scholarly journals Paget's Osteosarcoma — no Cure in Sight

Sarcoma ◽  
1999 ◽  
Vol 3 (3-4) ◽  
pp. 191-192 ◽  
Author(s):  
Phillip J. Shaylor ◽  
David Peake ◽  
Robert J. Grimer ◽  
Simon R. Carter ◽  
Roger M. Tillman ◽  
...  

PurposePaget' s osteosarcoma has a fearful reputation with a quoted survival of at best 5% at 5 years.We therefore reviewed our experience of 26 patients treated over the last 25 years using modern staging and limb salvage techniques to see if there had been any improvement in survival.Subjects:We identified 26 patients on the Royal Orthopaedic Hospital Oncological database with a diagnosis of sarcoma secondary to Paget's disease.Results:The survival rate was 53% at 1 year, 25% at 2 years and no patient survived for 5 years.The median survival was 21 months for those treated with curative intent and 7 months for those treated palliatively. Four of the five patients treated with limb-sparing surgery developed local recurrence between 5 and 12 months, the fifth died at 14 months.There was no difference in survival between amputation and limb salvage.Discussion:The development of sarcomatous change in Paget's disease is well recognised. It represents an important segment of primary bone tumours in patients over 40 years of age.The prognosis is appalling. Indeed only 15 of 368 cases (4%) from a number of historical series have survived more than 5 years. Our results are similarly disappointing with no survivors at 5 years despite modern methods of management of bone tumours.While there is no difference in local recurrence rates or survival between limb reconstruction and limb ablation the poor prognosis for both means that neither can be recommended at present. Sarcomatous change in Pagetoid bone should therefore be regarded as a different disease to primary osteosarcoma. It remains an incurable disease with a poor prognosis.

2021 ◽  
Vol 108 (Supplement_7) ◽  
Author(s):  
Jessica Lockhart ◽  
Damian McKay

Abstract Aim High quality operations with low rates of tumour perforation and circumferential resection margin (CRM) positivity are associated with improved long-term outcomes following surgery for rectal cancer. Previous audit has demonstrated lower rates of tumour perforation and CRM positivity by a single surgeon compared to the published standards. Our aim is to re-audit this surgeons’ outcomes for curative rectal resections. Methods Data was collected retrospectively for all potentially curative rectal resections over a 5-year period performed by a single surgeon using a local database and electronic care records. The CRM status and tumour perforation status were considered. Other end points included the rate of local recurrence, survival and length of stay. Results Fifty-one patients underwent rectal resections with curative intent, with a median age of 67. Complete resection (R0) was achieved in 94.1% of cases; 3.92% were found to have nodes less than 1mm from the margin and 1.96% were found to have tumour deposit less than 1mm from the margin – these cases were considered to be an R1 resection. Tumour perforation was present in 3.92% of cases, all of which had occurred pre-operatively. Local recurrence was found in 5.88% of cases and 90-day mortality was 1.96%. Median length of hospital stay was 7 days. Conclusion Our data demonstrates sustained high quality surgical outcomes with low tumour perforation rates and CRM positivity rates which compare favourably with the published standards to date. Local recurrence rates are comparable to published standards and 90-day mortality continues to be low.


2017 ◽  
Vol 27 (4) ◽  
pp. 791-793 ◽  
Author(s):  
Raghava Parashurama ◽  
Vivek Nama ◽  
Richard Hutson

