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Background: Dedifferentiated chondrosarcomas (CS) are a high-grade variant of CS that confers a 5-year survival of around 10–24%. Dedifferentiated CS arising from the pelvis confers an even worse prognosis. Questions: (1) What is the prognosis of patients with dedifferentiated CS of the pelvis? (2) Do wide margins or type of surgical intervention influence outcome? (3) Does the use of adjuvant therapy affect outcome? Methods: Patients were retrospectively reviewed from a prospectively collated musculoskeletal oncology database from 1995 to 2016. Thirty-one cases of dedifferentiated CS arising from the pelvis were included. Wide margins were defined as greater than 4 mm. The mean age was 55.6 years (range 33 to 76 years) and there were 19 males (61.3%) and 12 females (38.7%). Results: The disease presented at a locally or systemically advanced stage in 13 patients (41.9%). Eighteen patients (58.1%) underwent surgery with curative intent. Overall survival at 12 months was 15.4% for patients treated with palliative intent and 50% for those treated with surgery. In the surgical group, there were higher rates of disease-free survival in patients who underwent hindquarter amputation and those who received wide surgical margins (p = 0.047 and p = 0.019, respectively). Those who underwent hindquarter amputation were more likely to achieve wide margins (p = 0.05). Time to recurrent disease (local or systemic) was always less than 24 months. No hindquarter amputation for recurrent disease resulted in disease-free survival. No patient who received adjuvant therapy for palliative or recur- rent disease had disease control. Conclusions: Pelvic dedifferentiated CS often presents at an advanced local or systemic stage and confers a poor prognosis. Achieving wide surgical margins (> 4 mm) provided the highest rate of long-term disease-free survival. Fail- ing to achieve wide margins results in rapid disease recurrence, conferring deleterious consequences. Keywords: Chondrosarcoma, Dedifferentiated, Pelvis, Amputation, Limb-salvage, Sarcoma Introduction The most important factor for guiding management and Chondrosarcoma (CS) is a rare malignant bone tumour prognosis is determining the histological grade of the composed of cartilage matrix-producing cells. It is the tumour. There is a high degree of inter-observer variabil - second most common primary bone sarcoma with an ity when determining histological grade . Dedifferenti - incidence of 1 in 200,000/year. It may arise in the medul- ated CS is defined as one area of a lower grade cartilage lary cavity of bone (central CS) or secondary to a malig- tumour that lies directly adjacent to an area of high- nant transformation of a benign cartilage tumour . grade non-cartilaginous sarcoma . Only 10% of all CS dedifferentiate, which is fortunate as these high-grade tumours are associated with a 5-year survival of around 10–24% [1, 4, 5]. *Correspondence: email@example.com Chondrosarcomas, in general, are resistant to chem- Royal Orthopaedic Hospital, Birmingham B31 2AP, UK otherapy and conventional radiotherapy. Occasionally, Full list of author information is available at the end of the article © The Author(s) 2018. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creat iveco mmons .org/licen ses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creat iveco mmons .org/ publi cdoma in/zero/1.0/) applies to the data made available in this article, unless otherwise stated. Lex et al. Clin Sarcoma Res (2018) 8:23 Page 2 of 9 short-term local control can be achieved but has no Patients and methods proven benefit on distant spread or overall survival A retrospective review was conducted of a prospectively [6–8]. Consequently, surgery remains the mainstay of maintained database to identify patients with a histolog- treatment for dedifferentiated CS. Dedifferentiated ical diagnosis of dedifferentiated CS managed at a sin - CS arising from the pelvis is known to be a negative gle tertiary musculoskeletal oncology centre. Minimum prognostic factor, further lowering the survival rate [9, follow-up was 12 months or until death. 