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Analysis of the essence丨Professor Ma Jianmin shares the diagnosis and treatment strategy of lacrimal gland adenoid cystic carcinoma

Posting time:2023-01-31 09:24:58

Analysis of the essence丨Professor Ma Jianmin shares the diagnosis and treatment strategy of lacrimal gland adenoid cystic carcinoma

Editor's note: Lacrimal gland adenoid cystic carcinoma is the most common malignant epithelial tumor of the lacrimal gland. Due to the insignificant early symptoms and the complex orbital anatomy, early detection and subsequent treatment of the disease are difficult. In recent years, Professor Ma Jianmin of Beijing Tongren Hospital affiliated to Capital Medical University and his team have carried out systematic research from basic to clinical on lacrimal gland adenoid cystic carcinoma. At the 2022 Vision China International Forum on Innovation and Development (Vision China), Professor Ma shared a wonderful report on "Diagnosis and Treatment Strategies for Lacrimal Adenoid Cystic Carcinoma", and shared with colleagues in ophthalmology. Lacrimal gland adenoid cystic carcinoma "three highs" features: high recurrence rate, high metastasis rate, and high mortality. Lacrimal gland adenoid cystic carcinoma is the most common malignant epithelial tumor in the lacrimal gland area, and its incidence accounts for 25% of lacrimal gland epithelial tumors. ~30%, accounting for 60%~75% of lacrimal gland malignancies, 4.64% of orbital tumors, and 1% of head malignancies. After years of research, Professor Ma found that lacrimal gland adenoid cystic carcinoma has the characteristics of "three highs": high recurrence rate, as high as 77.14%, high metastasis rate, 16%~40%. The lungs, liver, bones, and parotid glands are the common metastatic sites. Among them, lung metastases are more common and have a high mortality rate. The pathogenesis of lacrimal gland adenoid cystic carcinoma in 44.4%-78% is still unclear. According to many studies, its occurrence and development , Metastasis and invasion may be related to cell signaling pathways and cytokines. The clinical manifestations of lacrimal gland adenoid cystic carcinoma lack specificity, which diseases are differentially diagnosed with? Adenoid cystic carcinoma of the lacrimal gland is a unilateral lesion with a lack of specific clinical manifestations, generally manifesting as eyelid swelling, ptosis, mass in the lacrimal gland, proptosis, and lowering of the eye position. It has been reported in the literature that most patients present with pain and discomfort in the orbital area because of their neurotrophic properties. Imaging manifestations are solid mass in the lacrimal gland area, with unclear boundaries, bone destruction of the lacrimal fossa, lesions can invade the orbital apex, and lesions can be enhanced; invading the cranial or nasal cavity is likely to cause cranio-orbital communication or nasal-orbital communication lesions. . The initial diagnosis is generally based on clinical manifestations and imaging changes. Histopathology is the gold standard for the diagnosis of lacrimal gland adenoid cystic carcinoma. Professor Ma pointed out that when diagnosing lacrimal adenoid cystic carcinoma, the differential diagnosis with the following diseases should be considered: Lymphoma of the lacrimal gland: Lymphoma is currently the most common adult ocular malignant tumor, which can occur in all tissue structures of the eye, including the lacrimal gland. It is one of its predisposing parts. Lacrimal gland lymphoma can occur in one or both eyes, and is more common in people over 50 years old. Common symptoms include eyelid swelling, lumps in the lacrimal gland area, and proptosis. Compared with lacrimal gland adenoid cystic carcinoma, lacrimal gland lymphoma is less prone to bone destruction. Lacrimal gland adenocarcinoma: The incidence is second only to lacrimal gland adenoid cystic carcinoma, and the clinical manifestations are monocular lesions; eyelid swelling, lacrimal gland mass, accompanied by ptosis, proptosis, and pain. It is difficult to differentiate lacrimal gland carcinoma from lacrimal gland adenoid cystic carcinoma based on clinical and imaging findings, and histopathology is currently the only basis for identifying these two diseases. Malignant transformation of lacrimal