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Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XIV | Pages 1 - 1
1 Apr 2012
Smith G Johnson G Pirrie S Stocken D Billingham L Grimer R
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Bone and Soft Tissue Sarcomas represent approximately 1% of all malignant tumours. Delays in diagnosis are frequent and the average size of Sarcomas at diagnosis has averaged 10cm for many years. In 1999 guidance was produced by NICE with the aim of leading to the earlier diagnosis of common cancers – including Sarcomas. We have attempted to analyze whether this guidance has had any impact on either the size of the tumours at diagnosis or the symptom duration prior to diagnosis experienced by the patients. Data for patients referred to the Royal Orthopaedic Hospital in Birmingham between 1992 and 2007 with Bone Sarcomas (n=1592) and Soft Tissue Sarcomas (n=2004) were analysed to determine the effect of the guidance. For Bone Sarcomas the mean size of the tumours decreased from 11.2cm prior to the guidance to 10.7cm after the guidance but the change was not statistically significant (p=0.09). The mean duration of symptoms increased from 18 to 21.2 weeks (p=0.01). For Soft Tissue Sarcomas, mean size fell from 10.8cm to 9.5cm (p<0.001), however the duration of symptoms actually increased from 27.3 to 32.1 weeks (p=0.01). Statistical modelling using restricted cubic splines confirmed these trends in the data. These results show that whilst there may have been a slight improvement in the size at diagnosis of Soft Tissue Sarcomas, overall most patients still experience a long delay between the onset of symptoms and diagnosis and commencement of treatment. It is difficult to conclude that the early diagnosis guidance produced in 1999 has had a significant effect on the basis of this study. Strategies to improve awareness of the symptoms and clinical features of Bone and Soft Tissue Sarcomas are still urgently required


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_IV | Pages 36 - 36
1 Mar 2012
Smith G Johnson G Grimer R
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Objective. Sarcomas are a rare group of tumours, which pose numerous problems regarding correct diagnosis and appropriate management. This study aimed to examine whether symptom duration and tumour size at diagnosis have changed over time, using size and symptom duration as methods of comparison. Methods. All patients diagnosed with sarcoma were identified retrospectively from a prospective database from 1963 to 2005. Demographic data concerning age at diagnosis, sex and diagnosis were recorded. Data were also collected on duration of symptoms and size of tumour at diagnosis. Symptom duration is defined as the length of time the patient was experiencing symptoms for until the date of diagnosis. Changes in size and symptom duration over time were compared by grouping date of diagnosis into time periods and comparing them against each other using non-parametric statistical analysis. Results. 4560 met our inclusion and exclusion criteria, with 2492 Bone Sarcomas (BS) and 2068 Soft Tissue Sarcomas (STS). Overall analysis of both BS and STS showed that size at diagnosis has decreased and symptom duration has increased. Analysis of STS showed that size has decreased over time, particularly following the introduction of referral guidelines in 1999 (median size pre 99 = 10cm, post = 8cm, Mann-Whitney-U test p=0.000 to 3 sig. fig.), but symptom duration has remained unaltered over time. Duration of symptoms reported for BS has significantly increased over time; however, size at diagnosis has remained unchanged over time. Conclusions. It appears from this study that STS are being diagnosed more quickly and that this is most likely due to the introduction of specific STS guidelines in 1999. This result is particularly heartening, and should encourage further promotion of such guidelines. Awareness of BS still appears to be low as size at diagnosis remains unchanged over time


Orthopaedic Proceedings
Vol. 92-B, Issue SUPP_IV | Pages 597 - 597
1 Oct 2010
Rodrigues LM Balacò I Lucas M Matos G Pombo R
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Introduction: Malignant bone tumors are rare. In a sample of 1000 pediatric tumors diagnosed in our hospital only 4% were primary bone tumors. Material and Methodology: The authors present a series of Primary Malignant Bone Tumors, in children and adolescents treated in their Department, referred to a period of 14 years (1991–2004). It’s a series of 45 cases, of which 41 were evaluated. There were excluded 3 malignant low-grade osteosarcomas and 1 Askin tumor (thoracic PNET). The authors evaluated 24 Osteosarcomas, 14 Ewing Sarcomas and 3 PNET. The cases correspond to a population of 24 girls and 17 boys. The study correlate survival rate with tumor histological characteristics, size, stage, chemotherapy protocol used, the percentage of necrotic induction after neoadjuvant chemotherapy and type of surgical plane of dissection. Results: From patients with high-grade osteosarcomas 71,2% are alive and without disease, with a minimum follow-up of 4 years (1 case) and a maximum of 17 years. On the Ewing Sarcomas/PNET the survival rate is 76%, with the same follow-up period. Discussion and Conclusions: Due to the improvement of imaging techniques a fast diagnostic orientation is possible. The stage evaluation, combined with chemo and radiotherapy advances, as well as the progress of the surgical techniques to preserve limbs, together contribute to a better prognosis of the disease. The high survival rates permit to face this pathology as a chronic disease