116 patients 10]. For this disease, there is limited evidence defining were diagnosed with dedifferentiated CS between 1995 the presenting disease stage, accuracy of pre-operative and 2016. diagnosis and treatment factors influencing outcome 31 patients were identified with pelvic dedifferenti - . ated CS. They had a mean age of 55.6 years (range 33 to Current treatment for pelvic dedifferentiated CS 76 years). There were 19 males (61.3%) and 12 females consists of either palliative, limb-salvage through pel- (38.7%). The ilium (P1) was involved in 51.6% (16/31) of vic resection with or without reconstruction, or limb- tumours. 48.4% of tumours (15/31) involved the periac- sacrifice with hindquarter amputation [5, 6]. Tumour etabular (P2) region either in isolation or in combination excision with wide margins should provide the best with other pelvic regions (Fig. 1). prognosis although this is often difficult due to the Local tumour staging comprised of plain radiography proximity of vital structures. This association and the and magnetic resonance imaging (MRI). Distant stag- probability of achieving wide margins with limb-sal- ing comprised of chest computed tomography (CT) and vage has yet to be described in pelvic dedifferentiated bone scintigraphy. All patients were managed by a spe- CS. cialist sarcoma multidisciplinary team (MDT). Histologi- We report our experience with dedifferentiated CS of cal diagnosis was based on biopsy material and reviewed the pelvis including diagnosis, survival and surgical out- by a specialist sarcoma pathologist prior to surgical treat- comes. The questions we attempted to answer in this ment. In 11/18 patients (61.1%) undergoing surgical study were; (1) what is the prognosis of patients with resection the histological diagnosis of a dedifferentiated dedifferentiated CS of the pelvis? (2) Do wide margins chondrosarcoma was made on the biopsy material pre- or type of surgical intervention influence outcome in operatively. In the remaining seven patients (38.9%) pre- patients for attempted curative resection? (3) Does the operative biopsy failed to accurately grade the tumour use of adjuvant therapy affect outcome? when compared to the post resection histology. Of these Fig. 1 Frequency of tumour location within the pelvis Lex et al. Clin Sarcoma Res (2018) 8:23 Page 3 of 9 seven patients, three had a biopsy that suggested a low- hemipelvectomies without reconstruction, three (23.1%) grade CS, two a high-grade CS and in two, a spindle cell had a stemmed acetabular prosthesis (“ice-cream” cone), sarcoma with no cartilaginous elements (Table 1). three (23.1%) had custom implants and one (7.7%) had Wide margins, the aim of surgery, were defined as combined internal hemipelvectomy and a proximal fem- greater than 4 mm on final pathological specimen analy - oral replacement. sis . Indications for hindquarter amputation (HQA) One patient died from a post-operative cardiac arrest were involvement of two of the following three struc- and another suffered a periprosthetic infection which tures: sciatic nerve, hip joint, external iliac vessels or was successfully treated through debridement, implant where it was deemed the safest way to achieve wide mar- retention and antibiotics. gins (Figs. 2a, b, 3a–c). Chemotherapy and radiotherapy Statistical analysis was performed using R and deducer was guided by oncologists on an individualised basis to statistical software packages, and considered statistically attempt systemic or local short-term tumour control. significant at p < 0.05. Analysis of local recurrence-free Patients were divided into palliative or surgical treat- survival (LRFS), metastasis-free survival (MFS), disease- ment groups. 