Last update 21 Mar 2024
Interdigitating Dendritic Cell Sarcoma
Last update 21 Mar 2024
Basic Info
Synonyms Dendritic Cell Sarcoma, Interdigitating, Interdigitating Cell Sarcoma, Interdigitating Cell Sarcoma/Tumor + [15] |
Introduction A rare sarcoma of INTERDIGITATING CELLS found in the lymph nodes and non-lymphoid organs. They exhibit a variable immunophenotype and lack Birbeck granules. |
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A Phase 2 Study of Pembrolizumab in Patients With Histiocyte/Dendritic Cell Neoplasms and Biologically Selected Subtypes of Relapsed/Refractory Aggressive Lymphomas
Single-center Trial Evaluating the Safety and Efficacy of MUC-1/WT-1 Peptide or Tumor Lysate-pulsed Dendritic Cell Immunotherapy for the Patients With Pancreatic Cancer
A Randomized Phase IIa Study: Natural Dendritic Cells for Immunotherapy of Chemo-naive Metastatic Castration-resistant Prostate Cancer Patients
100 Clinical Results associated with Interdigitating Dendritic Cell Sarcoma
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100 Translational Medicine associated with Interdigitating Dendritic Cell Sarcoma
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23 Nov 2023Haematologica
Massive parallel sequencing unveils homologous recombination deficiency in follicular dendritic cell sarcoma.
Article
Author: Luisa Lorenzi ; Torsten Haferlach ; Luigi Mori ; Matteo Simbeni ; Wencke Walter ; Piera Balzarini ; Manja Meggendorfer ; Claudia Döring ; Silvia Lonardi ; Mattia Bugatti ; Claudio Agostinelli ; Jay Mehta ; Anita Borges ; Abbas Agaimy ; Ingrid Simonitsch-Klupp ; José Cabeçadas ; Elias Campo ; Stefano Aldo Pileri ; Fabio Facchetti ; Martin Leo Hansmann ; Sylvia Hartmann
Standardized treatment options are lacking for patients with unresectable or multifocal follicular dendritic cell sarcoma (FDCS) and disease-related mortality is as high as 20%. Applying whole genome sequencing (WGS) in one case and whole exome sequencing (WES) in additional twelve, this study adds information on the molecular landscape of FDCS, expanding knowledge on pathobiological mechanisms and identifying novel markers of potential theragnostic significance. Massive parallel sequencing showed high frequency of mutations on oncosuppressor genes, particularly in RB1, CARS and BRCA2 and unveiled alterations on homologous recombination DNA damage repair related genes in 70% (9/13) of cases. This indicates that patients with high stage FDCS may be eligible for poly ADP ribose polymerase inhibition protocols. Low tumor mutational burden was confirmed in this study despite common PDL1 expression in FDCS arguing on the efficacy of immune checkpoint inhibitors. CDKN2A deletion, detected by WGS and confirmed by FISH in 41% of cases (9/22) indicates that impairment of cell cycle regulation may sustain oncogenesis in FDCS. Absence of mutations in the RAS/RAF/MAPK pathway and lack of clonal hematopoiesis related mutations in FDCS sanction its differences from dendritic cell-derived neoplasms of haematopoietic derivation. WGS and WES in FDCS provides additional information on the molecular landscape of this rare tumor, proposing novel candidate genes for innovative therapeutical approaches to improve survival of patients with multifocal disease.
07 Jul 2023The Lancet. Oncology
Pembrolizumab in patients with rare and ultra-rare sarcomas (AcSé Pembrolizumab): analysis of a subgroup from a non-randomised, open-label, phase 2, basket trial.
Article
Author: Jean-Yves Blay ; Sylvie Chevret ; Axel Le Cesne ; Mehdi Brahmi ; Nicolas Penel ; Sophie Cousin ; Francois Bertucci ; Emmanuelle Bompas ; Thomas Ryckewaert ; Pauline Soibinet ; Pascaline Boudou-Rouquette ; Esma Saada Bouzid ; Patrick Soulie ; Thibaud Valentin ; Jean-Pierre Lotz ; Diego Tosi ; Zoé Neviere ; Mathilde Cancel ; Isabelle Ray-Coquard ; Laetitia Gambotti ; Frédéric Legrand ; Assia Lamrani-Ghaouti ; Clotilde Simon ; Caroline Even ; Christophe Massard
BACKGROUND:
Sarcoma is a heterogeneous group of diseases with few treatment options. Immunotherapy has shown little activity in studies including unselected sarcomas, but immune checkpoint blockers have shown activity in specific histotypes. We evaluated the activity of pembrolizumab in rare and ultra-rare sarcomas.
