A 27-years-old married female patient presented with recurrent coughing and hemoptysis for more than ten years first came to our hospital in January 2019.According to the medical history information provided ,the patient was previously diagnosed as pneumonia and treated symptomatically in local clinical institution.The symptoms of patient were once relieved after the treatment, but recurrent frequently. The patient has no family history of primary lung malignancy and genetic disease and was 155 cm in height and 42 kg in weight with a lean body shape, symmetrical thorax without deformity. The trachea of the patient deviated ot the right slightly and there was no obvious rhonchi and moist rale as well as wheezing rale were heard.The heart rhythm was regular, the auscultation area of heart sound deviated to the right thorax obviously and no obvious abnormality was found in abdominal physical examination.
A chest computed tomography was performed for the patient and showed a giant abnormal space-occupying lesion in the hilum of right lung. The tumor was closely related to the right pulmonary artery and bronchus(Fig. 1) .The right lung was atelectasis and some of the left lung as well as heart were obviously deviated to the right thoracic cavity. A Chest MRI showed a mass abnormal signal tumor in the region of right hilum about 10.0 × 4.5 cm in size. The T1WI was isointense, while T2WI and DWI were both hyperintense.A 3D reconstruction of the hilar structures have shown a complete anatomical disorganization of right pulmonary artery and vein(Fig. 2).A neoplasm with smooth surface was observed in the right main bronchus by bronchoscopy examination, but a biopsy was not performed.Pulmonary function showed a severe obstructive mixed ventilation dysfunction. The forced expiratory volume in 1 second (FEV1)was 1.36L, accounting for 45% of the predicted value, and maximal voluntary ventilation(MVV) was 27.63L, accounting for 43% of the predicted value. The renal function, electrolyte, coagulation function, arterial blood gas analysis were all normal.Because of the continuous hemoptysis for quite a long time,the patient's blood routine examination showed a moderate anemia and hemoglobin was 69 g/L, biochemical examination showed moderate malnutrition and prealbumin was only 85 g/L, albumin was 31 g/L.In this patient,CA125 was 434.77u/ml, CEA was 12.43 ng/ml, CA724 was 233.3 u/ml, while CA242, CA199, AFP, SCCA, NSE were all normal.No sign of abnormality or metastasis was found in enhanced MRI of brain and bone scan.No abnormal and enlarged lymph nodes were observed in neck and supraclavicular region by ultrasound examination.
We diagnosed the patient with a huge tumor in the right lung and firstly considered a special type of malignancy.In view of the patient's personal willingness and equipment limitations, a preoperative biopsy of the tumor was not performed.After discussion with a multidisciplinary team which included thoracic surgeons, radiologists, and respiratory physicians, we decided to perform a surgical treatment for the patient with her permission.
A thoracotomy was performed under general anesthesia with left lung ventilation. The patient was in left 90°lateral position, a 20 centimeters long posterolateral incision at the 5th intercostal space was made overlying the right chest wall.Surgical exploration revealed a complete atelectasis of the right lung with obvious consolidation in lung tissue.The lingual segment and anterior segment of left upper lobe herniated into right upper thoracic cavity while the heart of patient also deviated into right lower thoracic cavity obviously.The lymph nodes of each group in mediastinum were checked in the operation and no significantly enlarged lymph nodes were observed.Because of the size and invasion of the tumor, a right pneumonectomy and mediastinal lymph nodes dissection was initially considered before surgery.The right main bronchus was significantly thicker than normal and the outer diameter of the right main bronchus was 2.4 cm. The bronchial arteries around the bronchus were extermely twisted and dilated. The upper lobe of the right lung was completely consolidated into a mass, the middle and lower lobes of the right lung were atelectatic.Subsequently, we performed a right pneumonectomy for this patient.
We check the surgical specimens and found that the tumor originated from the right main bronchus, about 1 cm away from the tracheal carina, grew distally along the lumen of the bronchus and completely blocked the lumen. The tumor was about 8.6 × 4.5 × 4.4 cm in size(Fig. 3) and pathological diagnosis showed a right main bronchus malignant tumor.The morphology of tumor cell under microscope demonstrate a primary acinic cell carcinoma of right lung.Three lymph nodes(LNs) in group 2 and 4, two LNs in group 7, three LNs in group 9 and one LN in group 10 were dissected and no tumor metastasis was found in the above lymph nodes. Immunohistochemistry of the tumor showed AE1/AE3(+), Ki-67(2%+), K7(+), Calponin(+/-), Vimentin(+), CK19(+), a-ACT(+), PAS(partial+). While CD56, S-100, P63, TTF-1, CDX2, CK5/6, SYN, Dog-1, SOX-10, Mammaglobin were all negative. (Fig. 4).
The patient complained of dyspnea on the second day after opeartion. Some significant inspiratory wheezing sounds were heard on retrosternal auscultation.The patient had no previous history of asthma and was unresponsive to treatment with bronchodilator.A Chest computed tomography then was performed and we were unexpected to find that the heart and mediastinum further deviated into the right thoracic cavity after right pneumonectomy. Because of the gravity of the heart, when the patient lay flat the left main bronchus was clamped between the mediastinum and the thoracic vertebra which resulting in an obvious compression on the left main bronchus(Fig. 5).The narrowest region of airway stenosis of the left main bronchus was only 3 mm.
Then we tried to turn the patient to left semi-prone and prone position to relieve the compression of the left main bronchus, and the patient's symptoms were partially relieved after changing body position.So we believe that some methods should be taken to reposit the patient's heart and mediastinum back to the left which may help to alleviate the patient's symptoms.After 600 ml of air was injected into the right thoracic cavity of the patient through drainage then clamped drainage tube,the symptom of the patient was partially relieved which effect is similar to the changing of body position.Over the next few days, we kept the patient's drainage clamped so that exudate and gas in the right thoracic cavity could alleviate the deviation of mediastinum.After clamping the drainage several days and confirming the symptoms did not recur, we removed the drainage on the 8th day after surgery and the patient discharged on the 14th day postoperatively.
At present, the patient has been followed up for 12 months after surgery. and there has been no evidence of recurrence and metastasis observed.All the clinical indexes and physical condition of the patient are normal.