Orthopaedic Proceedings
Vol. 96-B, Issue SUPP_10 | Pages 30 - 30
1 Jul 2014
McGoldrick N Butler J Sheehan S Dudeney S O'Toole G
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The purpose of this study is to present a series of soft tissue sarcomas requiring complex vascular reconstructions, and to describe their management and outcomes. Soft tissue sarcomas are rare mesodermal malignancies accounting for approximately 1% of all cancers diagnosed annually. Sarcomas involving the pelvis and extremities are of particular interest to the orthopaedic surgeon. Tumours that encase and invade large calibre vascular structures present a major surgical challenge in terms of safety of excision with acceptability of surgical margins. Technical advances in the fields of both orthopaedic and vascular surgery have resulted in a trend towards limb salvage with vascular reconstruction in preference to amputation. Limb-salvage surgery is now feasible due to the variety of reconstructive options available to the surgeon. Nevertheless, surgery with concomitant vascular reconstruction is associated with higher rates of complications including infection and amputation. We present a case series of soft tissue sarcomas with vascular compromise, requiring resection and vascular reconstruction. We treated four patients (n = 4, three females, and one male) with soft tissue masses, which were found to involve local vascular structures. Histology revealed leiomyosarcoma (n = 2) and alveolar soft part sarcomas (n = 2). Both synthetic graft and autogenous graft (long saphenous vein) techniques were utilised. Arterial reconstruction was undertaken in all cases. Venous reconstruction was performed in one case. One patient required graft thrombectomy at one month post-operatively for thrombosis. We present a series of complex tumour cases with concomitant vascular reconstructions drawn from our institution's experience as a national tertiary referral sarcoma service


Orthopaedic Proceedings
Vol. 88-B, Issue SUPP_II | Pages 300 - 301
1 May 2006
Babu L Nandhara G Baskaran K Kumar P Ng A Paul A
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Background: To look into the incidence of lymphatic spread in Soft Tissue Sarcomas (STS) of the extremities and its relevance to the patient’s prognosis. Patients & Methods: Retrospective review of 96 patients over a period of 5 years from 1999 to 2004 with a minimum follow up of 8 months. Complete data of every patient was reviewed with particular emphasis on lymph node and distant metastasis. All the patients were seen by one consultant and the histology reports were given by one Pathologist. 2 consultant radiologists were also involved in giving reports. Results: There were 39 males and 57 females with an average age of 51 years. The average duration of swelling at presentation was 6 months. There was a strong family history of cancer in first degree relatives in 23 patients (24%). Liposarcoma was the commonest tumour (22) followed by Leiomyosarcoma (19), Fibro sarcoma (14), Synovial Sarcoma (12), Rhabdomyosarcoma (10), Histiocytoma (9) and other rare sarcomas. The Trojani grade of the tumours was Grade 1 = 36, Grade 2 = 39 & Grade 3 = 21. No metastasis (mets) were found during pre op. screening in 71 patients (74%) while 11 (12%) had lung mets, 9 (10 %) had lymph node involvement and 5 had liver involvement (4 %). 4 had multiple organ involvement on presentation. All except 6 patients had either wide local or radical excision of the tumour. The average interval between presentation and definitive treatment was 28 days. 9 of the patients with lymph node mets underwent nodal clearance during primary surgery. 2 turned out to be reactive hyperplasia while 7 proved to be malignant. 22 of the 71 patients (31 %) with no mets pre op. developed mets during follow up at an average duration of 11 months (11 to lungs, 8 to regional lymph nodes, 2 to liver and one to bone. 4 patients had multiple mets) In addition to this, there was local recurrence in 12 patients of whom 9 had incomplete excision during primary surgery. Of the total 15 patients who had proven lymph node mets, 5 came from Rhabdomyosarcoma, 4 from Leiomyosarcoma, 3 each from Lipo & Synovial sarcoma. The average life span in patients with lymph node involvement was 13 months in total when compared to 31 months for others. As on 31-3-2005, 39 were dead and the rest were alive. The average life span of the patients who died from the time of confirmed diagnosis was 23 months. The average life span for Rhabdomyosarcoma was 8 months, Histiocytoma was 12 months, Liposarcoma was 19 months, Leiomyosarcoma was 28 months and Synovial sarcoma was 36 months. Patients with Trojani grade 3 STS died at an average of 9 months when compared to 38 months for grade 1. Discussion: There is a 16% spread to regional lymph nodes. It appears that lymph node involvement is indicative of micrometastatic disease elsewhere. Excision of the lymph nodes during primary surgery did not improve the life expectancy. Conclusion:. Lymph node involvement is a poor prognostic sign. While removal of clinically suspicious lymph nodes is reasonable, there appears to be little justification for treating clinically uninvolved draining regional lymph nodes. Therapeutic lymph node dissection might be indicated as part of the palliative management. The presence of regional lymph node metastasis at any time should be interpreted as an expression of systemic tumour spread and treated palliatively only


