Escudero Antonio García, Zarco Enrique Rodríguez, Arjona Juan Carlos Girón, Moreno María José Ríos, Rodríguez Katherine Gallardo, Benítez Ana Vallejo, Cámpora Ricardo González
Department of Anatomic Pathology, "Virgen Macarena" University Hospital, Avda, Doctor Fedriani s/n, Seville, 41009, Spain.
Department of Thoracic Surgery, "Virgen Macarena" University Hospital, Seville, Spain.
Virchows Arch. 2016 Sep;469(3):357-63. doi: 10.1007/s00428-016-1962-5. Epub 2016 Jun 14.
DIPNECH is characterized by neuroendocrine cell hyperplasia, tumorlets, and eventually carcinoid tumors. Although it is regarded by some authors as a preneoplastic condition, this issue is controversial. New pathologic criteria have recently been proposed for the diagnosis of DIPNECH, and a subgroup of carcinoid tumors expressing developing neural transcription factors (DNTFs), with clinicopathologic features similar to those of DIPNECH, has been recognized. This paper reports on the clinical and pathological findings in three cases of DIPNECH and investigates the expression of three DNTFs (TTF1, ASCL1, and POU3F2). All patients were female, with a mean age of 63 years, and all lesions were located in the periphery of the lung. In two cases, typical carcinoids were associated with a spindle-cell component. All neuroendocrine proliferations were DNTF positive. The morphologic (spindle-cell component), phenotypic (DNTF expression), and clinicopathologic (peripheral tumors, female predominance) similarities suggest that DIPNECH may be a preneoplastic lesion for peripheral carcinoids.
弥漫性特发性肺神经内分泌细胞增生症（DIPNECH）的特征为神经内分泌细胞增生、微瘤，最终发展为类癌肿瘤。尽管一些作者认为它是一种癌前病变，但这一问题存在争议。最近已提出用于诊断DIPNECH的新病理标准，并且已识别出一组表达发育性神经转录因子（DNTF）的类癌肿瘤，其临床病理特征与DIPNECH相似。本文报告了3例DIPNECH的临床和病理结果，并研究了三种DNTF（TTF1、ASCL1和POU3F2）的表达情况。所有患者均为女性，平均年龄63岁，所有病变均位于肺外周。在2例病例中，典型类癌伴有梭形细胞成分。所有神经内分泌增殖均为DNTF阳性。形态学（梭形细胞成分）、表型（DNTF表达）和临床病理（外周肿瘤、女性为主）的相似性表明，DIPNECH可能是外周类癌的癌前病变。
