Isolated liver involvement in Langerhans cell histiocytosis: A case report
We report here a case of LCH with solitary hepatic involvement in a 74 year-old patient. This case demonstrated molecular anomaly of the MAPK pathway, BRAF N486_P490del. Through this observation, we precise the epidemiological and histological aspects and diagnostic criteria of this rare disease.PMID:38216436 | DOI:10.1016/j.annpat.2023.12.001 (Source: Annales de Pathologie)
Source: Annales de Pathologie - January 12, 2024 Category: Pathology Authors: Pierre Allaume Eric Meneyrol Gontran Bernard Pauline Houssel-Debry Jean-Fran çois Emile Bruno Turlin Source Type: research

Oral manifestation as the only sign of Langerhans cell histiocytosis: A case report
Key Clinical MessageThe manifestations of Langerhans cell histiocytosis can be limited in the oral cavity, including gingival recession, tooth mobility, and bone destruction. Dentists play a crucial role in the early detection and management of these oral symptoms, which can significantly impact the overall prognosis and quality of life for patients with this serious disease.AbstractThe hyperplastic activity of bone marrow can lead to excessive histocyte proliferation, called Langerhans cell histiocytosis (LCH). Based on the accumulation location, it has various organ involvement, including skin, bone, and so forth. Oral m...
Source: Clinical Case Reports - January 11, 2024 Category: General Medicine Authors: Bahareh Nazemisalman, Mobina Sadat Zarabadi Tags: CASE REPORT Source Type: research

Peroxisome proliferator-activated receptor α/γ agonist pioglitazone for rescuing relapsed or refractory neoplasias by unlocking phenotypic plasticity
A series of seven clinical trials on relapsed or refractory (r/r) metastatic neoplasias followed the question: Are networks of ligand-receptor cross-talks that support tumor-specific cancer hallmarks, druggable with tumor tissue editing approaches therapeutically exploiting tumor plasticity? Differential recombinations of pioglitazone, a dual peroxisome-proliferator activated receptorα/γ (PPARα/γ) agonist, with transcriptional modulators, i.e., all-trans retinoic acid, interferon-α, or dexamethasone plus metronomic low-dose chemotherapy (MCT) or epigenetic modeling with azacitidine plus/minus cyclooxygenase-2 inhibiti...
Source: Frontiers in Oncology - January 11, 2024 Category: Cancer & Oncology Source Type: research

Successful treatment of xanthoma disseminatum with pulsed dye laser: A case report
Australas J Dermatol. 2024 Jan 8. doi: 10.1111/ajd.14209. Online ahead of print.ABSTRACTXanthoma disseminatum is a rare form of non-Langerhans cell histiocytosis with limited treatment options due to its unknown aetiology and diffuse skin lesions. This case report presents the successful treatment of a 31-year-old male with severe pan-facial xanthoma disseminatum lesions following a facial burn and traumatic brain injury resulting from a car accident. After 5 sessions of monthly pulsed dye laser treatment, there was a clinically significant reduction in the lesions. Over the course of 3 years, the patient underwent a serie...
Source: The Australasian Journal of Dermatology - January 8, 2024 Category: Dermatology Authors: Jin Woong Jung Joon-Goon Kim Albaraa Abdullatif A Alwabel Yeon Woo Jung Kee-Yang Chung Source Type: research

Successful treatment of xanthoma disseminatum with pulsed dye laser: A case report
Australas J Dermatol. 2024 Jan 8. doi: 10.1111/ajd.14209. Online ahead of print.ABSTRACTXanthoma disseminatum is a rare form of non-Langerhans cell histiocytosis with limited treatment options due to its unknown aetiology and diffuse skin lesions. This case report presents the successful treatment of a 31-year-old male with severe pan-facial xanthoma disseminatum lesions following a facial burn and traumatic brain injury resulting from a car accident. After 5 sessions of monthly pulsed dye laser treatment, there was a clinically significant reduction in the lesions. Over the course of 3 years, the patient underwent a serie...
Source: The Australasian Journal of Dermatology - January 8, 2024 Category: Dermatology Authors: Jin Woong Jung Joon-Goon Kim Albaraa Abdullatif A Alwabel Yeon Woo Jung Kee-Yang Chung Source Type: research