Bone & Joint Research
Vol. 10, Issue 9 | Pages 602 - 610
24 Sep 2021
Tsoi KM Gokgoz N Darville-O'Quinn P Prochazka P Malekoltojari A Griffin AM Ferguson PC Wunder JS Andrulis IL

Aims

Cell-free DNA (cfDNA) and circulating tumour DNA (ctDNA) are used for prognostication and monitoring in patients with carcinomas, but their utility is unclear in sarcomas. The objectives of this pilot study were to explore the prognostic significance of cfDNA and investigate whether tumour-specific alterations can be detected in the circulation of sarcoma patients.

Methods

Matched tumour and blood were collected from 64 sarcoma patients (n = 70 samples) prior to resection of the primary tumour (n = 57) or disease recurrence (n = 7). DNA was isolated from plasma, quantified, and analyzed for cfDNA. A subset of cases (n = 6) underwent whole exome sequencing to identify tumour-specific alterations used to detect ctDNA using digital droplet polymerase chain reaction (ddPCR).


Orthopaedic Proceedings
Vol. 104-B, Issue SUPP_11 | Pages 29 - 29
1 Nov 2022
Khan S Kapoor L Kumar V
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Abstract

Background

Reconstruction following resection of sarcomas of the upper extremity with methods described in the prevalent literature may not be possible in few selected cases. We describe Surgical Phocomelia or Phoco-reduction as a method of limb salvage in such cases of extensive sarcomas of the upper limb with its functional and oncological outcomes.

Methods

Evaluation of functional and oncological outcomes was performed for 11 patients who underwent surgical phocomelia or phocoreduction for extensive sarcomas of the upper limb between 2010 and 2019.


Orthopaedic Proceedings
Vol. 104-B, Issue SUPP_13 | Pages 94 - 94
1 Dec 2022
Lazarides A Novak R Burke Z Gundavda M Ghert M Rose P Houdek M Wunder JS Ferguson P Griffin A Tsoi K
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Radiation induced sarcoma of bone is a rare but challenging disease process associated with a poor prognosis. To date, series are limited by small patient numbers; data to inform prognosis and the optimal management for these patients is needed. We hypothesized that patients with radiation-induced pelvic bone sarcomas would have worse surgical, oncologic, and functional outcomes than patients diagnosed with primary pelvic bone sarcomas

This was a multi-institution, comparative cohort analysis. A retrospective chart review was performed of all patients diagnosed with a radiation-induced pelvic and sacral bone sarcoma between January 1st, 1985 and January 1st, 2020 (defined as a histologically confirmed bone sarcoma of the pelvis in a previously irradiated field with a minimum 3-year interval between radiation and sarcoma diagnosis). We also identified a comparison group including all patients diagnosed with a primary pelvic osteosarcoma/spindle cell sarcoma of bone (i.e. eligible for osteosarcoma-type chemotherapy) during the same time interval. The primary outcome measure was disease-free and overall survival.

