Actively Recruiting

Phase 2
All Genders
ID04079179

A Phase 2 Study to Assess the Safety and Efficacy of Cobimetinib in Refractory Langerhans Cell Histiocytosis, LCH-Associated Neurodegenerative Disease, and Other Histiocytic Disorders

Led by Carl Allen · Updated on 2025-09-18

90

Participants Needed

12

Research Sites

208 weeks

Total Duration

On this page

Sponsors

C

Carl Allen

Lead Sponsor

B

Baylor College of Medicine

Collaborating Sponsor

AI-Summary

What this Trial Is About

Researchers are studying a drug called cobimetinib in children and adults who have Langerhans cell histiocytosis (LCH) or other histiocytic disorders that have returned or do not respond to treatment. These disorders involve immune cells called histiocytes that grow abnormally due to genetic mutations, causing damage to tissues and organs. Some patients with LCH may develop neurodegeneration, which affects brain function. This phase 2 study aims to assess whether cobimetinib, which blocks a protein involved in abnormal cell growth, is safe and effective in treating these conditions, including patients with a specific mutation called BRAF-V600E. The study includes four groups of patients based on age and type of histiocytic disorder. Cobimetinib will be given orally as a tablet or suspension once daily for 21 days followed by 7 days off, making a 28-day treatment cycle. Children under 18 will receive up to 60 mg daily, and adults 18 or older will receive 40 mg daily. Participants will receive up to 12 cycles of treatment, approximately 12 months in total. Participants will be monitored regularly through clinical evaluations, imaging, and laboratory tests to measure overall response rates after 12 months using modified RECIST criteria. Researchers will also assess progression-free survival and record any side effects or adverse events during the study. Safety and effectiveness data will be collected throughout the treatment period to understand how patients respond to cobimetinib. The entire participation time is about one year of treatment and follow-up.

CONDITIONS

Brief Title

Cobimetinib in Refractory Langerhans Cell Histiocytosis (LCH), and Other Histiocytic Disorders

Who Can Participate

All Genders

Eligibility Criteria

Eligible

You may qualify if you...

  • For Group 1: Age at least 6 months and less than 21 years at enrollment
  • For Group 2: Age at least 6 months at enrollment
  • For Group 3: Age at least 6 months and less than 21 years at enrollment
  • For Group 4: Age 21 years or older at enrollment
  • Able to take medication by mouth or enteral route (tablet or suspension)
  • Biopsy proven Langerhans cell histiocytosis (LCH) or diagnosis of LCH-associated neurodegenerative disease with recent progression
  • Biopsy proven juvenile xanthogranuloma, Erdheim-Chester disease, Rosai-Dorfman disease, histiocytic sarcoma, or other histiocytic lesions with active disease
  • Karnofsky performance status ≥ 50% for patients older than 16 years, Lansky performance status ≥ 50% for patients 16 years or younger
  • Adequate blood, kidney, liver, and heart function according to defined laboratory and clinical criteria
  • Negative pregnancy test for females of childbearing potential and agreement to use effective birth control methods during and after the study
Not Eligible

You will not qualify if you...

  • Use of strong CYP3A4 inducers or inhibitors within 14 days before enrollment
  • Recent chemotherapy, immunotherapy, radiotherapy, or targeted therapy within defined time frames
  • Prior treatment with cobimetinib
  • Use of hematopoietic growth factors or anticoagulants within specified periods
  • Active infection or unresolved toxicity from previous treatments
  • Major surgery or significant injury within 28 days before enrollment
  • History of significant bowel malabsorption or pneumonitis
  • Significant eye conditions or risk factors for retinal vein occlusion
  • History of solid organ transplantation
  • Other medical or psychological conditions that increase risk
  • Clinically significant heart problems including unstable arrhythmias, angina, heart failure, or recent heart attack
  • Known HIV infection
  • Recent central nervous system hemorrhage
  • Pregnant or breastfeeding females

AI-Screening

AI-Powered Screening

Complete this quick 3-step screening to check your eligibility

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Your Study Journey

Screening

Duration - 2 to 4 weeks

Participants are screened for eligibility to participate in the trial.

1 visit (in-person) for screening and enrollment

Treatment

Duration - Approximately 12 months

Participants receive cobimetinib daily in 28-day cycles, with 21 days on medication followed by 7 days off, for up to 12 cycles (approximately 12 months).

Visits occur during each 28-day treatment cycle for monitoring and medication management

Trial Site Locations

Total: 12 locations

1

Phoenix Children's Hospital

Phoenix, Arizona, United States, 85016

Actively Recruiting

2

Arkansas Children's Hospital

Little Rock, Arkansas, United States, 72202

Actively Recruiting

3

Children's Hospital of Orange County

Orange, California, United States, 92868

Actively Recruiting

4

UCSF Benioff Children's Hospital

San Francisco, California, United States, 94158

Actively Recruiting

5

Children's National Hospital

Washington D.C., District of Columbia, United States, 20010

Actively Recruiting

6

John Hopkins University School of Medicine

Baltimore, Maryland, United States, 21287

Actively Recruiting

7

Dana Farber Cancer Institute, Boston Children's

Boston, Massachusetts, United States, 02215

Actively Recruiting

8

Memorial Sloan Kettering Cancer Center

New York, New York, United States, 10065

Actively Recruiting

9

NACHO Consortium

Memphis, Tennessee, United States, 38105

Not Yet Recruiting

10

Children's Medical Center- UTSW

Dallas, Texas, United States, 75235

Actively Recruiting

11

Texas Children's Hospital

Houston, Texas, United States, 77030

Actively Recruiting

12

University of Wisconsin-American Family Children's Hospital

Madison, Wisconsin, United States, 53792

Terminated

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Research Team

C

Carl E Allen, MD, PhD

O

Olive Eckstein, MD

How is the study designed?

Study Type

INTERVENTIONAL

Masking

NONE

Allocation

NON_RANDOMIZED

Model

PARALLEL

Primary Purpose

TREATMENT

Number of Arms

4

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