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首页> 外文期刊>The Internet Journal of Pulmonary Medicine >PET avid lung mass in a neurofibromatosis patient: a case report
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PET avid lung mass in a neurofibromatosis patient: a case report

机译：神经纤维瘤病患者的PET狂热肺部肿块：一例报告

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The differential diagnosis of a lung mass in a neurofibromatosis patient includes benign or malignant neurogenic neoplasm. Benign neurogenic neoplasms can transform to malignant and since this transformation is a major cause of mortality, the prognosis will depend on early detection. Computed tomography scans, magnetic resonance imaging and positron emission tomography are not able to differentiate benign from malignant neurogenic neoplasm. Needle biopsy has poor sensitivity and malignant neurogenic neoplasm can be misinterpreted as benign. We address these issues in the case of a 48 year old man with neurofibromatosis type 1 who incidentally was found to have a right apex lung mass. The needle biopsy showed spindle cell lesion of probable neurogenic origin. Given the concern about the adequacy of needle biopsy in providing an accurate diagnosis, a positron emission tomography scan was done and it showed high uptake within the lesion. The mass was resected and confirmed as neurofibroma Introduction Neurofibromatosis is a disorder of neural crest cells such Schwann cells, melanocytes, and endoneurial fibroblasts. These cell types proliferate excessively throughout the body forming neoplasms(1). The neoplasms may be harmless or may cause serious damage by either compressing adjacent tissues or transforming into malignancy (2,3). Malignant neoplasms have a very high rate of local recurrence and tendency to metastasize. Therefore differentiating benign neurogenic neoplasms from malignant cases is crucial since early detection of malignancy improve the prognosis. Case presentation A 48-year-old gentleman was recently admitted to the hospital with decompensated congestive heart failure (CHF). His echocardiogram showed diastolic dysfunction and moderately severe pulmonary hypertension. As part of his work up for his pulmonary hypertension a CT scan of the chest pulmonary embolus protocol was done which showed no evidence of pulmonary embolism, but a right apex pleural based mass measuring 2.7 cm. The patient was not aware of any prior history of lung mass. After treatment for his CHF, the patient only complained of a mild degree of dyspnea on excretion which was noted as a chronic symptom that had been stable for many years. The patient denied any chest pain or cough, fever or chills. His past medical history was significant for neurofibromatosis type 1 (NF-1), morbid obesity, hypertension, hyperlipidemia, obstructive sleep apnea syndrome, chronic obstructive pulmonary disease (COPD), allergic rhinitis, seizure disorder, depression, and chronic back pain. His medication list included Oxcarbazepine, Gabapentin, Atenolol, Lisinopril, Citalopram, Trazodone, Montelukast, Cetirizine, Tiotropium inhalation, and Ibuprofen. He has no known drug allergies. He smoked in the past but had quit many years ago. There was no history of alcohol or illicit drug abuse. His family history was significant for neurofibromatosis on his paternal side. His father and his paternal grandmother as well as his son have neurofibromatosis.His physical exam was significant for multiple neurofibromatosis lesions, most pronounced on his left eyelid (Figure.1). There was one on left knee and multiple lesions over his anterior chest wall, his abdomen, and his neck. There were also multiple cafe au lait spots on his abdominal wall. Otherwise the rest of his physical exam was unremarkable.He underwent CT guided transthoracic needle biopsy (figure 2) which showed spindle cell lesion of probable neurogenic origin. Given the concern about the adequacy of the needle biopsy in providing an accurate diagnosis, a FDG PET scan was ordered which showed high uptake within the lesion with a Standard Uptake Value (SUV) of 7 ( figure 3). The patient was referred for thoracic surgery and had an open thoracotomy revealing a mass arising from apical posterior region of the chest wall. The pathology was neurofibroma.
机译：神经纤维瘤病患者的肺部肿块的鉴别诊断包括良性或恶性神经源性肿瘤。良性神经源性肿瘤可以转化为恶性肿瘤，并且由于这种转化是导致死亡的主要原因，因此预后取决于早期发现。计算机断层扫描，磁共振成像和正电子发射断层扫描无法区分良性和恶性神经源性肿瘤。针头活检的敏感性较差，恶性神经源性肿瘤可被误解为良性。我们针对一名48岁的1型神经纤维瘤病男子偶然发现了右尖部肺部肿块的情况，解决了这些问题。穿刺活检显示可能是神经起源的梭形细胞病变。考虑到对穿刺活检是否足以提供准确的诊断的担忧，进行了正电子发射断层扫描，结果显示病灶内摄取率很高。将该肿块切除并确认为神经纤维瘤简介神经纤维瘤病是神经of细胞（如许旺细胞，黑素细胞和神经内膜成纤维细胞）的疾病。这些细胞类型在体内过度增殖，形成肿瘤（1）。肿瘤可能是无害的，或者可能由于压迫邻近组织或转化为恶性肿瘤而引起严重损害（2,3）。恶性肿瘤具有很高的局部复发率和转移趋势。因此，区分良性神经源性肿瘤与恶性肿瘤至关重要，因为早期发现恶性肿瘤可改善预后。病例介绍一名48岁的绅士因失代偿性充血性心力衰竭（CHF）最近入院。他的超声心动图显示舒张功能障碍和中度重度肺动脉高压。作为他为肺动脉高压所做的工作的一部分，对胸部肺栓塞方案进行了CT扫描，未显示出肺栓塞的迹象，但右胸膜的肿块为2.7 cm。病人没有任何肺部肿块的病史。在接受CHF治疗后，患者仅抱怨轻度的呼吸困难，这被认为是多年来一直稳定的慢性症状。病人否认有胸痛或咳嗽，发烧或发冷。他过去的病史对1型神经纤维瘤病（NF-1），病态肥胖，高血压，高脂血症，阻塞性睡眠呼吸暂停综合症，慢性阻塞性肺疾病（COPD），过敏性鼻炎，癫痫发作，抑郁症和慢性背痛具有重要意义。他的药物清单包括奥卡西平，加巴喷丁，阿替洛尔，利西普利，西酞普兰，曲唑酮，孟鲁司特，西替利嗪，噻托溴铵吸入和布洛芬。他没有已知的药物过敏。他过去吸烟，但很多年前就戒烟了。没有酒精或非法药物滥用史。他的家族史对父亲一方的神经纤维瘤病具有重要意义。他的父亲和他的祖母以及他的儿子患有神经纤维瘤病。他的体格检查对多发性神经纤维瘤病具有重要意义，在他的左眼皮上最为明显（图1）。左膝盖上有一个，在他的前胸壁，腹部和脖子上有多个病变。他的腹壁上也有多个咖啡厅点。否则，他的其余身体检查就不会很明显。他接受了CT引导的经胸针穿刺活检（图2），显示可能是神经源性梭形细胞病变。考虑到对穿刺活检是否足以提供准确的诊断的担忧，我们订购了FDG PET扫描，结果显示病变内的摄取率很高，标准摄取值（SUV）为7（图3）。该患者被转诊接受胸外科手术，并进行了开胸手术，发现肿块是由胸壁的顶端后部区域引起的。病理为神经纤维瘤。

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