We identified 85 patients with primary osteosarcoma of the pelvis (POP) and 39 patients with confirmed radiation induced sarcoma of the bony pelvis (RISB) undergoing surgical resection. Patients with RISB were older than patients with POP (50.5 years vs. 36.5 years, p67.7% of patients with POP underwent limb salvage as compared to 77% of patients with RISB; the type of surgery was not different between groups (p=.0.24). There was no difference in the rate of margin positive surgery for RISB vs. POP (21.1% vs. 14.1%, p=0.16). For patients undergoing surgical resection, the rate of surgical complications was high, with more RISB patients experiencing complications (79.5%) than POP patients (64.7%); this approached statistical significance (p=0.09).

15.4% of patients with RISB died perioperative period (within 90 days of surgery) as compared to 3.5% of patients with POP (p= 0.02). For patients undergoing surgical resection, 5-year OS was significantly worse for patients with RISB vs. POP (27.3% vs. 47.7%, p=0.02). When considering only patients without metastatic disease at presentation, a significant difference in 5-year survival remains for patients with RISB vs. POP (28.6% vs. 50%, p=0.03) was a trend towards poorer 5-year DFS for patients with RISB vs. POP (30% vs. 47.5%), though this did not achieve statistical significance (p=0.09).

POP and RISB represent challenging disease processes and the oncologic outcomes are similarly poor between the two; however, the disease course for patients with RISB appears to be worse overall. While surgery can result in a favorable outcome for a small subset of patients, surgical treatment is fraught with complications.


The Bone & Joint Journal
Vol. 105-B, Issue 6 | Pages 696 - 701
1 Jun 2023
Kurisunkal V Morris G Kaneuchi Y Bleibleh S James S Botchu R Jeys L Parry MC

Aims

Intra-articular (IA) tumours around the knee are treated with extra-articular (EA) resection, which is associated with poor functional outcomes. We aim to evaluate the accuracy of MRI in predicting IA involvement around the knee.

Methods

We identified 63 cases of high-grade sarcomas in or around the distal femur that underwent an EA resection from a prospectively maintained database (January 1996 to April 2020). Suspicion of IA disease was noted in 52 cases, six had IA pathological fracture, two had an effusion, two had prior surgical intervention (curettage/IA intervention), and one had an osseous metastasis in the proximal tibia. To ascertain validity, two musculoskeletal radiologists (R1, R2) reviewed the preoperative imaging (MRI) of 63 consecutive cases on two occasions six weeks apart. The radiological criteria for IA disease comprised evidence of tumour extension within the suprapatellar pouch, intercondylar notch, extension along medial/lateral retinaculum, and presence of IA fracture. The radiological predictions were then confirmed with the final histopathology of the resected specimens.


The Bone & Joint Journal
Vol. 104-B, Issue 2 | Pages 290 - 296
1 Feb 2022
Gosheger G Ahrens H Dreher P Schneider KN Deventer N Budny T Heitkötter B Schulze M Theil C

Aims

Iliosacral sarcoma resections have been shown to have high rates of local recurrence (LR) and poor overall survival. There is also no universal classification for the resection of pelvic sarcomas invading the sacrum. This study proposes a novel classification system and analyzes the survival and risk of recurrence, when using this system.

Methods

This is a retrospective analysis of 151 patients (with median follow-up in survivors of 44 months (interquartile range 12 to 77)) who underwent hemipelvectomy with iliosacral resection at a single centre between 2007 and 2019. The proposed classification differentiates the extent of iliosacral resection and defines types S1 to S6 (S1 resection medial and parallel to the sacroiliac joint, S2 resection through the ipsilateral sacral lateral mass to the neuroforamina, S3 resection through the ipsilateral neuroforamina, S4 resection through ipsilateral the spinal canal, and S5 and S6 contralateral sacral resections). Descriptive statistics and the chi-squared test were used for categorical variables, and the Kaplan-Meier survival analysis were performed.


