A recent literature review by Saygin et al reported 343 published cases of FDCS but no specific data are reported on intra-abdominal disease.18

Etiopathogenesis of FDCS remains unclear. A stromal cell derivation origin and a hematopoietic lineage origin have been proposed. Hwang So et al have reported a transformation of Castleman disease into an intra-abdominal FDCS.19

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Table 1 shows the clinical features of the 17 cases of intra abdominal FDCS reported so far.2–12,14,15 Complete surgical excision is mainly proposed for FDCS, but also for local resectable disease. 

According to small series and reviews, chemotherapy and radiotherapy have not yet proven their effectiveness for the treatment of FDCS.2,3,18,20 In the present case, the perigastric mass did not seem completely resectable and there was retroperitoneal lymph node involvement. The disease was considered locally advanced. Advanced intraperitoneal FDCS is a very severe condition where treatment options are not well established.

(To view a larger version of Table 1, click here.)

Since curative surgery was not possible for this patient, we opted for a medical treatment with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) chemotherapy (identical protocol as used for aggressive lymphomas).

The choice of this regimen in our case was based on a literature review where the association of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) was widely used and showed its efficacy.13 Other chemotherapeutic such as ICE (ifosfamid, carboplatin, etoposide), CHEOP, and ABVD (adriamycin, bleomycin, vincristine, dacarbazine) regi men have also been proposed.14

Given the bad prognosis of the disease, the recurrence after conventional chemotherapy and the availability of a familial donor, an allogeneic hematopoietic stem cell transplantation (HSCT) was decided following the report of a similar case in the literature.17

No rituximab was added because of the negativity of CD20. Even though a macroscopic mass was still in place after four cycles of chemotherapy, with reduced FDG capitation, a complete metabolic and radiologic response was achieved after six courses of chemotherapy.

This observation suggests that chemotherapy can be effective in locally advanced FDCS. Longer follow-up and further cases are definitely needed in order to draw any conclusion. However, six courses of CHOP chemotherapy might be considered an option for patients with unresectable FDCS lesions.