Anant Desai

Management of Recurrent Retroperitoneal Sarcoma (RPS) in the Adult: A Consensus Approach from the Trans-Atlantic RPS Working Group

IntroductionRetroperitoneal soft tissue sarcomas (RPS) are rare tumors. Surgery is the mainstay of curative therapy, but local recurrence is common. No recommendations concerning the best management of recurring disease have been developed so far. Although every effort should be made to optimize the initial approach, recommendations to treat recurring RPS will be helpful to maximize disease control at recurrence.MethodsAn RPS transatlantic working group was established in 2013. The goals of the group were to share institutional experiences, build large multi-institutional case series, and develop consensus documents on the approach to this difficult disease. The outcome of this document applies to recurrent RPS that is nonvisceral in origin. Included are sarcomas of major veins, undifferentiated pleomorphic sarcoma of psoas, ureteric leiomyosarcoma (LMS). Excluded are desmoids-type fibromatosis, angiomyolipoma, gastrointestinal stromal tumors, sarcomas arising from the gut or its mesentery, uterine LMS, prostatic sarcoma, paratesticular/spermatic cord sarcoma, Ewing sarcoma, alveolar/embryonal rhabdomyosarcoma, sarcoma arising from teratoma, carcinosarcoma, sarcomatoid carcinoma, clear cell sarcoma, radiation-induced sarcoma, paraganglioma, and malignant pheochromocytoma.ResultsRecurrent RPS management was evaluated from diagnosis to follow-up. It is a rare and complex malignancy that is best managed by an experienced multidisciplinary team in a specialized referral center. The best chance of cure is at the time of primary presentation, but some patients may experience prolonged disease control also at recurrence, when the approach is optimized and follows the recommendations contained herein.ConclusionsInternational collaboration is critical for adding to the present knowledge. A transatlantic prospective registry has been established.

Improving outcomes in gastric cancer over 20 years

BackgroundGastric cancer has in the past proven to be a difficult disease to cure. Surgery is the most effective treatment, although the extent of lymphadenectomy undertaken is controversial. This paper examines trends in the detection and treatment of gastric cancer, the move toward targeted surgery, and their impact on survival.MethodsFrom 1982 to 2001, 430 patients with gastric cancer were under the care of a single surgeon at the same institution. Copies of the operation records, discharge summaries, and histology reports were retained and subsequently reviewed.ResultsThirty-six patients were excluded from the analysis. Two hundred and five patients (52%) had potentially curative surgery for adenocarcinoma and 189 had incurable disease. During the 20-year period, overall 5-year survival increased significantly, from 15% to 41% (P < 0.01). The number of curative procedures also increased significantly, from 33% to 73% (P1 < 0.001) as disease was detected earlier, and 5-year survival in these patients increased from 26% to 46%. Eighty-seven D1, 92 D2, and 26 targeted procedures were performed, with 30-day mortalities of 5%, 9%, and 5%, respectively. The 5-year survival was 47% for D1, 42% for D2, and 66% for targeted surgery.ConclusionThis paper demonstrates an improvement in the stage at which disease was detected and improvement in long-term survival for patients with gastric cancer. The development of targeted surgery has shown promising early results.

Outcomes of isolated limb perfusion in the treatment of extremity soft tissue sarcoma: A systematic review

BACKGROUND Isolated limb perfusion (ILP) may provide a limb salvage option for locally advanced soft tissue sarcoma (STS) not amenable to local resection. METHODS A systematic review was performed for studies reporting outcome of ILP for locally advanced STS performed after 1980 in patients aged ≥ 12 years old. The main endpoints were tumour response and limb salvage rates. Complication and recurrence rates were secondary endpoints. RESULTS Eighteen studies were included, providing outcomes for 1030 patients. Tumour necrosis factor-alpha with melphalan was the commonest chemotherapy regime. When reported, 22% of cases achieved a complete tumour response (216/964, 15 studies) with an overall response rate of 72% (660/911, 15 studies). At median follow-up times ranging between 11 and 125 months, the limb salvage rate was 81% in patients who otherwise would have been subjected to amputation. However, 27% of patients suffered local recurrence and 40% suffered distant failure. ILP was associated with severe locoregional reactions in 4% (22/603) of patients. Amputation due to complications within 30 days was necessary in 1.2% of cases (7/586, nine studies). There was insufficient evidence to determine the effect of ILP on survival. CONCLUSION ILP induces a high tumour response rate, leads to a high limb salvage rate but is associated with a high recurrence rate. It provides a limb salvage alternative to amputation when local control is necessary.

Neoadjuvant and adjuvant strategies in retroperitoneal sarcoma

Extended surgery remains the mainstay of treatment in retroperitoneal sarcoma, although conflicting data exist on the benefit of neoadjuvant and adjuvant therapies, particularly with regard to tumour grade and histological type. Experience of radiotherapy and chemotherapy in extremity soft tissue sarcoma can inform treatment strategies, however these data cannot be universally extrapolated to the retroperitoneum where disease biology and anatomical considerations are different. The present review sets a historical context before discussing recent evidence and on-going multi-centre trials in retroperitoneal sarcoma. Promising data on histologically- and molecularly-targeted chemotherapy are discussed and the need for centralisation of retroperitoneal sarcoma services in order to facilitate large international collaborative trials is emphasised.

Alveolar soft part sarcoma presenting with back pain

A 35-year-old man was referred to the Sarcoma service with a 2-year history of worsening back pain, left-sided radicular pain and a mass on CT scan. In the 6 months following the onset of the back pain, he developed progressive numbness and paraesthesia in the L2 dermatome. This was worse on standing. On clinical examination, power was unaffected but somewhat limited by pain. No ‘red flag’ symptoms were present …

Peri-operative Therapies for Localized Retroperitoneal Soft Tissue Sarcoma

Retroperitoneal soft tissue sarcomas are rare solid tumors that usually arise in the retroperitoneum or the pelvis. They are often large at presentation and push adjacent structures, displacing them, anatomically. The most common adult histotypes are liposarcoma and leiomyosarcoma. Five-year overall survival is less than 50 % and is mainly determined by the high incidence of local relapse and to a lesser extent by their capacity to metastasize. Radical surgery is still the most effective treatment. While an aggressive surgical policy consisting of multivisceral resections ‘en bloc’ with the mass has undoubtedly decreased, the rate of local recurrence, prognosis remains poor. Retrospective analyses and early prospective data suggest a benefit from using neoadjuvant radiotherapy in order to improve local control. Chemotherapy plays an important role in advanced disease, but very little evidence supports a real advantage in delivering it prior to surgery to make the resection more likely to achieve clear margins or postoperatively to decrease the risk of systemic recurrence. Further studies are required to explore the role of these neoadjuvant/adjuvant therapies, particularly in the context of new developments in molecular therapeutic agents.