Dermatofibrosarcoma Protuberans Treated at a Single Institution: A Surgical Disease With a High Cure Rate

2005 ◽  
Vol 23 (30) ◽  
pp. 7669-7675 ◽  
Author(s):  
Marco Fiore ◽  
Rosalba Miceli ◽  
Chiara Mussi ◽  
Salvatore Lo Vullo ◽  
Luigi Mariani ◽  
...  
Keyword(s):  
Reconstructive Surgery ◽  
Residual Disease ◽  
Dermatofibrosarcoma Protuberans ◽  
Distant Metastases ◽  
Local Relapse ◽  
Primary Group ◽  
Low Grade ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Significant Difference

Purpose Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade, cutaneous sarcoma with autocrine overproduction of the platelet-derived growth factor (PDGF) β-chain from gene rearrangement as a key pathogenetic factor, now susceptible of molecular-targeted therapy. The aim of this retrospective analysis was to explore the outcome of patients with primary or recurrent DFSP. Patients and Methods Two hundred eighteen patients surgically treated at the Istituto Nazionale per lo studio e la cura dei Tumori (Milan, Italy) over 20 years were reviewed. Local relapse, distant metastasis, and survival were studied. Results One hundred thirty-six patients (62.4%) presented with a primary DFSP, while 82 patients (37.6%) had a recurrent disease. In the primary group, margins were microscopically positive in 11.8%, while in the recurrent group they were positive in 14.6% (P =.613). In the primary group, patients undergoing re-excision after inadequate previous surgery had residual disease in 62% of cases. Reconstructive surgery was needed in 30%, significantly more frequently in patients with a recurrence or a head and neck tumor. The crude cumulative incidence of local relapses was 4% at 10 years, and 2% at 10 years for distant metastases. No significant difference was found between primary and recurrent patients, as well as between positive and negative margins. Conclusion This being one of the largest mono-institutional series of DFSP, we confirm that long-term outcome is excellent, in terms of both local and distant control, after a wide excision with negative margins. Reconstructive surgery is often needed. Novel medical therapies will be of use in a limited subgroup of patients.

Allogeneic Stem Cell Transplantation (Allo-SCT) in the Management of Advanced Waldenstrom’s Macroglobulinemia (WM).

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 619-619
Author(s):  
Charalampia Kyriakou ◽  
C. Canals ◽  
A. Sureda ◽  
G. Taghipour ◽  
J. Cornelissen ◽  
...  
Keyword(s):  
Median Time ◽  
Relapse Rate ◽  
Residual Disease ◽  
Chronic Gvhd ◽  
Conditioning Regimen ◽  
Progression Free Survival ◽  
Mixed Chimerism ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Significant Difference

Abstract Despite effectiveness of standard chemotherapy regimens, complete response is infrequent in WM patients and there is no cure. The role of Allo-SCT has not been extensively explored and the available data are limited. In this retrospective European multicenter study we report the outcome of 106 WM patients (69 male) who underwent an Allo-SCT between 1989 and 2005 and were reported to the EBMT Lymphoma Database. The median age at transplant was 49 years (21–65), and the median time from diagnosis to SCT was 34 months (5–310). The median number of treatment lines prior to allo-SCT was 3(1–10) and 19 patients had failed a prior autograft. Ten (10%) patients were in 1st maximum response (MR), 35 (33%) in PR1, 29 (27%) in PR≥2 and 32 (30%) had refractory disease at the time of transplantation. Forty-four patients were treated with conventional (CT) conditioning protocols; [Cy/TBI n=24, Melphalan/TBI, n=6, BuCy n=14] and 62 with a reduced intensity protocol (RIC); [Fludarabine based regimen n=43, Low dose TBI/Cy n=19] With a median follow up of 31 months (3 to 169) 59 (56%) patients, are alive and free of disease. Forty-eight (45%) patients developed aGVHD [Grades I-II (n=34), Grades III-IV (n=14)] with no statistically significant difference between conventional and RIC groups. Five out of nine RIC patients developed aGVHD following the administration of donor lymphocytes for either residual disease or mixed chimerism. Sixteen patients (15%) developed limited and 11 (10%) extensive chronic GVHD. Seventeen (16%) patients relapsed at a median time of 8 (1–89) months after allo-SCT. Thirty-five (33%) patients died, 5 (5%) from disease relapse or progression and 30 (28%) from regimen toxicity. Non-relapse mortality rates were estimated of 30% and 33%, at 1 and 3 years, respectively, for the CT group, and 24% and 30% for the RIC group of patients. Relapse rates at 1 and 3 years were 10%, 12% for the CT group and 14% and 25% for the RIC. Progression free survival (PFS) rates were 60%, 54% and 54% at 1, 3 and 5 years for the CT and 61%, 44% and 39% for the RIC patients. Overall survival was 65%, 59% and 59% for the CT and 71%, 66% and 66% for the RIC at 1, 3 and 5 years, respectively. Multivariate analysis showed that chemorefractory disease at allo-SCT was associated with a significantly higher relapse rate [p<0.03; 95% CI 1.1–8.9] while the use of TBI in the conditioning regimen with a significantly lower relapse rate [p<0.02; 95% CI 1.1–9.3]. There were no differences in outcome when considering the intensity of the conditioning regimen. In conclusion, allo-SCT is a feasible and well-tolerated procedure in this group of elderly patients with advanced disease. In addition, relapse rate after the allogeneic procedure is low resulting in a good long-term outcome.

