Dendritic cell sarcoma: An analytic overview of the literature and presentation of original five cases

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Abstract

Interdigitating and follicular dendritic cell sarcoma (DCS) are very rare diseases, with approximately 184 cases being reported thus far, and their best treatment modality is still on debate. This is a review of all the cases of dendritic cell sarcoma reported from 1981 until April 2006. This review is enriched with the original contribution of five cases occurred at our Institution from 1994 to 2006.

The review of the literature pointed out that radical surgery alone was curative in approximately two thirds of these cases, the relapsing rate in patients who received adjuvant treatments being higher than 30%. We pinpoint new five cases of dendritic cell sarcoma (three FDCS and two IDCS).

Both the analytic revision of the literature and our data suggest that localized DCS may be effectively treated by radical surgery and do not support the use of adjuvant treatments after radical excision.

Introduction

Tumors arising from dendritic cells (follicular dendritic cell sarcoma/tumor, FDCS and interdigitating dendritic cell sarcoma/tumor, IDCS) (WHO) are exceedingly rare [1], [2]. Although most of dendritic cell sarcomas (DCS) arise in lymph nodes, especially of the cervical, mediastinal, and axillary region, approximately one-third of the cases involve extra-nodal sites. The disease is slightly more common in male (ratio male/female 1.4:1) [1], and may affect any age (range 14–80 years), although most of the cases have been reported in the middle age (mean, 46 years). The behavior of these tumors is akin to that of a low-grade soft tissue sarcoma, with a risk of developing local recurrences and distant metastases approaching 30%. Most of the patients have been treated by surgery, often followed by radiotherapy or chemotherapy, but the value of these adjuvant treatments in effectively improving survival has not been convincingly demonstrated. Here, we report our experience on five cases of nodal (two cases) and extra-nodal DCS (three cases of FDCS and two cases of IDCS): all the patients except one received surgical excision of the tumor as the unique treatment, and only one of them, with a hepatic FDCS, died because of the disease soon after the diagnosis, the remaining four being alive and free of disease. Similarly, the data stemming from the literature pointed out that DCS are mostly (59.7%) localized at diagnosis, and that radical surgery, without any adjuvant treatment, is curative in approximately two thirds (61.8%) of the cases [1], [2].

Section snippets

Histological features

According to WHO DCS are included in histiocytic and dendritic-cell neoplasms (Table 1) [3]. Macroscopically DCS appearance is similar to that of other sarcomas with a well-circumscribed tan grey cut surface. The histological appearance of FDCS is that of a spindled and ovoid cell proliferation forming fascicles and a storiform pattern with whirling, reminiscent of a meningioma. Tumor cells have plump eosinophilic cytoplasm and indistinct cell borders. Nuclei are elongated with vesicular or

Methods

References were obtained by the major indexed literature database Medline, using the keywords dendritic cell, sarcoma, and lymphoma. We reviewed all the cases of dendritic cell sarcoma reported from 1981 (first case published) until April 2006. For the analytic revision of the cases were included only the articles in English focused on clinical reports convincingly documenting the diagnosis, and clearly reporting the treatments administered and follow-up data.

Table 2 shows the clinical features

Methods

The cases of DCS were retrieved from the 1994–2006 files of the Division of Pathology of the European Institute of Oncology of Milan. For each patient, the following clinico-pathologic information were collected: past medical history, clinical status, Eastern Cooperative Oncology Group (ECOG) performance status, type of surgery, tumor size, histology and clinical stage, and adjuvant or neo-adjuvant treatment (radiotherapy and/or chemotherapy). In all the cases, the diagnosis was confirmed

Conclusion

Tumors composed by interdigitating and follicular dendritic cells are exceedingly rare, with less than two hundreds of cases reported thus far. Accordingly, we found only five cases of DCS in our 1994–2006 files, accounting for 0.4% of all soft tissue sarcomas. As a consequence, data concerning the clinical behavior of DCS are not consolidated, and their treatment is still a matter of debate. We describe the clinico-pathological characteristics of five cases of dendritic cell sarcoma (two cases

Reviewer

Dr. Alvaro Diaz De Liaño, Department of General Surgery, University Hospital Virgen del Camino, Pintor. Maeztu 2, 8°C, ES-31008 Pamplona, Navarra, Spain.

Tommaso Martino De Pas is senior deputy, Director of the Pharmacology and New Drugs Unit of European Institute of Oncology, Milan, Italy. He is referred especially lung and rare tumors. Member of the Board for the Italian Sarcoma Group, he authored over 50 articles on important international journals.

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    Tommaso Martino De Pas is senior deputy, Director of the Pharmacology and New Drugs Unit of European Institute of Oncology, Milan, Italy. He is referred especially lung and rare tumors. Member of the Board for the Italian Sarcoma Group, he authored over 50 articles on important international journals.

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