Last update 01 Nov 2024

Histiocytic and Dendritic Cell Neoplasm

Last update 01 Nov 2024

Basic Info

Synonyms

Histiocytic and Dendritic Cell Neoplasm, Histiocytic and Dendritic Cell Neoplasms, Histiocytic and Dendritic Cell Tumors

+ [1]

Introduction

A group of rare tumors that affect the hematopoietic and lymphoid tissues. The cells of origin are the histiocytes and accessory cells. This category includes the following: Langerhans cell histiocytosis, Langerhans cell sarcoma, indeterminate dendritic cell histiocytosis, interdigitating dendritic cell sarcoma, histiocytic sarcoma, disseminated juvenile xanthogranuloma, Erdheim-Chester disease, Rosai-Dorfman-Destombes disease, ALK-positive histiocytosis, and follicular dendritic cell sarcoma.

Drugs associated with Histiocytic and Dendritic Cell Neoplasm

Selinexor

Target

XPO1

Mechanism

XPO1 inhibitors

Active Org.

Karyopharm Therapeutics, Inc. [+7]

Originator Org.

Karyopharm Therapeutics, Inc.

Active Indication

Plasma cell myeloma refractory [+69]

Inactive Indication

Acute Promyelocytic Leukemia [+52]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

First Approval Date03 Jul 2019

Luvometinib

Target

MEK1 x MEK2

Mechanism

MEK1 inhibitors [+1]

Active Org.

Asymchem Life Science Tianjin Co. Ltd. [+2]

Originator Org.

Chongqing Fuchuang Pharmaceuticals Research Co., Ltd.

Active Indication

NF1 mutant Plexiform Neurofibroma [+7]

Inactive Indication

Melanoma [+1]

Drug Highest PhaseNDA/BLA

First Approval Ctry. / Loc.-

First Approval Date20 Jan 1800

Choline Salicylate

Target-

Mechanism-

Active Org.

National Cancer Institute

Originator Org.

Mayo Clinic

Active Indication

Histiocytic and Dendritic Cell Neoplasm [+9]

Inactive Indication

Diffuse Large B-Cell Lymphoma [+1]

Drug Highest PhasePhase 1

First Approval Ctry. / Loc.-

First Approval Date20 Jan 1800

Clinical Trials associated with Histiocytic and Dendritic Cell Neoplasm

NCT06508463 / RecruitingPhase 1IIT

Phase I Trial of Systemic Administration of Vesicular Stomatitis Virus Genetically Engineered to Express NIS and Human Interferon, in Patients With Relapsed or Refractory Multiple Myeloma, Acute Myeloid Leukemia, Lymphomas, or Histiocytic/Dendritic Cell Neoplasms

This phase I trial studies the best dose and side effects of recombinant vesicular stomatitis virus (VSV) carrying the human (h) sodium iodide symporter (NIS) and Interferon (IFN) beta (β) genes (VSV-hIFNβ-NIS) in combination with ipilimumab and cemiplimab in patients with T-cell lymphoma. A virus, called VSV-hIFNβ-NIS, which has been changed in a certain way, may be able to kill cancer cells without damaging normal cells. Immunotherapy with ipilmumab and cemiplimab may induce changes in body's immune system and may interfere with the ability of tumor cells to grow and spread.

Start Date05 Jan 2024

Sponsor / Collaborator

Mayo Clinic

[+1]

NCT04640779 / RecruitingPhase 1IIT

Phase Ib Trial of Low-Dose Selinexor (KPT-330) in Combination with Choline Salicylate (CS) for the Treatment of Patients with Non-Hodgkin Lymphoma (NHL), Hodgkin Lymphoma, Histiocytic/Dendritic Cell Neoplasms, or Multiple Myeloma

This phase Ib trial evaluates the side effects and best dose of choline salicylate given together with a low dose of selinexor in treating patients with non-Hodgkin or Hodgkin lymphoma, or multiple myeloma whose prior treatment did not help their cancer (refractory) or for patients with histiocytic/dendritic cell neoplasm. Anti-inflammatory drugs, such as choline salicylate lower the body's immune response and are used with other drugs in the treatment of some types of cancer. Selinexor may stop the growth of cancer cells by blocking a protein called CRM1 that is needed for cell growth. This trial may help doctors learn more about selinexor and choline salicylate as a treatment for with non-Hodgkin or Hodgkin lymphoma, histiocytic/dendritic cell neoplasm, multiple myeloma.