Germline mutations in cancer predisposition genes among pediatric patients with cancer and congenital anomalies
CONCLUSION: We uncovered a remarkable prevalence of germline deleterious CPG variants, highlighting the significance of a comprehensive genetic analysis in pediatric cancer, especially when coupled with additional clinical signs. Moreover, our findings emphasized the potential for oligogenic inheritance, wherein multiple genes synergistically increase cancer risk. Lastly, our investigation unveiled potentially novel genotype-phenotype associations, such as SETD5 in neuroblastoma, KAT6A in gliomas, JAG1 in hepatoblastomas, and TNFRSF13B in Langerhans cell histiocytosis.IMPACT: Novel gene-phenotype associations and candidate...
Source: Pediatric Research - January 5, 2024 Category: Pediatrics Authors: Gustavo D Dangoni Anne Caroline B Teixeira Silvia S da Costa Mar ília O Scliar Laura M L Carvalho Luciana N Silva Estela M Novak Carolina S C Vince Mariana C Maschietto Sofia M M Sugayama Vicente Odone-Filho Ana Cristina V Krepischi Source Type: research

Germline mutations in cancer predisposition genes among pediatric patients with cancer and congenital anomalies
CONCLUSION: We uncovered a remarkable prevalence of germline deleterious CPG variants, highlighting the significance of a comprehensive genetic analysis in pediatric cancer, especially when coupled with additional clinical signs. Moreover, our findings emphasized the potential for oligogenic inheritance, wherein multiple genes synergistically increase cancer risk. Lastly, our investigation unveiled potentially novel genotype-phenotype associations, such as SETD5 in neuroblastoma, KAT6A in gliomas, JAG1 in hepatoblastomas, and TNFRSF13B in Langerhans cell histiocytosis.IMPACT: Novel gene-phenotype associations and candidate...
Source: Cell Research - January 5, 2024 Category: Cytology Authors: Gustavo D Dangoni Anne Caroline B Teixeira Silvia S da Costa Mar ília O Scliar Laura M L Carvalho Luciana N Silva Estela M Novak Carolina S C Vince Mariana C Maschietto Sofia M M Sugayama Vicente Odone-Filho Ana Cristina V Krepischi Source Type: research

Germline mutations in cancer predisposition genes among pediatric patients with cancer and congenital anomalies
CONCLUSION: We uncovered a remarkable prevalence of germline deleterious CPG variants, highlighting the significance of a comprehensive genetic analysis in pediatric cancer, especially when coupled with additional clinical signs. Moreover, our findings emphasized the potential for oligogenic inheritance, wherein multiple genes synergistically increase cancer risk. Lastly, our investigation unveiled potentially novel genotype-phenotype associations, such as SETD5 in neuroblastoma, KAT6A in gliomas, JAG1 in hepatoblastomas, and TNFRSF13B in Langerhans cell histiocytosis.IMPACT: Novel gene-phenotype associations and candidate...
Source: Pediatric Research - January 5, 2024 Category: Pediatrics Authors: Gustavo D Dangoni Anne Caroline B Teixeira Silvia S da Costa Mar ília O Scliar Laura M L Carvalho Luciana N Silva Estela M Novak Carolina S C Vince Mariana C Maschietto Sofia M M Sugayama Vicente Odone-Filho Ana Cristina V Krepischi Source Type: research