METHODS:
AcSé Pembrolizumab is an ongoing phase 2, basket, multitumour study investigating the activity of pembrolizumab monotherapy in rare cancers. Here, we report the results obtained in patients with selected histotypes of rare sarcomas (incidence of less than one case per 1 000 000 people per year) recruited at 24 French hospitals. Key inclusion criteria were age 15 years or older, Eastern Cooperative Oncology Group performance status of 0-1, and advanced disease that was untreated and resistant to treatment. Patients were given pembrolizumab 200 mg intravenously on day 1 of every 21-day cycle for a maximum of 24 months. The primary endpoint was objective response rate at week 12 using Response Evaluation Criteria in Solid Tumours version 1.1, assessed by local investigators. The primary endpoint and safety were analysed in the intention-to-treat population. The AcSé Pembrolizumab study is registered with ClinicalTrials.gov, NCT03012620.
FINDINGS:
Between Sept 4, 2017, and Dec 29, 2020, 98 patients were enrolled, of whom 97 received treatment and were included in analyses (median age 51 years [IQR 35-65]; 53 [55%] were male; 44 [45%] were female; no data were collected on race or ethnicity). 34 (35%) patients had chordomas, 14 (14%) had alveolar soft part sarcomas, 12 (12%) had SMARCA4-deficient sarcomas or malignant rhabdoid tumours, eight (8%) had desmoplastic small round cell tumours, six (6%) had epithelioid sarcomas, four (4%) had dendritic cell sarcomas, three (3%) each had clear cell sarcomas, solitary fibrous tumours, and myxoid liposarcomas, and ten (10%) had other ultra-rare histotypes. As of data cutoff (April 11, 2022), median follow-up was 13·1 months (range 0·1-52·8; IQR 4·3-19·7). At week 12, objective response rate was 6·2% (95% CI 2·3-13·0), with no complete responses and six partial responses in the 97 patients. The most common grade 3-4 adverse events were anaemia (eight [8%] of 97), alanine aminotransferase and aspartate aminotransferase increase (six [6%]), and dyspnoea (five [5%]). 86 serious adverse events were reported in 37 patients. Five deaths due to adverse events were reported, none of which were determined to be related to treatment (two due to disease progression, two due to cancer, and one due to unknown cause).
INTERPRETATION:
Our data show the activity and manageable toxicity of pembrolizumab in some rare and ultra-rare sarcoma histotypes, and support the PD-1/PD-L1 pathway as a potential therapeutic target in selected histotypes. The completion of the basket study will provide further evidence regarding the activity and toxicity of pembrolizumab in identified rare types of cancer.
FUNDING:
The Ligue contre le cancer, INCa, MSD.
TRANSLATION:
For the French translation of the abstract see Supplementary Materials section.
03 Jul 2023Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
Malignant Histiocytosis Comprises a Phenotypic Spectrum that Parallels the Lineage Differentiation of Monocytes, Macrophages, Dendritic Cells, and Langerhans Cells.
Article
Author: Aishwarya Ravindran ; Surendra Dasari ; Gordon J Ruan ; Cody J Artymiuk ; Rong He ; David S Viswanatha ; Jithma P Abeykoon ; Saurabh Zanwar ; Jason R Young ; Gaurav Goyal ; Ronald S Go ; Karen L Rech ; Mayo Clinic-The University of Alabama at Birmingham Histiocytosis Working Group
The malignant histiocytoses (MH), or 'M group' of the Histiocyte Society classification, are characterized by neoplastic histiocytes with large pleomorphic nuclei. MH encompasses the diagnoses of histiocytic sarcoma, interdigitating dendritic cell sarcoma and Langerhans cell sarcoma. We aimed to define the phenotypic spectrum of MH and examine the genotypic features across this spectrum. By immunohistochemistry, we arranged the 22 cases into four subtypes that correspond to the lines of differentiation from monocytic and dendritic cell precursors : i) Macrophage (n=5): CD68+, CD163+, CD14+, Factor 13a+; ii) Monocyte-Macrophage (n=5): CD68+, CD163+, CD14+, S100+, OCT2+; iii) Dendritic cell (n=6): CD68+, CD11c+, S100+, lysozyme+, ZBTB46+, CD1a/langerin <5%; and iv) Langerhans cell (n=6): CD68+, CD11c+, S100+, ZBTB46+, CD1a and langerin+. The phenotypic subtypes align with those seen in low-grade histiocytic neoplasms: MH-macrophage type correlates with Erdheim-Chester disease phenotype; MH-monocyte-macrophage type with Rosai-Dorfman disease phenotype and MH-Langerhans cell type with Langerhans cell histiocytosis. Activating mutations in MAPK pathway genes were identified in 80% of MH cases; 29% had mutations in PI3k-AKT-mTOR pathway and 59% had mutations in epigenetic modulating genes. Strong expression of cyclin D1 was present in all cases, whereas p-ERK and p-AKT were not uniformly expressed. Eight of 22 (36%) MH cases were proven to be clonally related to a prior B-cell lymphoma. Defining the phenotypic spectrum of MH provides a guide to diagnosis and allows further exploration into the potential biological and clinical significance.
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