gland pleomorphic adenoma: The patient has a history of lacrimal gland pleomorphic adenoma, and the clinical manifestations are monocular lesions, eyelid swelling, single or multiple masses in the lacrimal gland area, accompanied by ptosis, proptosis, etc. The history of lacrimal pleomorphic adenoma is the clinical basis for differentiating malignant transformation of lacrimal pleomorphic adenoma from lacrimal adenoid cystic carcinoma, and the final identification is still based on the results of histopathological examination. Solitary fibroma: a single eye lesion, a single mass, accompanied by ptosis, proptosis, etc. Multiple locations of the orbit can occur. Inflammatory pseudotumor of the lacrimal gland: involving one or both eyes, swelling of the eyelid, swelling of the lacrimal gland, long course of disease, repeated disease, effective for glucocorticoid treatment. Langerhans histiocytosis: more common in children, more involved in one eye, relatively short course of disease; eyelid swelling, lacrimal gland area predilection; bone destruction. Treatment of adenoid cystic carcinoma of the lacrimal gland: surgery combined with radiation therapy According to the existing literature, the main point of treatment for adenoid cystic carcinoma of the lacrimal gland is surgical resection combined with radiation therapy. Surgical treatment: The choice of surgical approach is divided into sub-brow incision and double eyelid incision. Professor Ma prefers to choose sub-brow incision, which has better incision exposure and better surgical safety. Radiation therapy: Combined radiation therapy is required after surgery. Radiation therapy is very important for lacrimal adenoid cystic carcinoma, which can reduce the recurrence rate and metastasis rate and improve the prognosis. Radiation therapy includes particle I125 internal radiation therapy and external radiation therapy. Both radiotherapy methods can reduce the recurrence rate and prolong the survival time of patients to a certain extent. The study found that there was no significant difference in the effect of particle I125 internal radiotherapy and gamma knife radiotherapy. Chemotherapy: In addition to surgery and radiotherapy, chemotherapy is also used internationally for the treatment of lacrimal adenoid cystic carcinoma, but this method is relatively rare in China. Molecular targeted therapy: Targeted therapy is currently the most common treatment for malignant tumors, and specific gene mutations and gene fusions are the targets of future targeted therapy. At present, the research on targeted therapy for lacrimal gland adenoid cystic carcinoma mainly includes: MYB-NFIB gene fusion transcription, that is, translocation of chromosomes 6 and 9; bromodomain PHD domain transcription factor (BPTF) and Nocth pathway may be Potential targets for the treatment of low-grade ACC; AZD4547, a novel selective inhibitor of fibroblast growth factor receptor 1; Bcl-2 and NF-KB signaling pathways; matrix metalloproteinases and MAPK/MMP pathways; anti-vascular endothelial growth factor; PD-1 and PD-L1. Professor Ma emphasized that since lacrimal gland adenoid cystic carcinoma is a malignant lesion, surgical treatment is not a once-and-for-all, and there is the possibility of recurrence and metastasis, which requires long-term and close follow-up. In addition, the relationship between the location of the tumor and surrounding tissues, the degree of bone destruction, neurotropic properties, the pathological type of the tumor (solid tumors have a poor prognosis), the pathological stage of the tumor (T3~T4 tumors have a poor prognosis), the extent and method of surgical resection, Psychological factors and social factors all affect the prognosis of lacrimal gland adenoid cystic carcinoma. Finally, Professor Ma summarized the main points of diagnosis and treatment of lacrimal gland adenoid cystic carcinoma: lacrimal gland adenoid cystic carcinoma is a common malignant tumor originating in lacrimal gland tissue, and its clinical manifestations are not specific; histopathological examination is the gold standard for its diagnosis. ; The most basic treatment plan is surgical resection combined with local radiotherapy; focus on distant metastasis of lacrimal gland adenoid cystic carcinoma.

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