Sarcomas in the groin and inguinal canal �C often missed and difficult to manage

INTRODUCTION

This is a 7-year retrospective review summarising the North of England Bone and Soft Tissue Tumour Service''s experience of managing 13 cases of groin sarcoma requiring soft tissue flap reconstruction. This study was performed to try to identify where national referral guidelines in sarcoma management had been followed and reasons for any delays. The study also includes outcome data relating to these patients.

PATIENTS AND METHODS

A retrospective, case-note review was undertaken using the local sarcoma database to identify approriate patients.

RESULTS

In nine patients, national referral guidelines were not followed. This resulted in a mean delay of presentation to the multidisciplinary team of 4.4 months. Ten patients had unplanned excision or exploration of tumours before referral. There were no lower limb amputations. All patients with narrow margins or high grade tumours were referred for radiotherapy. Four patients died; three as a result of distant metastases and one as a result of local recurrence.

CONCLUSIONS

Despite delays in referral, treatment by wide excision and plastic surgical reconstruction allowed for local control of these tumours with functional limb salvage. Implementation of National Institute for Health and Clinical Excellence (NICE) guidelines and local strategies could improve the expedient management of these patients.     

‘Telangiectatic’ Transformation in Soft Tissue Sarcomas. A Clinicopathology Analysis of an Aggressive Feature of High-grade Sarcomas

Background ‘Telangiectatic’ change, which contains a large fluid hemorrhagic component, occurs in a variety of high-grade soft tissue sarcomas. Methods In a retrospective database review, we identified 20 consecutive patients (3%) with ‘telangiectatic’ change in soft tissue sarcomas. Results Tumors were located in the thigh (55%), shoulder (15%), calf (15%), upper arm (10%), and buttock in one patient. All 20 tumors were high grade. Histological diagnoses were MFH (40%), leiomyosarcoma (15%), synovial sarcoma (10%), and one each of seven other sarcomas (35%). Tumor size was often large—more than 10 cm (35%), between 5 and 10 cm (60%), and less than 5 cm in one case. A history of contusion to the tumor site followed by swelling was recorded in 30% of patients and 80% presented with a painful mass. On MRI imaging, 60% of tumors appeared to contain more than 50% blood, 50% had a hemosiderin-laden rim, and 55% had well-defined tumor nodules within the wall of the hematoma. Limb-sparing surgery was carried out in 90% of patients, the other 10% underwent primary amputation. The 5-year, event-free survival rate was 30%. Of the patients, 15% presented initially with metastatic disease; in 53%, it developed within 2 years of diagnosis. The overall local recurrence rate was 30%. Conclusions Telangiectatic transformation in soft tissue sarcomas is a rare feature of aggressive high-grade soft tissue sarcomas and is unique in its clinical presentation, MRI characteristics, pathological pattern, and a tendency for a worse-off prognosis. This investigation was performed at the Department of Orthopedic Oncology, Washington Cancer Institute, Washington Hospital Center, Washington DC.     

Litigation related to casting in Orthopaedics—An analysis of claims against the National Health Service in England

Orthopaedic casts have been used to treat musculoskeletal conditions for hundreds of years and are still a fundamental component of treating a variety of disorders. As surgical techniques have advanced the frequency of use of orthopaedic casts has declined. With Orthopaedics being is one of the most litigious specialties in medicine we sough to evaluate how this related to casting in Orthopaedics and how we could learn from past mistakes.We analysed litigation claims related to Orthopaedic casts from 1995 to 2010 in which the claims were closed. 43 cases were related to orthopaedic casts. The total costs of these claims were over £2.3 million with an average total cost of £48,500 per claim. The most common cause for claim was harm caused when a cast was applied too tight and secondly from removing the cast. This is the first study to evaluate litigation claims related to Orthopaedic casts and highlights potential complications that if avoided will certainly improve the care of the patients and avoid unnecessary litigation.     

Retroperitoneal paraganglioma—Is pre operative embolization useful?

IntroductionParagangliomas (PG) are rare tumors derived from chromaffin cells that are located outside the adrenal gland and are capable of producing catecholamines. The treatment is based on a surgical resection, and there is controversy regarding the usefulness of previously carrying out an embolization and what is the most adequate surgical approach.Clinical caseWe will present a 17-year-old woman with a retroperitoneal tumour in contact with the aorta and the inferior vena cava, treated with embolization prior to the surgical resection via laparotomy.DiscussionThe PG tumors are very infrequent and originate in the extra-adrenal chromaffin cells that exist in the vicinity of the components of the autonomic nervous system. Most of them (86%) produce catecholamines, are unique, sporadic, benign and more frequent in middle-aged women. Since they are radioresistant tumors, the only possibility for a cure is by a complete surgical excision. The preoperative embolization has been described mainly as the treatment of cervical PG, although its use in abdominal PG is more controversial and is not done in a systematic manner.ConclusionWe can conclude that the embolization of abdominal PG is not free of risks and that it has not been demonstrated that it significantly reduces the peri-operative bleeding or the surgical time. Probably, the embolization should be reserved for intensively hypervascularized and larger PGs.     

Can a Bayesian Belief Network Be Used to Estimate 1-year Survival in Patients With Bone Sarcomas?

