title: Secretion of interleukin-6 and vascular endothelial growth factor by spindle cell sarcoma complicating Castleman's disease (so-called 'vascular neoplasia').
abstract: So-called 'vascular neoplasia' (VN) is a rare tumour of unknown origin that complicates hyaline vascular type Castleman's disease (CD). This paper reports a case of VN complicating CD of hyaline vascular type, in which neoplastic cells were shown to secrete interleukin-6 (IL-6) and vascular endothelial growth factor (VEGF). In this case, VN first occurred in the retroperitoneum of a 60-year-old male. The lesion showed typical morphology, with three distinct areas: (1) a lymph node-like area with regressively transformed lymph follicles showing hyaline vascular changes and with a hypervascular interfollicular region filled with slit-like vascular channels; (2) an area composed of spindle cell sarcoma; and (3) an area showing angiolipomatous hamartoma. A proportion of the cells in the spindle cell area showed severe pleomorphism. Subcutaneous recurrence after 8 months was composed purely of pleomorphic spindle cells. A karyotypic analysis of the recurrent tumour showed 47, XXY with some instability. Supernatant from primary culture contained high levels of IL-6 and VEGF, suggesting high secretion of these cytokines from neoplastic cells. Immunohistochemically, p53 overexpression was identified only in the pleomorphic spindle cells of the primary lesion and metastatic tumour. No features suggestive of vascular origin were shown on immunohistochemical or electron microscopic analysis of the neoplastic cells. Human herpesvirus type 8 was not detected by immunohistochemistry or PCR analysis. High levels of IL-6 and/or VEGF have been reported to play a role in CD. This is the first case report that clarifies the site of such cytokine production, showing the possibility of CD as a paraneoplastic phenomenon.
authors.affiliation: Department of Pathology, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
authors.forename: Chihiro
authors.initials: C
authors.lastname: Kakiuchi
authors.forename: Tsuyoshi
authors.initials: T
authors.lastname: Ishida
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Sato
authors.forename: Harutaka
authors.initials: H
authors.lastname: Katano
authors.forename: Tositaka
authors.initials: T
authors.lastname: Ishiko
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Mukai
authors.forename: Mieko
authors.initials: M
authors.lastname: Kogi
authors.forename: Naoki
authors.initials: N
authors.lastname: Kasuga
authors.forename: Kengo
authors.initials: K
authors.lastname: Takeuchi
authors.forename: Kenichi
authors.initials: K
authors.lastname: Yamane
authors.forename: Masashi
authors.initials: M
authors.lastname: Fukayama
authors.forename: Shigeo
authors.initials: S
authors.lastname: Mori
mesh_terms: D005871:Castleman Disease / Q000150:complications*; D016228:Endothelial Growth Factors / Q000378:metabolism*; D017809:Fatal Outcome; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D015850:Interleukin-6 / Q000378:metabolism*; D007621:Karyotyping; D008222:Lymphokines / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D012186:Retroperitoneal Neoplasms / Q000209:etiology* / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D012509:Sarcoma / Q000209:etiology* / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D014407:Tumor Cells, Cultured; D042461:Vascular Endothelial Growth Factor A; D042442:Vascular Endothelial Growth Factors
chemical_list: D016228:Endothelial Growth Factors; D015850:Interleukin-6; D008222:Lymphokines; D042461:Vascular Endothelial Growth Factor A; D042442:Vascular Endothelial Growth Factors