Al Ghulaiga Faris M, Alkhiary Hattan, AlKhalidi Hisham, Alkatan Hind M
Department of Ophthalmology, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Department of Pathology & Laboratory Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia; King Saud University Medical City, King Saud University, Riyadh, Saudi Arabia.
Int J Surg Case Rep. 2022 Oct;99:107623. doi: 10.1016/j.ijscr.2022.107623. Epub 2022 Sep 10.
Adenoid cystic carcinoma (ACC) is an uncommon malignant epithelial tumor of the salivary and lacrimal glands. Orbital ACC is rare, originating commonly from the lacrimal gland, with only a few cases reported without lacrimal gland involvement. Deep orbital ACC may be associated with extension into skull base structures, and further intracranial invasion.
We report a 47-year-old gentleman who presented with insidious onset of bilateral proptosis, left ophthalmoplegia, and loss of vision. Imaging revealed left orbital infiltrative mass with intracranial invasion and bilateral cavernous sinus extension. The lacrimal gland was not involved clinically nor radiologically. Histopathology showed ACC with classical cribriform pattern. There was no evidence of primary source of tumor or metastasis.
ACC of the orbit commonly originates from the lacrimal gland. Only a few cases of orbital ACC without lacrimal gland involvement were found in English literature. Workup for our patient did not reveal a primary source of tumor. Tumor may have risen from ectopic orbital lacrimal gland tissue, extension from non-orbital sites, or through perineural or hematogenous spread. Only one case of bilateral cavernous sinus extension has been previously reported. Treatment for advanced orbital ACC is exenteration in most cases. However, due to the advanced nature of disease in our patient, palliative radiotherapy was the treatment of choice.
Orbital ACC of non-lacrimal origin is rare and is associated with high morbidity and mortality. Early recognition and treatment are key for preventing organ and life-threatening complications such as advanced intracranial spread.
腺样囊性癌（ACC）是一种罕见的唾液腺和泪腺恶性上皮性肿瘤。眼眶腺样囊性癌较为罕见，通常起源于泪腺，仅有少数病例报道未累及泪腺。眼眶深部腺样囊性癌可能与向颅底结构的扩展及进一步的颅内侵犯有关。
我们报告一名47岁男性，表现为隐匿性双侧眼球突出、左侧眼肌麻痹和视力丧失。影像学检查显示左侧眼眶浸润性肿块伴颅内侵犯及双侧海绵窦扩展。临床及影像学检查均未发现泪腺受累。组织病理学显示为具有典型筛状结构的腺样囊性癌。未发现肿瘤的原发灶或转移证据。
眼眶腺样囊性癌通常起源于泪腺。英文文献中仅发现少数眼眶腺样囊性癌未累及泪腺的病例。对我们的患者进行的检查未发现肿瘤的原发灶。肿瘤可能起源于异位眼眶泪腺组织、非眼眶部位的扩展、或通过神经周围或血行播散。此前仅报道过一例双侧海绵窦扩展的病例。大多数情况下，晚期眼眶腺样囊性癌的治疗方法是眶内容摘除术。然而，由于我们患者的病情已属晚期，姑息性放疗是首选的治疗方法。
非泪腺起源的眼眶腺样囊性癌罕见，且与高发病率和死亡率相关。早期识别和治疗是预防诸如晚期颅内播散等危及器官和生命的并发症的关键。
