Answer and Discussion of Quiz Set: N-007

4.    Which of the following tumor is likely to be assocated with significant lymphoplasmacytic infiltration and occasionally with hematological manifestations including Castleman syndrome?   Answer

A.  Chordoid meningioma, WHO grade II

B.  Chordoid glioma, WHO grade II

C.  Clear cell meningioma, WHO grade II

D.  Germinoma

E.  Secretory meningioma, WHO grade I

Answer and Discussion: The answer is (A). Chordoid meningioma is a WHO grade II tumor. Although they do not have atypical features that would justify them as an atypical meningioma and mitoses are not readily seen in most cases, but they have a strong tendency to recur. It is rare and comprises about 0.5-1.0% of all meningiomas. Similar to to other meningioims, chordoid meningiomas occur predominantly in adults and most of them are supratentorial followed by posterior  and then spinal cord. In contrast to other meningiomas, it is equally prevalent in male and feamle. Systemic manifestations of Castleman syndrome including iron-resistant hypochromic, microcytic anemia, dysgammaglobulinemia and bone marrow plasmacytosis have been associated with some cases of chordoid meningioma. These case are found almost exclusively in children and adolescents but not in adults. Prominent lymphoplasmacytic infiltrates are often present in this type of patients and the clinical manifestations of Castleman  syndrome may reverse upon removal of these tumors.

Germinoma, like seminioma, is associated with substantial amount of lymphocytic infiltration that may mask the true nature of the tumor. Occasionally, granulomatous inflammation will be extensive enough to suggest a granulomatous inflammation or infection rather than a genuine malignant neoplasm. However, germinoma is not associated with manifestations of Castleman syndrome.