Impact of centralization of services on outcomes in a rare tumour: Retroperitoneal sarcomas.

BACKGROUND AND AIM: The retroperitoneal tumor (RPT) service in the North West costal region of England was centralized in May 2011 by the merger of the Merseyside, Cheshire and Lancashire, Cumbria sarcoma networks. Our aim was to analyze the impact of centralization of services on patient outcomes. METHODS: An analysis from 01/12/2004 to 30/11/2017 was undertaken from prospectively maintained database and electronic patient records; follow-up was until 30/04/2018. This time period encompassed 6.5 years before and after centralization of services took place. Survival analysis was done for Retroperitoneal Sarcomas (RPS) and also compared the impact of centralization. RESULTS: 72 patients (27 men), median age 69 (21-90) years) underwent 95 operations with an intention to excise RPS. Overall there were 52 (54.7%) multi-visceral resections (MVR). 91/95 (95.8%) patients with primary tumors had surgery with a curative (R0/1) intent. 30-day and 90-day operative mortality was 3.2% (n = 3) and 4.2% (n = 4) respectively. The 5-year survival for patients undergoing resection for RPTs was 51.3%. 79 (83.1%) of the resections in this series occurred in the 6.5-years post-centralization with an increase in MVR between the two time points (p < 0.0006). Despite the more radical nature of surgery post-centralization, there was no difference in 5-year survival for RPS patients when compared to pre-centralization, p = 0.575. However the 5-yr survival post-centralization compared favorability to national outcomes. CONCLUSION: Centralization in the management of RPS has resulted in an increase in resection rates and more complex MVRs, without compromising R0/1 resection rates; peri-operative mortality or overall survival.

Giant schwannoma of the medial plantar nerve.

In this article the authors present an exceptionally rare case of giant schwannoma of the foot in a 23 year old male. This article demonstrates the key principles of investigation and management of such cases as conducted by a regional sarcoma centre.

Stewart-Treves syndrome following idiopathic leg lymphoedema: remember sarcoma.

Patients with skin complaints secondary to oedema are commonly encountered and can be a diagnostic challenge. Here, we present the case of a 78-year-old lady with Stewart-Treves syndrome, a rare cutaneous angiosarcoma. The histology showed angiocutaneous sarcoma with poorly defined margins. The prognosis remains extremely poor. A successful transfemoral amputation reduced the massive tumour burden and cleared a source of sepsis from this fungating tumour. Our aim is to highlight this rare but high-grade sarcoma which results from chronic lymphoedema. The red flag signs of sarcoma are masses greater than 5 cm, tumours which are rapidly growing, deep to fascia or painful, and tumours that recur after previous surgery. Non-healing or progressive ulceration in the background of chronic lymphoedema should raise the possibility of underlying malignancy. We need a low threshold for biopsy and early referral to an appropriate multidisciplinary team for the optimum management of the patient.

Giant osteochondroma of the talar neck.

Giant osteochondroma is an uncommon entity and it is rare in the foot and ankle region. It is extremely rare to originate from the talus. In this case report we present a case of giant osteochondroma arising from the talar neck measuring 100 mm × 90 mm × 30 mm. It is unique because of the size, site and the age at presentation.

Hindquarter amputation: is it still needed and what are the outcomes?

A total of 157 hindquarter amputations were carried out in our institution during the last 30 years. We have investigated the reasons why this procedure is still required and the outcome. This operation was used as treatment for 13% of all pelvic bone sarcomas. It was curative in 140 and palliative in 17, usually to relieve pain. There were 90 primary procedures (57%) with the remaining 67 following the failure of previous operations to control the disease locally. The indication for amputation in primary disease was for large tumours for which limb-salvage surgery was no longer feasible. The peri-operative mortality was 1.3% (n = 2) and major complications of wound healing or infection arose in 71 (45%) patients. The survival at five years after hindquarter amputation with the intent to cure was 45%, and at ten years 38%. Local recurrence occurred in 23 patients (15%). Phantom pain was a significant problem, and only 20% used their prosthesis regularly. Functional scores were a mean of 57%. With careful patient selection the oncological results and functional outcomes of hindquarter amputation justify its continued use.

Clinical implication of pretreatment neutrophil to lymphocyte ratio in soft tissue sarcoma.