Start Date08 Feb 2021

Sponsor / Collaborator

Mayo Clinic

[+1]

NCT03017820 / RecruitingPhase 1IIT

This phase I trial studies the best dose and side effects of recombinant vesicular stomatitis virus carrying the human NIS and IFN beta genes (VSV-hIFNbeta-sodium iodide symporter [NIS]) with or without cyclophosphamide or ipilimumab and nivolumab or cemiplimab in treating patients with multiple myeloma, acute myeloid leukemia (AML) or lymphoma that has come back or does not respond to treatment. A virus, called VSV-hIFNbeta-NIS, which has been changed in a certain way, may be able to kill cancer cells without damaging normal cells. Cyclophosphamide is in a class of medications called alkylating agents. It works by damaging the cell's DNA and may kill cancer cells. It may also lower the body's immune response. Immunotherapy with ipilmumab and nivolumab or cemiplimab may induce changes in body's immune system and may interfere with the ability of tumor cells to grow and spread. Giving VSV-hIFNbeta-NIS and ruxolitinib phosphate may work better at treating multiple myeloma, acute myeloid leukemia and T-cell lymphoma.

Start Date04 Apr 2017

Sponsor / Collaborator

Mayo Clinic

[+1]

100 Clinical Results associated with Histiocytic and Dendritic Cell Neoplasm

100 Translational Medicine associated with Histiocytic and Dendritic Cell Neoplasm

0 Patents (Medical) associated with Histiocytic and Dendritic Cell Neoplasm

Literatures (Medical) associated with Histiocytic and Dendritic Cell Neoplasm

01 Jan 2024·Modern Pathology

Cutaneous Manifestations of Myeloid Neoplasms Exhibit Broad and Divergent Morphologic and Immunophenotypic Features but Share Ancestral Clonal Mutations With Bone Marrow

Article

Author: Luskin, Marlise R ; Kim, Annette S ; Rodig, Scott J ; Pinkus, Geraldine S ; Shanmugam, Vignesh ; Lane, Andrew A ; Pozdnyakova, Olga ; Garcia, Jacqueline S ; Zemmour, David ; Lucas, Fabienne ; Hergott, Christopher B ; Sadigh, Sam ; Morgan, Elizabeth A ; Tsai, Harrison K ; Winer, Eric S ; DeAngelo, Daniel J ; Lovitch, Scott B ; Hasserjian, Robert P

In this study, we performed a comprehensive molecular analysis of paired skin and peripheral blood/bone marrow (BM) samples from 17 patients with cutaneous myeloid or cutaneous histiocytic-dendritic neoplasms. The cutaneous manifestations included 10 patients with cutaneous acute myeloid leukemia (c-AML), 2 patients with full or partial Langerhans cell differentiation, 2 patients with blastic plasmacytoid dendritic cell neoplasms (BPDCN), 1 patient with both Langerhans cell differentiation and BPDCN, and 2 patients with full or partial indeterminate dendritic cell differentiation. Seven of the 10 c-AML patients (70%) exhibited concurrent or subsequent marrow involvement by acute myeloid leukemia, with all 7 cases (100%) demonstrating shared clonal mutations in both the skin and BM. However, clonal relatedness was documented in one additional case that never had any BM involvement. Nevertheless, NPM1 mutations were identified in 7 of the 10 (70%) of these c-AML cases while one had KMT2A rearrangement and one showed inv(16). All 3 patients (100%) with Langerhans cell neoplasms, 2 patients with BPDCN (100%), and one of the 2 patients (50%) with other cutaneous dendritic cell neoplasms also demonstrated shared mutations between the skin and concurrent or subsequent myeloid neoplasms. Both BM and c-AML shared identical founding drivers, with a predominance of NPM1, DNMT3A, and translocations associated with monocytic differentiation, with common cutaneous-only mutations involving genes in the signal transduction and epigenetic pathways. Cutaneous histiocytic-dendritic neoplasms shared founding drivers in ASXL1, TET2, and/or SRSF2. However, in the Langerhans cell histiocytosis or histiocytic sarcoma cases, there exist recurrent secondary RAS pathway hits, whereas cutaneous BPDCN cases exhibit copy number or structural variants. These results enrich and broaden our understanding of clonally related cutaneous manifestations of myeloid neoplasms and further illuminate the highly diverse spectrum of morphologic and immunophenotypic features they exhibit.

