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Abstract：
BACKGROUND: Alveolar soft part sarcoma (ASPS) occurs most often in the deep muscles or fascia of the extremities in adults, with only 3.4% of these tumours originating from the head, face and neck. To date, only 17 cases of buccal ASPS have been reported, including the case presented here. Only one case of ASPS recurrence at the primary site, similar to our case, has been reported thus far. Immune checkpoint inhibitors (ICPis)-associated diabetes, with an estimated incidence of 0.43%, is usually seen in older cancer patients and has not been reported in younger people or in patients with ASPS.
METHODS: A 24-year-old male patient presented with a slowly progressing right cheek mass with a clinical history of approximately 28 months. Sonographic imaging revealed a hypoechoic mass, which was considered a benign tumour. However, a pathological diagnosis of ASPS was made after excision of the mass. Five days later, functional right cervical lymph node dissection was performed. No other adjuvant therapy was administered after surgery. In a periodic follow-up of the patient six months later, blood-rich tumour growth was noted at the primary site, and Positron emission tomography-computedtomography (PET-CT) ruled out distant metastasis in other areas. The patient was referred to the Ninth People\'s Hospital of Shanghai Jiaotong University. Due to the large extent of the mass, the patient received a combination of a Programmed Cell Death Ligand 1(PD-L1) inhibitor and a targeted drug. Unfortunately, the patient developed three episodes of severe diabetic ketoacidosis after the administration of the drugs. A confirmed diagnosis of ICPis-associated diabetes was confirmed. After the second operation, the postoperative pathological diagnosis was ASPS, and the margins were all negative. Therefore, we made a final clinical diagnosis of ASPS recurrence at the primary site. Currently in the follow-up, the patient is alive, has no distant metastases, and undergoes multiple imaging examinations every 3 months for the monitoring of their condition.
CONCLUSIONS: In analysing the characteristics of all previously reported cases of buccal ASPS, it was found that the clinical history ranged from 1 to 24 months, with a mean of approximately 3 to 9 months. Tumour recurrence at the primary site has been reported in only one patient with buccal ASPS, and the short-term recurrence in our patient may be related to the extraordinarily long 28-month history. ICPis-associated diabetes may be noted in young patients with rare tumours, and regular insulin level monitoring after use is necessary.
METHODS: A 24-year-old male patient presented with a slowly progressing right cheek mass with a clinical history of approximately 28 months. Sonographic imaging revealed a hypoechoic mass, which was considered a benign tumour. However, a pathological diagnosis of ASPS was made after excision of the mass. Five days later, functional right cervical lymph node dissection was performed. No other adjuvant therapy was administered after surgery. In a periodic follow-up of the patient six months later, blood-rich tumour growth was noted at the primary site, and Positron emission tomography-computedtomography (PET-CT) ruled out distant metastasis in other areas. The patient was referred to the Ninth People\'s Hospital of Shanghai Jiaotong University. Due to the large extent of the mass, the patient received a combination of a Programmed Cell Death Ligand 1(PD-L1) inhibitor and a targeted drug. Unfortunately, the patient developed three episodes of severe diabetic ketoacidosis after the administration of the drugs. A confirmed diagnosis of ICPis-associated diabetes was confirmed. After the second operation, the postoperative pathological diagnosis was ASPS, and the margins were all negative. Therefore, we made a final clinical diagnosis of ASPS recurrence at the primary site. Currently in the follow-up, the patient is alive, has no distant metastases, and undergoes multiple imaging examinations every 3 months for the monitoring of their condition.
CONCLUSIONS: In analysing the characteristics of all previously reported cases of buccal ASPS, it was found that the clinical history ranged from 1 to 24 months, with a mean of approximately 3 to 9 months. Tumour recurrence at the primary site has been reported in only one patient with buccal ASPS, and the short-term recurrence in our patient may be related to the extraordinarily long 28-month history. ICPis-associated diabetes may be noted in young patients with rare tumours, and regular insulin level monitoring after use is necessary.
摘要：
背景：肺泡软组织肉瘤（ASPS）最常见于成人四肢的深层肌肉或筋膜，这些肿瘤中只有3.4%来自头部，脸和脖子迄今为止,仅报告了17例颊部ASPS，包括这里介绍的案例。只有一例ASPS在原发部位复发，和我们的情况类似,到目前为止已经有报道。免疫检查点抑制剂（ICPis）相关糖尿病，估计发病率为0.43%，通常见于老年癌症患者，在年轻人或ASPS患者中没有报道。
方法：一名24岁男性患者表现为缓慢进展的右脸颊肿块，临床病史约28个月。超声成像显示低回声肿块，被认为是良性肿瘤.然而,切除肿块后对ASPS进行病理诊断.五天后,行功能性右颈淋巴结清扫术。手术后不给予其他辅助治疗。在六个月后对患者进行的定期随访中，在原发部位观察到富含血液的肿瘤生长，和正电子发射断层扫描（PET-CT）排除了其他区域的远处转移。患者转诊至上海交通大学第九人民医院。由于质量很大，患者接受了程序性细胞死亡配体1(PD-L1)抑制剂和靶向药物的联合治疗.不幸的是,患者在服用这些药物后出现了3次严重的糖尿病酮症酸中毒.确认了ICPis相关糖尿病的确诊。第二次手术后，术后病理诊断为ASPS,利润率都是负值。因此,我们对原发灶ASPS复发进行了最终临床诊断.目前在后续行动中,病人还活着,没有远处转移,并每3个月进行多次影像学检查以监测其病情。
结论：在分析所有先前报道的口腔ASPS病例的特征时，发现临床病史为1至24个月，平均约3至9个月。据报道，只有一名口腔ASPS患者在原发部位肿瘤复发。我们患者的短期复发可能与超长的28个月病史有关.ICPis相关糖尿病可能在罕见肿瘤的年轻患者中出现，和使用后定期监测胰岛素水平是必要的。
方法：一名24岁男性患者表现为缓慢进展的右脸颊肿块，临床病史约28个月。超声成像显示低回声肿块，被认为是良性肿瘤.然而,切除肿块后对ASPS进行病理诊断.五天后,行功能性右颈淋巴结清扫术。手术后不给予其他辅助治疗。在六个月后对患者进行的定期随访中，在原发部位观察到富含血液的肿瘤生长，和正电子发射断层扫描（PET-CT）排除了其他区域的远处转移。患者转诊至上海交通大学第九人民医院。由于质量很大，患者接受了程序性细胞死亡配体1(PD-L1)抑制剂和靶向药物的联合治疗.不幸的是,患者在服用这些药物后出现了3次严重的糖尿病酮症酸中毒.确认了ICPis相关糖尿病的确诊。第二次手术后，术后病理诊断为ASPS,利润率都是负值。因此,我们对原发灶ASPS复发进行了最终临床诊断.目前在后续行动中,病人还活着,没有远处转移,并每3个月进行多次影像学检查以监测其病情。
结论：在分析所有先前报道的口腔ASPS病例的特征时，发现临床病史为1至24个月，平均约3至9个月。据报道，只有一名口腔ASPS患者在原发部位肿瘤复发。我们患者的短期复发可能与超长的28个月病史有关.ICPis相关糖尿病可能在罕见肿瘤的年轻患者中出现，和使用后定期监测胰岛素水平是必要的。