Orthopaedic Proceedings
Vol. 102-B, Issue SUPP_8 | Pages 4 - 4
1 Aug 2020
Basile G Alshaygy I Mattei J Griffin A Ferguson P Wunder JS
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Lymph node metastasis are a rare occurrence in soft tissue sarcomas of the extremity, arising in less than 5% of patients. Few studies have evaluated the prognosis and survival of patients with a lymph node metastasis. Early reports compared lymph node involvement to lung metastasis, while others suggested a slightly better outcome. The purpose of this study was to evaluate the impact of lymph node metastasis on patient survival and to investigate the histologic and clinical features associated with lymph node involvement.

A retrospective review was done of the prospectively collected soft tissue sarcoma database at our institution. Two thousand forty-five patients had surgery for soft tissue sarcoma of an extremity between January 1986 and August 2017. Included patients either presented with a synchronous lymph node metastasis or were diagnosed with a lymph node metastasis after their initial treatment. Demographic, treatment, and outcome data for patients with lymph node involvement were obtained from the clinical and radiographic records.

Lymph node metastases were identified as palpable adenopathy by physical examination and were further characterized on cross-sectional imaging by computed tomography (CT) or magnetic resonance imaging (MRI) scans. All cases were confirmed by pathologic examination of biopsy specimens. A pathologist with expertise in sarcoma determined the histologic type and graded tumors as 1, 2, or 3.

One hundred eighteen patients with a mean age of 55.7 (SD=18.9) were included in our study. Seventy-two (61.3%) out of 119 patients were male. Thirty six patients (57.1%) had lymph node involvement at diagnosis. The mean follow-up from the date of the first surgery was 56.3 months. The most common histological diagnoses were Malignant fibrous histiocytoma (35) and liposarcoma (12). Ninety eight patients (89%) underwent surgical treatment of the lymph node metastasis while 21 (17.6%) were treated with chemotherapy and/or radiation therapy. The mean survival was 52.6 months (range 1–307).

Our results suggest that patients with a lymph node metastasis have a better prognosis than previously described. Their overall survival is superior to patients diagnosed with lung metastasis. A signifant proportion of patients may expect long term survival after surgical excision of lymph node metastasis. Furthermore, our study also indicates that different histological subtypes such as liposarcoma or malignant peripheral nerve sheath tumor (MPNST) may also be responsible for lymph node metastasis. Additional studies to further improve the treatment of soft tissue sarcoma nodal metastasis are warranted.


Orthopaedic Proceedings
Vol. 103-B, Issue SUPP_3 | Pages 62 - 62
1 Mar 2021
Lee J Perera J Trottier ER Tsoi K Hopyan S
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Paediatric bone sarcomas around the knee are often amenable to either endoprosthetic reconstruction or rotationplasty. Cosmesis and durability dramatically distinguish these two options, although patient-reported functional satisfaction has been similar among survivors. However, the impact on oncological and surgical outcomes for these approaches has not been directly compared.

We retrospectively reviewed all wide resections for bone sarcoma of the distal femur or proximal tibia that were reconstructed either with an endoprosthesis or by rotationplasty at our institution between June 2004 and December 2014 with a minimum two year follow-up. Pertinent demographic information, surgical and oncological outcomes were reviewed. Survival analysis was performed using the Kaplan-Meier method with statistical significance set at p<0.05.

Thirty eight patients with primary sarcomas around the knee underwent wide resection and either endoprosthetic reconstruction (n=19) or rotationplasty (n=19). Groups were comparable in terms of demographic parameters and systemic tumour burden at presentation. We found that selection of endoprosthetic reconstruction versus rotationplasty did not impact overall survival for the entire patient cohort but was significant in subgroup analysis. Two-year overall survival was 86.7% and 85.6% in the endoprosthesis and rotationplasty groups, respectively (p=0.33). When only patients with greater than 90% chemotherapy-induced necrosis were considered, overall survival was significantly better in the rotationplasty versus endoprosthesis groups (100% vs. 72.9% at two years, p=0.013). Similarly, while event-free survival was not affected by reconstruction method (60.2% vs. 73.3% at two years for endoprosthesis vs rotationplasty, p=0.27), there was a trend towards lower local recurrence in rotationplasty patients (p=0.07). When surgical outcomes were considered, a higher complication rate was seen in patients that received an endoprosthesis compared to those who underwent rotationplasty. Including all reasons for re-operation, 78.9% (n=15) of the endoprosthesis patients required a minimum of one additional surgery compared with only 26.3% (n=5) among rotationplasty patients (p=0.003). The most common reasons for re-operation in endoprosthesis patients were wound breakdown/infection (n=6), limb length discrepancy (n=6) and periprosthetic fracture (n=2). Excluding limb length equalisation procedures, the average time to re-operation in this patient population was 5.6 months (range 1 week to 23 months). Similarly, the most common reason for a secondary procedure in rotationplasty patients was wound breakdown/infection, although only two patients experienced this complication. Average time to re-operation in this group was 23.8 months (range 5 to 49 months).

