Synchronous Multiple Pituitary Neuroendocrine Tumors of Different Cell Lineages
Synchronous Multiple Pituitary Neuroendocrine Tumors of Different Cell Lineages
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DOI：
10.1007/s12022-018-9545-4
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发表时间：
2018-12-01
影响因子：
4.4
通讯作者：
Asa, Sylvia L.
中科院分区：
文献类型：
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作者：
Mete, Ozgur;Alshaikh, Omalkhaire M.;Asa, Sylvia L.
我们报告了一系列不同细胞系的同步多发性垂体神经内分泌肿瘤（PitNETs）的临床病理特征。对2001年至2016年的病理记录进行回顾性审查，从使用垂体细胞系转录因子，腺垂体激素和其他生物标志物分类的1055个PitNET中确定了13个同步多个PitNET。我们回顾了这些肿瘤的临床、影像学和组织病理学特征。该系列包括7名女性和6名男性。诊断时的平均年龄为55.23岁（范围36-73岁）。4名患者无法获得成像;在其他9名患者中，平均肿瘤大小为2.23 cm（范围0.9-3.9）。5例患者患有肢端肥大症，4例患有库欣病，4例患有临床无功能性肿瘤。12个有两个PitNET;一个有三个PitNET。最常见的肿瘤类型是促肾上腺皮质激素细胞（n=8; 6例密集颗粒细胞和1例稀疏颗粒细胞和1例Crooke细胞; 3例密集颗粒细胞和1例稀疏颗粒细胞临床无症状）、促性腺激素细胞肿瘤（n=8）和促生长激素细胞肿瘤（n = 8）（n=5; 4例稀疏颗粒状和1例密集颗粒状生长激素细胞），其次是催乳素细胞瘤（n=4;均为稀疏颗粒状）、低分化Pit-1谱系肿瘤（n=1）和不常见的多激素肿瘤（n=1）。一名患有库欣病的54岁男性患有MEN 1驱动的克鲁克细胞和促性腺激素细胞肿瘤。三重pitNET由多系多激素肿瘤与促性腺激素细胞和稀疏颗粒催乳细胞肿瘤。Ki 67（可从10个标本中获得）在个体肿瘤中的范围为1%至5%。分别有10名和11名患者接受了放射学和生化随访。在3例双PitNET患者中确定了放射学肿瘤持续性/复发，包括稀疏颗粒促乳细胞和促性腺细胞肿瘤（n=1），稀疏颗粒促生长细胞和无症状促肾上腺皮质细胞肿瘤（n=1），促性腺细胞和无症状促肾上腺皮质细胞肿瘤（n=1）伴海绵窦浸润。在4例双PitNET患者中观察到生化持久性，包括稀疏颗粒生长激素和沉默促肾上腺皮质激素肿瘤（n=2），促性腺激素和克鲁克细胞肿瘤（n=1），密集颗粒生长激素和沉默促肾上腺皮质激素肿瘤（n=1）。多个PitNET约占PitNET的1%，并且通常由于至少一种肿瘤成分而具有激素过量。临床表现可能是由于微量成分，特别是在库欣病患者。侵袭性生长和侵袭性组织学亚型预测疾病持续/复发。这一系列还强调了常规应用垂体细胞谱系转录因子沿着激素来区分和分型多种同步PitNET的重要性。
We report clinicopathological features of a large series of synchronous multiple pituitary neuroendocrine tumors (PitNETs) of different cell lineages. Retrospective review of pathology records from 2001 to 2016 identified 13 synchronous multiple PitNETs from 1055 PitNETs classified using pituitary cell-lineage transcription factors, adenohypohyseal hormones, and other biomarkers. Clinical, radiological, and histopathological features of these tumors were reviewed. The series included seven females and six males. Mean age at diagnosis was 55.23years (range 36-73). Imaging was unavailable for four patients; among the other nine, mean tumor size was 2.23cm (range 0.9-3.9). Five patients had acromegaly, four had Cushing disease, and four had clinically non-functional tumors. Twelve had double PitNETs; one had a triple PitNET. The most common tumor type was corticotroph (n=8; six densely and one sparsely granulated and one Crooke cell; three densely and one sparsely granulated were clinically silent), gonadotroph tumors (n=8), and somatotroph tumors (n=5; four sparsely granulated and one densely granulated somatotroph) were followed by lactotroph tumors (n=4; all sparsely granulated), poorly differentiated Pit-1 lineage tumor (n=1), and unusual plurihormonal tumor (n=1). A 54-year-old man with Cushing disease had MEN1-driven Crooke cell and gonadotroph tumors. The triple pitNET consisted of a multilineage plurihormonal tumor associated with a gonadotroph and a sparsely granulated lactotroph tumor. The Ki67 (available from 10 specimens) ranged from 1 to 5% in individual tumors. Radiological and biochemical follow-up was available for 10 and 11 patients, respectively. Radiological tumor persistence/recurrence was identified in three patients with double PitNETs consisting of sparsely granulated lactotroph and gonadotroph tumors (n=1), sparsely granulated somatotroph and silent corticotroph tumors (n=1), and gonadotroph and silent corticotroph tumors (n=1) with cavernous sinus invasion. Biochemical persistence was noted in four patients with double PitNETs consisting of sparsely granulated somatotroph and silent corticotroph tumors (n=2), gonadotroph and Crooke cell tumors (n=1), and densely granulated somatotroph and silent corticotroph tumors (n=1). Multiple PitNETs represent about 1% of PitNETs and usually have hormone excess due to at least one tumor component. Clinical manifestations may be due to the minor component, especially in patients with Cushing disease. Invasive growth and aggressive histological subtypes predicted disease persistence/recurrence. This series also highlights the importance of routine application of pituitary cell lineage transcription factors along with hormones to distinguish and subtype multiple synchronous PitNETs.