Table 2

Pembrolizumab response status based on RECIST and irRECIST for 57 patients with rare cancers

Primary cancerBased on RECISTBased on irRECIST
Controlled disease
(N=20)
Non-responders (N=37)Controlled disease
(N=24)
Non-responders
(N=33)
Adrenocortical carcinoma5 (25)4 (11)6 (25)3 (9)
Carcinoma of unknown primary2 (10)6 (16)4 (12)4 (12)
Medullary renal carcinoma1 (5)3 (8)1 (4)3 (9)
Other rare histologies*5 (25)5 (15)5 (20)5 (15)
Paraganglioma-pheochromocytoma3 (15)2 (5)3 (13)2 (6)
Penile carcinoma0 (0)1 (3)0 (0)1 (3)
Small cell malignancies of non-pulmonary origin0 (0)3 (8)1 (4)2 (6)
Spindle cell sarcoma of retroperitoneum†0 (0)1 (3)0 (0)1 (3)
Squamous cell carcinoma of the skin1 (5)3 (8)1 (4)3 (9)
Testicular carcinoma/germ cell tumor2 (10)7 (19)2 (8)7 (21)
Vascular sarcoma1 (5)2 (5)1 (4)2 (6)
  • All data are no. of patients (%).

  • *Vaginal squamous cell carcinoma and others.

  • †The one case of spindle cell sarcoma of retroperitoneum was initially diagnosed as adrenocortical carcinoma.

  • irRECIST, immune-related RECIST; RECIST, Response Evaluation Criteria in Solid Tumors.