Korean J Gastroenterol.  2013 Aug;62(2):131-134. 10.4166/kjg.2013.62.2.131.

Transformation of Castleman's Disease into Follicular Dendritic Cell Sarcoma, Presenting as an Asymptomatic Intra-abdominal Mass

• Hwang SO
• Lee TH
• Bae SH
• Cho HD
• Choi KH
• Park SH
• Kim CH
• Kim SJ

• KMID: 1775752
• DOI: http://doi.org/10.4166/kjg.2013.62.2.131

Abstract

Follicular dendritic cell (FDC) sarcoma is an extremely rare malignant neoplasm arising from FDCs. The exact origin of FDCs remains unclear; both a hematopoietic lineage origin and a stromal cell derivation have been proposed. Proliferation of FDCs can lead to benign reactive lesions or generate neoplastic conditions. The lesions are most commonly found in lymph nodes and usually involve the head and neck area. Castleman's disease is a rare non-neoplasitic lymphoproliferative disorder. Rare cases of hyaline-vascular Castleman's disease have been associated with FDC sarcoma, but a clonal relationship has not been convincingly demonstrated. A pathway toward tumor evolution, beginning with hyperplasia and dysplasia of FDCs, has been proposed. Despite this known association between Castleman's disease and FDC sarcoma, there have only been few reported cases of sarcoma arising as a complication of pre-existing Castleman's disease, especially in abdominal lesions. We describe here a 51-year-old female with an FDC sarcoma arising from unicentric, hyaline-vascular type Castleman's disease in an intra-abdominal mass. Pathologically, the lesion showed a series of changes during the process of transformation from Castleman's disease to FDC sarcoma.

Keyword

Follicular dendritic cell sarcoma; Castleman's disease

MeSH Terms

Abdomen/ultrasonography
Abdominal Neoplasms/*diagnosis/etiology/pathology
Dendritic Cell Sarcoma, Follicular/*diagnosis/etiology/pathology
Female
Giant Lymph Node Hyperplasia/complications/*diagnosis
Humans
Middle Aged
Positron-Emission Tomography
Tomography, X-Ray Computed

Figure

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  Fig. 1. Radiological findings. (A) A 4-cm well-enhancing mass was located around the liver, stomach, and pancreas (enhanced CT). (B) A 4.4-cm highly signal intensity mass lesion was noted in same lesion (MRCP, T2 weighted image).

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  Fig. 2. Gross findings after surgical resection. The encapsulated mass measured 6.0×5.0×3.0 cm and showed a yellowish-to-brown color with hemorrhagic spots.

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  Fig. 3. Histological features of the mass (H&E, ×400). Microscopic findings indicated an atrophic germinal center with a penetrating vessel and follicular dendritic cell hyperplasia (A), as well as fascicles of spindle cells with atypical vesicular nuclei and eosinophilic cytoplasm, with admixed lymphocytes (B).

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