Surgical resection of chest wall sarcomas: an analysis of survival and predictors of outcome at an Australian multidisciplinary sarcoma service.

BACKGROUND: Chest wall sarcomas are a rare group of tumours. Surgical resection is considered the mainstay of curative treatment, however, resection and reconstruction of chest wall defects presents complex issues for the clinician. METHODS: A retrospective analysis of 59 patients undergoing surgical management of chest wall sarcoma between December 1996 and July 2020 was conducted across a multidisciplinary sarcoma service in Melbourne, Australia. Patient demographics, pathologic data, and long-term outcomes were recorded. RESULTS: Mean age at surgery was 48.4 years (SD 18.3), and 66.1% were male. Median follow-up was 29 months (IQR 11.8, 51.0 months). Fifty-one patients presented with primary tumours, while the others had secondary tumours resected. Most tumours arose in bone (72.9%) as opposed to soft tissues (27.1%). Chondrosarcoma was the most common histologic subtype (50.8%). The most common reconstructive techniques involved the use of mesh (79.7%) or mesh supplemented with bone cement (33.9%). Overall survival at 1 and 5 years was 92% and 70%, respectively. Seven patients died of metastatic sarcoma during the follow up period with a median survival time of 27 months. Twelve patients had evidence of disease recurrence during the follow-up period. Stage 4 disease, soft tissue tumours, secondary tumours, leiomyosarcoma and UPS subtypes, and plating reconstruction were associated with increased disease recurrence. CONCLUSIONS: The results suggest that outcomes for chest wall sarcoma are similar to extremity sarcomas and may be treated in a similar manner. Patients requiring adjuvant radiotherapy and those who develop disease-recurrence are more likely to have worse overall survival outcome despite complete surgical resection.

Pulmonary carcinoid tumours: A multi-centre analysis of survival and predictors of outcome following sublobar, lobar, and extended pulmonary resections.

BACKGROUND: Pulmonary carcinoids are rare neoplasms, accounting for approximately 1%-2% of all lung malignancies. A retrospective analysis was undertaken of all patients who underwent surgical resection of pulmonary carcinoid tumours across multiple institutions in Melbourne, Australia. METHODS: From May 2000 through April 2020, 241 patients who underwent surgical resection of pulmonary carcinoid tumours were retrospectively reviewed. Patient demographics, pathologic data, and long-term outcomes were recorded. RESULTS: Median age was 57.7 years and the majority of patients were female (58.9% vs. 41.1%). Typical carcinoid was present in 77.1%. Histological subtype was associated with several factors. Atypical carcinoid was more likely to have larger tumour size and nodal involvement. Overall survival for typical carcinoid at 5, 10, and 15 years was 98%, 95%, and 84%, and for atypical carcinoid was 88%, 82%, and 62%, respectively. Histological subtype and age were found to be independent predictors of overall survival, with worse outcomes for atypical and those above 60 years of age. Disease-free survival was related to sublobar resection (p < 0.001, sub-hazard ratio (SHR): 6.89), lymph node involvement (p = 0.022, SHR: 3.18), and atypical histology (p < 0.001, SHR: 9.89). CONCLUSION: Excellent long-term outcomes can be achieved following surgical resection of pulmonary carcinoids. Atypical histology and lymph node involvement are significant prognostic factors, and sublobar resection should not be considered in patients with either of the above features. Typical carcinoid tumour without nodal involvement may be appropriate for sublobar resection. Typical and atypical carcinoid tumours should be considered distinct disease entities, and as such treated accordingly.

Tissue "valve-over-valve" implantation in previous mechanical Bentall.

Multiple studies have reported the adverse impact of audible clicks from mechanical heart valves on the quality of life. Sadly, this can become a lifelong liability in younger patients necessitating explantation. Bioprosthetic valve replacement is the treatment for this distressing condition associated with an isolated mechanical valve. However, patients who have undergone a mechanical Bentall's procedure represent a unique surgical challenge. In view of the significant complications associated with a redo aortic root operation, the alternative of a "valve-over-valve" implantation is an attractive option that might present reduced morbidity. We report a unique case of valve noise intolerance in a patient who underwent a bioprosthetic valve-over-valve replacement following previous Bentall's procedure with a mechanical valved conduit.

Resection of a functioning intrapericardial paraganglioma associated with succinate dehydrogenase B mutation.

Functional paragangliomas are rare neuroendocrine tumours that secrete catecholamines and are infrequently found in the mediastinum. We report a case of a young male with symptoms of catecholamine excess and a personal and family history of the paraganglioma predisposing succinate dehydrogenase subunit B mutation. The lesion had anatomical intrapericardial juxtaposition to important cardiac anatomy and posed the significant challenge of dissection at surgery. The lesion was successfully resected via sternotomy on cardiopulmonary bypass and confirmed histopathologically as paraganglioma. Intrapericardial paraganglioma is rare and treatment is difficult and time critical considering the proximity of cardiac anatomy as well as malignant potential.

Removal of Peritnoeo-venous-atrial shunt thrombus without cardiopulmonary bypass.

Thrombus formation is not uncommon in longstanding intracardiac catheters, but formation of a thrombus at the tip of a Peritnoeo-venous-atrial shunt, causing obstruction of the tricuspid valve, is a rare complication and frequently unrecognized. A large intracardiac thrombus causing valve obstruction requires surgical removal with the support of cardiopulmonary bypass which is associated with significant morbidity. We successfully removed a thrombus attached to the tip of peritoneovenous shunt without cardiopulmonary bypass in a 25-year-old man.

Sternal-Sparing Aortic Valve Replacement in a Patient with Osteogenesis Imperfecta: A Case Report.

The case described here is the first reported case of successful isolated aortic valve replacement (AVR) via a right anterior thoracotomy (RAT) in a patient with osteogenesis imperfecta (OI). The most common reported complication in patients with OI undergoing AVR or other cardiac surgery is bleeding and sternal complications. By using a RAT approach, it was possible to replace the aortic valve without major bleeding, transfusion, or sternal complications.

Choice of valve prosthesis in a rare clinical condition: aortic stenosis due to alkaptonuria.

Alkaptonuria is a rare inherited disorder of tyrosine metabolism, which results in deposition of homogentisic acid in the connective tissues. The accumulation of homogentisic acid in connective tissue causes the syndrome known as ochronosis, which is typically manifested by skin pigmentation, degenerative arthropathy and discolouration of urine. Cardiovascular involvement is a much less common complication of alkaptonuria but poses a greater risk to the patient's health. We present the case of a 65 year-old man with aortic stenosis and a previous diagnosis of alkaptonuria who underwent successful aortic valve replacement with a mechanical prosthesis.