This phase II trial studies nivolumab and ipilimumab in treating patients with rare tumors.
Immunotherapy with monoclonal antibodies, such as nivolumab and ipilimumab, may help the
body's immune system attack the cancer, and may interfere with the ability of tumor cells to
grow and spread.
This trial enrolls participants for the following cohorts based on condition:
Epithelial tumors of nasal cavity, sinuses, nasopharynx: A) Squamous cell carcinoma with
variants of nasal cavity, sinuses, and nasopharynx and trachea (excluding laryngeal,
nasopharyngeal cancer [NPC], and squamous cell carcinoma of the head and neck [SCCHN])
Adenocarcinoma and variants of nasal cavity, sinuses, and nasopharynx (closed to
Epithelial tumors of major salivary glands (closed to accrual 03/20/2018)
Salivary gland type tumors of head and neck, lip, esophagus, stomach, trachea and lung,
breast and other location (closed to accrual)
Undifferentiated carcinoma of gastrointestinal (GI) tract
Adenocarcinoma with variants of small intestine (closed to accrual 05/10/2018)
Squamous cell carcinoma with variants of GI tract (stomach small intestine, colon,
rectum, pancreas) (closed to accrual 10/17/2018)
Fibromixoma and low grade mucinous adenocarcinoma (pseudomixoma peritonei) of the
appendix and ovary (closed to accrual 03/20/2018)
Rare pancreatic tumors including acinar cell carcinoma, mucinous cystadenocarcinoma or
serous cystadenocarcinoma. Pancreatic adenocarcinoma is not eligible
Intrahepatic cholangiocarcinoma (closed to accrual 03/20/2018)
Extrahepatic cholangiocarcinoma and bile duct tumors (closed to accrual 03/20/2018)
Sarcomatoid carcinoma of lung
Bronchoalveolar carcinoma lung. This condition is now also referred to as adenocarcinoma
in situ, minimally invasive adenocarcinoma, lepidic predominant adenocarcinoma, or
invasive mucinous adenocarcinoma
Non-epithelial tumors of the ovary: A) Germ cell tumor of ovary B) Mullerian mixed tumor
and adenosarcoma (closed to accrual 03/30/2018)
Trophoblastic tumor: A) Choriocarcinoma (closed to accrual)
Transitional cell carcinoma other than that of the renal, pelvis, ureter, or bladder
(closed to accrual)
Cell tumor of the testes and extragonadal germ tumors: A) Seminoma and testicular sex
cord cancer B) Non seminomatous tumor C) Teratoma with malignant transformation (closed
Epithelial tumors of penis - squamous adenocarcinoma cell carcinoma with variants of
Squamous cell carcinoma variants of the genitourinary (GU) system
Spindle cell carcinoma of kidney, pelvis, ureter
Adenocarcinoma with variants of GU system (excluding prostate cancer) (closed to accrual
Odontogenic malignant tumors
Pancreatic neuroendocrine tumor (PNET) (formerly named: Endocrine carcinoma of pancreas
and digestive tract.) (closed to accrual)
Neuroendocrine carcinoma including carcinoid of the lung (closed to accrual 12/19/2017)
Pheochromocytoma, malignant (closed to accrual)
Paraganglioma (closed to accrual 11/29/2018)
Carcinomas of pituitary gland, thyroid gland parathyroid gland and adrenal cortex
(closed to accrual)
Peripheral nerve sheath tumors and NF1-related tumors (closed to accrual 09/19/2018)
Malignant giant cell tumors
Chordoma (closed to accrual 11/29/2018)
Adrenal cortical tumors (closed to accrual 06/27/2018)
Tumor of unknown primary (Cancer of Unknown Primary; CuP) (closed to accrual 12/22/2017)
Not Otherwise Categorized (NOC) Rare Tumors [To obtain permission to enroll in the NOC
cohort, contact: S1609SC@swog.org] (closed to accrual 03/15/2019)
Adenoid cystic carcinoma (closed to accrual 02/06/2018)
Vulvar cancer (temporarily closed to accrual)
MetaPLASTIC carcinoma (of the breast) (closed to accrual)
Gastrointestinal stromal tumor (GIST) (closed to accrual 09/26/2018)
Perivascular epithelioid cell tumor (PEComa)
Apocrine tumors/extramammary Paget's disease (closed to accrual)
Peritoneal mesothelioma (temporarily closed to accrual 05/08/2020)
Basal cell carcinoma (temporarily closed to accrual 04/29/2020)
Clear cell cervical cancer
Esthenioneuroblastoma (closed to accrual)
Endometrial carcinosarcoma (malignant mixed Mullerian tumors) (closed to accrual)
Clear cell ovarian cancer (closed to accrual)
Gestational trophoblastic disease (GTD)
Small cell carcinoma of the ovary, hypercalcemic type
PD-L1 amplified tumors
High-grade neuroendocrine carcinoma (pancreatic neuroendocrine tumor [PNET] should be
enrolled in Cohort 22; prostatic neuroendocrine carcinomas should be enrolled into
Cohort 52). Small cell lung cancer is not eligible (temporarily closed to accrual
Treatment-emergent small-cell neuroendocrine prostate cancer (t-SCNC)
I. To evaluate the Response Evaluation Criteria in Solid Tumors (RECIST) version (v)1.1
overall response rate (ORR) in subsets of patients with advanced rare cancers treated with
ipilimumab plus nivolumab combination immunotherapy.
II. To evaluate the overall response rate (ORR) in patients with gestational trophoblastic
tumors treated with ipilimumab plus nivolumab combination immunotherapy.
III. To evaluate the RECIST v1.1 overall response rate (ORR) in patients PD-L1 amplified
cancers treated with nivolumab immunotherapy.
I. To evaluate toxicities in each cohort. II. To estimate overall survival (OS),
progression-free survival (PFS), clinical benefit rate; and to estimate immune related
(i)RECIST ORR (iORR), and iRECIST PFS (iPFS) across cohorts and within each cohort.
OUTLINE: Patients are assigned to 1 of 2 arms.
ARM I (ALL COHORTS EXCEPT THE PD-L1 AMPLIFIED COHORT): Patients receive nivolumab
intravenously (IV) over 30 minutes on days 1, 15, and 29 and ipilimumab IV over 60 minutes on
day 1. Treatment repeats every 42 days for up to 17 cycles (2 years) in the absence of
disease progression or unacceptable toxicity. Patients who complete 17 cycles (2 years) of
therapy, may continue receiving the same treatment with nivolumab and ipilimumab, or receive
nivolumab once every 14 or 28 days (2 weeks or 4 weeks) per physician discretion in the
absence of disease progression or unacceptable toxicity. Patients who stop treatment prior to
the completion of 17 cycles of therapy may receive nivolumab once every 14 or 28 days (2
weeks or 4 weeks) in the absence of disease progression or unacceptable toxicity.
ARM II (PD-L1 AMPLIFIED COHORT): Patients receive nivolumab IV over 30 minutes on days 1, 15
and 29. Treatment repeats every 42 days for up to 17 cycles (2 years) in the absence of
disease progression or unacceptable toxicity. After 17 cycles (2 years) of therapy, patients
may receive nivolumab once every 14 or 28 days (2 weeks or 4 weeks) in the absence of disease
progression or unacceptable toxicity.
After completion of study treatment, patients are followed up for 10 years from registration.
Clinical Study Identifier
National Cancer Institute (NCI)
Last Modified on
26 November 2020
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