scholarly journals De Novo Angiosarcoma of the Thoracic Outlet: A Rare Entity

2020 ◽  
pp. 1-3
Author(s):  
Kalliopi Athanassiadi ◽  
Kalliopi Athanassiadi ◽  
Ilias Samiotis ◽  
Evangelia Chatzimichali ◽  
Aikaterini Katsandri ◽  
...  

Angiosarcoma is a rare soft tissue highly malignant tumor of vascular origin, accounting for only 1% to 2% of these tumors. We present a rare case of De novo (unrelated to irradiation or pyothorax) angiosarcoma of the thoracic outlet with a 10-year disease free survival. Α 49-year-old male was admitted to our department due to a tumor of the thoracic outlet referred by the neurologists to whom he addressed complaining for vertigo and instability. After a thorough examination including chest X-ray, CT scan and MRI, a well circumscribed vascularized lesion of a diameter of 5cm was detected in the thoracic outlet. A head and neck angiography was performed along with a full staging in order to exclude metastatic disease. The patient was submitted to high axillary thoracotomy. The adjacent structures were dissected, small arterioles arising from the subclavian artery, neo-vessels were ligated or cauterized and the soft largely encapsulated tumor was excised. Gross observation of the resected specimen demonstrated a regular-shaped neoplasm. Histology revealed a well differentiated angiosarcoma comprised of multiple anastomosing blood vessels lined by endothelial cells showing malignant features but with little nuclear pleomorphism. The recovery was uneventful. The patient did not receive postoperative radiation or chemotherapy and 10 years postoperatively is free of disease. In conclusion, de novo primary pleural angiosarcoma are rare entities that should not be misdiagnosed. The immunohistochemical examination is the key for diagnosis and will offer the definite histotype. Although the prognosis is bad, early detection will give the patient the best chance for successful surgical treatment. Our patient being well and asymptomatic after 10 years follow-up represents a rare case and the first mentioned in the literature with a location of the lesion in the thoracic outlet.

2020 ◽  
Vol 35 (3) ◽  
pp. 41-49
Author(s):  
Lorenzo Scappaticcio ◽  
Pierpaolo Trimboli ◽  
Frederik A. Verburg ◽  
Luca Giovanella

Objective Clinical and laboratory guidelines recommend thyroglobulin antibodies (TgAbs) measurement with every thyroglobulin (Tg) measurement for the follow-up of differentiated thyroid cancer (DTC) patients. However, no evidence exists on the need for perpetual TgAbs testing in patients who are TgAb-negative at baseline. Our study was carried out to evaluate the prevalence, the dynamic changes, and the clinical significance of TgAbs that appeared de novo during the follow-up of DTC patients who were TgAb-negative at baseline. Methods The data of DTC patients with negative pre-ablation TgAbs were reviewed retrospectively. The main characteristics of patients with both transient and sustained de novo TgAbs appearance were analyzed. DTC patients with persistently negative TgAbs served as controls. Results Among 119 patients with pre-ablation negative TgAbs, 14 cases (11.7%) with de novo TgAbs appearance (10 and 4 patients with a transient and sustained de novo TgAbs appearance, respectively) were detected. No differences in disease-free survival were observed in patients with de novo TgAbs appearance compared to controls. The TgAbs peak value was higher in patients with sustained de novo appearance compared to patients with transient de novo. Two of 14 patients with de novo TgAbs developed structural disease with concurrently detectable Tg in both cases. Conclusions Transient de novo TgAbs appearance is not infrequent during DTC patients’ follow-up, and it has no apparent clinical impact. Sustained de novo TgAbs appearance is rare and may predict structural recurrences; however, similar disease-free survival was observed in patients with sustained de novo TgAbs and TgAb-negative DTC patients.


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 169-169 ◽  
Author(s):  
Beverly J. Lange ◽  
Franklin O. Smith ◽  
Patricia A. Dinndorf ◽  
Carola A.S. Arndt ◽  
Dorothy R. Barnard ◽  
...  

