A Case of Giant Mediastinal Liposarcoma of Thymic Origin: A Rare Clinical Entity

Thymoliposarcoma is an exceedingly rare tumor of thymus with a very few cases reported till date. This case study presents a 45-year male with rare type of thymoma. On the contrast-enhanced CT images, there was a large mass lesion of predominantly fat attenuation in the pre-vascular compartment of the mediastinum insinuating on both sides of the visceral compartment of the mediastinum, and extending upto the bilateral cardio phrenic and anterior costophrenic angles, anterior to the right ventricle with loss of fat plane with the pericardium, with few sub-centimetric lymph nodes in the right paratracheal and AP window and a calcified right hilar lymph node, suggestive of well-differentiated liposarcoma/thymoliposarcoma. Initial CT guided tru-cut tissue biopsy was inconclusive, and the repeat biopsy revealed as fibro-collagenous tissue with area of necrosis, focal myxoid changes in the background with presence of cells which are spindle to oval in shape with mild nuclear pleomorphism and negative for S100, Cytokeratin, CD34, desmin. The entire tumor was resected en masse after meticulous dissection without the support of cardiopulmonary bypass (CPB) with an intact pericardium. Final histopathology report of the surgical biopsy specimens is consistent with dedifferentiated thymoliposarcoma with focal ganglionic cell differentiation. Postoperative follow-up CECT of thorax revealed no evidence of residual mass in the pre-vascular compartment. The patient is disease-free and asymptomatic after 6-month and he is under routine follow-up under Radiotherapy department since he received 30 Gy of postoperative radiotherapy (PORT).

Konno Procedure for Managing Small Aortic Root during Aortic Valve Replacement Surgery: An Experience of 12 Cases

Background: Small aortic annulus during aortic valve replacement can lead to implanting a smaller sized valve compared to the body surface area thereby causing patient prosthesis mismatch. Various aortic root enlargement techniques have been described depending on anterior or posterior approach. Konno procedure uses anterior approach for aortic root enlargement. In this study, we reviewed results of Konno procedure done from 2011 to 2019 by a single surgeon. Methods: 12 adult patients who underwent aortic valve replacement along with Konno procedure for small aortic root by a single surgeon at a single center between 2011 and 2019 were reviewed. Echocardiographic and demographic data and post-operative data were obtained from medical records. Symptomatic profile was assessed as per New York Heart Association Classification. Intraoperative findings and post-operative period findings were noted. Follow up symptom profile was assessed for these patients. Results: 12 patients underwent Konno procedure between 2011 and 2019 for small aortic root along with valve replacement. The main indication for surgery was aortic stenosis with small aortic annulus, with or without involvement of the mitral valve. Preoperatively, 3 patients had NYHA class II and 9 patients had NYHA class III symptoms. Mean age at operation was 26.42 years, minimum age 10 years, and maximum age 39 years. 3 were females and 9 were males. Mean bypass time was 106.4 minutes and aortic cross clamp time was 80.67 minutes. Mechanical aortic valves were implanted in all patients. Mean post-operative blood loss was 134.2 ml and duration of ventilation before extubation was 14.5 hours. Mean duration of intensive care unit (ICU) stay was 2.83 days and hospital stay was 9.1 days. Mean gradient in the post-operative period was 10.75 mm Hg. There was no mortality in these 12 patients and no reoperation was needed in the follow up period. Follow up in the outpatient department suggested all patients had NYHA class I symptoms and anticoagulation with warfarin adjusted to prothrombin time— International normalised ratio. Conclusion: Konno procedure is effective for managing small aortic root as bigger outflow orifice area through the larger valve prosthesis improves ventricular outflow and hence, improves the outcomes.