INTRODUCTION: Elevated neutrophil to lymphocyte ratio has been identified as a prognostic indicator in malignancies whereas; its association with extremity and trunk soft tissue sarcoma remain unclear. The aim of this study is to determine the utility of full blood neutrophil lymphocyte ratio (NLR) in preoperative diagnosis and its predictive value for survival in patients managed for soft tissue sarcoma of the trunk and extremities. METHOD: 223 patients who presented with a soft tissue tumor were retrospectively reviewed. The study period was from January 2002-December 2009. Preoperative NLR as well as demographics, clinical and histopathological data were analysed. RESULTS: Full blood NLR was significantly higher in patient with a soft tissue sarcoma compared to benign soft tissue tumors (p < 0.001). Cox regression analysis demonstrated that elevated NLR >5 (p < 0.05) may be an adverse prognostic factor for Overall Survival. CONCLUSION: The preoperative NLR is a simple, investigation predicting the preoperative diagnosis of a soft tissue sarcoma and a predictor of worse overall survival for patient with a soft tissue sarcoma.

Pathological fracture of the proximal femur in osteosarcoma: need for early radical surgery?

Seventeen patients underwent treatment for a pathological fracture of the proximal femur due to osteosarcoma. Their age range was from 9 to 84 (mean age 42) with nine patients under the age of 40 and eight above the age of 40. Twelve patients had a fracture at diagnosis and five developed a fracture after the diagnosis. Seven patients had metastatic disease at diagnosis. Five patients were referred after internal fixation of the fracture prior to diagnosis. Chemotherapy was used when appropriate and eight patients then underwent limb salvage surgery, six had an amputation, and three had palliative treatment. The estimated five-year survival was 14%. These results are significantly worse than expected, and it proved impossible to identify any group who fared well. The high incidence of metastases both at diagnosis and subsequently suggests this group of patients are at very high risk. Review of multicentre data may suggest an optimum treatment for this patient group.

Outcome of pathologic fractures of the proximal femur in nonosteogenic primary bone sarcoma.

UNLABELLED: Pathologic fractures of the proximal femur resulting from primary bone sarcomas are often difficult to treat and some are often not recognized resulting in inappropriate interventions prior to the correct diagnosis being made. The aim of the study was to identify factors determining the survival after pathologic fractures of the proximal femur resulting from nonosteogenic primary bone sarcomas. PATIENTS AND METHODS: We retrospectively reviewed 72 patients with nonosteogenic primary bone sarcomas of the proximal femur (chondrosarcoma in 34 patients, spindle cell sarcoma in 25 and Ewing's sarcoma in 13). There were 40 male and 32 female patients. The mean age was 49 years. We analyzed patient, tumor, and treatment factors in relation to overall survival. The mean follow up was 55 months (range 0-302 months). RESULTS: The overall 5-year survival rate was 44% (Ewing's sarcoma 60%, chondrosarcoma 57%, spindle cell sarcoma 30%, and dedifferentiated chondrosarcoma 0%). The diagnosis of spindle cell sarcoma and dedifferentiated chondrosarcoma was the only significant factor adversely affecting survival. There was no difference in survival resulting from timing of the fracture, site of the fracture, age at diagnosis, surgical margin, limb salvage or amputation. CONCLUSIONS: The key-factor adversely affecting the survival after pathologic fractures of the proximal femur resulting from nonosteogenic primary bone sarcomas is the histological diagnosis of dedifferentiated chondrosarcoma and spindle cell sarcoma.

Management of radiation-induced sarcomas in a tertiary referral centre: a review of 25 cases.

INTRODUCTION: Radiation-induced sarcomas are a rare and late complication of radiotherapy for breast carcinoma which often have poor prognosis. METHODS: This study is a retrospective review of 25 patients referred to a regional sarcoma unit between 1978 and 2009. RESULTS: Radiation-induced sarcoma following the diagnosis and treatment of breast cancer occurred after a mean period of 156 months. Anatomical sites involved were the breast, chest wall, clavicle, scapula, humerus and axilla. Twenty one patients had wide local excision followed by chest wall reconstruction, latissimus dorsi flap cover or limb amputation. The estimated five years survival following the diagnosis of the radiation-induced sarcoma was 27% and the local recurrence rate 52%. CONCLUSION: Radiation-induced sarcoma following breast cancer has high local recurrence rate and poor prognosis. They should be managed in a multi-disciplinary setting. Long-term follow-up of patients treated with radiotherapy for breast cancer is therefore advisable.

