PMC6313888_01
Primary tumor in the posterior mediastinum. (A) Enhanced CT scan showed a faintly enhanced solid mass, which was 7 cm in diameter and was surrounded by the trachea, aorta and thoracic vertebrae. (B and C) MRI showed heterogeneous T1-hypointesnse and T2-hyperintense signal tumors invaded into 6th and 7th thoracic vertebral columns. Ao, aorta; Br, bronchi; Es, esophagus; LA, left atrium; PA, pulmonary artery; Tr, trachea.
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Cypra at 3.1 ng/ml and neuron specific enolase (NSE) at 17.3 ng/ml, others were normal, such as carcinoembryonic antigen (CEA) at 0.8 ng/ml, CA19-9 at 5.7 U/ml, alpha fetoprotein at 4 ng/ml, and interleukin-2 receptor at 259 U/ml. Computed tomography (CT) showed a faintly enhanced solid mass, 7 cm in diameter, that was surrounded by the trachea, aorta, esophagus and thoracic vertebrae (Fig. 1).
The primary tumor sampled by endobronchial ultrasound fine-needle aspiration. (A) Epithelial and spindle-shaped tumor cells showed alveolar growth solid. (B) Immunohistochemical examination showed positive for c-kit. Magnification, x200.
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Sequential CT scans showing change in size and numbers of lung metastases but not change in size of the primary tumor (arrow). The volume of bloody pulmonary effusion was increased. (A and B) after radiation; (C and D) after the second course of sunitinib; and (E and F) 2 weeks after imatinib.
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Although the primary tumor was unchanged after 2 courses of sunitinib, the lung metastases were enlarged and there was a bloody pleural effusion that increased (Fig. 3).
Histological features of metastasized lung tumors (A-C) and adrenal glands (D and E) by autopsy. (A-C) The majority of tumor cells showed a rounded and middle size of cells. There were small amounts of large and pleomorphic cells or mucous cells (magnification, x200). (D and E) Adrenomedullary hyperplasia expanded diffusely (E, right adrenal gland) and invaded into the adrenal cortex (D, left adrenal gland). Magnification, x20.
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The majority of tumor cells showed rounded, mid-size cells (Fig. 4A-C). In addition, histological examination showed bilateral adrenomedullary hyperplasia, which diffusely expanded adrenal medulla and invaded partially into the adrenal cortex (Fig. 4D and E).
Immunohistochemical features of the metastasized tumors (A-H) and control of liver (I). (A) c-kit, (B) NSE, (C) neurofilament, (D) symaptophysin, (E) ki67, (F) p53 and (G-I) SDHB. Magnification, x200. NSE, neuron specific enolase; SDHB, succinate dehydrogenase B.
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Immunohistochemical examination showed that the tumor cells were positive for Ki67 and p53 (Fig. 5, Table I). c-kit was positive in less than 1% of tumor cells, with no staining for either DOG1 or CD34. Although tumor cells in low-density nests expressed succinate dehydrogenase (SDH)-B, those in high-density areas did not show much expression (Fig. 5).