Department of Ophthalmology, New York Eye and Ear Infirmary, New York, NY, USA.
Ophthalmic Plast Reconstr Surg. 2011 Sep-Oct;27(5):e119-21. doi: 10.1097/IOP.0b013e318201cb90.
A rare variant of adenoid cystic carcinoma is the dedifferentiated sarcomatoid form, which has previously been reported in the hard and soft palate, maxillary sinus, submandibular glands, and nasal cavity. The authors report the first case of a dedifferentiated sarcomatoid adenoid cystic carcinoma occurring in the lacrimal gland, that of a 52-year-old man. The patient presented with a 4-month history of diplopia, decreased vision, and right upper eyelid swelling. Radiographic imaging showed a soft tissue mass in the extraconal compartment superolateral to the right eye. The patient subsequently underwent surgical debulking. Histologic examination of the tissue revealed classic cribiform adenoid cystic carcinoma and a sarcomatous component consisting of malignant spindle cells and fusiform cells arranged in whorls. Dedifferentiation is a well-established phenomenon in salivary gland tumors that is associated with aggressive behavior and poor prognosis; however, the exact nature of such dedifferentiated neoplasms remains unclear.
腺样囊性癌的一种罕见变体为去分化肉瘤样型，此前已在硬腭和软腭、上颌窦、颌下腺和鼻腔中报道过。作者报告了首例发生在泪腺的去分化肉瘤样腺样囊性癌，患者为 52 岁男性。该患者出现复视、视力下降和右上眼睑肿胀 4 个月。影像学检查显示右眼外上方眶隔外有软组织肿块。随后患者接受了手术切除术。组织学检查显示典型的筛状腺样囊性癌和肉瘤样成分，由恶性梭形细胞和呈漩涡状排列的梭形细胞组成。去分化是唾液腺肿瘤中一种已确立的现象，与侵袭性行为和不良预后相关；然而，这种去分化肿瘤的确切性质仍不清楚。