Abstract CCG-2961 tested an intensively timed induction therapy consisting of cytarabine (AC), etoposide, thioguanine, dexamethasone, idarubicin and daunorubicin. Patients in remission after induction were randomized to a second induction course (Arm A) or a 3-drug combination of fludarabine, AC, and idarubicin (Arm B). Course 3 for patients with related donors was bone marrow transplantation (BMT); for those without donors, high dose AC/l-asparaginase. After Course 3 patients without donors were randomized to 14 infusions of Interleukin 2 (IL2) over 18 days or follow-up. CNS prophylaxis was intrathecal AC. Eligibility included all subtypes of de novo AML except acute promyelocytic leukemia and AML in patients with Down syndrome. CCG-2961 opened in Oct.1996 and closed in Dec. 2002. The DSMC suspended the study between Oct. 1999 and May 2000 while the 2961 Committee developed supportive care policies to reduce treatment-related mortality (TRM). CCG-2961 enrolled 900 de novo patients aged 3 days to 21 years, with 495 and 405 patients accruing pre-and post suspension respectively. Remission induction rate is 88.5%. With median follow-up of 3.6 years (range: 0 – 8.1 years), event-free survival (EFS) at 3 years is 44±3% and survival (OS) 57±3%. Disease-free survival (DFS) following Course 2 Arms A and B are not different, although relapse is significantly higher in Arm A (7.3% .vs. 3.1% P=0.018) and TRM more common in Arm B (7.9% vs.4.2% P=0.059), despite 7 less days of neutropenia in Arm B (P<0.001). DFS is 65±9% for patients with a donor versus 50±5% for patients without a donor (P=0.005); respective OS, 74±8% and 66±5% (P=0.221). However, among 98 patients in CR1 with t(8;21) or inv(16) cytogenetics, outcomes in those without and with a donor were no different: DFS (61±12% vs. 72±18%, P = 0.49) and OS (78±10% vs. 77±17%, l P= 0.85). DFS with and without IL2 is 55±9% and 60±8%(P=0.606). Outcomes improved progressively over time. EFS pre- and post-suspension are 41±4% and 47±5%(P=0.038); OS, 52±5% and 63±5%(P=0.005); TRM is 17±3% pre- and 12±3% post-suspension (P=0.039). Factors predictive of inferior EFS are age >17 years, Afro-American and Hispanic ethnicity, body mass index <10th or >95th percentile for age, absence of related marrow donor, WBC > 50,000/mm3, karyotype with −7/7q, −5/5q- or > cytogenetic 5 abnormalities, FLT3/ITD, >15 % morphologic blasts on day 14 or >0.5% immunologically detectable blasts at the end of induction. CCG-2961 confirms the efficacy and high TRM of intensively timed therapy. Neither fludarabine nor IL2 increases DFS or OS, and availability of a donor does not improve outcomes in those with favorable cytogenetics.


Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4461-4461
Author(s):  
Eugene Choi ◽  
Lingyi Chen ◽  
Srikanth Nagalla ◽  
Vamshi Kaveti ◽  
Regina Mullaney ◽  
...  

