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Follicular dendritic cell sarcoma of the spleen
Bjoern Sander1, Peter Middel, Bastian Gunawan
1Department of Pathology, University of Göttingen, 37099 Göttingen, Germany.
Insights
Diagnosing rare primary spindle cell tumors of the spleen is difficult. This case highlights a follicular dendritic cell (FDC) sarcoma with specific immunophenotypic and cytogenetic markers, including CD21, CD23, CD35, and Xp loss.
Area of Science:
- Oncology
- Pathology
- Genetics
Background:
- Primary spindle cell tumors of the spleen are rare and diagnostically challenging.
- Limited immunophenotypic and cytogenetic data exist for these entities.
Observation:
- A case of primary follicular dendritic cell (FDC) sarcoma in a 44-year-old woman is presented.
- Immunohistochemistry revealed positive staining for CD21, Ki-M4P, CD14, fascin, CD23, and CD35.
Findings:
- Cytogenetic analysis identified multiple clonal chromosomal aberrations.
- Unbalanced translocations resulted in gains at 3q, 7p, 8q, 9q and losses at Xp, 8p, 9p, 10p.
- Loss at chromosome Xp was noted, a recurrent finding in FDC tumors.
Implications:
- This case expands the understanding of FDC sarcoma immunophenotype and cytogenetics.
- The recurrent Xp loss suggests a potential role in FDC sarcoma pathogenesis.
- Improved characterization aids in the diagnosis and management of splenic spindle cell neoplasms.
Abstract:
Diagnosis of primary spindle cell tumors of the spleen is challenging because of the limited immunologic and cytogenetic characterization of this rare entity. We report a case of primary follicular dendritic cell (FDC) sarcoma of the spleen in a 44-year-old woman. Indications for FDC included positive staining for CD21, Ki-M4P, CD14, and fascin. Expression of both standard FDC markers CD23 and CD35 was detected immunohistochemically using tyramide signal amplification. Cytogenetic analysis revealed multiple clonal chromosomal aberrations involving unbalanced translocations of chromosomes X, 3, 5, 7, 8, 9, and 10, leading to net gains at 3q, 7p, 8q, and 9q and net losses at Xp, 8p, 9p, and 10p. Loss at Xp has been described previously in another tumor with FDC features, suggesting that this aberration might play a common role in this malignancy.
