Follicular dendritic cell sarcoma complicated by hyaline‐vascular type Castleman's disease in a schizophrenic patient

Abstract

Follicular dendritic cell (FDC) sarcoma is an exceedingly rare neoplasm of unknown pathogenesis. A case of FDC sarcoma complicated by the hyaline-vascular type Castleman's disease occurring in a schizophrenic male is presented. Swelling of the left cervical lymph node appeared in a 44-year-old male schizophrenic who had been medicated with major tranquilizers for 20 years. He had had a history of cervical lymphadenopathy 14 years before, for which a diagnosis of hyaline-vascular type Castleman's disease had been made. The present specimen, obtained from the same site, was an enlarged lymph node heavily infiltrated with oval to spindle-shaped atypical cells but was uninvolved at the periphery. The infiltrating cells showed nodular or sheet-like growth, occasionally taking fascicular or storiform patterns. Follicular involvement was also common. Peculiarly, various amounts of small lymphocytes were intermingled with the neoplastic cells. The atypical cells expressed two FDC-specific antigens, DRC-1 and Ki-M4 antigen, together with a few other markers that are shared by FDC, including CD21 and HLA-DR. These findings clearly show the tumor to be FDC sarcoma. In addition, a peculiar fibro-hyalinous change in the lymph follicle, which is compatible with hyaline-vascular type Castleman's disease, was noted at the periphery of the lymph node where neoplastic cells had not infiltrated. Surprisingly, similar hyaline-vascular changes were observed in the previous biopsy taken 14 years ago. Meanwhile, Kaposi's sarcoma-associated herpesvirus, which is often identified from generalized Castleman's disease, was not identified in the present case by polymerase chain reaction study. Thus, this case is unique in two aspects: (i) the overlap of FDC sarcoma with hyaline-vascular type follicular changes; and (ii) its occurrence in a schizophrenic patient.

Dendritic cell neoplasms: An overview

“…Moreover, it usually contains distinct small [39,43] to medium [30] nucleoli, although prominent [44] nucleoli has also been reported. Mitotic figures are common but they are highly variable ranging from absent to 35 mf/10 hpf [25, 27,43]. The cytoplasm is eosinophilic, occasionally foamy or vacuolated, with undefined and indistinct borders [27,30,37,40,44,45].…”

“…The nucleus can exhibit one of various shapes: indented [32,33], walnut, cerebriform, bean-shaped [30], ovoid [39][40][41], and poly lobated [42]. Moreover, it usually contains distinct small [39,43] to medium [30] nucleoli, although prominent [44] nucleoli has also been reported. Mitotic figures are common but they are highly variable ranging from absent to 35 mf/10 hpf [25, 27,43].…”

“…R4/23, Ki-M4, and Ki-FDC1 are also specific for FDCT/S, but only R4/23 is commercially available [25,27,50]. Other positive markers include vimentin [25] and HLA-DR [30,43]. Variable positivity has been noted with S-100 [30,34,38,45], CD68 [27,34,35,38,45], Desmoplakin [27], and EMA [25,27].…”

“…CD21 and CD35 markers are most specific and can be performed on formalin fixed paraffin embedded materials [27], with almost universal positivity [25,27,28,35,[38][39][40][41][42][43][44][45][46][47][48][49]. It is important to note that frequent pathological mimickers such as thymoma, meningioma, and malignant fibrous histiocytoma lack these markers.…”

Follicular Dendritic Cell Sarcoma of Parapharyngeal Space: A Case Report and Review of the Literature

“…Few cases of FDCS have been associated with paraneoplastic pemphigus and hyaline vascular type Castleman disease [13,14]. FDCS is most often present as a painless slow growing mass, with median size of 5.0 cm [4].…”