Abstract INTRODUCTION: Autologous PBSC transplant is an important yet evolving treatment modality for patients with AML. However, the ideal mobilization regimen from which to collect PBSC remains in question. Previous reports have indicated that highdose cytarabine with etoposide is both safe and effective in terms of successful PBSC procurement, subsequent engraftment, and disease outcome. METHODS: At our institution from 1994 to 2007, 38 consecutive patients with de novo non-promyelocytic AML in first complete remission following conventional induction chemotherapy were consolidated with high-dose cytarabine (2000mg/m2 IV q12h × 8 doses, days 1–4) and etoposide (40mg/kg IV over 96h) followed by G-CSF 5 mg/kg subcutaneously starting d14 until completion of PBSC collection. Patients underwent myeloablative therapy with busulfan (1mg/kg po q6h × 16 doses, days –7 to -4) and etoposide (60 mg/kg IV over 10h, day -3) with PBSC infusion occurring on day 0 with daily G-CSF 5 mg/kg. Data regarding stem cell yield, engraftment and patient outcome was collected retrospectively. RESULTS: The average patient age was 44 years (range 19–70). Following consolidation, at least 2×106 CD34 cells/kg were isolated from all 38 patients with a median of 9.4×106 (range 2.2–43) CD34 cells/kg over a mean of 4 collections (range 1–11). Overall, 36 of 38 (95%) remained in CR and went onto PBSC transplant (one died from infectious complications during consolidation, one relapsed before transplant). The median number of stem cells infused was 8.8×106 CD 34 cells/kg (range 2.2–47). All 36 patients engrafted with the mean number of days to neutrophil recovery (ANC>500) being 11 (range 8–17) and the mean number of days to platelet recovery (>20,000) being 12 (range 8–19). Disease-free outcomes in patients undergoing PBSC transplant while in CR1 are presented in Figure 1. The 3y overall survival in all pts was 66%, and 56% at 5y. For good-risk cytogenetic patients, 3y OS was 78% and the 5y OS was 75%. For intermediate-risk cytogenetic patients, OS was 47% and 36% at 3y and 5y respectively. Three patients with poor cytogenetics were autulogously transplanted. One patient relapsed at day 111 and expired at day 450. The second patient remains in CR at day 246. The third patient relapsed at day 104 and expired at day 322. CONCLUSION: In patients with de novo non-promyelocytic AML in CR1, consolidation with high-dose cytarabine plus etoposide is safe and provides excellent yield of PBSCs upon growth factor accelerated hematological recovery. Subsequent engraftment after autologous transplanation is rapid. Our outcomes support the viability of this regimen in patients with good and intermediate-risk cytogenetics. Figure 1: Kaplan-Meier analysis of disease-free survival following autologous PBSC transplant. Cytogenetic analysis was unavailable in 5 patients, and 1 patient was lost to follow-up. Figure 1:. Kaplan-Meier analysis of disease-free survival following autologous PBSC transplant. Cytogenetic analysis was unavailable in 5 patients, and 1 patient was lost to follow-up.


2012 ◽  
Vol 30 (4_suppl) ◽  
pp. 406-406
Author(s):  
Joshua S. Hill ◽  
Safia Rafeeq ◽  
Matthew H.G. Katz ◽  
Michael J. Overman ◽  
Laura A. Lambert ◽  
...  

406 Background: Well-differentiated appendiceal adenocarcinomas (WDAA) are rare tumors characterized by peritoneal spread. Lymph node metastasis can occur, yet the association between nodal spread and recurrence is poorly understood. Methods: A single institution retrospective review of patients seen between August 1993 and January 2010 with a pathologic diagnosis of WDAA who underwent colectomy was conducted. Patients with zero lymph nodes found during pathologic review were excluded. Parameters evaluated included demographics, presence of lymph node metastasis, completeness of cytoreduction and time to recurrence. Results: Of 688 patients with appendiceal neoplasms, 160 (23.3%) had WDAA. The mean age at diagnosis was 50.7 years and 81 (50.6%) were male. Median follow-up after diagnosis was 58.5 months. One hundred patients (62.5%) had regional or distant metastasis present at the time of colectomy. Seventy-eight (48.8%) colectomies were performed at outside institutions. The median number of nodes examined was 12. Twelve patients (7.5%) were found to have nodal metastasis. The rate of peritoneal metastasis did not correlate with the presence of nodal metastasis (node positive 9/12, 75% versus node negative 91/148, 61.5%; p=0.35). No difference in the ability to perform complete cytoreduction existed for those with and without nodal metastasis (66% versus 51% p=0.19). Examining patients with complete cytoreduction and ≥ 12 months of follow-up, there was an increased risk of recurrence among patients with lymphatic metastasis compared to those without (5/8, 62.5% versus 15/61, 24.6%; p=0.03). In this subset, median disease-free-survival in lymph node positive patients was 53 months compared to 109.1 months in patients without nodal metastasis (p=0.08). Conclusions: Patients with WDAA tumors have an overall favorable prognosis; however, patients with lymph node metastasis appear to have an increased risk of recurrence and apparent shortened disease free survival. Right colectomy may be warranted in this patient population.


