PDF(Pubmed)
Abstract：
We present an interesting case of mediastinal small cell carcinoma (MSCC), an exceedingly rare entity, comorbid with idiopathic pulmonary fibrosis (IPF). A 66-year-old female was first seen in the pulmonology office for abnormal chest computed tomography (CT) findings of right apical bronchiectasis and subpleural fibrotic changes with focal pleural thickening along the fissures, along with a right lower lobe nodule. Pulmonary function testing (PFT) showed an obstructive pattern with modest bronchodilator response, although subsequent PFT showed a worsening restrictive pattern with a worsening DLCO. On a follow-up CT one year later, a soft tissue density with peripheral calcification was found in the anterior mediastinum, later found to be hypermetabolic on a PET scan. Radiographically, fibrosis worsened with the appearance of worsening diffuse bilateral coarse reticular interstitial changes with lower lobe predominance, honeycombing, and areas of ground-glass opacity. A biopsy of the mediastinal lesion showed a high-grade neuroendocrine tumor. Cam5.2, insulinoma-associated protein-1, synaptophysin, and thyroid transcription factor-1 immunostains were positive. She underwent four cycles of chemotherapy with cisplatin and etoposide with a total of 60 Gy of radiation. Mediastinal mass started to decrease in size. Her respiratory status, imaging, and PFTs continued to show evidence of IPF progression. Prednisone resulted in modest clinical and radiographic response. Steroid-sparing therapy with mycophenolate mofetil, although effective, had to be discontinued due to GI bleeding. Anti-fibrotic therapy was deferred due to evidence showing a lack of clinical improvement. We discuss the existing evidence available on IPF management and proceed to highlight the deficiencies in existing data available on the management of IPF and MSCC in these patients. Most of the cases of MSCC reported in the past have managed MSCC using guidance from treatment practices for small cell lung cancer. No reported cases discuss or describe the management of IPF and MSCC in the very rare cohort of patients our case represents.
摘要：
我们提出了一个有趣的纵隔小细胞癌（MSCC）的病例，一个极其罕见的实体，合并特发性肺纤维化（IPF）。首次在肺科发现一名66岁的女性，原因是胸部计算机断层扫描（CT）异常发现右根尖支气管扩张和胸膜下纤维化改变，并伴有沿裂隙的局灶性胸膜增厚，连同右下叶结节。肺功能测试（PFT）显示阻塞性模式与适度的支气管扩张剂反应，尽管随后的PFT显示限制性模式恶化，DLCO恶化。一年后的CT随访中，在前纵隔发现软组织密度伴周围钙化，后来在PET扫描中发现是高代谢。射线照相,纤维化随着弥漫性双侧粗网状间质改变的恶化而恶化，下叶占优势，蜂窝,和毛玻璃不透明的区域。纵隔病变的活检显示高度神经内分泌肿瘤。Cam5.2，胰岛素瘤相关蛋白-1，突触素，甲状腺转录因子-1免疫染色阳性。她接受了四个周期的顺铂和依托泊苷化疗，总共60Gy放疗。纵隔肿块开始减小。她的呼吸状况,成像,和PFTs继续显示IPF进展的证据。泼尼松导致适度的临床和影像学反应。霉酚酸酯保留类固醇治疗，虽然有效,因为消化道出血不得不停药.由于证据显示缺乏临床改善，因此推迟了抗纤维化治疗。我们讨论了IPF管理的现有证据，并继续强调这些患者IPF和MSCC管理的现有数据的不足。过去报道的大多数MSCC病例都使用小细胞肺癌治疗实践的指导来管理MSCC。在我们的病例代表的非常罕见的患者队列中，没有报告病例讨论或描述IPF和MSCC的管理。
