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Tropomyosin receptor kinase antagonism in cylindromatosis

Not Applicable
Completed
Conditions
Topic: Cancer, Genetics, Dermatology
Subtopic: Head and Neck Cancer, Genetics Research and Congenital Disorders (all subtopics), Skin (all Subtopics)
Disease: Head and Neck, Genetics Research and Congenital Disorders, Dermatology
Skin and Connective Tissue Diseases
Registration Number
ISRCTN75715723
Lead Sponsor
ewcastle upon Tyne Hospitals NHS Foundation Trust (UK)
Brief Summary

2017 protocol in: https://www.ncbi.nlm.nih.gov/pubmed/28270164 2018 results in: https://www.ncbi.nlm.nih.gov/pubmed/29955768

Detailed Description

Not available

Recruitment & Eligibility

Status
Completed
Sex
All
Target Recruitment
28
Inclusion Criteria

Cohort 1:
1. Males and females age 18 years and older
2. Patients from genotyped pedigrees with known CYLD mutations; or if they have a clinical phenotype compatible with this diagnosis
3. Patients that are suitable for the trial will have at least one eligible tumour
4. The eligible tumour will be scheduled for removal >4 weeks from consent
5. The eligible tumour must be no more than 3cm in size
6. For women of childbearing age: a negative pregnancy test is required prior to study entry, and on completion of trial treatment. The patient must be using an adequate contraception method and agree to continue using this throughout the trial and for at least 2 weeks after stopping trial medication
7. Sexually active men must agree to use barrier forms of contraception
8. The recruiting clinician must be confident that the patient understands the consent process and has the capacity and willingness to provide fully informed consent for participation in the trial

Cohort 2:
1. Males and females age 18 years and older
2. For women of child bearing age: a negative pregnancy test is required prior to study entry, and on completion of trial treatment. The patient must be using an adequate contraception method and agree to continue using this throughout the trial and for at least 2 weeks after stopping trial medication
3. Patients from genotyped pedigrees with known CYLD mutations, or if they have a clinical phenotype compatible with this diagnosis
4. Patients will optimally have 8-10 eligible tumours
5. Eligible tumours will be less than 1 cm in diameter and no more than 2 cm in diameter at the base
6. Eligible tumours must be spaced at least 1 cm apart from other eligible tumours to avoid crosscontamination
7. The recruiting clinician must be confident that the patient understands the consent process and has the capacity and willingness to provide fully informed consent for participation in the trial
8. Patients who have completed Phase 1b without adverse reaction and after completing a minimum 2 week treatment free washout period

Exclusion Criteria

Cohort 1:
1. Patients aged <18 years
2. Patients without CYLD defective tumours
3. CYLD defective tumours which are ulcerated (these tumours will be managed according to standard practice of care)
4. The eligible tumour is due to be removed <4 weeks from consent
5. Pregnancy or lactation
6. Women of childbearing age and sexually active men whom do not wish to use contraception whilst on the study
7. Severe incapacity of higher function such that fully informed consent cannot be achieved, to be determined by clinical judgement
8. Use of any other topically administered treatments at the treatment site

Cohort 2:
1. Patients aged <18 years
2. Patients without multiple CYLD defective tumours
3. Pregnancy or lactation
4. Women of childbearing age and sexually active men whom do not wish to use contraception whilst on the study
5. CYLD defective tumours which are ulcerated, have recently changed or are painful (these tumours will be managed according to standard practice of care)
6. Severe incapacity of higher function such that fully informed consent cannot be achieved, to be determined by clinical judgement
7. Significant concurrent illness
8. Patients who developed an adverse reaction to CT327 in cohort 1(score of 4 or above on the modified Draize score)
9. Patients who have taken part in cohort 1and not completed a minimum 2 week treatment free washout period
10. Large tumours >2cm base diameter will not be eligible
11. Any tumour within 10cm of an excision scar of a cohort 1 treated site will not be eligible
12. Use of any other topically administered treatments at the treatment site

Study & Design

Study Type
Interventional
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
Cohort 1: Number of patients with severe treated skin site reactions as determined by Modified Draize score.<br>Cohort 2: The proportion of tumours responding to treatment by 12 weeks.
Secondary Outcome Measures
NameTimeMethod
Cohort 1: <br>1. Patient reported quality of life using patient reported QoL tools (EQ5D, DLQI)<br>2. Acceptability of treatment according to patient treatment questionnaire<br>3. Adverse events within a planned 4-week treatment period<br>4. Compliance including reasons for non-compliance<br><br>Cohort 2: <br>1. Change in tumour volume from baseline (pre-randomisation) to 12 weeks<br>2. Adverse events within a planned 12-week treatment period<br>3. Compliance including reasons for non-compliance<br>4. Confirmation of the definition of response (currently according to WHO RECIST criteria where response is defined as >30% reduction in tumour volume, this will be used as a benchmark)<br>5. Expression of targets of TRK signalling in tumour biopsies as determined by QPCR and immunohistochemistry<br>6. Patient reported quality of life using patient reported QoL tools (EQ5D, DLQI)<br>7. Assessment of acceptability of trial treatment according to patient treatment questionnaire
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