No abstract available.
Dendritic Cell Sarcoma, Interdigitating* ; Dendritic Cells* ; Lymphoma*

Dendritic Cell Sarcoma, Interdigitating ; Humans ; Lymph Nodes ; Male ; Middle Aged

Dendritic Cell Sarcoma, Interdigitating ; Dendritic Cells ; Hematopoietic Stem Cell Transplantation ; Humans ; Lymphoma, Non-Hodgkin

Dendritic Cell Sarcoma, Follicular ; pathology ; Dendritic Cell Sarcoma, Interdigitating ; pathology ; Female ; Humans ; Middle Aged ; Stomach Neoplasms ; pathology

Adult ; Dendritic Cell Sarcoma, Interdigitating ; complications ; surgery ; Humans ; Male ; Paraneoplastic Syndromes ; etiology ; Pemphigus ; etiology

Interdigitating dendritic cell sarcoma (IDCS) is a very rare disease around the world and its prognosis is known to be aggressive. This reports a case diagnosed as IDCS of the axillary region treated in Soonchunhyang University Hospital. A 57-year-old female visited Soonchunhyang University Hospital with a left axillary mass. The mass was hard and fixed. Computed tomography observed a 7 cm lymph node at the left axilla, and core biopsy suspected sarcoma. In another study, there was no specific finding except the axillary lesion. Left axillary lymph node dissection (level I, II) was conducted and the pathologic report finally showed IDCS. The patient was treated with only radiotherapy and followed up without recurrence for 13 months up to now. IDCS is a very rare sarcoma that is hard to diagnose and progresses fast. Thus, treatment is very difficult. Proper treatment can be better established after more experiences.
Axilla ; Biopsy ; Dendritic Cell Sarcoma, Interdigitating ; Dendritic Cells ; Female ; Humans ; Lymph Node Excision ; Lymph Nodes ; Middle Aged ; Polyenes ; Prognosis ; Rare Diseases ; Recurrence ; Sarcoma

Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare neoplasm arising from the antigen-presenting cells of the immune system. This disease usually involves the lymph nodes, and rarely, extranodal sites may be affected. The authors report a case of extranodal IDCS presenting in the pleura. A 32-yr-old man presented with progressive chest pain. Imaging studies showed diffuse pleural thickening with pleural effusion. Morphological and immunohistochemical analysis of an incisional biopsy of the pleura were consistent with a diagnosis of IDCS; tumor cells were positive for S100 and CD45, but negative for CD1a, CD21, CD35, B cell and T cell markers. The patient was administered chemotherapy, but died of progressive disease. Although its incidence is extremely rare, this case suggests that extranodal IDCS should be considered in the differential diagnosis of undifferentiated neoplasms and that immunohistochemical staining be performed using appropriate markers.
Adult ; Dendritic Cell Sarcoma, Interdigitating/diagnosis/*pathology ; Fatal Outcome ; Humans ; Male ; Pleura/*pathology ; Pleural Neoplasms/diagnosis/*pathology ; Tumor Markers, Biological

OBJECTIVETo describe the clinical and pathological features, treatment and prognosis of dendritic cell sarcoma (DCS).

METHODSA group of DCS was described, including two cases of follicular dendritic cell sarcoma (FDCS), one each of interdigitating dendritic cell sarcoma (IDCS) and langerhans cell sarcoma (LCS). The related English literatures were reviewed.

RESULTSTwo patients with IDCS were a 19-year-old man and a 45-year-old woman respectively, both exhibited fever of unknown origin and painless lymphadenopathy. Pathological diagnosis of lymph node biopsy was FDCS with positive CD21 and CD35. Both patients achieved complete remission (CR) after 6 cycles of chemotherapy (CHOP: cyclophosphamide, epirubicin, vindesine, and prednisolone). However, the male patient relapsed 5 months later and another patient was still in CR at 5 months follow-up. One case of IDCS was a 42-year-old man, who manifested as paraneoplastic pemphigus. Biopsy of mediastinal lymph node demonstrated IDCS and immunohistochemistry showed positive S-100 staining. This patient died of pneumonia after two cycles of CHOP. One patient of LCS was a 54-years-old woman with fever, painless lymphadenopathy and diffused pulmonary nodules. The diagnosis of LCS was established after excisional biopsy was taken from inguinal lymph node. Positive staining of CD1a and S-100 was displayed by immunohistochemistry. Electron microscope examination confirmed the presence of Birbeck granule in tumor cells. Four cycles of chemotherapy (including ECHOP, FND) were administered, but the disease progressed.

CONCLUSIONDCS is a group of very rare sarcoma, FDCS, IDCS and LCS have different characteristic clinical features, immunophenotype and prognosis. The prognosis of most patients is poor.

Adult ; Dendritic Cell Sarcoma, Interdigitating ; pathology ; therapy ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Prognosis ; Young Adult

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.
Biopsy ; Bleomycin ; Dendritic Cell Sarcoma, Interdigitating* ; Dendritic Cells* ; Diagnosis ; Doxorubicin ; Drug Therapy ; Lymph Nodes ; Mediastinum ; Neck ; Neoplasm Metastasis ; Parotid Gland* ; Radiotherapy ; Spine ; Thorax ; Vinblastine ; Vincristine

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