Alectinib In the Treatment of Systemic Juvenile Xanthogranuloma of Infancy With <i>ALK</i> Translocation

Xu, Jiaosheng; Yao, Xingfeng; Wen, Yang; Lian, Hongyun; Han, Xiaofeng; Xu, Zigang

doi:10.1001/jamadermatol.2023.4027

JAMA Dermatol

2023

DOI: 10.1001/jamadermatol.2023.4027

|View full text |Cite

Alectinib In the Treatment of Systemic Juvenile Xanthogranuloma of Infancy With ALK Translocation

Jiaosheng Xu,

Xingfeng Yao,

Yang Wen

et al.

Abstract: This observational case series examines the diagnosis and treatment of 2 patients with systemic juvenile xanthogranuloma treated with alectinib.

Help me understand this report

Search citation statements

Order By: Relevance

Paper Sections

Select...

Citation Types

Supporting

Mentioning

Contrasting

Year Published

2024

Publication Types

Select...

Article3

Relationship

Self Cite1

Independent2

Authors

Journals

Cited by 3 publications

(4 citation statements)

References 7 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…4 Supporting our notion, ALK-positive histiocytosis was recently added as a distinct entity to the fifth edition of the World Health Organization Classification of Haematolymphoid Tumours and the International Consensus Classification of Mature Lymphoid, Histiocytic, and Dendritic Cell Tumors. 5 The 2 cases described by Xu et al 1 are prime examples of ALK-positive histiocytosis. Notably, both were female individuals with lung involvement, 1 had neurologic disease, and the clinical and radiologic images bear striking similarities to those from patients in our study.…”

mentioning

confidence: 99%

“…The 2 cases described by Xu et al are prime examples of ALK -positive histiocytosis. Notably, both were female individuals with lung involvement, 1 had neurologic disease, and the clinical and radiologic images bear striking similarities to those from patients in our study .…”

mentioning

confidence: 99%

“…To the Editor It is with great interest that we read the recent article by Xu et al that described 2 infants with systemic histiocytic neoplasms harboring ALK translocations who were successfully treated with the ALK inhibitor alectinib. To our surprise, the authors classified the disease as systemic juvenile xanthogranuloma (JXG) without any mention of ALK -positive histiocytosis and stated that the treatment method had not been previously reported.…”

mentioning

confidence: 99%

See 2 more Smart Citations

ALK-Positive Histiocytosis—A Distinct Histiocytic Entity Deserving Recognition

Kemps,

Picarsic,

Emile

2024

JAMA Dermatol

View full text Add to dashboard Cite

mentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

See 1 more Smart Citation

ALK-Positive Histiocytosis—A Distinct Histiocytic Entity Deserving Recognition

Kemps,

Picarsic,

Emile

2024

JAMA Dermatol

View full text Add to dashboard Cite

“…In Reply We read the Letter to the Editor by Kemps et al with interest. We believe that these cases belong to the spectrum of juvenile xanthogranuloma (JXG) . Conventionally, JXG is divided into 3 clinical stages during the disease process (the early developed, xanthomatous, and regression stages) and 3 histopathological changes are observed accordingly (the early, classic, and transitional stages) .…”

mentioning

confidence: 99%

ALK-Positive Histiocytosis—A Distinct Histiocytic Entity Deserving Recognition—Reply

