A case of inﬂammatory pseudotumor-like
厚生連医誌
follicular/fibroblastic
第30巻 dendritic
1 号 31〜33
cell sarcoma
2021

Brief report

A case of inflammatory pseudotumor-likefollicular/fibroblastic dendritic cell sarcoma
Genetic laboratory, Department of pathology, Nagaoka Central HospitalToshihiko Ikarashi
The patient was female, 79 y/o, and complained
abdominal pain and vomiting. Radiographic study revealed
several abdominal masses: intra-pelvic tumor (Tp), expansive growth and 8.5cm in diameter, occupied mesentery
close to superior mesenteric vein and ileocolic artery, andhepatic expansive tumors occupied both S7 segment (TS7),6.5cm in diameter, and S8 (TS8), 5.5cm (Fig. 1, 2).
These tumors showed same macroscopic findings:
solid, white-and yellow colored, medium-consistency, andspotty necrosis. Microscopic study showed marked inﬂammatory change and several nodular lesions consisted ofpolygonal and spindle cells. These nodular areas were foundin Tp mass, and showed whorl formation consisted ofspindle cells without atypism and separated with thickbands of inflammatory cells around vessels (Fig. 3, 4).
Immunohistochemical analysis was positive for CD21,
CD35, fascin, and MDM2, which suggested follicular dendritic cell sarcoma (FDCS) (Table 1, Fig. 5). Genetic analysis with both PCR-electrophoresis and real time PCR
showed positivity for Epstein-Barr (EBV) infection andnegativity for immunoglobulin heavy chain (IgHVDJ) andT cell receptor (TCR)γ.
Based on positive EBV and predilection of aged
female patient, “inﬂammatory pseudotumor-like follicular/
fibroblastic dendritic cell (FDC/FRC) sarcoma” was suggested rather than simple “FDC sarcoma”.
Key words: nﬂammatory pseudotumor-like follicular/fibroblastic dendritic cell sarcoma, follicular dendritic cellsarcoma, FDC/FRC, CD21, CD35, fascin, MDM2,
Epstain-Barr virus infection, aged female
References
1 ．Pileri SA et al. Histiocytc and dendritic cell neoplasms
Chapter 17. In WHO Classification of tumours of haematopoietic and lymphoid tissues. Ed. Swerdlow SH etal. Lyon: International Agency for Research on Cancer,
2017. p465-584.
2 ．Pileri SA et al. Histiocytc and dendritic cell neoplasms.
Fig. 17.01, Table 17.02. In WHO Classification oftumours of haematopoietic and lymphoid tissues. Ed.
Swerdlow SH et al. Lyon: International Agency for
Research on Cancer, 2017. p466-7.

Acknowledgements.
The author wishes to express thanks to Drs. Tamotu
Sugai, Takashi Sato, Noriuki Uesugi (Iwate MedicalSchool), and Iwao Emura for their expert assistances.
和文抄録短報
炎症性偽腫瘍様濾胞／線維芽細胞肉腫の 1 症例
JA 新潟県厚生連長岡中央綜合病院，病理部・遺伝子検査室
五十嵐俊彦
症例は骨盤内腫瘍と肝臓内多発腫瘍を認めた高齢女性で，免疫組織検査で濾胞樹状細胞肉腫が示唆された．
高齢・女性，高度の炎症，Epstein-Barr ウイルス感染陽性より，炎症性偽腫瘍様濾胞／線維芽細胞肉腫が妥当と判断された．
キーワード：炎症性偽腫瘍様濾胞／線維芽細胞肉腫，濾胞樹状細胞肉腫，免疫組織学的検査，FDC/FRC, CD21, CD35, fascin, MDM2, Epstain-Barr ウ
イルス感染，高齢，女性－ 31 －

厚生連医誌

第30巻

1号

31〜33

2021

Table 1. Immunophenotypic markers of macrophage and dendritic cell (DC)markerDCmacrophage

dermal/interstitial DC

Langerhans
cellinterdigitatingDC

plasmacytoidDCfollicular DC

this caseCD21++

CD35++++

CD68+

fascinS100++

langerinlysozyme++++++

MDM2+

DC: dendritic cell
Sources: Pileri SA et al. Histiocytc and dendritic cell neoplasms. Fig. 17.01, Table 17.02.
In WHO Classiﬁcation of tumours of haematopoietic and lymphoid tissues.
Ed. Swerdlow SH et al. Lyon: International Agency for Research on Cancer, 2017. p466-7.

Fig. 1. Intrapelvic tumor (Tp)

Fig. 2. Hepatic tumor (TS8)－ 32 －

A case of inﬂammatory pseudotumor-like follicular/fibroblastic dendritic cell sarcoma

Fig. 3. Tp nodular lesion (Hematoxylin eosin stain, loupe)

Fig. 4. Whorl formation in Fig. 4 (HE stain)

Fig. 5. Positive immunostain for fascin.

－ 33 －