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Histiocytic and Dendritic Cell Neoplasms
Published in Dongyou Liu, Tumors and Cancers, 2017
Classified along with mature B-cell neoplasms, mature T and NK neoplasms, Hodgkin lymphoma, and post-transplant lymphoproliferative disorders (PTLD) under the mature lymphoid, histiocytic, and dendritic neoplasms category, histiocytic and dendritic cell neoplasms consist of (i) histiocytic sarcoma (HS), (ii) Langerhans cell histiocytosis (LCH), (iii) Langerhans cell sarcoma (LCS), (iv) indeterminate dendritic cell tumor (IDCT), (v) interdigitating dendritic cell sarcoma (IDCS), (vi) follicular dendritic cell sarcoma (FDCS), (vii) disseminated juvenile xanthogranuloma (DJX), and (viii) Erdheim–Chester disease (ECD) (Table 27.1) [1].
Successful Treatment of Interdigitating Dendritic Cell Sarcoma Presenting as Multiple Parotid Tumors
Published in Acta Oto-Laryngologica Case Reports, 2019
Yasuyuki Kajimoto, Naoki Otsuki, Masanori Teshima, Yukiko Morinaga, Tomoo Itoh, Ken-Ichi Nibu
The surgical specimens were whitish in color and soft (Figure 2(B)). Microscopic examination of the tumor specimens from the parotid gland revealed that large clear cells propagated between neutrophils and lymphocytes. Tumor cells contained large nuclei with unique Notch complexes. The findings from the specimen of the lymph node were similar, but increased nuclear pleomorphism with unusual giant cells, and brisk mitotic activity were observed. Immunohistochemical studies showed diffuse positivity for S-100, CD163, and CD4. The specimen tested negative for all other markers: CD1a, CD21, CD30, HMB45, AE1/3, αSMA, and langerin (Figure 3). The positivity of S-100, CD163, and CD4 indicated that the tumor was a histiocytic/dendritic cell tumor. The negativity of CD21 and CD23 did not correspond to a diagnosis of follicular dendritic cell sarcoma, whereas the negativity of CD1a and langerin did not correspond to a diagnosis of Langerhans cell sarcoma. Based on these finding, the final pathological diagnosis of IDCS was made.
A case of spindle cell dominant histiocytic sarcoma showing a complete remission after first-line chemotherapy with doxorubicin and ifosfamide
Published in Journal of Chemotherapy, 2020
Yusuke Nakamura, Akihiro Takemasa, Yoshitomo Kushima, Sayo Soda, Naoya Ikeda, Ryo Arai, Kazuyuki Chibana, Yoshimasa Nakazato, Tomoyuki Yokose, Yasuo Shimizu, Seiji Niho
Histiocytic sarcoma (HS) is an exceedingly rare but aggressive hematopoietic tumor in which the tumor cells show histiocytic features on immunostaining,1,2 and accounts for less than 1% of hematolymphoid neoplasms.2,3 The median age of patients with HS is 46–55 years old, and tends to be slightly male predominant (male/female = 2.6:1).2,3 HS is typically composed of round to oval tumor cells with limited spindle cell foci,2 but some tumors are spindle cell predominant.4–6 A definite diagnosis of HS is made by immunostaining for histiocyte markers (CD63 and CD163) and excluding other malignacies,2 e.g., B cell or T cell lymphoma, Langerhans cell sarcoma (LCS), and dendritic cell sarcoma. Lymph nodes are the most susceptible primary sites for HS (about 15%), followed by the gastrointestinal tract, spleen, soft tissues, and skin.2 Surgical resection or radical radiation therapy is performed in patients with early disease and localized lesions, while patients with advanced stages are usually treated with multidrug chemotherapy.4,7 The commonly used and reported multidrug chemotherapy regimens are: CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone), ICE (ifosfamide, carboplatin, and etoposide), ProMACE-MOPP (prednisone, methotrexate, doxorubicin, cyclophosphamide, etoposide, mechlorethamine, vincristine, and procarbazine), and CLAG-M (cladribine, cytarabine, G-CSF, and mitoxantrone).7,8 Recently, a case report was published, which showed the effectiveness of AI therapy for stage IV HS;9 however, HS was not diagnosed at the initial treatment. The initial diagnosis was Erdheim–Chester disease (ECD), a rare non-Langerhans histiocytic neoplasm.10 Given the lack of response to three ECD treatment regimens, empiric chemotherapy with AI therapy was started. Complete remission (CR) with AI therapy was achieved after two cycles. In general, HS shows multidrug chemotherapy resistance or early relapses.4 The response rate to chemotherapy as well as progression-free survival and overall survival are uncertain due to the rarity of HS; however, most patients are reported to die of progressive disease within two years.2