Report of two cases of chordoid meningioma in patients with Castleman syndrome

Abstract

We read with great interest the article by Lin et al. on the evidence and specific profile of chordoid meningioma in adult patients [1]. They reported a comprehensive clinicopathologic study of 11 adult patients suffering from this uncommon tumor, adding useful information regarding the existence of such tumors in all ages. Interestingly, of their 11 patients who had a mean age of 60.8 years at first surgery, none of them presented any systemic manifestations of Castleman syndrome such as iron-refractory hypochromic/microcytic anemia and dysgamaglobulinemia [1]. For this reason, they arrive at their main observation that ‘‘chordoid meningiomas (CMs) are not always associated with Castleman’s Syndrome’’. They further speculate that ‘‘CMs in young patients are mainly formed by B cells due to the association with the Castleman syndrome, while a predominance of T cells is observed in adult cases’’. However, the mechanism through which these tumors are associated with the Castleman syndrome needs to be further discussed. In presenting our observations of two adult patients with chordoid meningioma associated with systemic manifestations, we hope to add some useful information to this controversial relationship. The first patient, a 48-year-old woman, presented with a long history of iron-refractory hypochromic anemia. Peripheral blood cell examination confirmed the presence of an inflammatory syndrome with hypochromic anemia (HGB = 9.61 g/dl), elevated erythrocytes sedimentation (ERS = 29 mm/h), moderate leucocytosis (WBC = 16,000), and thrombocytosis (PLT = 707,000). Cerebral CT and MRI revealed a right cerebellar mass with homogenous contrast enhancement and marked perifocal edema (Fig. 1). She underwent a total surgical removal of the tumor, histological examination of which revealed the presence of nest and cords of epithelioid cells embedded in basophilic extracellular material producing the chordoid meningioma pattern (Fig. 2). In the early post-operative course, refractory anemia and inflammatory syndrome disappeared (HGB = 13.1 g/dl, ERS = 14 mm/h, WBC = 11,100). Cerebral MRI 39 months after the surgery confirmed the absence of any recurrence. The blood picture was within normal ranges. The second patient, a 47-year-old man, presented with a 2-month history of headaches. Peripheral blood cell examination revealed the presence of hypochromic and microcytic anemia (HGB = 9.6 g/dl), elevated erythrocytes sedimentation (ERS = 28 mm/h), moderate leucocytosis (WBC = 16,000), and thrombocytosis (PLT = 707,000). Cerebral CT scan showed a right temporal lesion with homogenous contrast enhancement exerting a marked mass effect on median structures (Fig. 3). Histopathological examination of the totally removed lesion confirmed the presence of a meningothelial neoplasm with whorl formation and composed of cells with intranuclear inclusions. Neoplastic cells are embedded in a mucoid matrix (Fig. 2). The surrounding tissue consisted of marked lymphoplasmacytic inflammatory infiltrate and siderophages. Proliferation index (Ki-67) was 2%. The tumor showed diffuse positive immunoreactivity with vimentin and EMA. In the G. Kaloshi (&) G. Vreto A. Lame I. Kerri T. Bushati A. Rroji M. Petrela Department of Neurosurgery, Tirana School of Medicine, University Hospital Center ‘‘Mother Theresa’’, 327 Rr e Dibres, Tirana, Albania e-mail: g_kaloshi@yahoo.com