Endoprosthetic reconstruction and rotationplasty are both viable limb-salvage options following wide resection of high-grade bony sarcomas located around the knee in the paediatric population. Endoprosthetic reconstruction is associated with a higher complication rate and may negatively impact local recurrence. Study of a larger number of patients is needed to determine whether the reconstructive choice affects survival.


Orthopaedic Proceedings
Vol. 90-B, Issue SUPP_I | Pages 88 - 88
1 Mar 2008
Younge D Rabbani S Ilyas I
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Twenty cases of malignant sarcomas of the foot and ankle included: four osteosarcomas, three Ewing’s sarcomas, three chondrosarcomas, three fibrosarcomas, five synovial cell sarcomas, one clear cell sarcoma and one malignant schwannoma.

Five-year survival was: three of the nine who had B-K amputation, five of the nine who had more distal amputation or local resection, for a total of eight of eighteen.

More distal amputation than B-K or local resection was offered if plantar sensation and stability of the foot could be retained, and was accepted by half of the patients. There were no local recurrences of eighteen operated.

Twenty cases of malignant sarcomas of the foot and ankle seen over a ten-year period from 1985 to 1995 were reviewed retrospectively.

Eleven tumors arose from bone and nine from soft tissue. The bone tumors were: four osteosarcomas, three Ewing’s sarcomas, three chondrosarcomas, and one fibrosarcoma. The soft tissue sarcomas were: five synovial cell sarcomas, two fibrosarcomas, one clear cell sarcoma and one malignant schwannoma. The average age for all patients was twenty-four years.

Two patients presented with chest metastases, both had palliative radiation and one had palliative B-K amputation.

The surgical treatment given for the eighteen non-metastatic cases was: B-K amputation nine, Symes, Chopart, or ray amputation six, and wide excision of the tumor three patients.

Five-year survival was eight of eighteen: three of the nine who had B-K amputation, four of the six who had more distal amputation, and one of the three who had local resection. There were no local recurrences, all deaths were from distant metastases.

Although B-K amputation is often recommended, in our series more distal amputation or local resection was offered for the non-metastatic patient if plantar sensation and stability of the foot could be retained. This gave good function in nine of eighteen patients, with no local recurrences.

A long duration of symptoms before presentation or referral influenced the outcome of the disease.

In selected patients, amputation more distal than B-K level or wide local resection can give good function without compromising the prognosis.


Orthopaedic Proceedings
Vol. 86-B, Issue SUPP_II | Pages 133 - 134
1 Feb 2004
Gracia-Alegría I Pérez-Moreno F Peirò-Ibáñez A Doncel-Cabot A Majò-Buigas J
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Introduction and Objectives: Extraskeletal osseous sarcomas are very rare high-grade tumours. They include osteosarcoma, chondrosarcoma, and Ewing’s soft tissue sarcoma and their respective variants. We present a retrospective study of 25 cases covering the period from December 2002 to January 2003.

Materials and Methods: This study of 25 cases shows that this heterogeneous group of soft tissue sarcomas primarily affects adults (mean age 50.68; range 17 to 70 years). The thigh (36.60%) and the elbow-forearm (18.18%) are the most common locations for these tumours. In these cases, 14 chondrosarcomas, 8 osteosarcomas, and 3 Ewing’s sarcomas were diagnosed. Mean follow-up time was 48.9 months with a range of 16 to 197 months. There were 16 surgeries performed with wide or compartmental margins, while in the 5 remaining cases, amputations were performed, and one case of Ewing’s sarcoma of the pelvis was treated by means of chemotherapy and radiation therapy, as it was considered non-resectable. All cases of osteosarcoma and Ewing’s sarcoma were treated by means of pre- and postoperative chemotherapy and postoperative radiation therapy.

