Korean J Hematol.  2012 Jun;47(2):150-153. 10.5045/kjh.2012.47.2.150.

Successful treatment of disseminated interdigitating dendritic cell sarcoma with adriamycin, bleomycin, vinblastine, and dacarbazine chemotherapy

• Lee SY
• Lee SR
• Chang WJ
• Kim HS
• Kim BS
• Kim IS

• KMID: 2083611
• DOI: http://doi.org/10.5045/kjh.2012.47.2.150

Abstract

Interdigitating dendritic cell sarcoma (IDCS) is a very rare and aggressive neoplasm that arises from antigen presenting cells. IDCS usually involves lymph nodes; however, extra-nodal involvement has also been reported. Because a consistent standard therapy for IDCS has not been established to date, we report a case of the successful treatment of disseminated IDCS using ABVD chemotherapy (doxorubicin, bleomycin, vinblastine, and dacarbazine). A 64-year-old man was diagnosed with IDCS on the basis of immunohistochemical findings of a biopsy specimen of the inferior nasal concha. Immunohistochemical staining showed a positive reaction for CD68, leukocyte common antigen, and S-100 protein, but a negative reaction for CD34, CD1a, and CD21. Imaging studies showed cervical and axillary lymphadenopathies, subcutaneous nodules, and a soft tissue lesion in the nasal cavity. Treatment with the ABVD regimen resulted in complete remission after 8 cycles of chemotherapy.

Keyword

IDCS; ABVD chemotherapy; Complete remission

MeSH Terms

Antigen-Presenting Cells
Antigens, CD45
Biopsy
Bleomycin
Dacarbazine
Dendritic Cell Sarcoma, Interdigitating
Dendritic Cells
Humans
Middle Aged
Nasal Cavity
S100 Proteins
Turbinates
Vinblastine
Antigens, CD45
Bleomycin
Dacarbazine
S100 Proteins
Vinblastine

Figure

• 

  Fig. 1 Histological features of the nasal cavity include diffuse infiltration of spindle cells, large pleomorphic cells, foamy histiocytes, and various inflammatory cells (A). Immunohistochemical staining shows a strong positive reaction for S-100 protein (B).

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  Fig. 2 Computed tomography (CT) scans show multiple lymphadenopathies in both axillae (A) and lymphadenopathies on both sides of the neck (B).

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  Fig. 3 CT scans after 8 cycles of ABVD (adriamycin, bleomycin, vinblastine, and dacarbazine) shows complete resolution of axillary lymphadenopathies (A) and cervical lymphadenopathies (B).

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