Germline mutations in cancer predisposition genes among pediatric patients with cancer and congenital anomalies
CONCLUSION: We uncovered a remarkable prevalence of germline deleterious CPG variants, highlighting the significance of a comprehensive genetic analysis in pediatric cancer, especially when coupled with additional clinical signs. Moreover, our findings emphasized the potential for oligogenic inheritance, wherein multiple genes synergistically increase cancer risk. Lastly, our investigation unveiled potentially novel genotype-phenotype associations, such as SETD5 in neuroblastoma, KAT6A in gliomas, JAG1 in hepatoblastomas, and TNFRSF13B in Langerhans cell histiocytosis.IMPACT: Novel gene-phenotype associations and candidate...
Source: Pediatric Research - January 5, 2024 Category: Pediatrics Authors: Gustavo D Dangoni Anne Caroline B Teixeira Silvia S da Costa Mar ília O Scliar Laura M L Carvalho Luciana N Silva Estela M Novak Carolina S C Vince Mariana C Maschietto Sofia M M Sugayama Vicente Odone-Filho Ana Cristina V Krepischi Source Type: research

Solitary Langerhans cell histiocytosis of the sternum in a 21 ‐year‐old woman
We present an instance of sternal LCH with adult onset. By fusing and reconstructing chest computed tomography, it was possible to determine the extent of surrounding soft tissue invasion. Because LCH is so uncommon, it could be challenging to recall when we see a sternal lesion. Adult Patients who arrive with anterior chest discomfort and an osteolytic sternal lesion should include LCH on their differential diagnosis list. (Source: Clinical Case Reports)
Source: Clinical Case Reports - January 3, 2024 Category: General Medicine Authors: Neda Soleimani, Massood Hosseinzadeh, Armin Amirian, Masha Hassani, Sahand Mohammadzadeh Tags: CASE REPORT Source Type: research

Treatment of children with refractory/relapse high risk langerhans cell histiocytosis with the combination of cytarabine, vindesine and prednisone
The patients with multisystem and risk organ involvement Langerhans cell histiocytosis (MS-RO  + LCH) have poor prognosis. The patients with MS-LCH who failed front-line therapy have a high mortality rate and t... (Source: BMC Pediatrics)
Source: BMC Pediatrics - January 3, 2024 Category: Pediatrics Authors: Wenqian Wang, Jian Ge, Honghao Ma, Hongyun Lian, Lei Cui, Yunze Zhao, Zhigang Li, Tianyou Wang and Rui Zhang Tags: Research Source Type: research

Cancers, Vol. 16, Pages 180: Addressing Genetic Tumor Heterogeneity, Post-Therapy Metastatic Spread, Cancer Repopulation, and Development of Acquired Tumor Cell Resistance
Heudobler The concept of post-therapy metastatic spread, cancer repopulation and acquired tumor cell resistance (M-CRAC) rationalizes tumor progression because of tumor cell heterogeneity arising from post-therapy genetic damage and subsequent tissue repair mechanisms. Therapeutic strategies designed to specifically address M-CRAC involve tissue editing approaches, such as low-dose metronomic chemotherapy and the use of transcriptional modulators with or without targeted therapies. Notably, tumor tissue editing holds the potential to treat patients, who are refractory to or relapsing (r/r) after conventional chemother...
Source: Cancers - December 29, 2023 Category: Cancer & Oncology Authors: Dennis Christoph Harrer Florian L üke Tobias Pukrop Lina Ghibelli Albrecht Reichle Daniel Heudobler Tags: Review Source Type: research

The role of < sup > 18 < /sup > F-FDG PET/CT imaging in paediatric Langerhans disease: Case report
Hell J Nucl Med. 2023 Sep-Dec;26(3):215-218. doi: 10.1967/s002449912611.ABSTRACTLangerhans cell histiocytosis (LCH) is a haematological disorder, affecting single or multiple organs, characterized by abnormal proliferation of Langerhans cells in children. Accurate tumour delineation (number of lesions, organs involved) is crucial for staging/re-staging, and follow-up (response to therapy). Conventional imaging techniques (computed tomography (CT), magnetic resonance imaging (MRI)) have been employed for initial diagnosis, staging and assessment of response to therapy focusing on the healing effect therapeutic protocols hav...
Source: Hellenic Journal of Nuclear Medicine - December 27, 2023 Category: Nuclear Medicine Authors: Ioanna Sevaslidou Periklis Papavasileiou Maria Gavra Margarita Baka Sophia Polychronopoulou Lida Gogou Source Type: research