Pineal parenchymal tumor of intermediate differentiation

Changed by Frank Gaillard, 20 Mar 2017

Updates to Article Attributes

Body was changed:

Pineal parenchymal tumours with intermediate differentiation (PPTID) are, as the name suggests, tumours which fall between pineocytoma (well differentiated, WHO grade I) and pineoblastomas (poorly differentiated, WHO grade IV) and are considered WHO grade II / III tumours 2. This is a tumour encountered at all ages, but particularly of middle age adults (20-70 years of age) with male and females affected equallyslight female predilection, similar to that of pineocytomas 4

Their pathology, radiographic appearance and biological behaviour are also intermediate. They may invade adjacent structures and also spread along CSF (imaging of the intire craniospinal axis is required 3).

Pathology

Macroscopic appearance

Pathologically they do not demonstrate the highly differentiatedPineal parenchymal tumour with intermediate differentiation have similar macroscopic appearance ofto pineocytomas, but also lackappearing relatively well circumscribed with heterogeneous cut surface 4.

Microscopic appearance

Two microscopic patterns are encountered, sometimes co-existing 4:

  1. lobulated
  2. diffuse

The lobulated pattern, poorly defined lobules are separated from each other by large fibrous vessels 4.

The diffuse pattern is reminiscent to oligodendrogliomas and/or central neurocytomas, with the malignant small blue cell appearancelarge pineocytomatous rosettes characteristic of a pineoblastomapineocytomas not evident 4.

Immunopheontype
  • -<p><strong>Pineal parenchymal tumours with intermediate differentiation (PPTID)</strong> are, as the name suggests, tumours which fall between <a href="/articles/pineocytoma">pineocytoma</a> (well differentiated, <a href="/articles/who-classification-of-cns-tumours-1">WHO</a> grade I) and <a href="/articles/pineoblastoma">pineoblastomas</a> (poorly differentiated, WHO grade IV) and are considered WHO grade II / III tumours <sup>2</sup>. This is a tumour of middle age adults with male and females affected equally. </p><p>Their pathology, radiographic appearance and biological behaviour are also intermediate. They may invade adjacent structures and also spread along CSF (imaging of the intire craniospinal axis is required <sup>3</sup>).</p><p>Pathologically they do not demonstrate the highly differentiated appearance of pineocytomas, but also lack the malignant small blue cell appearance of a pineoblastoma. </p>
  • +<p><strong>Pineal parenchymal tumours with intermediate differentiation (PPTID)</strong> are, as the name suggests, tumours which fall between <a href="/articles/pineocytoma">pineocytoma</a> (well differentiated, WHO grade I) and <a href="/articles/pineoblastoma">pineoblastomas</a> (poorly differentiated, WHO grade IV) and are considered WHO grade II / III tumours <sup>2</sup>. This is a tumour encountered at all ages, but particularly of middle age adults (20-70 years of age) with slight female predilection, similar to that of pineocytomas <sup>4</sup>. </p><p>Their radiographic appearance and biological behaviour are also intermediate. They may invade adjacent structures and also spread along CSF (imaging of the intire craniospinal axis is required <sup>3</sup>).</p><h4>Pathology</h4><h5>Macroscopic appearance</h5><p>Pineal parenchymal tumour with intermediate differentiation have similar macroscopic appearance to pineocytomas, appearing relatively well circumscribed with heterogeneous cut surface <sup>4</sup>.</p><h5>Microscopic appearance</h5><p>Two microscopic patterns are encountered, sometimes co-existing <sup>4</sup>:</p><ol>
  • +<li>lobulated</li>
  • +<li>diffuse</li>
  • +</ol><p>The lobulated pattern, poorly defined lobules are separated from each other by large fibrous vessels <sup>4</sup>.</p><p>The diffuse pattern is reminiscent to <a title="Oligodendroglioma" href="/articles/oligodendroglioma">oligodendrogliomas </a>and/or <a title="Central neurocytoma" href="/articles/central-neurocytoma">central neurocytomas</a>, with the large pineocytomatous rosettes characteristic of <a title="Pineocytoma" href="/articles/pineocytoma">pineocytomas </a>not evident <sup>4</sup>.</p><h5>Immunopheontype</h5><ul>
  • +<li>
  • +<a href="/articles/synaptophysin">synaptophysin</a>: positive <sup>4</sup>
  • +</li>
  • +<li>NFP: variable</li>
  • +<li>
  • +<a title="NeuN" href="/articles/neun">NeuN</a>: negative</li>
  • +</ul><p> </p>

References changed:

  • 4. Hasselblatt M, Huang A, Vasiljevic A, Jones DTW, Orr BA, Snuderl M, Pineal parenchymal tumour of intermediate differentiation. In: WHO Classification of Tumours Editorial Board. Central nervous system tumours. Lyon (France): International Agency for Research on Cancer; 2021. (WHO classification of tumours series, 5th ed.; vol. 6). <a href="https://publications.iarc.fr/601.">https://publications.iarc.fr/601</a>

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