Pathology Department, Shaoxing People's Hospital, Shaoxing, People's Republic of China.
Department of Hepatobiliary Surgery, Shaoxing People's Hospital, Shaoxing, People's Republic of China.
Medicine (Baltimore). 2024 Aug 2;103(31):e39147. doi: 10.1097/MD.0000000000039147.
Neuroendocrine neoplasms (NENs) originating from neuroendocrine cells occur in the thyroid, respiratory, and digestive systems, with Gallbladder Neuroendocrine Carcinoma (GB-NEC) accounting for only 0.5% of all NENs and 2.1% of gallbladder cancers. Due to its rarity, little is known about GB-NEC's clinical presentation and treatment.
We report a case of a 52-year-old male presenting with acute upper right abdominal pain, leading to further investigation.
Initial diagnostic workup, including abdominal ultrasound and contrast-enhanced CT, suggested gallbladder malignancy. Post-surgical pathology confirmed GB-NEC, with immunohistochemistry supporting the diagnosis.
The patient underwent radical cholecystectomy, followed by etoposide plus cisplatin chemotherapy. After disease progression indicated by CT, the patient received additional cycles of chemotherapy with cisplatin and irinotecan, plus targeted therapy with anlotinib and immunotherapy with paimiplimab.
The patient showed a partial response to initial treatment. Subsequent liver biopsy confirmed NEC, consistent with small cell carcinoma. With continued treatment, the patient maintains a good survival status.
GB-NEC is associated with poor prognosis, emphasizing the importance of early detection and multimodal treatment strategies. Our case underlines the potential benefit of a comprehensive treatment plan, including aggressive surgery and chemotherapy, with further research needed to standardize treatment for this rare condition.
神经内分泌肿瘤(NENs)起源于神经内分泌细胞,可发生于甲状腺、呼吸系统和消化系统,胆囊神经内分泌癌(GB-NEC)仅占所有 NENs 的 0.5%和胆囊癌的 2.1%。由于其罕见性,对 GB-NEC 的临床表现和治疗知之甚少。
我们报告了一例 52 岁男性,表现为急性右上腹疼痛,进一步检查。
初步诊断工作,包括腹部超声和增强 CT,提示胆囊恶性肿瘤。术后病理证实为 GB-NEC,免疫组化支持诊断。
患者接受根治性胆囊切除术,随后接受依托泊苷加顺铂化疗。CT 提示疾病进展后,患者接受了顺铂和伊立替康联合化疗以及安罗替尼靶向治疗和帕姆单抗免疫治疗。
患者对初始治疗有部分反应。随后的肝活检证实为 NEC,符合小细胞癌。随着持续治疗,患者保持良好的生存状态。
GB-NEC 预后不良,强调早期发现和多模式治疗策略的重要性。我们的病例强调了综合治疗方案的潜在益处,包括积极的手术和化疗,需要进一步研究来规范这种罕见疾病的治疗。