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Case Report Open Access

Unicentric Castleman disease with paraneoplastic pemphigus and follicular dendritic cell sarcoma: A case report

  • Authors:
    • Yukun Li
    • Fanchen Zeng
    • Zeyao Wang
    • Ganjian Zhao
    • Qi Zhou
  • View Affiliations / Copyright

    Affiliations: General Surgery Department, HuiYa Hospital of The First Affiliated Hospital, Sun Yat‑sen University, Huizhou, Guangdong 516200, P.R. China
    Copyright: © Li et al. This is an open access article distributed under the terms of Creative Commons Attribution License.
  • Article Number: 55
    |
    Published online on: November 27, 2025
       https://doi.org/10.3892/ol.2025.15408
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Abstract

Castleman disease (CD) is a rare lymphoproliferative disorder that may be complicated by paraneoplastic pemphigus (PNP) and follicular dendritic cell sarcoma (FDCS), although their concurrence is uncommon. The present study reported on a 67‑year‑old woman with progressive oral ulceration and generalized purple skin spots. Imaging demonstrated a 114mm x96mm x118 mm lesion in the left pelvis. Serology was positive for anti‑desmoplakin‑1 and ‑3 and anti‑BP230 antibodies. Histopathology revealed hyaline‑vascular unicentric CD with focal low‑to‑intermediate‑grade FDCS; immunohistochemistry showed CD21, CD23, CD35, CD68, CXCL13 expression and Ki‑67 ~10%. Initial therapy comprised high‑dose methylprednisolone, intravenous immunoglobulin and thalidomide, followed by surgical resection and adjuvant thalidomide‑cyclophosphamide‑prednisone. Postoperatively, inflammatory indices normalized and mucocutaneous lesions partially improved; nevertheless, the patient died of respiratory failure 6 months after surgery. In summary, the present case report aimed to provide valuable references and experiences for clinicians in the diagnosis and treatment of CD featuring PNP and FDCS.
View Figures

Figure 1

The skin symptoms of the patient. (A)
Lips of the patient were ulcerated, with black crust formation and
yellow secretion exudation. (B) White scurf on the labia majora and
ulceration on the labia minora side. (C) Angular purplish purple
skin spots with a clear boundary is seen in perineum area. (D) The
appearance of the improved lip after treatment (from July to August
2024).

Figure 2

Laryngoscopy and imaging findings. (A)
Erosions and white pseudomembranous exudates on laryngoscopy. (B)
The imaging revealed bronchitis and mild bronchial dilation in both
lungs (indicated by red arrows). (C) Pelvic CT plain scan exhibited
a solitary mass in the left pelvic region (indicated by red arrow).
(D) Transvaginal ultrasonography showing a hypoechoic mass
measuring ~114×86×118 mm.

Figure 3

Pathological manifestations of tumors.
(A) H&E staining demonstrated regional differences: The left
side exhibited a concentric (‘onion-skin’, which marked with red
arrows) configuration with attenuation of lymphoid follicles,
whereas the right side exhibited effacement of the normal
follicular architecture with replacement by a compressive mass
consistent with progression to a follicular dendritic cell tumor
(which marked with yellow arrows). (magnification, ×100; scale bar,
100 µm). Immunohistochemical staining of the pelvic mass
demonstrated dark areas of (B) CD21, (C) CD23 and (D) CD35, which
were focally positive, suggesting follicular dendritic cell sarcoma
(magnification, ×100; scale bar, 100 µm).

Figure 4

Timeline of the clinical course of the
patient. The minus sign represents the time before admission and
the plus sign represents the time after admission. PNP,
paraneoplastic pemphigus; UCD, unicentric Castleman disease; FDCS,
follicular dendritic cell sarcoma.
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Copy and paste a formatted citation
Spandidos Publications style
Li Y, Zeng F, Wang Z, Zhao G and Zhou Q: Unicentric Castleman disease with paraneoplastic pemphigus and follicular dendritic cell sarcoma: A case report. Oncol Lett 31: 55, 2026.
APA
Li, Y., Zeng, F., Wang, Z., Zhao, G., & Zhou, Q. (2026). Unicentric Castleman disease with paraneoplastic pemphigus and follicular dendritic cell sarcoma: A case report. Oncology Letters, 31, 55. https://doi.org/10.3892/ol.2025.15408
MLA
Li, Y., Zeng, F., Wang, Z., Zhao, G., Zhou, Q."Unicentric Castleman disease with paraneoplastic pemphigus and follicular dendritic cell sarcoma: A case report". Oncology Letters 31.2 (2026): 55.
Chicago
Li, Y., Zeng, F., Wang, Z., Zhao, G., Zhou, Q."Unicentric Castleman disease with paraneoplastic pemphigus and follicular dendritic cell sarcoma: A case report". Oncology Letters 31, no. 2 (2026): 55. https://doi.org/10.3892/ol.2025.15408
Copy and paste a formatted citation
x
Spandidos Publications style
Li Y, Zeng F, Wang Z, Zhao G and Zhou Q: Unicentric Castleman disease with paraneoplastic pemphigus and follicular dendritic cell sarcoma: A case report. Oncol Lett 31: 55, 2026.
APA
Li, Y., Zeng, F., Wang, Z., Zhao, G., & Zhou, Q. (2026). Unicentric Castleman disease with paraneoplastic pemphigus and follicular dendritic cell sarcoma: A case report. Oncology Letters, 31, 55. https://doi.org/10.3892/ol.2025.15408
MLA
Li, Y., Zeng, F., Wang, Z., Zhao, G., Zhou, Q."Unicentric Castleman disease with paraneoplastic pemphigus and follicular dendritic cell sarcoma: A case report". Oncology Letters 31.2 (2026): 55.
Chicago
Li, Y., Zeng, F., Wang, Z., Zhao, G., Zhou, Q."Unicentric Castleman disease with paraneoplastic pemphigus and follicular dendritic cell sarcoma: A case report". Oncology Letters 31, no. 2 (2026): 55. https://doi.org/10.3892/ol.2025.15408
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