Curettage of aneurysmal bone cysts of the feet.

BACKGROUND: Aneurysmal bone cysts (ABC) mainly occur in the long bones but only rarely in the bones of the feet. This study reviews the 25-year experience of a tertiary orthopaedic oncology referral center treating ABC's in the bones of the feet, using prospectively collected data to document their incidence, treatment and the outcome. MATERIALS AND METHODS: Information on the diagnosis, treatment, complications and outcomes were collected for all eligible patients from our prospectively collected database. Radiologic healing was used as our primary outcome measure. Cases were considered to have failed treatment if there was progression of the lesion that needed further treatment. RESULTS: Of 273 patients with ABC, 16 patients (5.9%) were found to have their lesions in the foot. There were ten female and six male patients, aged from 4 to 52 (mean, 18.2) years. The metatarsals were the most commonly affected site (44%). Curettage of the lesion without additional adjuvant measures (14 of 16 patients) was sufficient for the treatment in 11 of 14 patients (79%). This gave a recurrence rate of 21% (three of 14 patients) in those treated by curettage alone and 19% (three of 16 patients) if the whole cohort is considered. CONCLUSION: Although relatively rare, there is no reason to assume that ABCs of the feet will respond to treatment or recur any differently from ABCs that occur elsewhere in the body. Our experience has been that surgical curettage alone is sufficient to treat most ABCs of the feet.

Unipolar proximal femoral endoprosthetic replacement for tumour: the risk of revision in young patients.

We undertook a cemental unipolar proximal femoral endoprosthetic replacement in 131 patients with a mean age of 50 years (2 to 84). Primary malignant tumours were present in 54 patients and 67 had metastatic disease. In addition, eight patients had either lymphoma or myeloma and two had non-oncological disorders. The mean follow-up was 27 months (0 to 180). An acetabular revision was required later in 14 patients, 12 of whom had been under the age of 21 years at the time of insertion of their original prosthesis. The risk of acetabular revision in patients over 21 years of age was 8% at five years compared with 36% in those aged under 21 years. All the unipolar hips in this younger age group required revision within 11 years of the initial operation. We conclude that unipolar replacement should not be used in younger patients and should be avoided in patients with a life expectancy of more than five years.

Adamantinoma of the calcaneum--a case report.

Adamantinoma is rare, representing less than 1% of all primary malignant bone tumours. It is a slow growing low grade malignant tumour which is often clinically, radiologically and histologically mistaken for many other tumours like Ewing's sarcoma, synovial sarcoma, fibrosarcoma, metastatic carcinoma, fibrous cortical defect and osteofibrous dysplasia. Adamantinoma commonly occurs in the tibia. It is also known to occur in the fibula, femur, humerus, radius and ulna. Adamantinoma of the foot is extremely rare. There are few reported cases of metatarsal and cuneiform bone involvement. To our knowledge adamantinoma of the calcaneum has not been previously reported in the literature. We report the case of a male patient, aged 33 years, who was treated for adamantinoma of the calcaneum and followed for 16 years highlighting the difficulty in diagnosis and management of this rare tumor occurring at a rare site.

Modular endoprosthetic replacement for tumours of the proximal femur.

Endoprosthetic replacement of the proximal femur may be required to treat primary bone tumours or destructive metastases either with impending or established pathological fracture. Modular prostheses are available off the shelf and can be adapted to most reconstructive situations for this purpose. We have assessed the clinical and functional outcome of using the METS (Stanmore Implants Worldwide) modular tumour prosthesis to reconstruct the proximal femur in 100 consecutive patients between 2001 and 2006. We compared the results with the published series for patients managed with modular and custom-made endoprosthetic replacements for the same conditions. There were 52 males and 48 females with a mean age of 56.3 years (16 to 84) and a mean follow-up of 24.6 months (0 to 60). In 65 patients the procedure was undertaken for metastases, in 25 for a primary bone tumour, and in ten for other malignant conditions. A total of 46 patients presented with a pathological fracture, and 19 presented with failed fixation of a previous pathological fracture. The overall patient survival was 63.6% at one year and 23.1% at five years, and was significantly better for patients with a primary bone tumour than for those with metastatic tumour (82.3% vs 53.3%, respectively at one year (p = 0.003)). There were six early dislocations of which five could be treated by closed reduction. No patient needed revision surgery for dislocation. Revision surgery was required by six (6%) patients, five for pain caused by acetabular wear and one for tumour progression. Amputation was needed in four patients for local recurrence or infection. The estimated five-year implant survival with revision as the endpoint was 90.7%. The mean Toronto Extremity Salvage score was 61% (51% to 95%). The implant survival and complications resulting from the use of the modular system were comparable to the published series of both custom-made and other modular proximal femoral implants. We conclude that at intermediate follow-up the modular tumour prosthesis for proximal femur replacement provides versatility, a low incidence of implant-related complications and acceptable function for patients with metastatic tumours, pathological fractures and failed fixation of the proximal femur. It also functions as well as a custom-made endoprosthetic replacement.