13 patients (41.9%) were in the palliative free (DFS) and overall survival (OS) was performed using group. Patients were not offered surgery due to either the Kaplan–Meier survival method with 95% confidence advanced metastatic disease (in 10 patients), the tumour intervals (CI). To assess the effect of different factors on was deemed unresectable (entire hemipelvis involve- survival outcomes, the Cox proportional hazards (PH) ment, including the sacrum and sciatic notch), too model was used. medically unfit for surgery or died during neoadjuvant chemotherapy (one patient each) (Table 2). Patients in the surgical group were analysed according to the surgi- Results cal procedure and surgical margins. Palliative group 18 patients (58.1%) underwent attempted curative 41.9% of patients (n = 13) were considered incurable by treatment by surgery. 13 patients underwent limb-sal- resection of the primary tumour and were offered pal - vage surgery (LSS) and five HQA. None of the patients liative treatment only. In this group, the median survival in the surgical group had metastases at the time of diag- was 3 months (IQR 2 to 8 months); two patients (15.4%) nosis. For those treated by LSS, six (46.2%) had internal in this group survived beyond 12 months. The longest Table 1 Patients demographics and outcomes who underwent attempted curative resection Patient Age Sex Surgery Margin Correct Time to LR Time Time to death Time alive Status pre-op to metastases diagnosis 1 61 F AMPHQ Intralesional Yes 14 13 33 DOD 2 62 M AMPHQ Wide Yes 121 NED 3 52 F AMPHQ Marginal Yes 4 3 4 DOD 4 55 M AMPHQ Wide Yes 51 NED 5 55 F AMPHQ Wide Yes 12 NED 6 72 M LSS Intralesional No 0 DOD 7 51 M LSS Marginal No 2 2 5 DOD 8 49 M LSS Intralesional Yes 10 12 12 DOD 9 43 F LSS Marginal Yes 13 13 15 DOD 10 71 M LSS Marginal Yes 2 4 DOD 11 47 M LSS Intralesional No 5 5 9 DOD 12 71 F LSS Marginal No 2 3 DOD 13 44 F LSS Wide No 20 24 29 DOD 14 63 F LSS Wide Yes 100 NED 15 47 F LSS Marginal No 63 NED 16 57 F LSS Intralesional Yes 3 0 7 DOD 17 48 M LSS Intralesional No 2 11 24 AWD 18 54 M LSS Intralesional Yes 2 2 3 DOD F female, M male, AMPHQ hindquarter amputation, LSS limb salvage surgery, DOD dead of disease, NED no evidence of disease, AWD alive with disease Lex et al. Clin Sarcoma Res (2018) 8:23 Page 4 of 9 Fig. 2 Pre-operative pelvis radiograph (a) and MRI (b) of a patient who underwent HQA. In this case, there was a large tumour involving both the hip joint and femoral neurovascular bundle survival (34 months), was seen in a patient treated with palliative radiotherapy alone as they were unfit for abla - tive surgery. Curative group Of the patients (n = 18, 58.1%) who underwent attempted curative resection with wide margins, the Fig. 3 Pre-operative pelvis radiograph (a) and MRI (b) and OS at 12 months was 50.0% (95% CI 31.5%–79.4%), at post-operative radiograph (c) of a patient who underwent LSS 36 months was 29.2% (95% CI 13.4%–63.4%) (Fig. 4a). through an internal hemipelvectomy and no reconstruction (flail hip) At 12 months, the LRFS and MFS were 56.1% (95% with wide margins (> 20 mm) and long-term survival achieved CI 36.1%–87.2%) and 47.2% (95% CI 28.6%–78.1%), Lex et al. Clin Sarcoma Res (2018) 8:23 Page 5 of 9 respectively. At 36 months, the LRFS and MFS were The DFS for the LSS cohort was 15.4% (95% CI 4.3%– 32.7% (95% CI 15.2%–70.2%) and 28.3% (95% CI 13.0%– 55.0%) and 60% for the HQA group. The DFS was sig - 61.7%), respectively. The mean time to local recurrence nificantly greater in patients who underwent HQA and metastasis was 7.5 months (range 2 to 20 months) (p = 0.047) (Fig. 4b). There was no significant difference and 7.4 months (range 0.5 to 24 months), respectively. between groups in time to local recurrence or metastases There were five patients with long-term DFS (27.8%) (p = 0.776 and p = 0.914, respectively). after undergoing surgery with curative intent. There 90% of patients with recurrence developed local was no difference in age (p = 0.842), size (p = 0.191) recurrence and metastases synchronously (maximum or proportion of isolated P1 area tumours (p = 0.260) 4 months between events). One patient developed local between survivors and those with disease recurrence or recurrence at 2 months with metastases detected at death. 11 months. 