Clamshell Approach for Growing Teratoma Syndrome

The patient was a 21-year-old man with a history of worsening cough, chest pain, and increasing chest wall asymmetry during the previous six months. Imaging demonstrated a large heterogeneous mass of the left hemithorax, causing compression of the heart, pulmonary artery, left lung, and displacing the mediastinum to the right. Alpha-fetoprotein and beta-HCG were increased. CT scan confirmed the absence of other masses or enlarged lymph nodes and a testicular ultrasound was normal. Fine needle aspiration demonstrated teratocarcinoma.

The patient underwent four cycles of chemotherapy. Following chemotherapy, serum markers were normalized. However, the mass did not shrink and symptoms worsened. In fact, chest CT demonstrated a further increase in size. The authors suspected a case of growing teratoma syndrome and the patient was evaluated for surgery. Clamshell incision was the chosen approach. The patient preparation included a thoracic epidural catheter and a double lumen endotracheal tube, placed to allow selective one-lung ventilation. The patient was prepared for cardiopulmonary bypass. Transesophageal echocardiogram was positioned as well as jugular central venous cannula. The femoral artery and vein were isolated. The patient was lying in a supine position with arms abducted. The anterior chest was prepped and draped in sterile fashion, leaving the midaxillary lines free on both sides. The clamshell incision allows for the exposure of the mediastinum and both pleural spaces. Median sternotomy was not chosen due to the potential involvement of left PA and veins. The tumor was too large and anterior for a posterolateral thoracotomy, since it could put the patient at risk of mediastinal compression during positioning and manipulation. A bilateral submammary incision was made, extending from one midaxillary line to the opposite. The pectoralis major was separated from its inferior attachments. Laterally, division of the serratus was required to allow for wider exposure. Intercostal muscles were divided, giving access to the fourth intercostal space. After opening the bilateral intercostal spaces, the internal mammary vessels were isolated and ligated. A substernal space was obtained by blunt finger dissection. The sternum was cut with a transverse incision using a sternal saw. A Sellor rib spreader was placed on either side. Cautious opening of the retractors exposed both pleural cavities and the mediastinum. Development of a dissection plane, anterior to the pericardium, was achieved by a combination of sharp, blunt finger dissection and cautery. Circumferential dissection of all adhesions was performed and the entire mass was gradually exposed. Care must be taken to both achieve en bloc resection and avoid inadvertent breach of the capsule during manipulation. The left superior pulmonary vein was encircled to ensure its control in case of lung involvement. The underlying left lung was freed of the compressing mass, which was a 2500 g tumor measuring 23 x 16 x 15 cm. A small area of consolidation of the superior lobe was resected because it appeared solid and covered with fibrotic pleura. Double lung ventilation was restored, with satisfactory reinflation of left lung parenchyma.

Prior to closure, four 28F chest drains were inserted. Three PDS Cord pericostal sutures were placed on either side to approximate the intercostal space, and two were used to close the sternum. The serratus anterior and pectoralis major muscles were approximated with number 1 Vycril. The subcutaneous tissue layer was closed using 2-0 Vycril. The skin was closed with subcuticular suture using 4-0 Monosyn. The postoperative period was uneventful. Pain was controlled with an epidural catheter which was removed on postoperative day five. Postoperative chest X-ray demonstrated fully expanded lungs. After ten days, he was transferred to a Pulmonary Rehabilitation Center where he stayed during the following three weeks. Final pathology report demonstrated a mature teratoma. Resected parenchymal wedge was a pulmonary hemorrhagic infarction. Follow-up at three months revealed complete resolution of symptoms. The surgical wound healed well. Seven months after resection, he is alive and well, with no signs of recurrence. Growing teratoma syndrome describes the phenomenon by which germ cell tumors enlarge after chemotherapy, despite normalization of serum markers and complete eradication of malignant cells.

Surgical management of these tumors is challenging. However, despite their massive size, successful resection can be achieved.

References

1. Kesler KA, Patel JB, Kruter LE, Birdas TJ, Rieger KM, Okereke IC, et al. The “growing teratoma syndrome” in primary mediastinal nonseminomatous germ cell tumors: Criteria based on current practice. J Thorac Cardiovasc Surg. 2012 Aug;144(2):438–443.
2. Afifi HY, Bosl GJ, Burt ME. Mediastinal growing teratoma syndrome. Ann Thorac Surg. 1997 Aug 1;64(2):359–362.
3. Agatsuma T, Koizumi T, Kubo K, Saito G, Kondo R, Yoshida K, et al. Mediastinal growing teratoma syndrome succesfully Tteated by multiple modality therapies. Intern Med. 2011;50(6):607–610.
4. Sachdeva AK, Penumadu P, Kohli P, Dubashi B, Munuswamy H. Growing teratoma syndrome in primary mediastinal germ cell tumor: our experience. Asian Cardiovasc Thorac Ann. 2019 Feb 1;27(2):98–104.

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