Variability in the presentation of synovial sarcoma in children: a plea for greater awareness.

We have analysed the pattern of symptoms in patients presenting with synovial sarcoma to identify factors which led to long delays in diagnosis. In 35 children, the early symptoms and the results of clinical and radiological investigation were reviewed, along with the presumed diagnoses. The duration of symptoms was separated into patient delay and doctor delay. Only half of the patients had one or more of the four clinical findings suggestive of sarcoma according to the guidance of the National Institute for Clinical Excellence at the onset of symptoms. Of the 33 children for whom data were available, 16 (48.5%) presented with a painless mass and in ten (30.3%) no mass was identified. Seven (21.2%) had an unexplained joint contracture. Many had been extensively investigated unsuccessfully. The mean duration of symptoms was 98 weeks (2 to 364), the mean patient delay was 43 weeks (0 to 156) and the mean doctor delay was 50 weeks (0 to 362). The mean number of doctors seen before referral was three (1 to 6) and for 15 patients the diagnosis was obtained after unplanned excision. Tumours around the knee and elbow were associated with a longer duration of symptoms and longer doctor delay compared with those at other sites. Delays did not improve significantly over the period of our study of 21 years, and we were unable to show that delay in diagnosis led to a worse prognosis. Our findings highlight the variety of symptoms associated with synovial sarcoma and encourage greater awareness of this tumour as a potential diagnosis in childhood.

Elastofibroma dorsi: an uncommon benign pseudotumour.

Elastofibroma dorsi is an uncommon benign soft tissue pseudotumour usually located at the lower pole of the scapula, deep to serratus anterior, and often attached to the periosteum of the ribs, presenting with long history of swelling and occasionally pain and discomfort. This lesion is usually seen in patients over the age of 50 years and is not uncommonly mistaken as a malignant tumour because of its size and location deep to the periscapular muscles. Review of the orthopaedic oncology database of 17 500 patients revealed that there were 15 patients with elastofibroma dorsi. There were 12 males and 3 females, mean age at diagnosis of 68.4 years range 51-79 years. The diagnosis was confirmed by MRI in 3 patients, excision biopsy in 3 patients, trucut biopsy in 8 patients and open biopsy in 1 patient. Eight patients had excision of the lesion which was symptomatic. There have been no recurrences. We highlight the clinical and radiological presentation of elastofibroma dorsi to increase awareness of its existence and management.

The effect of an unplanned excision of a soft-tissue sarcoma on prognosis.

We investigated whether our policy of routine re-excision of the tumour bed after an unplanned excision of a soft-tissue sarcoma was justified. Between April 1982 and December 2005, 2201 patients were referred to our hospital with the diagnosis of soft-tissue sarcoma, of whom 402 (18%) had undergone an unplanned excision elsewhere. A total of 363 (16.5%) were included in this study. Each patient was routinely restaged and the original histology was reviewed. Re-excision was undertaken in 316 (87%). We analysed the patient, tumour and treatment factors in relation to local control, metastasis and overall survival. Residual tumour was found in 188 patients (59%). There was thus no residual disease in 128 patients of whom 10% (13) went on to develop a local recurrence. In 149 patients (47%), the re-excision specimen contained residual tumour, but it had been widely excised. Local recurrence occurred in 30 of these patients (20%). In 39 patients (12%), residual tumour was present in a marginal resection specimen. Of these, 46% (18) developed a local recurrence. A final positive margin in a high-grade tumour had a 60% risk of local recurrence even with post-operative radiotherapy. Metastases developed in 24% (86). The overall survival was 77% at five years. Survival was related to the grade of the tumour and the finding of residual tumour at the time of re-excision. We concluded that our policy of routine re-excision after unplanned excision of soft-tissue sarcoma was justified in view of the high risk of finding residual tumour.