3/13 (23%) patients in the LSS group underwent sec- ondary HQA for locally recurrent disease. The mean time Limb-salvage versus amputation from surgery to additional operation was 11.7 months. A Of the five patients who underwent HQA, three (60%) wide resection margin at subsequent HQA was achieved are still alive with no evidence of disease at 12, 51 and in two patients and a marginal margin in one. One patient 121 months follow-up. All three had wide margins of died from a complication of the amputation, one lived excision. The remaining two patients (40%) had mar - an additional 9 months and the other remains alive with ginal and intralesional excision margins and both devel- local and systemic disease 21 months following HQA. oped local recurrence (4 and 14 months, respectively), metastases (3 and 13 months, respectively) and died (4 Margins and 33 months, respectively) (Table 1). The type of surgery significantly affected the margins Of the 13 patients who underwent LSS, three patients achieved. HQA achieved wide margins more frequently remain alive at a mean 62.3 months since operation, than LSS (p = 0.05) (Table 3). of which, two have no evidence of disease, at 100 and Disease-free survival was greater in patients who 63 months, one had a wide and the other a marginal received wide resection margins (p = 0.019). Of the five excision, respectively. The remaining 10 patients have patients presumed cured, with no evidence of disease at died (one dying in perioperative period from car- last follow-up, four (80%) had wide resection margins, diac arrest). The LRFS and MFS at last follow-up was the last had marginal margins. Overall, 80% of patients 22.2% (95% CI 6.7%–73.8%) and 16.8% (95% CI 4.7%– who had wide margins were cured, 16.7% of patients 59.4%), respectively. The mean time to local recurrence with marginal margins were cured and no patients with was 7.1 months (range 2 to 20 months), mean time to intralesional margins were disease-free at last follow- metastases was 7.3 months (range 0 to 24 months) and up (Table 1 and Fig. 4c). the mean time to death was 8.7 months (range 0 to 29 months). Table 2 Patient demographics by treatment group Overall cohort Limb-salvage (LSS) Hindquarter Palliative p-value (between amputation LSS and HQA (HQA) groups) Patient number (n, %) 31 13 (41.9%) 5 (16.1%) 13 (41.9%) Age (mean, range) 55.6 (33–76) 55.2 (43–72) 57 (52–62) 55.5 (33–76) 0.716 Enneking stage (n, %) Stage 2b 21 (67.7%) 13 (100%) 5 (100%) 3 (23.1%) 1.000 Stage 3b (metastases) 10 (32.3%) 0 0 10 (76.9%) 1.000 Biopsy diagnosis correct (n, %) 11 (61.1%) 6 (46.1%) 5 (100%) N/A 0.036 Tumour location, solitary P1 lesions (n, %) 11 (35.5%) 1 (7.7%) 3 (60%) 7 (53.8%) 0.017 Tumour size (max diameter, cm) 110.9 (45–200) 108.8 (45–200) 117.5 (90–180) N/A 0.756 Chemotherapy (n, %) 15 (48.4%) 6 (46.1%) 2 (40%) 7 (53.8%) 0.814 Radiotherapy (n, %) 7 (22.6%) 2 (15.4%) 1 (20%) 4 (30.8%) 0.814 Surgery complications 2 (11.8%) 2 (15.4%) 0 N/A 0.352 Lex et al. Clin Sarcoma Res (2018) 8:23 Page 6 of 9 Chemotherapy and radiotherapy Patients who underwent primary HQA had higher Chemotherapy was not used routinely, but was used rates of disease-free survival than those who underwent palliatively or postoperatively following the devel- LSS, 60% vs 15.4%, respectively. This likely correlated to opment of recurrent disease. In most cases where the higher rates of wide margins achieved with HQA. In chemotherapy was used, it consisted of cisplatin and patients who had LSS, 66.7% had local recurrence and doxorubicin. One patient had neoadjuvant chemo- in the those who did not develop local recurrence, two therapy, 4 cycles of cisplatin and doxorubicin, after the are long term survivors and the other two died at 3 and initial biopsy suggested the diagnosis of spindle cell 4 months. Other studies of predominantly non-dediffer - sarcoma. Following resection, dedifferentiated CS was entiated pelvic CS have revealed satisfactory margins can confirmed. This patient had < 10% necrosis, a marginal be achieved with limb-salvage [6, 12, 13]. However, one margin and is alive at 63 months following surgery study identified a significantly higher chance of