2017 ◽  
Vol 35 (15_suppl) ◽  
pp. e22531-e22531
Author(s):  
Daniela Greto ◽  
Carlotta Becherini ◽  
Calogero Saieva ◽  
Giulio Francolini ◽  
Domenico Andrea Campanacci ◽  
...  

e22531 Background: Liposarcoma (LPS) are classified into subtypes: well-differentiated (WDLPS), de-differentiated (DDLPS), myxoid (MLPS) and pleomorphic (PLS). We report a single institution cohort of patients with LPS, undergoing surgery and radiotherapy, to explore prognostic factors related to outcome and toxicity. Methods: This retrospective analysis included 186 LPS patients. Patient, tumor, and treatment variables were analyzed for local recurrence (LR-DFS) and distant metastasis (DM-DFS) disease free survival and overall survival (OS). Results: At a median follow-up of 8.6 years (range, 0.1-27.3 years), Kaplan-Meier (KM) survival analysis showed that LR, DM and OS were 75.5%, 76.6% and 48.1%, respectively. KM analysis showed that Age>56, DDLPS and lower limb localization were related to LR (p=0,001, p=0,0001 and p=0,0001, respectively). Association between LR, Age and DDLPS persisted both at univariate (p=0,003 and p=0,0001, respectively) and multivariate Cox regression (CR) analysis (p=0,024 and p=0,002). Age, tumor depth and grading influenced distant recurrence, both at KM (p=0,023, p = 0.026 and p = 0.016) and univariate CR (p=0,026, p=0,042 and p=0,012). Age and grading were confirmed at multivariate analysis (p=0,009 and p 0,017). Patients with WDLPS and wide excision had significantly better OS (p=0,001 and p=0,03, respectively), while histologic G3 and age>56 were related with worse OS (p= 0,008 and p=0,0001, respectively). Age, DDLPS and Grade were related to OS at univariate (p=0,0001, p=0,0001 and p=0,03, respectively) and multivariate CR analysis (p=0,031, p=0,0001 and p=0,001, respectively). Conclusions: Our analysis confirmed that Grade, tumor depth and histological subtype influenced survival. Further studies are needed in order to explore the influence of hystopathologic features on treatment outcomes. [Table: see text]


2021 ◽  
Vol 12 (5) ◽  
Author(s):  
Sha Zhou ◽  
Jianhong Peng ◽  
Liuniu Xiao ◽  
Caixia Zhou ◽  
Yujing Fang ◽  
...  

AbstractResistance to chemotherapy remains the major cause of treatment failure in patients with colorectal cancer (CRC). Here, we identified TRIM25 as an epigenetic regulator of oxaliplatin (OXA) resistance in CRC. The level of TRIM25 in OXA-resistant patients who experienced recurrence during the follow-up period was significantly higher than in those who had no recurrence. Patients with high expression of TRIM25 had a significantly higher recurrence rate and worse disease-free survival than those with low TRIM25 expression. Downregulation of TRIM25 dramatically inhibited, while overexpression of TRIM25 increased, CRC cell survival after OXA treatment. In addition, TRIM25 promoted the stem cell properties of CRC cells both in vitro and in vivo. Importantly, we demonstrated that TRIM25 inhibited the binding of E3 ubiquitin ligase TRAF6 to EZH2, thus stabilizing and upregulating EZH2, and promoting OXA resistance. Our study contributes to a better understanding of OXA resistance and indicates that inhibitors against TRIM25 might be an excellent strategy for CRC management in clinical practice.


2000 ◽  
Vol 18 (5) ◽  
pp. 987-987 ◽  
Author(s):  
Howard S. Hochster ◽  
Martin M. Oken ◽  
Jane N. Winter ◽  
Leo I. Gordon ◽  
Bruce G. Raphael ◽  
...  

PURPOSE: To determine the toxicity and recommended phase II doses of the combination of fludarabine plus cyclophosphamide in chemotherapy-naive patients with low-grade lymphoma. PATIENTS AND METHODS: Previously untreated patients with low-grade lymphoma were entered onto dosing cohorts of four patients each. The cyclophosphamide dose, given on day 1, was increased from 600 to 1,000 mg/m2. Fludarabine 20 mg/m2 was administered on days 1 through 5. The first eight patients were treated every 21 days; later patients were treated every 28 days. Prophylactic antibiotics were required. RESULTS: Prolonged cytopenia and pulmonary toxicity each occurred in three of eight patients treated every 3 weeks. The 19 patients treated every 28 days, who were given granulocyte colony-stimulating factor as indicated, did not have undue nonhematologic toxicity. Dose-limiting toxicity was hematologic. At the recommended phase II/III dose (cyclophosphamide 1,000 mg/m2), grade 4 neutropenia was observed in 17% of all cycles and 31% of first cycles. Grade 3 or 4 thrombocytopenia was seen in only 1% of all cycles. The median number of cycles per patient was six (range, two to 11) for all patients enrolled. The response rate was 100% of 27 patients entered; 89% achieved a complete and 11% a partial response. Nineteen of 22 patients with bone marrow involvement had clearing of the marrow. Median duration of follow-up was more than 5 years; median overall and disease-free survival times have not been reached. Kaplan-Meier estimated 5-year overall survival and disease-free survival rates were 66% and 53%, respectively. CONCLUSION: The recommended dosing for this combination in patients with previously untreated low-grade lymphoma is cyclophosphamide 1,000 mg/m2 day 1 and fludarabine 20 mg/m2 days 1 through 5. The regimen has a high level of activity, with prolonged complete remissions providing 5-year overall and disease-free survival rates as high as those reported for other therapeutic approaches in untreated patients.


