Primary apocrine adenocarcinoma of the orbit with lacrimal sac invasion

Periocular apocrine adenocarcinoma presenting as an orbital mass: clinicopathological features and management in four patients

“…Interestingly, all orbital tumors associated with AC described in the literature and our cases were in the medial part of the orbit. 5,12–17 The recurrent tumor in our 3 rd patient developed in the lateral orbit and lacrimal gland probably due to slow perineural invasion.…”

“…Based on reported cases, exenteration emerges as the safest therapeutic approach for AC with orbital involvement. 5,12–18 Most patients managed likewise did not have any recurrence for a follow-up period ranging from 1.5 to 5 years. 5,12–17 Three patients additionally received orbital radiotherapy because of the presence of risk factors including lymph node and extensive orbital involvement.…”

“…5,12–18 Most patients managed likewise did not have any recurrence for a follow-up period ranging from 1.5 to 5 years. 5,12–17 Three patients additionally received orbital radiotherapy because of the presence of risk factors including lymph node and extensive orbital involvement. 12,16,17 In our first patient who had a tumor-free periosteum, exenteration alone provided a disease-free survival for 10 years.…”

“…5,[12][13][14][15][16][17] Three patients additionally received orbital radiotherapy because of the presence of risk factors including lymph node and extensive orbital involvement. 12,16,17 In our first patient who had a tumor-free periosteum, exenteration alone provided a disease-free survival for 10 years. Our second patient received radiotherapy after exenteration because of periosteal involvement and this provided a 6-year recurrence-free survival.…”

“…In two of these patients, AC developed in the lacrimal caruncle and the main bulk of the tumor grew into the orbit. 12,13 The caruncle may contain a small number of sweat glands, and Popoff's glands which are ectopic accessory lacrimal glands, all having the potential to give rise to AC. 14 In our cases we did not identify any histopathological evidence to pinpoint the caruncle as the origin of the tumor.…”