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This video highlights the case of a 55-year-old female who presented with facial swelling, breathlessness -NYHA class 3 and cough for 15 days. She had a known case of hypertension and a cerebrovascular accident (CVA) in the past, though she recovered without any neurological deficit. She required diuretics and noninvasive ventilation (NIV) support on admission.
A computed tomography (CT) chest scan showed bilateral pleural effusion (right >left), a large lobulated mediastinal mass measuring 5.6 x 5.7 x 4.4 cms extending from the superior mediastinum to the subcarinal region. Eythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were raised. A peripheral smear showed mild neutrophilic leucocytosis and the antinuclear antibodies (ANAs) profile was negative. Complement C3, C4, Alpha fetoprotein, LDH, and serum beta HCG were normal. Pleural fluid was negative for malignancy and acid-fast bacillus (AFB).
A CT-guided biopsy showed metastatic deposits of signet ring cell adeno carcinoma to the subcarinal node. An endobronchial ultrasound-guided fine needle biopsy from the subcarinal lymph node showed atypical cells with some signet cell appearance favoring metastasis. A medical oncology opinion was sought, and 10 cycles of paclitaxel and carboplatin chemotherapy were given.
A repeat positron emission tomography (PET) scan showed a large, ill-defined heterogeneously enhancing conglomerated soft tissue density mass involving the pretracheal, precarinal, and subcarinal regions encasing the right main pulmonary artery closely abutting the superior vena cava (SVC) (infiltration) measuring 61 x 45 x 40 mm and showing intense FDG activity (SUV max 15.1); previously it measured 69 x 49 x 46 mm with SUV max 11.99. Lung parenchyma appeared normal, and the mediastinal vascular structures were within normal limits. After consultation with a medical oncologist, the patient was taken to surgery because the tumor did not respond to chemotherapy. Surgery was chosen after chemotherapy, with the intent of curative near-total resection. SVC reconstruction was done for symptom relief and complete oncologic clearance.
Surgical Technique
A triple lumen central line was inserted into the femoral vein and an arterial line in the radial artery. A midline sternotomy was done.
Off-Pump SVC Reconstruction
The SVC was badly stuck to the mass. Both brachiocephalic veins were dissected and clamped, and a purse-string suture was placed at the SVC-right atrium (RA) junction. The SVC was divided at the brachiocephalic junction close to RA. Hemodynamics were maintained with inotropes (dobutamine and noradrenaline) and fluids. The thrombus was removed from both brachiocephalic veins and the RA. The brachiocephalic veins junction was reconstructed with 6-0 polypropylene. A ringed PTFE graft was sutured to the RA and brachiocephalic junction reconstructing the SVC without using cardiopulmonary bypass. PTFE is preferred over pericardial tube because it is easy to retract during dissection of a mass without hemodynamic compromise.
Mediastinal Mass Excision
The aorta was retracted with stay sutures, and the mediastinal mass was carefully dissected from the right pulmonary artery (RPA), trachea, and carina. It was excised in two pieces. The main bulk of tumor mass was removed first. The tumor around the trachea was shaved off completely without damaging the airway. The RPA was completely mobilized and freed from the tumor. A subcarinal mass and lymph node were excised. There was near total resection.
The postoperative course was uneventful. The patient was on Ecosprin and heparin for three days and then switched to rivaroxaban. Facial swelling and upper arm swelling were completely reduced. The histopathology examination was suggestive of invasive mucinous adenocarcinoma of pulmonary origin with metastatic deposits in lymph nodes. Immunohistochemistry -Cytokeratin 7, TTF 1 and Napsin were positive. Cytokeratin 20, CDX2, p63 were negative. Next generation sequencing lung panel EML4: ALK fusion variant 3 was positive. The patient was started on targeted therapy -adjuvant ALECTINIB 600mg BD for two years. The chance of disease-free survival was improved due to the near total resection of the mediastinal mass. Follow-up echocardiogram at six months showed a patent graft and good heart function. No recurrence was shown on the follow-up PET scan.
References
- Patriarcheas V, Grammoustianou M, Ptohis N, Thanou I, Kostis M, Gkiozos I, Charpidou A, Trontzas I, Syrigos N, Kotteas E, Dimakakos E. Malignant Superior Vena Cava Syndrome: State of the Art. Cureus. 2022 Jan 4;14(1):e20924.
- Lepper PM, Ott SR, Hoppe H, Schumann C, Stammberger U, Bugalho A, Frese S, Schmücking M, Blumstein NM, Diehm N, Bals R, Hamacher J. Superior vena cava syndrome in thoracic malignancies. Respir Care. 2011 May;56(5):653-66.
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6 Comments
I wondered what is the advantage of removing this complex mass without cardiopulmonary bypass. Decompressing the heart would facilliate the dissection, provide a safetynet if a great vessel was violated, and decompress the brain. Percutaneous venous cannulation via the femoral vein and either juggularwould keep the field clear. The authors have done a great job, but for we ordinary marksmen, it is safer to face big game with heavy weapons.
Thanks for your comment
We have a good experience in doing Offpump Bidirectional glenn ,which we have published .This patient has venous collateral to decompress brain . Also when we give full dose heparin for CPB, dissection planes becomes difficult .
I’m curious about whether a preop cytological diagnosis was obtained, via EBUS, which could have provided a cytological diagnosis of mucinous adenoCA, as well as establishing advanced clinical staging of lung cancer, with at least stage IIIa N2 disease. The role of primary surgical resection in advanced stage IIIA disease with unknown primary is undefined, as survival will be more dependent upon sensitivity of tumor to chemotherapy and immunotherapy than it will to surgical resection a priori.
A CT-guided biopsy showed metastatic deposits of signet ring cell adeno carcinoma to the subcarinal node. An endobronchial ultrasound-guided fine needle biopsy from the subcarinal lymph node showed atypical cells with some signet cell appearance favoring metastasis.
A medical oncology opinion was sought, and 10 cycles of paclitaxel and carboplatin chemotherapy were given.There was no change in tumour size.
The patient was taken to surgery because the tumor did not respond to chemotherapy. Surgery was chosen after chemotherapy, with the intent of curative near-total resection. SVC reconstruction was done for symptom relief and complete oncologic clearance.
Very impressive dissection, and as Said above properly not something we would consider resectable in our hospital but you did macroscopically remove it and obviously relieved obstruction of the superior Vena cava. Presumably the patient got radiotherapy afterwards as well.?
Thanks for your comment
No patient didn”t get radiotherapy
The histopathology examination was suggestive of invasive mucinous adenocarcinoma of pulmonary origin with metastatic deposits in lymph nodes. Immunohistochemistry -Cytokeratin 7, TTF 1 and Napsin were positive. Cytokeratin 20, CDX2, p63 were negative.
Next generation sequencing lung panel EML4: ALK fusion variant 3 was positive.
Our oncologist started targeted therapy -adjuvant ALECTINIB .