Primary intrapulmonary thymomas (PITs) are uncommon lung tumors. To the best of our knowledge, 32 cases of PITs have been reported in the English and Japanese literature since 1951, not counting our cases [2, 4–11].
Here, we present a summary of the clinicopathologic features of all 34 cases. The patients’ ages ranged from 14 to 79 years, and half the patients were females. The site of occurrence was the right pulmonary lobe in 21 cases, the left lobe in 12 cases, unknown in 1 case, the upper lobe in 22 cases, and the lower lobe in 11 cases. Three cases, including our second case 2, were multifocal within the same lung. The sizes of the lesions ranged from 9 to 128 mm in diameter. The presenting symptoms of PITs have included chest pain, cough, and hemoptysis, which also occur in patients with lung cancer, but most lesions were discovered incidentally on radiographic examination in asymptomatic individuals. Based on the WHO classification , there were 7 PIT type A cases, 8 type AB, 6 type B1, 5 type B2, and 7 type B3; 1 case was not classified. There were 3 patients with PIT and myasthenia gravis, and 2 patients with PIT and immunodeficiency, which is known as Good syndrome.
PIT appears on CT as a well-circumscribed, heterogeneous mass, such as seen in our patients. The differential radiologic diagnosis of PIT includes low-grade malignant tumor, metastatic lung tumor, hamartoma, sclerosing hemangioma, lipoma, and angiolipoma. Performing a preoperative diagnosis is difficult, and most PITs are diagnosed postoperatively .
Histological features of the tumor showed that the lesion was composed of dense proliferation of spindle cells with a minority arranged in pseudoglands and a cystic lesion in case 1, and there were lobulated lesions with vascular septa, consisted of epithelial cells palisading along the septa and centrally located small lymphocytes in case 2. The differential diagnosis of PIT includes a wide range lymphoma and primary or metastatic carcinoma of the lung. Immunohistochemical staining is helpful in this differentiation. Epithelial cells are stained by AE1/AE3 or EMA, and lymphocytes in thymomas are stained by T cell phenotypes . Epithelial cell-dominant thymoma shares the same immunoreactivity for AE1/AE3 or EMA as metastatic carcinoma or poorly differentiated carcinomas of the lung, but PIT appeared as well-circumscribed lesions, with low mitotic activity and minimal atypia. The antigen CD20 is also helpful for diagnosis of type A thymoma because CD20-positive B cells are found if focal micronodular areas with a lymphoid stroma is present . In our case, these tumors were diagnosed type A thymoma and type B2 thymoma in the WHO classification, respectively. Immunohistochemical staining combined with gross and microscopic pattern of the tumor and patient’s clinical history is helpful in differentiating a PIT from any other intrathoracic neoplasm .
FDG-PET can be applied to the diagnosis of malignant neoplasms because it is designed to detect and assess the degree of carbohydrate metabolism in malignant tissue . The PITs of our two patients had different histopathologic manifestations and FDG-PET findings. The SUVmax values of the lesions of case 2 were markedly higher than the value of the lesion of case 1. It has been reported that FDG accumulation was highly correlated with WHO histologic subtypes of thymic epithelial tumors [14, 15]. Notably, the degree of FDG uptake has been positively correlated with the grade of malignancy of PITs as with the case of thymic epithelial tumors.
Most case reports of PIT have described the tumors as slow-growing, indolent, and asymptomatic until large enough to cause localized effects such as pain and bronchial obstruction . The best therapy for PIT is complete resection, which for these tumors leads to good outcomes [6, 8, 10]. In their systematic review of 25 cases of PIT, Myers et al.  reported that the survival of surgically managed patients was significantly better than the survival of conservatively managed patients (log-rank test, P = 0.039). Histologic subtype (WHO classification) and tumor size did not significantly affect survival. Myasthenia gravis and the Good syndrome significantly decreased survival (log-rank test, P = 0.039). Because complete surgical resection was obtained for both of our cases, we believe that their postoperative outlook is good.