obtaining (being the only survivor with a marginal margin). a clear margin with amputation . Our data suggests Radiotherapy was used palliatively or for manage- that wide margins are more achievable with HQA, which ment of local recurrence. The radiotherapy dose ranged was translated into improved local control and survival. from 30 to 70 Grays depending on adjacent anatomical The primary operation to consider in those with dediffer - structures, tumour site and size. No patients who had entiated CS should be a HQA with wide margins to offer radiotherapy survived. patients the best chance of cure. In this series, none of the three patients who had HQA with wide margins died, compared to only three survivors in the 15 patients who Discussion had any other combination of surgery and margin type. In all chondrosarcomas, survival correlates with his- Obtaining a wide margin has been reported to be a tological grade, and the dedifferentiated CS subtype is positive prognostic indicator for non-dedifferentiated known to confer the worst prognosis [1, 9, 10, 12]. In CS [11, 14, 22]. However, it remains unclear whether accordance with other studies, dedifferentiated CS has achieving a wide margin around dedifferentiated CS a peak incidence in the 6th decade of life, a male pre- impacts local recurrence [12–14, 23]. In our series, there dilection, and a male-to-female ratio of 3:2 observed in was an 80% chance of achieving disease cure when wide our series [12–14]. margins, greater than 4 mm, were obtained. Of the five Dedifferentiated CS has been associated with a poor patients who were completely disease-free at last follow- prognosis . The best chance of cure was linked to up, four had a wide margin and the other a marginal excision of the tumour with clear margins . Achiev- margin. There were no long-term survivors in those with ing clear margins is much more difficult in the pelvis, intralesional tumour resection. even with HQA. The role of chemotherapy and radio - Another issue with pelvic dedifferentiated CS, reflect - therapy is largely palliative [7, 8, 15–18]. The presenting ing the tumour heterogeneity and large volume, is that clinical scenario and treatment of pelvic dedifferenti - 7 of the 18 curative patients did not have the correct ated CS has not been specifically examined previously, diagnosis on initial biopsy. Three of these patients were however, data extrapolated from previous studies thought to have low-grade CS and LSS was carried out clearly reveals pelvic disease confers a dreadful progno- with very close margins. Although one died postopera- sis (Table 4) [5, 9, 10, 12, 19, 20]. tively the other two both developed recurrent disease at In the present study concerning dedifferentiated 2 months. This highlights the importance of obtaining CS of the pelvis, 42% of patients presented with such wide margins in any pelvic CS. Four of the five patients advanced disease that surgery was not a viable treat- who were disease-free at last follow-up had accurate pre- ment option. Unsurprisingly, patients able to undergo operative biopsies. Saifuddin et al. have suggested tak- surgery with curative intent had greater overall survival ing biopsies from areas of reduced signal intensity on than the palliative group. The only long-term survi - T2-weighted MRI may result in higher detection rates of vors with no evidence of disease (16.1% of the cohort) dedifferentiated CS . underwent attempted curative surgical resection with For isolated local recurrence of dedifferentiated CS wide or marginal margins. treated with amputation, no patients achieved a cure (See figure on next page.) Fig. 4 a Overall survival between patients treated with either curative or palliative intent (p = 0.002). b. Disease-free survival between amputation and limb-salvage surgery (p = 0.047). AMPHQ = hindquarter amputation; LSS = Limb-salvage surgery. c. Kaplan–Meier graph comparing overall survival for patients who received intralesional and marginal resections to wide resections (p = 0.019). IL intralesional, M marginal Lex et al. Clin Sarcoma Res (2018) 