Journal of Experimental & Clinical Cancer Research Case report Follicular dendritic cell sarcoma of the neck: Report of a case treated by surgical excision and COP plus (PEG)-liposomal doxorubicin Francesco Pisani*1, Mirella Marino2, Steno Sentinelli2 and Maria Concetta Petti1 BioMed Central Open Access Address: 1Department of Hematology, Regina Elena National Cancer Institute, Rome, Italy and 2Department of Phatology, Regina Elena National Cancer Institute, Rome, Italy Email: Francesco Pisani* - fr.pisani@tiscali.it; Mirella Marino - mirellamarino@inwind.it; Steno Sentinelli - steno8@virgilio.it; Maria Concetta Petti - mcpetti@ifo.it * Corresponding author Published: 2 September 2008 Journal of Experimental & Clinical Cancer Research 2008, 27:33 doi:10.1186/1756-9966-27-33 This article is available from: http://www.jeccr.com/content/27/1/33 Received: 12 June 2008 Accepted: 2 September 2008 © 2008 Pisani et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Abstract Background: Follicular dendritic cell (FDC) sarcoma is a rare neoplasm arising in lymph nodes but also in extranodal sites from accessory cells of the immune system that are essential for the function of antigen presentation and germinal center reaction regulation. FDC sarcoma has a significant recurrent and metastatic potential and for these reason it should be viewed as an intermediate grade malignancy. Methods: We report the case of a 49-year old woman patient who showed persistent, enlarged, hard, cervical lymph node. The most common histologic feature was the presence of oval to spindle cells with elongated nuclei, vesicular or stippled chromatin and scant eosinophilic cytoplasm. Immunohistochemically, tumor cells were diffusely positive for follicular dendritic cell markers CD21, CD23 and negative for cytokeratin. The patient after complete excision of the lymph node underwent five courses of adjuvant chemotherapy with COP plus PEG-liposomal doxorubicin, considering the propensity of the tumor to metastasize. Results: No hematological or cardiac toxicity were registered and among the other extra hematological effects only transitory palmar erythrodysesthesia is worthy of mention. After a follow up of 5 years the patient is alive and in CR. Conclusion: These results suggest that this therapeutic modality may be useful in the management of FDC sarcoma. Background Follicular dendritic cell sarcoma is a rare tumor arising from antigen-presenting cells of the B-cell follicles of nodal and extranodal sites; these cells are important for germinal center reaction regulation. It was first described in 1986 by Monda et al [1] and in recent years there has Page 1 of 4 (page number not for citation purposes)