RINGSIDE: A Phase 2/3, Randomized, Multicenter Study to Evaluate AL102
in Patients with Progressing Desmoid Tumors

Part A
1. At least 18 years of age (inclusive) at the time of signing the ICF.
2. Histologically confirmed desmoid tumor (aggressive fibromatosis) by local
pathologist (prior to informed consent).
3. Disease progression, assessed by the investigator, defined as having at
least one of the following:
a) Unidimensional growth of desmoid tumor(s) by >=10%, using the sum of the
largest diameters of target lesion(s), within 18 months of the
screening MRI
b) Having desmoid tumor-related pain that is not adequately controlledwith
non-opioid medication
4. At least 1 measurable lesion amenable to volume measurements byMRI at
screening
5. One of the following:
• Treatment naïve subjects for whom, in the opinion of the investigator, the IP
is deemed appropriate; OR
• Recurrent/refractory disease following at least one line of therapy
(including surgery, radiation, or systemic therapy).
6. A desmoid tumor in which continued progressing disease will not result in
immediate significant risk to the subject.
7. Agrees to provide formalin-fixed paraffin embedded (FFPE) archival or fresh
tumor tissue.
8. Must be able to swallow whole capsules with no GI condition affecting
absorption (not including history of colectomy); nasogastric or G-tube
administration is not allowed.
9. Male or female subjects.
10. Women of childbearing potential (WOCBP) must have a negative serum or urine
pregnancy test (minimum sensitivity 25 IU/L or
equivalent units of human chorionic gonadotropin [hCG]) within 24 hours prior
to the start of investigational product (IP). An extension up
to 72 hours is permissible in situations where results cannot be obtained
within the standard 24 hour window.
11. WOCBP and men who are sexually active with WOCBP must agree to follow
instructions for method(s) of contraception for the duration of the treatment
with IP plus 120 days post-treatment completion.
Contraception methods should be consistent with local regulations.
12. Capable of giving signed informed consent which includes compliance with
the requirements and restrictions listed in the ICF and in this protocol.

Part B
1. >=12 years of age (inclusive) in countries which allow participation of
adolescents and >= 40 kg at the time of signing the ICF.
2. Histologically confirmed desmoid tumor (aggressive fibromatosis) by local
pathologist (prior to informed consent) that has progressed per
RECIST v1.1 (>=20% or new lesion) by investigator within 12 months of the
screening visit scan.
3. Evidence of measurable disease by CT/MRI scan. Measurable lesions are
defined according to RECIST v1.1.
4. One of the following:
• Recurrent/refractory disease following at least one line of therapy
(including surgery, radiation, or systemic therapy); OR
• Treatment naïve subjects for whom, in the opinion of the investigator,
surgery or radiation therapy is not deemed appropriate;
5. A desmoid tumor in which continued progressing disease will not result in
immediate significant risk to the subject.
6. Agrees to provide FFPE archival or fresh tumor tissue.
7. Must be able to swallow whole capsules with no GI condition affecting
absorption (not including history of colectomy or proctocolectomy);
nasogastric or G-tube administration is not allowed.
Gender and Reproductive Considerations
8. Male or female subjects.
9. Premenstrual female subjects with a history of ovulatory dysfunction may be
enrolled
10.WOCBP must have a negative serum or urine pregnancy test (minimum
sensitivity 25 IU/L or equivalent units of hCG) within 24
hours prior to the start of IP.
11. WOCBP and men who are sexually active with WOCBP must agree to follow
instructions for method(s) of contraception for the duration of the treatment
with IP plus 120 days post-treatment completion.
Contraception methods should be consistent with local regulations.
12. Subject and/or legally authorized representative (i.e. parent/guardian)
must be capable of giving signed informed consent, which includes compliance
with the requirements and restrictions listed in the ICF.
14.Minor subjects must be capable of giving written assent as appropriate per
the applicable age (per local regulatory requirements).

OLE
1. One of the following:
a. Participated in Part A (including MRI at Week 16) and were still on study at
time that Part B/OLE dose selection was made, OR
b. Participating in Part B and were noted to have radiographic progressive
disease by BICR, OR
c. Are on study after completion of Part B
2. Must be able to swallow whole capsules with no GI condition affecting
absorption; nasogastric or G-tube administration is not allowed.
3. Subject and/or legally authorized representative (i.e. parent/guardian) must
be capable of giving signed informed consent, which includes compliance with
the requirements and restrictions listed in the ICF.
4. Minor subjects must be capable of giving written assent as appropriate per
the applicable age (per local regulatory requirements)