scholarly journals A retrospective study on prophylactic regional lymphadenectomy versus nodal observation only in the management of dogs with stage I, completely resected, low-grade cutaneous mast cell tumors

2021 ◽  
Author(s):  
Silvia Sabattini ◽  
Matti Kiupel ◽  
Riccardo Finotello ◽  
Damiano Stefanello ◽  
Eugenio Faroni ◽  
...  
Keyword(s):  
Mast Cell ◽  
Stage I ◽  
Low Grade ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Mast Cell Tumors ◽  
Regional Lymphadenectomy ◽  
Significant Difference ◽  
Cutaneous Mast Cell ◽  
Prophylactic Lymphadenectomy

Abstract Background. While lymphadenectomy of metastatic 27 lymph nodes (LNs) has been associated with improved outcome, the clinical utility of prophylactic lymphadenectomy in dogs with stage I cutaneous mast cell tumors (cMCTs) remains a controversial topic. To assess the therapeutic role of lymphadenectomy of uninvolved regional LNs, the long-term outcome of cMCT-bearing dogs with cytologically negative and surgically unresected regional LNs (observation only, OO) was compared with that of dogs with a surgically resected and histologically negative regional LNs (prophylactic regional lymphadenectomy, PRL).Results. A retrospective analysis of 64 dogs with a low-grade, completely resected stage I cMCT was performed: (54.7%) dogs were subjected to OO and 29 (45.3%) underwent PRL. Dogs were monitored for a median of 813 and 763 days in the OO group and PRL group, respectively. The number of dogs undergoing MCT progression was significantly higher in the OO group (P = 0.028) and curve comparison revealed a tendency to a better time to progression in the PRL group (P = 0.058). No significant difference in survival time (P = 0.294) was observed between dogs in the OO and PRL groups.Conclusions. Our results showed that lack of immediate lymphadenectomy was associated with a higher risk for tumor progression. This preliminary judgement, reinforced by the findings that lymphadenectomy was well tolerated in all cases, and that histopathology provides the definitive assessment of the nodal pathological status, may suggest that prophylactic lymphadenectomy is indicated in the management of stage I MCTs. Larger prospective studies are warranted for generating clinical evidence of this latter hypothesis.

Solitary fibrous tumor: A retrospective analysis on 65 patients from a reference center

2009 ◽  
Vol 27 (15_suppl) ◽  
pp. 10572-10572
Author(s):  
E. Palassini ◽  
S. Stacchiotti ◽  
P. Collini ◽  
S. Pilotti ◽  
U. Pastorino ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Local Recurrence ◽  
Locally Advanced ◽  
Distant Metastases ◽  
Local Relapse ◽  
Mesenchymal Tumors ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Free Survival ◽  
Fibrous Tumor

10572 Background: Solitary fibrous tumors (SFT) are very rare mesenchymal tumors with an unpredictable malignant potential. We retrospectively reviewed the long-term outcome of patients operated on at the Istituto Nazionale Tumori (INT), Milan, in a 18-year time span. Methods: All patients who underwent surgery at INT from 1990 to 2008 for primary SFT or first local relapse were retrospectively reviewed. Clinical and pathologic data were recorded. Local recurrence, distant metastases and overall survival were analyzed both from time of diagnosis and from time of local recurrence/distant metastasis. Biomolecular studies are ongoing for those cases whose frozen material is available. Results: 65 patients were identified (M/F: 33/32 - age: mean 54 yrs, range 19–85 yrs - site: peritoneum 45%, pleura 21%, lower-limb 17%, other 18% - extension at diagnosis: localized 94%, locally-advanced/unresectable 3%, metastatic 3% - size: median 11 cm, range 2–40 cm). The median follow-up was 54 months (range 0–185 months). The overall 5/10-year survival (OS), local relapse-free survival (LRFS) and metastasis-free survival (DRFS) were, respectively, 92/91%, 89/86%, 94/89%. The 5-year OS, LRFS, DMRFS after the first local relapse were, respectively, 100%, 67%, 89%. The overall incidence of metastases was 17% (lung was the commonest site). The 5-year post-metastasis OS was 45%. Multivariate analysis on clinical and pathologic/molecular prognostic factors is ongoing. Conclusions: SFT arise from a variety of anatomic sites. Surgery is curative in most patients, and local relapse has a limited impact on prognosis. Pathologic prognostic factors are under investigation to help identify the minority of patients with a poor clinical outcome. No significant financial relationships to disclose.