Xu,

Yao,

2024

JAMA Dermatol

Self Cite

View full text Add to dashboard Cite

COMMENT & RESPONSEIn Reply We read the Letter to the Editor by Kemps et al with interest. We believe that these cases belong to the spectrum of juvenile xanthogranuloma (JXG). 1 Conventionally, JXG is divided into 3 clinical stages during the disease process (the early developed, xanthomatous, and regression stages) and 3 histopathological changes are observed accordingly (the early, classic, and transitional stages). 2 In our previous study, 3 approximately 34.8% of systemic JXG exhibited ALK-positive immunostaining and ALK translocation verified via ALK fluorescence in situ hybridization assays. Furthermore, 17.4% were BRAFV600E-variants. This portion of JXG with genetic aberrations in our study mainly showed the early developed and xanthomatous clinical stages with corresponding histopathological presentations, including the pathological changes described by Kemps et al. In the case reported by Wolter et al, 4 ALK translocation was observed in the lesion involving the single subglottic mucous membrane. The lesion showed a diffuse infiltrate of histiocytic cells mixed with spindle cells, clinicopathologically exhibiting the transitional stages of JXG with ALK translocations. Hence, the clinical and histopathological presentations of JXG with ALK translocations belong to JXG.Although rare, JXG is the most common type of non-Langerhans cell histiocytosis, with a predilection for children and frequent cutaneous involvement. JXG includes the meanings of juvenile, xanthomatous, and granulomatous, emphasizing its clinical and histopathological characteristics. These characteristics provide the clues for medical professionals, especially dermatologists and pediatricians confronting JXG. The term ALK-positive histiocytosis suggests the importance of genetics and histopathology in etiology and pathogenesis. However, it is less favorable to identify the disease based on the clinical findings to fulfill the term ALK-positive histiocytosis.We have conducted studies in JXG for decades and maintained a high interest in ALK-positive histiocytosis. Considerable overlaps exist between systemic JXG with ALK translocations and ALK-positive histiocytosis, as mentioned in our previous study. 3 Similarly, commonalities exist between BRAFV600E-variant JXG and Erdheim-Chester disease. Therefore, we suggest genetic testing and systemic screening as novel modalities in the diagnosis and management of JXG, espe-

show abstract

Multisystem ALK‐Positive Histiocytosis With DCTN1::ALK Fusion in an Adult, Responsive to Alectinib: Case Report and Literature Review

Phillips,

Knapp,

Olsen

et al. 2024

J Cutan Pathol

View full text Add to dashboard Cite

Anaplastic lymphoma kinase (ALK)‐positive histiocytosis has emerged as a clinically relevant diagnosis featuring a wide span of clinical presentations, which are unified by the presence of ALK‐positive histiocytes on histopathology and molecular drivers involving the ALK kinase gene. This report presents an adult case of multisystem ALK‐positive histiocytosis with xanthogranuloma‐like features on histopathology that was responsive to ALK inhibition, and includes a review of ALK‐positive histiocytoses with cutaneous involvement reported in the literature. A 56‐year‐old male developed a widespread eruption of red‐brown papules on the face, trunk, and upper extremities. Histopathological evaluation revealed a well‐circumscribed, nodular dermal infiltrate of epithelioid histiocytes with Touton giant cells, rare bizarre multinucleated cells, and focal emperipolesis. The lesional cells were positive for CD68 and ALK1 immunohistochemical stains, and negative for CD1a. Next‐generation sequencing identified a DCTN1::ALK fusion. On imaging, he was found to have bone, lung, soft tissue, and salivary gland involvement. ALK inhibition was initiated with alectinib, resulting in rapid improvement of cutaneous lesions and eventual complete resolution of abnormal imaging findings, which was sustained at 24 months of follow‐up. This case adds to the spectrum of ALK‐positive histiocytoses and further demonstrates the positive response with targeted therapy.

show abstract

scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.

Contact Info

customersupport@researchsolutions.com

10624 S. Eastern Ave., Ste. A-614

Henderson, NV 89052, USA

This site is protected by reCAPTCHA and the Google Privacy Policy and Terms of Service apply.

Blog Terms and Conditions API Terms Privacy Policy Contact Cookie Preferences Do Not Sell or Share My Personal Information

Made with 💙 for researchers

Part of the Research Solutions Family.

Alectinib In the Treatment of Systemic Juvenile Xanthogranuloma of Infancy With ALK Translocation

Abstract: This observational case series examines the diagnosis and treatment of 2 patients with systemic juvenile xanthogranuloma treated with alectinib.

Cited by 3 publications

References 7 publications

ALK-Positive Histiocytosis—A Distinct Histiocytic Entity Deserving Recognition

ALK-Positive Histiocytosis—A Distinct Histiocytic Entity Deserving Recognition

ALK-Positive Histiocytosis—A Distinct Histiocytic Entity Deserving Recognition—Reply

Multisystem ALK‐Positive Histiocytosis With DCTN1::ALK Fusion in an Adult, Responsive to Alectinib: Case Report and Literature Review

Contact Info

Product

Resources

About