01 Sep 2019·Surgical Pathology Clinics

Histiocytic and Dendritic Cell Neoplasms

Review

Author: Pan, Zenggang ; Xu, Mina L

Histiocytic and dendritic cell neoplasms are very rare, belonging to a group that share morphologic, immunophenotypic, and ultrastructural characteristics of mature histiocytic/dendritic neoplasms. Histiocytic and dendritic cell neoplasms may arise de novo or in association with B-cell, T-cell, or myeloid neoplasms. Recent molecular findings, particularly the discoveries of the mutations in the RAS-RAF-MEK-ERK pathway, have greatly advanced the diagnosis and treatment options. Histiocytic and dendritic cell neoplasms may closely resemble each other, non-hematopoietic neoplasms, and even reactive processes. Therefore, it is essential to understand the clinicopathologic characteristics, differential diagnoses, and pitfalls of each entity.

01 Nov 2018·Annals of HematologyQ3 · MEDICINE

Histiocytic cell neoplasms involving the bone marrow: summary of the workshop cases submitted to the 18th Meeting of the European Association for Haematopathology (EAHP) organized by the European Bone Marrow Working Group, Basel 2016

Q3 · MEDICINE

Article

Author: Kremer, Markus ; Tzankov, Alexandar ; Gianelli, Umberto ; Leguit, Roos ; van der Walt, Jon ; Orazi, Attilio ; Hebeda, Konnie M

The bone marrow is a preferential site for both reactive and neoplastic histiocytic proliferations. The differential diagnosis ranges from reactive histiocyte hyperplasia in systemic infections, vaccinations, storage diseases, post myeloablative therapy, due to increased cell turnover, and in hemophagocytic lymphohistiocytosis, through extranodal Rosai-Dorfman disease to neoplasms derived from histiocytes, including histiocytic sarcomas (HS), Langerhans cell histiocytoses (LCH), Erdheim-Chester disease (ECD), and disseminated juvenile xanthogranuloma (JXG). One of the most important recent developments in understanding the biology of histiocytic neoplasms and in contributing to diagnosis was the detection of recurrent mutations of genes of the Ras/Raf/MEK/ERK signaling pathway, in particular the BRAF^V600E mutation, in LCH and ECD. Here, we summarize clinical and pathological findings of 17 histiocytic neoplasms that were presented during the bone marrow symposium and workshop of the 18th European Association for Haematopathology (EAHP) meeting held in Basel, Switzerland, in 2016. A substantial proportion of these histiocytic neoplasms was combined with clonally related lymphoid (n = 2) or myeloid diseases (n = 5, all ECD). Based on the latter observation, we suggest excluding co-existent myeloid neoplasms at initial staging of elderly ECD patients. The recurrent nature of Ras/Raf/MEK/ERK signaling pathway mutations in histiocytic neoplasms was confirmed in 6 of the 17 workshop cases, illustrating their diagnostic significance and suggesting apotential target for tailored treatments.

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