scholarly journals LGG-06. LONG-TERM OUTCOME OF NEWLY DIAGNOSED LOW GRADE GLIOMA

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii367-iii367
Author(s):  
Nongnuch Sirachainan ◽  
Attaporn Boongerd ◽  
Samart Pakakasama ◽  
Usanarat Anurathapan ◽  
Ake Hansasuta ◽  
...  
Keyword(s):  
Surgical Management ◽  
Progression Free Survival ◽  
Low Grade Glioma ◽  
Low Grade ◽  
Newly Diagnosed ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Common Location ◽  
Suprasellar Region

Abstract INTRODUCTION Low grade glioma (LGG) is the most common central nervous system (CNS) tumor in children accounted for 30–50%. Regarding benign characteristic of disease, surgical management remains the mainstay of treatment. However, surgical approach is limited in some conditions such as location at brainstem or infiltrative tumor. Chemotherapy and radiation treatments have been included in order to control tumor progression. The 5-years survival rate is approach 90% especially in patients who receive complete resection. However, the outcome of children with LGG in low to middle income is limited. Therefore, the aim of the study was to determine long-term outcome of children with newly diagnosed LGG. METHODS A retrospective study enrolled children aged &lt;18 years who were newly diagnosed LGG during January 2006- December 2019. Diagnosis of LGG was confirmed by histological findings of grade I and II according to WHO criteria. RESULTS A total of 40 patients, female to male ratio was 1:1.35 and mean (SD) for age was 6.7 (4.0) years. The most common location was optic chiasmatic pathway (42.5%), followed by suprasellar region (25.0%). Sixty percent of patients received at least partial tumor removal. Chemotherapy and radiation had been used in 70% and 10.0% respectively. The 10-year progression free survival was 74.1±11.4% and overall survival was 96.2±3.8%. SUMMARY: Treatment of Pediatric LGG mainly required surgical management, however, chemotherapy and radiation had been used in progressive disease. The outcome was excellent.

Favorable long-term outcome of low-grade oligodendrogliomas irrespective of 1p/19q status when treated without radiotherapy

2010 ◽  
Vol 102 (3) ◽  
pp. 443-449 ◽  
Author(s):  
Yasuo Iwadate ◽  
Tomoo Matsutani ◽  
Yuzo Hasegawa ◽  
Natsuki Shinozaki ◽  
Yoshinori Higuchi ◽  
...  
Keyword(s):  
Low Grade ◽  
Term Outcome ◽  
Long Term Outcome

Early Aneurysm Surgery and Preventive Therapy with Intravenously Administered Nimodipine: A Multicenter, Double-Blind, Dose-Comparison Study

Neurosurgery ◽  
1990 ◽  
Vol 26 (3) ◽  
pp. 458-464 ◽  
Author(s):  
Joachim M. Gilsbach ◽  
Hans J. Reulen ◽  
Bengt Ljunggren ◽  
Lennart Brandt ◽  
Hans v. Holst ◽  
...  
Keyword(s):  
Preventive Therapy ◽  
Neurological Dysfunction ◽  
Comparison Study ◽  
Double Blind ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Dose Comparison ◽  
Significant Difference ◽  
Low Incidence

Abstract A European, multicenter. prospective, randomized. double-blind, dose-comparison study on preventive therapy with intravenously administered nimodipine was performed to evaluate the efficacy and tolerability of two different doses: 2 and 3 mg/h. Two hundred four patients fulfilled the criteria for enrollment in the study; surgery within 72 hours after the last subarachnoid hemorrhage, and age between 16 and 72 years. All patients who had Hunt and Hess grades of I to III were operated upon: patients who had poor Hunt and Hess grades (IV-V) were operated on according to the surgeon's choice. This treatment regimen was associated with a low incidence of delayed neurological dysfunction with no significant difference between the two dosage groups: three patients (1.5%) remained severely disabled and two (1%) moderately disabled due to vasospasm with or without additional complications. Among the patients with Hunt and Hess grades of IV or V. the long-term outcome was favorable (good-fair) for 40% and unfavorable for 60%. Among the patients with grades of I to III, the long-term outcome was favorable for 89% and unfavorable for 11%.

scholarly journals Hypospadias: clinical approach, surgical technique and long-term outcome

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Pier Luca Ceccarelli ◽  
Laura Lucaccioni ◽  
Francesca Poluzzi ◽  
Anastasia Bianchini ◽  
Diego Biondini ◽  
...  
Keyword(s):  
Functional Outcome ◽  
Surgical Repair ◽  
Adult Life ◽  
Clinical Approach ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Initial Surgery ◽  
Significant Difference ◽  
Corrective Procedures

