99    
    Comments [Add]    
    Cited by others 1    

Recommend this journal