Huwer H, Kalweit G, Schäfer H, Eltze E
Klinik für Thoraxchirurgie, Lungenzentrum Saar, Völklingen.
Pneumologie. 2011 Dec;65(12):742-4. doi: 10.1055/s-0031-1286635. Epub 2011 Nov 2.
Pulmonary paraganglioma is a very rare condition with 40 cases reported in the literature. In the vast majority of cases the correct diagnosis could not be yielded preoperatively.
We report a case of paraganglioma of the lung. Computed tomographic scan showed a solitary pulmonary nodule. Diagnostic thoracotomy was performed and a tumor in the left lower lobe was resected. Frozen section evaluation showed an epithelial tumor with neuroendocrine differentiation and low grade features. Accordingly, lobectomy was performed. The study of the paraffin-embedded specimen yielded furthermore a neuroendocrine differentiated tumor, but mitotic figures were rare. Immunhistochemically the final diagnosis paraganglioma was made.
In patients with pulmonary paraganglioma, the correct preoperative diagnosis is in general not available. Solitary pulmonary nodules or minor tumors of unknown histology are resected by wedge resection and sent to frozen section evaluation. Frozen section evaluation results in the diagnosis neuroendocrine tumor with more or less mitoses and mostly specified as carcinoid tumor. According to the literature biologic behaviour of carcinoid tumor and pulmonary paraganglioma is similar and thus the incorrect result of frozen section evaluation leads to a correct resection mode. If frozen section evaluation shows low grade features, surgical overtreatment may occur.
肺副神经节瘤是一种非常罕见的疾病，文献中仅报道了40例。在绝大多数病例中，术前无法做出正确诊断。
我们报告一例肺副神经节瘤病例。计算机断层扫描显示一个孤立性肺结节。进行了诊断性开胸手术，切除了左下叶的一个肿瘤。冰冻切片评估显示为具有神经内分泌分化和低级别特征的上皮性肿瘤。因此，进行了肺叶切除术。对石蜡包埋标本的研究进一步证实为神经内分泌分化肿瘤，但有丝分裂象罕见。免疫组化最终诊断为副神经节瘤。
对于肺副神经节瘤患者，一般无法在术前做出正确诊断。孤立性肺结节或组织学不明的小肿瘤通过楔形切除术切除并送冰冻切片评估。冰冻切片评估结果为或多或少有有丝分裂象的神经内分泌肿瘤，大多诊断为类癌肿瘤。根据文献，类癌肿瘤和肺副神经节瘤的生物学行为相似，因此冰冻切片评估的错误结果导致了正确的切除方式。如果冰冻切片评估显示低级别特征，可能会出现手术过度治疗的情况。
