Kukkar Sandeep Ramesh, Panchal Harsha, Anand Asha
Department of Medical and Paediatric Oncology, Gujarat Cancer and Research Institute, Ahmedabad, Gujarat, India.
Indian J Cancer. 2017 Jul-Sep;54(3):556-559. doi: 10.4103/ijc.IJC_295_17.
Primary neuroendocrine tumors of the thymus are unusual anterior mediastinal tumors with a variable prognosis. A retrospective analysis of five patients with primary neuroendocrine tumors of the thymus admitted to the Gujarat Cancer and Research Institute, Ahmedabad, between 2012 and 2016, was done to study the clinical profile and outcome of these patients. The role of various prognostic factors such as surgical resection, histological grade, and Masaoka-Koga staging was also analyzed. Majority of the patients present with signs and symptoms related to a rapidly expanding mediastinal mass, such as breathlessness, facial puffiness, edema over the neck and extremities, chest pain, and other features of superior vena cava (SVC) syndrome. Collateral venous dilatation over the neck and chest and edema over neck were the most common physical signs. All the patients enrolled in the study presented in advanced stages with a poor differentiation on histopathological examination. Thymic neuroendocrine tumors usually manifest as large, lobulated, and locally invasive anterior mediastinal masses surrounding the great vessels of the neck and thorax. None of the diagnosed patients underwent surgical resection in view of extensive vascular encasement in the neck and thorax, and all of them were started on platinum-based palliative chemotherapy. The median survival of the patients was 12 months with the longest survival of 16 months for one patient. Possibility of this potentially rare entity should be kept in mind when a patient presents with features of SVC syndrome and large mediastinal mass. Complete surgical resection of the tumor is prognostic of improved treatment outcome and long-term survival. Large tumor size could be a determinant of poor overall outcome, and tumor size should strongly be considered as a factor in the revised (NETT) Neuroendocrine tumours of thymus staging. Histological grade and Masaoka-Koga stage are the important prognostic factors, but this study emphasizes the utmost need to further validate the prognostic factors.
胸腺原发性神经内分泌肿瘤是不常见的前纵隔肿瘤，预后各异。对2012年至2016年间入住艾哈迈达巴德古吉拉特癌症与研究所的5例胸腺原发性神经内分泌肿瘤患者进行了回顾性分析，以研究这些患者的临床特征和预后。还分析了手术切除、组织学分级和Masaoka-Koga分期等各种预后因素的作用。大多数患者表现出与迅速增大的纵隔肿块相关的体征和症状，如呼吸困难、面部肿胀、颈部和四肢水肿、胸痛以及上腔静脉综合征的其他特征。颈部和胸部的侧支静脉扩张以及颈部水肿是最常见的体征。纳入研究的所有患者均处于晚期，组织病理学检查显示分化不良。胸腺神经内分泌肿瘤通常表现为围绕颈部和胸部大血管的大的、分叶状且局部浸润的前纵隔肿块。鉴于颈部和胸部广泛的血管包绕，所有确诊患者均未接受手术切除，全部开始接受铂类姑息化疗。患者的中位生存期为12个月，其中1例患者生存期最长，为16个月。当患者出现上腔静脉综合征和大纵隔肿块的特征时，应考虑到这种潜在罕见疾病的可能性。肿瘤的完整手术切除对改善治疗效果和长期生存具有预后意义。肿瘤体积大可能是总体预后不良的决定因素，在修订的胸腺神经内分泌肿瘤（NETT）分期中，肿瘤大小应被视为一个重要因素。组织学分级和Masaoka-Koga分期是重要预后因素，但本研究强调迫切需要进一步验证这些预后因素。