1. Diagnosed with a malignancy in the past 2 years, unless for protocol defined
allowed malignancies
2. Current or recent (within 2 months of IP administration) GI disease or
disorders that increase the risk of diarrhea, such as inflammatory bowel
disease and Crohn's disease
3. Evidence of uncontrolled, active infection, requiring systemic
antibacterial, anti-viral or anti-fungal therapy <=7 days prior to
administration of IP
4. Myocardial infarction within 6 months prior to enrollment, greater than
Class 1 angina pectoris, or has NYHA Class III or IV heart failure, symptomatic
ventricular arrhythmias, sustained ventricular tachycardia, TdP, the long QT
syndrome, pacemaker dependence, or electrocardiographic evidence of acute
ischemia
5. History of additional risk factors for TdP
6. Unstable or severe uncontrolled medical condition or any important medical
illness or abnormal laboratory finding
7. Pregnant or breastfeeding or expecting to conceive children during the study
8. ECOG performance status >=2
9. Abnormal organ and marrow function at Screening defined as: a. Neutrophils
<1500/mm3; b. Platelet count <100,000/mm3; c. Hemoglobin <9 g/dL; d.
Electrolytes (potassium, calcium, magnesium, and phosphorus, using corrected
value if low serum albumin level is
present) outside the normal limits of the local laboratory; e. Total bilirubin
>1.5x ULN (except known Gilbert's syndrome >3x ULN); f. Aspartate
aminotransferase (AST) and alanine aminotransferase (ALT) >2.5x ULN; g. Serum
or plasma creatinine > ULN and creatinine clearance (CrCl) <60 mL/min; h.
Uncontrolled triglyceride >=Grade 2 elevations per CTCAE v5.0 (>300 mg/dL or
>3.42 mmol/L)
10. ECG Exclusions : a. Mean QT interval corrected for heart rate using
Fridericia's formula (QTcF) >=450 msec; b. QRS duration > 110 ms; c. PR interval
> 240 ms; d. Marked ST-T wave abnormalities which would make it difficult to
measure the QT interval
11. Any treatments for desmoid tumors within 4 weeks prior to first dose
12. Chronic NSAIDs for the treatment of desmoid tumors within 4 weeks of first
dose
13. Prior treatment with GSI or other agents targeting the Notch pathway
14. Use of strong inhibitors of CYP3A4 or strong inducers of CYP3A4
18. Contraindication to MRI

Part B
1. Diagnosed with a malignancy in the past 2 years, unless for protocol defined
allowed malignancies
2. Current or recent (within 2 months of IP administration) GI disease or
disorders that increase the risk of diarrhea, such as inflammatory bowel
disease and Crohn's disease
3. Evidence of uncontrolled, active infection, requiring systemic
antibacterial, anti-viral or anti-fungal therapy <=7 days prior to
administration of IP
4. Myocardial infarction within 6 months prior to enrollment, greater than
Class 1 angina pectoris, or has NYHA Class III or IV heart failure,
symptomatic ventricular arrhythmias, sustained ventricular tachycardia, TdP,
the long QT syndrome, pacemaker dependence, or electrocardiographic evidence of
acute ischemia
5. History of additional risk factors for TdP
6. Unstable or severe uncontrolled medical condition or any important medical
illness or abnormal laboratory finding
7. Pregnant or breastfeeding or expecting to conceive children during the study
8. ECOG performance status >=2
9. Abnormal organ and marrow function at Screening defined as: a. Neutrophils
<1500/mm3; b. Platelet count <100,000/mm3; c.
Hemoglobin <9 g/dL; d. Electrolytes (potassium, calcium, magnesium, and
phosphorus, using corrected value if low serum albumin level is present)
outside the normal limits of the local laboratory; e. Total bilirubin >1.5x ULN
(except known Gilbert's syndrome >3x ULN); f. AST and ALT >2.5x ULN; g. Serum
or plasma creatinine > ULN and CrCl <60 mL/min (calculation of CrCl will be
based on acceptable institution
standard); h. Uncontrolled triglyceride >=Grade 2 elevations per CTCAE v5.0
(>300 mg/dL or >3.42 mmol/L); i.Any other laboratory abnormality >=Grade 3
10. ECG Exclusions: a. Mean QTcF >=450 msec; b. Second or third degree AV block
11. Any treatments for desmoid tumors within 4 weeks prior to first dose
12. Chronic NSAIDs for the treatment of desmoid tumors within 4 weeks of first
dose
13. Prior treatment with GSI or other agents targeting the Notch pathway
14. Use of strong inhibitors of CYP3A4 or strong inducers of CYP3A

OLE
1. Unstable or severe uncontrolled medical condition or any important medical
illness, abnormal ECG or laboratory finding.
2. Ongoing TEAE from Part A or Part B that requires discontinuation.
3. Breastfeeding or expecting to conceive children within the projectedduration
of the study.
4. Use of any therapy that is prohibited in Part A or Part B of the study.
5. Concurrent enrollment in another clinical study unless it is an
observational (non-interventional) clinical study.
6. Hypersensitivity to AL102 and any of its excipients.
Other protocol defined criteria could apply