Results: The postoperative duration of symptoms ranged from 2 weeks to 6 years with a mean of 6 months. Local recurrence following surgery with wide or compartmental margens occurred in 8 cases, representing 35.45% (3 chondrosarcomas, 4 osteosarcomas, and 1 Ewing’s sarcoma). The total number of cases surviving without recurrence is 14 (63.6%), surviving cases with one or more recurrences is 5 (22.72%), one case is alive with metastasis (4.59%), and 3 sarcomas (2 ulcerated upon admission) had disseminated and the patients died (10.05%).

Discussion and Conclusions: All these tumours were high-grade sarcomas. The most common metastatic localizations were lungs, regional ganglia, and skeleton. The recommended treatment is surgery with wide or compartmental margins, if possible in the early stages, in conjunction with chemotherapy and radiation therapy. The worst prognosis corresponds to the osteosarcoma.


The Journal of Bone & Joint Surgery British Volume
Vol. 47-B, Issue 3 | Pages 542 - 547
1 Aug 1965
Singh AD Scudder IB


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXX | Pages 32 - 32
1 Jul 2012
Cosker T White HB James A Athanisou N Whitwell D Gibbons M
Full Access

Introduction

Primary soft tissue sarcomas of the extremities are uncommon. Many such lesions will present to specialists in other clinics such as specialist Hand or Foot and Ankle clinics. Many are of a small size at presentation and may appear to be alternate, more common pathology.

Methods

We collected data from all those patients with acral soft tissues sarcomas and referred to the Oxford Sarcoma Service, Nuffield Orthopaedic Centre, Oxford over a thirteen year period from 1997 – 2010. Data were collected regarding the primary suspected diagnosis, the final diagnosis, the referral route and whether patients had undergone previous inadvertent excision.


Orthopaedic Proceedings
Vol. 90-B, Issue SUPP_II | Pages 401 - 401
1 Jul 2008
Murata H Kalra S Ahrens H Abudu A Grimer R Carter S Tillman R
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99 patients with new diagnosed soft tissue sarcomas involving the pelvic region were studied to determine the outcome and prognostic factors for survival and local recurrence.

The mean age at diagnosis was 57 years. There were 55 males and 44 females. The mean tumor size was 12cm. The tumor was deep in 79 patients and superficial in 20. Surgical treatment was excision in 93 patients and hindquarter amputation in 6 patients. Histological grade was low grade in 23 and high grade in 75 patients. 7% of the patients had metastases at presentation.

The 5 year overall survival was 57% and local recurrence occurred in 22% of the patients. The risk of inadequate surgical margins in patients with tumors within the pelvic brim was 50% compared to 18% for those with tumors located outside the pelvic brim. The significant predictors of local recurrence were inadequate margins and location of the tumor within the pelvic brim. Tumor size, grade and depth did not influence development of local recurrence. Significant predictors of survival included metastases at presentation, tumor grade and depth. The cumulative 5 year survival for patients with deep high grade tumors, deep low grade tumors, superficial high grade and superficial low grade tumors were 45%, 74%, 63% and 100% respectively (p=0.01). The 5-year overall survival was 66% in those patients without local recurrence compared to 37% in those who develop local recurrence (p=0.005). Multivariate analysis revealed that development of local recurrence was the most important determinant of overall and metastases free survival.

We conclude that patients with pelvic soft tissue sarcoma who develop local recurrence have an extremely poor prognosis. Patients with high grade and inadequate surgical margins represent a particular group with very high risk of metastases and death even with radiotherapy and perhaps should be considered for other adjuvant treatment.


