Table 1.
Patient 1 | Patient 2 | Patient 3 | Patient 4 | Patient 5 | Patient 6 (our case) |
|
Age at onset, y | 47 | 61 | 64 | 61 | 45 | 56 |
Sex | F | M | F | F | F | F |
Author | Ogawa A | Higo K | Minatsuki S | Fukuda I | Fukuda I | Kubota K |
Year of report | 2013 | 2014 | 2015 | 2015 | 2016 | 2017 |
Primary disease | Gastric adenocarcinoma (signet-ring cells) | Colorectal cancer | Gastric adenocarcinoma (signet-ring cells) | Breast cancer | Breast cancer | Gastric adenocarcinoma (signet-ring cells) |
Drugs administered | Imatinib 100 mg bosentan 93.75 mg epoprostenol 27 ng/kg/min | Imatinib 50→200 mg bosentan tadalafil | Imatinib 200 mg sildenafil 60 mg ambrisentan 10 mg | Imatinib 200→400 mg tadalafil 40 mg | Imatinib 200→400 mg | Imatinib 200 mg bosentan 62.5 mg tadalafil 40 mg |
Mean PAP after administration of imatinib | 47→25 mm Hg | 48→35 mm Hg | 48→33 mm Hg | 60→56 mm Hg | 27→30 mm Hg | 47→22 mm Hg |
Chemotherapy | TS-1 40 mg | Bevacizumab 5 mg/kg TS-1 100 mg |
TS-1 | Eribulin | Capecitabine | TS-1 oxaliplatin |
Surgical therapy | (–) | (–) | Total gastrectomy | (–) | (–) | (–) |
Survival time | 9 months | 12 months | 12 months | 54 days | 24 days | 7 months |
Cause of death | Metastasis | Infection | Metastasis | Respiratory failure | Respiratory failure | Metastasis |
PAP, pulmonary arterial pressure; PTTM, pulmonary tumour thrombotic microangiopathy; TS, titanium silicate.