Double pituitary neuroendocrine tumors (double PitNETs) are two distinct tumors in the same gland and are infrequent in clinical practice. In typical double PitNETs, an MRI detects two separate tumors that are diagnosed by pathology; they could also appear as a single tumor, and pathology would then identify the two independent tumors. A literature review was conducted, and 142 cases were analyzed to determine the characteristics of double PitNETs. Of these cases, acromegaly (45.5%) was the most common clinical feature, followed by Cushing’s disease (35.1%) and prolactinoma (17.9%), indicating that double PitNETs are usually noticed by hormonal excess symptoms due to at least one functional tumor. The pathological analysis of 284 tumors showed that somatotroph (28.9%) and corticotroph (26.8%) tumors were predominant, with a recent increase in the proportion of gonadotroph tumors. Regarding transcription factors, 51.1% were of GH-PRL-TSH PIT1-lineage, 26.1% ACTH TPIT-lineage, and 17.9% LH-FSH SF1-lineage. The radiological analysis of 82 cases revealed that double tumors (45.1%) and single tumors (47.6%) were comparable, suggesting that double PitNETs are often detected as a single tumor, and attention should be paid to hidden micro-tumors during surgery. Double PitNETs are complicated by a wide variety of clinical, radiological, and pathological findings, but diagnostic and therapeutic approaches are advancing.
双重垂体神经内分泌肿瘤(双重PitNETs)指同一腺体内存在两种不同的肿瘤,在临床实践中较为少见。在典型双重PitNETs中,MRI可检测到两个独立的肿瘤并经病理学确诊;也可能表现为单一肿瘤,而病理学检查会识别出两个独立的肿瘤。通过文献回顾分析142例病例,以明确双重PitNETs的特征。其中肢端肥大症(45.5%)是最常见的临床表现,其次为库欣病(35.1%)和泌乳素瘤(17.9%),这表明双重PitNETs通常因至少一个功能性肿瘤引起的激素过量症状而被发现。对284个肿瘤的病理学分析显示,生长激素细胞瘤(28.9%)和促肾上腺皮质激素细胞瘤(26.8%)占主导地位,促性腺激素细胞瘤的比例近期有所上升。在转录因子方面,51.1%属于GH-PRL-TSH PIT1谱系,26.1%为ACTH TPIT谱系,17.9%为LH-FSH SF1谱系。对82例病例的影像学分析显示,双重肿瘤(45.1%)与单一肿瘤(47.6%)比例相当,提示双重PitNETs常以单一肿瘤形式被检出,手术中应关注隐藏的微肿瘤。双重PitNETs的临床表现、影像学特征和病理学结果复杂多样,但诊断和治疗方法正在不断进步。