A Case of Disseminated Extranodal Interdigitating Dendritic Cell Sarcoma Arising from Parotid Gland

Article information

Kosin Med J. 2015;30(2):163-169
Publication date (electronic) : 2015 January 20
doi : https://doi.org/10.7180/kmj.2015.30.2.163
1Department of Internal Medicine and School of Medicine, Incheon, Korea
2Department of Pathology, Inha University Hospital and School of Medicine, Incheon, Korea
Corresponding Author: Chul Soo Kim, Department of Internal Medicine, Inha University Hospital and School of Medicine, 27, Inhang-ro, Jung-gu, Incheon 22332, Korea Tel: +82-32-890-2588 Fax: +82-32-890-2585 E-mail: cskimmd@inha.ac.kr
Received 2014 September 24; 2014 October 21; Accepted 2014 November 01.

Abstract

Abstract

Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare tumor derived from professional antigen presenting cell and primarily found in lymph nodes, with rarer case report about extranodal presentation of IDCS. A 71-yr-old man was admitted with progressively enlarging and painless mass in the right parotid area for 2 months. Computed tomography of the neck and chest revealed enhancing mass in right parotid gland, multiple lymphadenopathies around neck and mediastinum, and an osteolytic metastasis at thoracic spine. Morphological and immunohistochemical analysis of an excisional biopsy specimen from parotid mass were consistent with a diagnosis of IDCS. Palliative chemotherapy with 6 cycles of CHOP (cyclophosphamide, adriamycin, vincristine, and prednisolone) regimen and 2 cycles of ABVD (adriamycin, bleomycin, vinblastine, and dacarbazine) regimen plus radiotherapy on parotid mass failed in tumor reduction. We describe a rare case of disseminated extranodal IDCS arising from parotid gland.

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Demographic and clinical features of reported and Korean cases.

References

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Table 1.

Demographic and clinical features of reported and Korean cases.

  Reported cases (N = 100) Korean cases (N = 5)
Age    
Median (range), yr 56.5 (1.8 – 88) 57 (32 – 73)
Sex    
Male:female (ratio) 58:42 (1.38:1) 2:3 (1:1.5)
Presentationa    
Localized 59% (45/76) 20% (1/5)
Locally advanced 5.3% (4/76) 40% (2/5)
Metastatic 35.6% (27/76) 40% (2/5)
Involved sites (number of cases)    
Lymph nodes    
Cervical 41 2
Axillary 22 2
Mediastinal 9
Intraabdominal 18
Exrtanodal sites    
Tonsil 3 1
Nasopharynx 3 2
Soft tissue 1 1
Bone 5 1
Pleura 3 1
Treatment (number of cases)a    
Surgery alone 21/76
Surgery plus adjuvant therapy 18/76 1/5
Radiation therapy alone 6/76 1/5
Chemotherapy alone 26/76 2/5
Chemotherapy plus radiation therapy 2/76 1/5
No treatment 2/76
Unknown 1/76
a

Data obtained from 76 out of 100 reported cases were analyzed.