Background

Extremity sarcoma has a preponderance to present late with advanced stage at diagnosis. It is important to know why these patients die early from sarcoma and to predict those at high risk. Currently we have mid- to long-term outcome data on which to counsel patients and support treatment decisions, but in contrast to other cancer groups, very little on short-term mortality. Bayesian belief network modeling has been used to develop decision-support tools in various oncologic diagnoses, but to our knowledge, this approach has not been applied to patients with extremity sarcoma.

Questions/purposes

We sought to (1) determine whether a Bayesian belief network could be used to estimate the likelihood of 1-year mortality using receiver operator characteristic analysis; (2) describe the hierarchal relationships between prognostic and outcome variables; and (3) determine whether the model was suitable for clinical use using decision curve analysis.

Methods

We considered all patients treated for primary bone sarcoma between 1970 and 2012, and excluded secondary metastasis, presentation with local recurrence, and benign tumors. The institution’s database yielded 3499 patients, of which six (0.2%) were excluded. Data extracted for analysis focused on patient demographics (age, sex), tumor characteristics at diagnosis (size, metastasis, pathologic fracture), survival, and cause of death. A Bayesian belief network generated conditional probabilities of variables and survival outcome at 1 year. A lift analysis determined the hierarchal relationship of variables. Internal validation of 699 test patients (20% dataset) determined model accuracy. Decision curve analysis was performed comparing net benefit (capped at 85.5%) for all threshold probabilities (survival output from model).

Results

We successfully generated a Bayesian belief network with five first-degree associates and describe their conditional relationship with survival after the diagnosis of primary bone sarcoma. On internal validation, the resultant model showed good predictive accuracy (area under the curve [AUC] = 0.767; 95% CI, 0.72–0.83). The factors that predict the outcome of interest, 1-year mortality, in order of relative importance are synchronous metastasis (6.4), patient’s age (3), tumor size (2.1), histologic grade (1.8), and presentation with a pathologic fracture (1). Patient’s sex, tumor location, and inadvertent excision were second-degree associates and not directly related to the outcome of interest. Decision curve analysis shows that clinicians can accurately base treatment decisions on the 1-year model rather than assuming all patients, or no patients, will survive greater than 1 year. For threshold probabilities less than approximately 0.5, the model is no better or no worse than assuming all patients will survive.

Conclusions

We showed that a Bayesian belief network can be used to predict 1-year mortality in patients presenting with a primary malignancy of bone and quantified the primary factors responsible for an increased risk of death. Synchronous metastasis, patient’s age, and the size of the tumor had the largest prognostic effect. We believe models such as these can be useful as clinical decision-support tools and, when properly externally validated, provide clinicians and patients with information germane to the treatment of bone sarcomas.

Clinical Relevance

Bone sarcomas are difficult to treat requiring multidisciplinary input to strategize management. An evidence-based survival prediction can be a powerful adjunctive to clinicians in this scenario. We believe the short-term predictions can be used to evaluate services, with 1-year mortality already being a quality indicator. Mortality predictors also can be incorporated in clinical trials, for example, to identify patients who are least likely to experience the side effects of experimental toxic chemotherapeutic agents.

     

Retroperitoneal sarcoma: Time for a change in attiude?

Background : Retroperitoneal sarcoma (RPS) is considered a disease with poor prognosis partly because of the difficulty with diagnosis at an early stage. This review assesses the current best practice principles for RPS and finds evidence suggesting a better outlook for appropriately managed cases. Recommendations are made for improving diagnostic certainty before laparotomy and inappropriate transperitoneal biopsy occur. Methods : A critical review of the English language literature was conducted using MEDLINE software and searching the terms ‘retroperitoneal sarcoma’ alone or in combination with ‘prognosis’, ‘surgery’ and ‘adjuvant therapy’. Conclusions : Retroperitoneal sarcoma is a rare disease but when appropriately managed the disease‐free survival can be improved and may even approach that of extremity soft tissue sarcoma. One of the greatest barriers to improving outcome is the misinterpretation of clinical signs and an over‐reliance on ultrasound diagnosis in pelvic presentations, or misinterpretation of clinical signs and/or computer tomography (CT) scans in abdominal masses. Physicians referring patients with a retroperitoneal mass should consider more frequently the less common differential diagnoses of an abdominopelvic mass including retroperitoneal sarcoma. This is especially true in circumstances where there is a circumscribed, predominantly solid tumour, with clinical or radiological signs of vascular or rectal displacement, ureteric obstruction and/or classic renal rotational displacement. The more frequent use of CT scans with intravenous and oral contrast with referral prior to inappropriate transperitoneal biopsy is recommended. In atypical cases where preoperative biopsy is necessary, extraperitoneal routes are preferable. Complete en bloc surgical excision at the first laparotomy is the treatment of choice in RPS. Macroscopic clearance may necessitate resection of adjacent viscera, neurovascular structures or abdominopelvic walls but, if achieved, may lead to long‐term survival depending on individual tumour biology.     

Retroperitoneal Sarcoma: Is It Time to Change the Surgical Policy?

Background  

Complete tumor resection is the mainstay of treatment for retroperitoneal sarcoma (RPS), but the size and quality of surgical margins for radical resection in RPS are unknown. They are believed to be pushing tumors, but recently, aggressive surgical policies leading to multivisceral resection have seemed to suggest better local control compared with simple tumor resection. We analyzed a single-institution series of RPS to provide information useful to surgical decision-making.