Reference Age Sex Presentation Duration Management Radiotherapy Lymph nodes Distant metastasis Outcome [3] 68 M Painless mass 6 months Fronto-temporal craniotomy and en-bloc orbitectomy  with tumor resection 60 Gy RT given No evidence No evidence Alive at 10 months [4] 67 M Painless mass NA En-bloc tumor resection with frontal craniotomy 40 Gy RT given to the subdural metastasis No evidence 2 years post surgery, Subdural metastasis  in temporal lobe, -resected Alive at 2 years [5] 46 M Painless proptosis with neurofibromat-osis 2 years Lid-sparing orbital exenteration  RT given No evidence No evidence Alive at 19 months [6] 59 M Blepharoptosis with painless mass 15 years  blepharo-ptosis Lid-sparing orbital exenteration RT planned Parotid and cervical lymph nodes No evidence Alive at 6 months [7] 67 M Decreased vision and mass lesion NA En-bloc tumor resection RT given No evidence Metastasis to Brain, liver, lungs, pancreas, common biliary duct Died at 2 years 10 months [8] 47 M Mass in lacrimal gland region NA Orbital exenteration RT given Mediastinal nodes and skin lymphatics Metastasis to spine, pelvis, femur, cerebellum 10 years after surgery Died at 17 years [9] 50 M Non tender mass with low vision 2 years Lateral orbitotomy with en-bloc tumor removal Not given No evidence No evidence Alive at 10 months [10] 78 M Painless palpable mass NA Lid-sparing exenteration Not given Parotid and cervical lymph nodes  No evidence Died at 2 years [11] 75 M Swelling of upper eyelid 3 months No surgery of orbital mass Carbon ion radiotherapy 58 Gy Submandib-ular lymph nodes Metastasis to lung, chemotherapy given Died at 2 years 67 M Swelling and ptosis of upper eyelid 6 months Orbital exenterationwith bone removal No RT given Submandib-ular lymph nodes Multiple metastasis to bone and liver Died at 1.3 years 53 M NA 18 months Orbital exenteration RT given 60 Gy No evidence Metastasis to spine, brain and liver Died at 4.3 years 39 M NA 6 months Orbital exenteration with bone removal RT given 50 Gy No evidence Metastasis to lung and brain Alive at 10 years 46 F NA 1month Tumor resection with globe sparing surgery Not given No evidence No evidence Alive at 5.5 years Our case 38 F Painful swelling in upper eyelid 2 months Tumor resection with globe sparing surgery RT given 60 Gy after recurrence No evidence CNS metastasis Died at 6 months

Table 1: Clinical data, management and outcomes of the published cases of primary ductal adenocarcinoma of the lacrimal gland (in chronological order).