Interdigitating dendritic cell sarcoma presenting simultaneously with acute myelomonocytic leukemia: report of a rare case and literature review.

Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare tumor derived from interdigitating dendritic cells. We report the first case of a 64-year-old Chinese woman who was diagnosed with simultaneous IDCS and acute myelomonocytic leukemia (AML-M4). The patient had undergone chemotherapy for breast cancer 6 years previously. Based on the laboratory results, both the IDCS and the AML-M4 in this patient were determined to be of myelogenous origination. Furthermore, a review of 62 IDCS cases (Medline database, key word: IDCS) reported to date revealed that as many as 17 % of the patients had malignant disease and received radiotherapy and/or chemotherapy prior to developing IDCS, and that this group of patients showed worse prognosis compared with counterparts. The patient in the present report showed poor response to four cycles of sequential chemotherapy, and died 6 months after the initial diagnosis.

Interdigitating dendritic cell sarcoma of the liver and lung: a case report with morphohological and immunohistochemical features of tumor.

Interdigitating dendritic cell sarcoma is extremely rare neoplasm that mainly occurs in the lymph nodes. Only 45 cases have been reported in the literature to date. We report a case of this sarcoma arising from the liver and lung, a previosly unreported site for this neoplasm. An 19-year-old girl deteriorated rapidly after artificial abortion and died 4 weeks later. Autopsy showed markedly enlarged liver and lung with numerous nodules up to 0.5 centimeters in diameter. Microscopically, nodules was composed of large pleomorphic cells that were immunohistochemically positive for proteins S-100 and vimentin, some of them expressed positivity to fascin and CD 68, with a rich small CD3 positive T lymphocytic infiltrateite around them. Based of these findings, the present case was diagnosed as interdigitating dendritic cell sarcoma, a neoplasm that remains a diagnostic and clinical challenge, because it can mimic a wide variety of other malignant tumors and tumor-like lesions.

Interdigitating dendritic cell sarcoma involving bone marrow in a liver transplant recipient.

Liver transplantation is an effective treatment for patients with many kinds of liver diseases. However, an increased risk of de novo malignancy has been reported in liver transplant recipients; immunosuppressive drugs have generally been identified as the primary culprit. Interdigitating dendritic cell sarcoma (IDCS) is an exceedingly rare neoplasm arising from antigen-presenting cells of the immune system. In this study, we have reported a case of IDCS with bone marrow involvement occurring in a 61-year-old female liver transplant recipient at 2 years after the procedure. She was admitted to our center with fever, cough, and expectoration. Physical examination revealed firm and painless nodes in both cervical and axillary fossae. Routine examination revealed an abnormal white blood cell count and elevated serum lactate dehydrogenase. Computerized tomography of the chest, abdomen, and pelvis were negative. Viral infections were also excluded. To obtain a definite diagnosis, we performed an excisional lymph node biopsy and a bone marrow biopsy. Microscopically, the tumor was composed of spindle cells with pale to eosinophilic cytoplasm, ill-defined cell borders, and large pleomorphic nuclei with prominent nucleoli. Immunophenotypic analysis demonstrated positive staining for S-100, vimentin, CD163, and CD68. Follicular dendritic cell, lymphoid, epithelial, myoepithelial, and melanoma markers were negative. Histology revealed bone marrow involvement. Taken together, the above features were consistent with IDCS with bone marrow involvement. She responded to chemotherapy. This case demonstrates the importance of cancer prevention and early detection for liver transplant recipients.

Metastatic interdigitating dendritic cell sarcoma masquerading as a skin primary tumor: a case report and review of the literature.

We report an unusual case of a lymph node interdigitating dendritic cell sarcoma (IDCS), metastatic to skin, in a 73-year-old patient. The patient initially presented as having a primary skin tumor with lymph node metastasis. The metastatic IDCS was initially read as an atypical fibroxanthoma. However, the morphology seen on the lymph node excision, paired with immunohistochemistry and electron microscopy studies, was diagnostic for an IDCS. Additional immunohistochemistry was performed on the shave biopsy, confirming that the skin tumor was a metastasis. IDCS is a rare tumor that belongs to the histiocytic and dendritic cell group of tumors. Diagnosing this entity is difficult without the aid of ancillary testing such as immunohistochemistry and electron microscopy. In the workup of a spindle cell neoplasm, IDCS should be included in the differential diagnosis.