Pasireotide as Maintenance Treatment with monthly deep intramuscular injection in SSTR2/3/5-Expressing Synovial Sarcoma and Desmoplastic Small Round Cell Tumor (PAMSARC)

Prior treatment

• Prior treatment with somatostatin analog
• Concurrent or previous treatment within 30 days in another interventional clinical trial / Participation in other ongoing clinical trials
• Uncontrolled concurrent disease, in particular diabetes mellitus
• Is taking or requiring any of the prohibited medication listed in 6.4.2

Other illness

• History of hypersensitivity to the investigational medicinal product or to any drug with similar chemical structure or to any excipient present in the pharmaceutical form of the investigational medicinal product
• Bleeding disorder
• Heart rate at rest < 60/min
• Severe neurologic or psychiatric disorder

Lab values

• fasting glucose level > 110mg/dl

Other

• Pregnancy/lactation
• Therapeutic anticoagulation which cannot be paused temporarily in order to ensure safe intramuscular injection

• Reference pathological proven diagnosis of DSRCT in any stage; or Reference pathological proven diagnosis of SySa, IRS III, metastatic or relapsed disease
• Female patients of childbearing potential and male patients with partners of childbearing potential who are sexually active must agree to the use of two forms of contraception in combination (male condom and one highly effective method). These should be started immediately after signing the informed consent form and continued throughout the period of study treatment plus 3 months for female and male patients. Male patients should refrain from fathering a child or donating sperm during the trial and for at least 3 months following the last dose.
• Adequate bone marrow, renal, and hepatic function defined by laboratory tests within 14 days prior to study treatment (for details refer to protocol)
• High SSTR2/3/5 mRNA expression, as determined by RNA sequencing within DKFZ/NCT/DKTK MASTER program
• Stable disease, partial or complete response after completion of standard treatment
• Age from 13 to 50 years
• Bodyweight ≥ 30 kg
• Lansky-Index ≥ 80% in patients < 16 years of age and Karnofsky-Index ≥ 80% in patients ≥ 16 years of age (including adults).
• No curative treatment option
• Time from last chemotherapy (at least 2 chemotherapy cycles) to enrollment <8 weeks
• For women of childbearing potential negative urine pregnancy test at screening as well as highly effective forms of contraception have to be in place thereafter