Orthopaedic Proceedings
Vol. 88-B, Issue SUPP_I | Pages 189 - 189
1 Mar 2006
Balaco I Casanova J Matos P Teixeira C Tavares P Garcia H Laranjo A Portela J
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In the last ten years, 172 bone sarcomas and 126 soft tissue sarcomas, were treated by our Orthopaedic Oncology Unit. From those patients 49 (16.5%) meet criteria for pulmonary metastasis resection. Patient group were 27 males (55%) and 22 females (45%); median age 28.8 years (range 12–71); histology of the primary tumours were in 33 cases bone lesions (67%): 17 osteosarcomas, 8 Ewing sarcomas, 3 Malignant Fibrous Histiocytoma (MFH) and 1 Giant Cell Tumor and 16 cases (23%) for soft tissue tumours: 5 synovial sarcoma, 3 schwannoma and MFH, 2 leiomiosarcoma and liposarcoma and 1 rhabdomyosarcoma. From those sarcomas 8 (17.8%) were metastatic at presentation. Treatment included surgical resections of the secondary lesions and chemotherapy. The lung metastasis were bilateral in 20 cases (41%) and unilateral in 29 cases (59%). The number of metastasis range from one to 24 and the surgical sessions for each patient were from one to eight. The median disease free survival in the patients with no metastatic lesions at presentation was 17.8m (range 2–88). From this group of patients 25 (51%) were dead of disease (DOD), and 24 (49%) were survivors (median follow-up 42 month: range 12–120 month), being 14 with no evidence of disease (NED) and 10 alive with disease (AWD).

The authors perform a statistical analysis relating survival with local recurrence and the amount of necrosis in the surgical specimen.


Orthopaedic Proceedings
Vol. 86-B, Issue SUPP_III | Pages 299 - 299
1 Mar 2004
Isidro G Doncel A Roca D Maj— J C‡ceres E
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Aims: Extraskeletal bone tumours are rare and high-grade tumours including osteosarcoma (OS), chondrosarcoma (CHO) and Ewingñs sarcoma (EW) of the soft tissues and its variants. A retrospective study of 22 cases were made in our Hospital in the period from 1983–2000. Methods: The study of 22 cases revealed that this tumours affect adults (median age 50.86; range 17 to 70 years). The thigh (36.36%) and the arm-elbow (18.18%) were the most common tumour locations. For diagnostic type of tumours 11 were CHO, 8 OS and 3 EW. The median follow-up was 48.9 months with a range 6 to a97 months. All of the cases of OS and EW were treated with preoperative chemotherapy and postoperative radiotherapy. Results: The preoperative duration of symptoms was ranged from 6 weeks to 6 years (median 6 months). Local recurrences after wide and radical surgery developed in 8 cases Ð35.45%- (3 CHO, 4 OS and 1 EW) and distant metastases developed in 3 cases (13.18%). 14 cases were alive with no evidence of recurrence (63.6%), 5 were alive with one or more recurrences (22.72%), 1 were alive with metastases (4.59%) and 2 ulcerated tumours were exitus form metastases (10.05%). Five amputations were performed. Conclusions: 1. All cases were high-grade tumours. 2. The prevailing sites of metastases were the lung, the regional lymph nodes and the skeleton. 3. Cure may be achieved by wide or radical local excision of the tumour at and early stage of the disease (combined with radiation and chemotherapy).


Orthopaedic Proceedings
Vol. 93-B, Issue SUPP_I | Pages 79 - 79
1 Jan 2011
White HAB Giele H Critchley P Whitwell D Gibbons CLM
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Introduction: Acral sarcomas present as small tumours to specialised orthopaedic services. There is a high incidence of inadequate resection compromising clinical and functional outcome.

It is advised that lumps which are greater than 5cm should be referred to a sarcoma centre for management and that small lesions cause less harm with unplanned excision.

Method: Data from the Oxford Registry was collated of all those patients with acral sarcomas who underwent surgery in a period from 1997 – 2008 at The Nuffield Orthopaedic Centre including site, size, histological subtype, of tumours, history of previous surgical excisions, staging, and functional scores.

Results: 27 patients were treated (17 foot and ankle cases, 10 hand tumours). The commonest acral lesions were synovial sarcomas (30%).

Of the hand patients 7 of the 10 patients had wide excision of the lesion with 3 an amputation. 5 of the cases were for inadequate previous excision (50%).

7 of the 17 in the foot and ankle group underwent amputation (41%) the rest wide excision. 29% of cases were for second time surgery due to inadequate previous excision.

Discussion: Our study shows that up to 50% of patients with acral tumours are undergoing further surgery due to previous inadequate excision. The belief that 5cm should be a benchmark for specialist referral should not be applied to such lesions.