A 38-year-old female presented to the general department with a history of intermittent right upper abdominal pain, back pain, nausea and vomiting for 18 months. The patient is a college teacher with good living habits and no history of addiction, smoking or family disease. The physical examination was normal. The symptoms did not decrease after the oral administration of an acid-inhibitory drug and cholagogue at the local hospital.
Ultrasound of the abdomen showed a retroperitoneal tumour (4.8*2.3 cm) (Fig. 1). To further confirm the diagnosis, contrast-enhanced computed tomography (CT) was performed and showed a right retroperitoneal tumour (3.4*2.7 cm) extending to the renal veins and into the partially patent IVC lumen, and it was pushing the pancreas forward. There were no enlarged lymph nodes around the tumour (Fig. 2). To determine the primary site of the tumour and whether metastasis had occurred, we performed head magnetic resonance imaging (MRI), chest CT and cardiac ultrasound. The patient refuses to undergo PET-CT examination in order to save money. The cardiac ultrasound and brain MRI results were normal. The CT scan of the chest showed a tumour in the lingual segment of the upper lobe of the left lung, consisting of ground-glass nodules with unclear boundaries; the tumour was approximately 1.3 cm×1.1cm in size and had multiple short burls on the edges, and there were no enlarged lymph nodes in the mediastinum (Fig. 3). Bronchoscopy was performed to clarify the nature of the tumour, showing acute inflammation of the bronchial mucosa. We considered lung nodules as metastases from the IVC tumour, primary lung cancer or benign lung nodules. Therefore, the patient was discussed in multidisciplinary tumor board for management. The results that Inferior vena cava sarcoma without effective treatment in addition to surgery. We can be conducted first resection of the inferior vena cava sarcoma in our department without preoperative pulmonary biopsy, and then resection of pulmonary tumour by thoracoscope after recovery from the first operation.
A full-length right upper rectus abdominus incision was initially performed. The patient underwent resection of the sarcoma of the inferior vena cava, which was located at the confluence of the renal vein into the inferior vena cava. In order to save operation time, the inferior vena cava was reconstructed with artificial blood vessels. During the surgical procedure, there were no enlarged lymph nodes in the abdominal cavity and no metastatic foci in the abdominal cavity or pelvis. The lateral retroperitoneum of the duodenum was cut, and the duodenum was pulled up to the left. A solid and prominent tumour (6*4*3 cm) was located in the middle segment, including the anterolateral wall of the vena cava, as well as the renal veins. Vascular clamps were used to clamp the proximal and distal portions of the IVC tumours, renal arteries and renal veins, and then complete removal of the tumours was performed. Proximal and distal to the IVC, the renal veins were anastomosed with the artificial vessel by 5 − 0 Prolene sutures. The vascular clamp was opened to restore blood flow after vascular anastomosis, and no active bleeding was detected. Bleed loss about 400 ml during the operation. There were no obvious complications and without intensive care unit after the operation. The patient was discharged from the hospital on the 13th day after the operation.
The postoperative pathological specimens showed that the tumour was 6×4×3 cm in size, lobulated, and had an intact capsule, and there was no metastasis in the surrounding lymph nodes. The histopathological examination of the excised mass showed hypercellular mesenchymal tumours that were arranged in fascicles, bundles and interlacing patterns (Fig. 4). The tumour cells were spindle-shaped and elongated, with abundant cytoplasm. The nuclei were hyperchromatic, blunt-ended, centrally located and had some degree of atypia and pleomorphism (Fig. 5). A few fused nuclei were also seen. Immunohistochemical staining showed positivity for smooth muscle actin (SMA), myoglobin, desmin, Epithelial Membrane Antigen (EMA), Transducin-Like Enhancer of split 1 (TLE-1) and h-caldesmon; Ki-67 showed 40% positivity, and vimentin and CD99 were focally positive; and CD 117, CKp, CK7, Bcl-2, S100, CD31, CD34, Dog-1 and STAT-6 were negative. These findings were suggestive of IVC leiomyosarcoma (Fig. 5).
After one month, the patient underwent thoracoscopic wedge-shaped pneumonectomy. There was a mass less than 1 cm in diameter in the anterior segment of the left upper lobe after entering the pleural cavity. No obvious pleural contraction or lymph node enlargement was seen in the hilum or mediastinum. The lung tissue, including the tubercle in the left upper lung, was removed more than 2 cm from the edge of the tubercle with a linear cutting closure device. There were no obvious blood loss and complications during or after the operation. The patient was discharged from the hospital 4 days after thoracoscopic wedge-shaped partial pneumonectomy.
The postoperative pathological specimens showed lung tissue with a size of approximately 5×4×2 cm, and a tumour with a diameter of 0.8 cm was seen in the centre. The resection margin was larger than 2 cm from the tumour, and the mediastinal lymph nodes were not dissected. The histopathological examination of the excised mass showed lung adenocarcinoma (Fig. 6). Immunohistochemical staining showed positivity for CK7, napsin A and TTF-1, Ki-67 showed 20% positivity, and CK5/6, p63, p40, Syn, CgA, CD56 and LCA were negative. CK7, napsin A and TTF-1 were positive in lung adenocarcinoma, while P63 and CK5/6 were positive in lung squamous cell carcinoma. Additional, the lung tissue has carried on the consultations to assist in the diagnosis in the cancer hospital of Fudan University and Gansu provincial cancer hospital, and experts from two hospitals pathology agreed with the diagnosis of lung adenocarcinoma. The patient received Gefitinib Tablets treatment 250 mg per day for 18 months during the perioperative period. We required the patient to return to the hospital every 6 months for CT scans of the lung and abdomen, ultrasound scans of the abdomen, and tests for blood tumour markers. Followed up to 18 months after thoracoscopic wedge-shaped partial pneumonectomy, there have two lump about 1.2 cm and 0.7 cm in diameter in upper lobe and lower lobe of the right lung by contrast-enhanced CT images(Fig. 7). According to the examination results, it was suggested that the metastasis of inferior vena cava sarcoma or the recurrence of lung adenocarcinoma, and thoracoscopic resection was decided after the discussion by thoracic surgery department. The immunohistochemical results of postoperative specimens were consistent with the metastasis of inferior vena cava sarcoma(Fig. 8). Immunohistochemical staining showed that SMA, desmin, vimentin and h-caldesmon and Ki-67 showed 70% were positive; CD117, S100, CD34, and Dog-1 were negative. The histopathological examination of the right lung mass and the discussion of the pathological expert group suggested that it was the metastasis of inferior vena cava sarcoma. The patient received daily 12 mg treatment with Anlotinib Hydrochloride Capsules after the last operation. Regular outpatient follow-up showed that there was no obvious recurrence and metastasis as of April 2021 (6 months after operation).