Abstract Background Hypospadias is one of the most common congenital abnormalities in male newborn. There is no universal approach to hypospadias surgical repair, with more than 300 corrective procedures described in current literature. The reoperation rate within 6–12 months of the initial surgery is most frequently used as an outcome measure. These short-term outcomes may not reflect those encountered in adolescence and adult life. This study aims to identify the long-term cosmetic, functional and psychosexual outcomes. Methods Medical records of boys who had undergone surgical repair of hypospadias by a single surgical team led by the same surgeon at a single centre between August 2001 and December 2017 were reviewed. Families were contacted by telephone and invited to participate. Surgical outcome was assessed by combination of clinical examination, a life-related interview and 3 validated questionnaires (the Penile Perception Score-PPS, the Hypospadias Objective Score Evaluation-HOSE, the International Index of Erectile Function-5-IIEF5). Outcomes were compared according to age, severity of hypospadias, and respondent (child, parent and surgeon). Results 187 children and their families agreed to participate in the study. 46 patients (24.6%) presented at least one complication after the repair, with a median elapsed time of 11.5 months (6.5–22.5). Longitudinal differences in surgical corrective procedures (p < 0.01), clinical approach (p < 0.01), hospitalisation after surgery (p < 0.01) were found. Cosmetic data from the PPS were similar among children and parents, with no significant differences in child’s age or the type of hypospadias: 83% of children and 87% of parents were satisfied with the cosmetic result. A significant difference in functional outcome related to the type of hypospadias was reflected responses to HOSE amongst all groups of respondents: children (p < 0.001), parents (p=0.02) and surgeon (p < 0.01). The child’s HOSE total score was consistently lower than the surgeon (p < 0.01). The HOSE satisfaction rate on functional outcome was 89% for child and 92% for parent respondents. Conclusion Surgeons and clinicians should be cognizant of the long-term outcomes following hypospadias surgical repair and this should be reflected in a demand for a standardised approach to repair and follow-up.

Short and Long Term Outcome In Patients with Calcified Lesions Requiring Rotational Artherectomy

2021 ◽  
Vol 35 (2) ◽  
pp. 140-146
Author(s):  
Lima Asrin Sayami ◽  
Al Fazir Omar ◽  
Sheikh Ziarat Islam ◽  
Subasni Govindan ◽  
Zulaikha Zainal ◽  
...  
Keyword(s):  
P Value ◽  
Success Rates ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Operator Experience ◽  
Kaplan Meier ◽  
Significant Difference ◽  
Short And Long Term ◽  
Procedural Success

Objective: Despite the evolution of interventional techniques and operator experience, percutaneous revascularization of complex coronary lesions especially calcified lesions remains challenging because of lower procedural success and higher restenosis rates. Limited data are available on the effect of rotational atherectomy (RA) plus stenting in the treatment of complex calcified lesions of coronary artery disease. This study was aimed to investigate the characteristics, short and long term outcomes in patients undergoing RA. Material and Methods: A database search was performed from the year 2008 to 2013 in National Heart institute, Malaysia. A total of 16009 patients who underwent PCIs were enrolled in 2 groups, RA group (258 patients) and non RA group (15751 patients). The Chi square test and Kaplan - Meier analysis were used. Results: Male patients (73.6%) and elderly population (63.2%) were predominant in this study.The RA group had more co-morbidities such as diabetic on insulin (34%) and chronic kidney disease (57%). The lesions in RA group were more complex with higher Type C lesion (68.8%) and longer lesion (20.6%) compared to non RA group. Despite higher patient risk profile, the success rate of revascularization remains high in RA group (99.3%) as in non RA group (97%) (p value 0.89%). More importantly there were no significant difference in in-hospital mortality, myocardial infarction and stent thrombosis in both group (p value 0.1). In 1 year Kaplan - Meier survival graph, there were better survival noted in non RA group (97.7%) compare to RA (89.6%) (p value <0.005), Conclusion: The use of RA allows debulking of a calcified lesion and possibly explains the higher acute procedural success rates. However, the lower 1-yearsurvival in the RA group highlights the higher associated baseline comorbitidity in this group. Therefore, besides coronary intervention, this RA group requires aggressive medical therapy through a multi-disciplinary approach. Bangladesh Heart Journal 2020; 35(2) : 140-146

Intermediate to long term outcome after reconstructive surgery

2006 ◽  
Vol 22 (1) ◽  
pp. 86-86
Author(s):  
SK Varma ◽  
KM Rao ◽  
S Ramanathan ◽  
P Chandrasekar ◽  
S Muralidharan
Keyword(s):  
Reconstructive Surgery ◽  
Term Outcome ◽  
Long Term Outcome
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