Blood ◽  
1991 ◽  
Vol 78 (11) ◽  
pp. 2814-2822 ◽  
Author(s):  
CA Linker ◽  
LJ Levitt ◽  
M O'Donnell ◽  
SJ Forman ◽  
CA Ries

Abstract We treated 109 patients with adult acute lymphoblastic leukemia (ALL) diagnosed by histochemical and immunologic techniques. Patients were excluded only for age greater than 50 years and Burkitt's leukemia. Treatment included a four-drug remission induction phase followed by alternating cycles of noncrossresistant chemotherapy and prolonged oral maintenance therapy. Eighty-eight percent of patients entered complete remission. With a median follow-up of 77 months (range, 48 to 111 months), 42% +/- 6% (SEM) of patients achieving remission are projected to remain disease-free at 5 years, and disease-free survival for all patients entered on study is 35% +/- 5%. Failure to achieve remission within the first 4 weeks of therapy and the presence of the Philadelphia chromosome are associated with a 100% risk of relapse. Remission patients with neither of these adverse features have a 48% +/- 6% probability of remaining in continuous remission for 5 years. Patients with T-cell phenotype have a favorable prognosis with 59% +/- 13% of patients achieving remission remaining disease-free compared with 31% +/- 7% of CALLA-positive patients. Intensive chemotherapy may produce prolonged disease-free survival in a sizable fraction of adults with ALL. Improved therapy is needed, especially for patients with adverse prognostic features.


2021 ◽  
Author(s):  
Jane E. Rogers ◽  
Michael Lam ◽  
Daniel M. Halperin ◽  
Cecile G. Dagohoy ◽  
James C. Yao ◽  
...  

We evaluated outcomes of treatment with 5-fluorouracil (5-FU), doxorubicin, and streptozocin (FAS) in well-differentiated pancreatic neuroendocrine tumors (PanNETs) and its impact on subsequent therapy (everolimus or temozolomide). Advanced PanNET patients treated at our center from 1992 to 2013 were retrospectively reviewed. Patients received bolus 5-FU (400 mg/m2), streptozocin (400 mg/m2) (both IV, days 1-5) and doxorubicin (40 mg/m2 IV, day 1) every 28 days. Overall response rate (ORR) was assessed using RECIST version 1.1. Of 243 eligible patients, 220 were evaluable for ORR, progression-free survival (PFS), and toxicity. Most (90%) had metastatic, nonfunctional PanNETs; 14% had prior therapy. ORR to FAS was 41% (95% confidence interval [CI]: 36-48%). Median follow-up was 61 months. Median PFS was 20 (95% CI: 15-23) months; median overall survival (OS) was 63 (95% CI: 60-71) months. Cox regression analyses suggested improvement with first-line vs subsequent lines of FAS therapy. Main adverse events ≥ grade 3 were neutropenia (10%) and nausea/vomiting (5.5%). Dose reductions were required in 32% of patients. Post-FAS everolimus (n=108; 68% second line) had a median PFS of 10 (95% CI: 8-14) months. Post-FAS temozolomide (n=60; 53% > fourth line) had an ORR of 13% and median PFS of 5.2 (95% CI: 4-12) months. In this largest reported cohort of PanNETs treated with chemotherapy, FAS demonstrated activity without significant safety concerns. FAS did not appear to affect subsequent PFS with everolimus; this sequence is being evaluated prospectively. Responses were noted with subsequent temozolomide-based regimens although PFS was possibly limited by line of therapy.


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