8:23 Page 7 of 9 Lex et al. Clin Sarcoma Res (2018) 8:23 Page 8 of 9 Table 3 Surgical margins achieved by the operation conducted Surgical margins Overall Limb salvage Hindquarter amputation Secondary hindquarter amputation Intralesional (n,%) 7 (33.3%) 6 (46.1%) 1 (20.0%) 0 Marginal (n, %) 7 (33.3%) 5 (38.5%) 1 (20.0%) 1 (33.3%) Wide (n, %) 7 (33.3%) 2 (15.4%) 3 (60.0%) 2 (66.7%) Table 4 Survival outcomes for all patients with dedifferentiated CS of the pelvis treated with either palliative or curative intent from available literature over the last 30 years Study Number Mortality rate Time to disease Notes of patients recurrence, months Frassica et al.  23 19–21 (82.6%–91.3%) N/A Calculated from available data (pelvic data not explicitly reported) Liu et al.  13 12 (92.3%) 10.6 Grimer et al.  95 N/A N/A No pelvis survival outcomes reported Mavrogenis et al.  32 22 (68.8%) 13 Calculated from available data on Kaplan–Meier curves Weber et al.  1 1 (100%) 8 Sheth et al.  13 10 (76.9%) 4.5 Current study 31 25 (80.6%) 6.2 be important areas to research in the future. Addition- or long-term survival (mean survival = 9.7 months). LR ally, the sample size was small, reflecting the rarity of this and metastases occurred synchronously in 90%. A study disease, therefore whether amputation is truly superior at reporting surgical outcomes for recurrent CS of the pel- achieving radical section margins cannot be proven, and vis also showed no overall survival advantage when treat- a larger multi-institutional review would help clarify this. ing recurrent high-grade CS tumours . Therefore, the priority should be initial curative resection to reduce the chance of recurrence as rapid disease progression, with Conclusion subsequent death can be anticipated. Dedifferentiated CS of the pelvis confers a poor prog - In our series, one of the notable findings was that all nosis. 12-month survival is 15.4% in those treated pal- recurrent disease, whether local or systemic arose within liatively and 55.6% when treated with curative intent. 24 months. This is likely a reflection of the extremely It is a particularly aggressive disease, presenting at an aggressive nature of this tumour. A high chance of cure advanced, inoperable state in almost half of patients. can be expected if a patient lacks any evidence of disease The factors that influenced disease cure were achieving after 24 months follow-up. A short time to recurrent dis- a wide surgical margin (greater than 4 mm), which was ease was also evident in previous studies (Table 4). more common with HQA. Margins less than 4 mm have We found no benefit for chemotherapy or radiotherapy a very high risk of local recurrence and death. HQA for used in a palliative setting, reaffirming the belief that local recurrence did not result in disease control and 90% chemotherapy and radiotherapy are ineffective at con - of disease recurrence occurred synchronously. Obtaining trolling dedifferentiated CS . the correct pre-operative diagnosis is also an important The limitations to this series are that the data is based factor. We recommend early surgery with the widest pos- on a retrospective analysis from a single institution, with sible margins to optimise chances for long-term survival. inherent selection bias both in those treated surgically and in the type of surgical procedure. This study was not Abbreviations able to analyse whether having wide margins from the CS: chondrosarcoma; MRI: magnetic-resonance imaging; CT: computed dedifferentiated component of the tumour or if having a tomography; MDT: multidisciplinary team; HQA: hindquarter amputation; LSS: limb-salvage surgery; LRFS: local recurrence-free survival; MFS: metastasis-free small proportion of dedifferentiation within the tumour survival; DFS: disease-free survival; OS: overall survival; CI: confidence interval. was associated with increased survival as this was not routinely reported by pathologists, however, these would Lex et al. Clin Sarcoma Res (2018) 8:23 Page 9 of 9 Authors’ contributions 7. 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