BackgroundExtramammary Paget’s disease is a rare condition, and the vulva is a common site for it to occur. Despite this, there is a paucity of literature on Paget’s disease of the vulva (VPD). A Cochrane meta-analysis could not draw any conclusions on interventions in VPD. Our aim was to review our practice and improve further management of VPD in our center.MethodsWe reviewed all the cases presented to Leeds Gynaecological Oncology Centre between 1988 and 2016. All cases identified in this interval were followed up until April 2016. All case notes and electronic patient data were retrieved to collate the data.ResultsWe identified 18 cases of VPD. The median age at presentation was 76.9 years. Primary surgery was used in 18 cases. Eight patients had wide local excision with graft reconstruction. Ten women had wide local excision with primary reconstruction. Margins were negative in 27% of the excisions. Sixty percent of patients with clear surgical margins had a recurrence, and 69% of patients with positive margins had a recurrence; there was no statistical difference between the 2 groups for recurrence (P > 0.05). Fifty-eight percent of patients who had recurrence had coexisting malignancy. Logistic regression showed no correlation of recurrence rates due to either age, margin status, or coexisting malignancies.ConclusionsPaget’s disease of the vulva is a rare condition. Our experience indicates that most cases may be amenable to surgical treatment at first presentation. Negative margin status does not reduce the chance of recurrence, and hence patients should be under follow-up for life. The benefit of radical surgery in the absence of reduced recurrences, based on margin status, is questionable. Radiotherapy and imiquimod are options for extensive lesions or recurrent settings. Coexisting malignancies are associated with VPD.


2017 ◽  
Vol 7 (1) ◽  
Author(s):  
Lujia Wang ◽  
Chenchen Feng ◽  
Minwei Zhou ◽  
Zhongwen Zhou ◽  
Guanxiong Ding ◽  
...  

Skin Cancer ◽  
2011 ◽  
Vol 26 (1) ◽  
pp. 79-84
Author(s):  
Munezumi FUJITA ◽  
Katsumi HORIUCHI ◽  
Kunihiro KAWASHIMA ◽  
Tetsunori YOSHIDA ◽  
Hikaru IKEDA ◽  
...  

2008 ◽  
Vol 26 (33) ◽  
pp. 5368-5373 ◽  
Author(s):  
Alessandra Longhi ◽  
Costantino Errani ◽  
Daniel Gonzales-Arabio ◽  
Cristina Ferrari ◽  
Mario Mercuri

Purpose We reviewed the outcome of osteosarcoma patients older than 65 years, an age group usually excluded from protocols, to determine the different clinical features and prognostic factors in this age group compared with younger patients. Patients and Methods Patients treated at our institute who had high-grade osteosarcoma and were older than 65 years were observed. Results Forty-three patients were eligible to be enrolled onto this study; of these, 22 were male and 21 were female. The median age of this group was 69 years (range, 65 to 80 years). Of the 43 patients, 29 patients had localized disease, and 14 patients had metastatic disease. Localizations were appendicular in 33 patients, and axial in 10 patients. Twenty-nine patients had a primary osteosarcoma, 13 patients (30%) had a sarcoma in Paget's disease, and one patient had postradiotherapy (RT) osteosarcoma. The median interval from onset of symptoms to diagnosis was 4 months (range, 0 to 73 months).Thirty-two of 43 patients received surgery for a primary tumor. Of these, 18 patients had limb salvage, 13 patients had an amputation, and one patient had palliative surgery; the remaining 11 patients received palliative RT. Fourteen patients received chemotherapy; two deaths related to chemotherapy were observed. Median overall survival (OS) for all 43 patients was 19 months (range, 3 to 229 months); 5-year OS was 22% (SE = 3%) for the whole group, and 45% OS for those patients with localized primary osteosarcoma. Multivariate analysis demonstrated that stage, volume, and surgery were significant prognostic factors. Insignificant prognostic factors were sex, type of surgery, chemotherapy, and Paget's disease. Conclusion Patients older than 65 years with osteosarcoma have a worse prognosis compared with younger patients. This older age group is characterized by a longer time lapse from the onset of symptoms to diagnosis, more metastatic cases at diagnosis, less use of limb salvage, fewer patients receiving chemotherapy, and more patients excluded from clinical trials than a younger age group.


2016 ◽  
Vol 38 (1) ◽  
pp. 29-31
Author(s):  
Haldun Kar ◽  
Necat Cin ◽  
Cengiz Tavusbay ◽  
Kursat Yemez ◽  
Yasin Peker ◽  
...  

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