Low-Grade Appendiceal Mucinous Neoplasms: A Single Institution Experience of 64 Cases With Clinical Follow-up and Correlation With the Current (Eighth Edition) AJCC Staging

2019 ◽  
Vol 28 (3) ◽  
pp. 252-258 ◽  
Author(s):  
Mary Wong ◽  
Brad Barrows ◽  
Alexandra Gangi ◽  
Stacey Kim ◽  
Richard B. Mertens ◽  
...  

Background. In this single-institution study, we applied the current (eighth edition) American Joint Committee on Cancer pathologic staging criteria to 64 low-grade mucinous neoplasms of the appendix (LAMNs), examined their histopathologic features, and studied the patients’ clinical outcomes. Design. Sixty-four LAMNs, with a median follow-up of 52 months, were reviewed. Results. The distribution of pathologic stages was pTis (n = 39), pT3 (n = 1), pT4a (n = 5), pT4aM1a (n = 8), and pT4aM1b (n = 11). Recurrence was observed in only 2 patients (both with pT4aM1b disease), one of whom died of disease. All remaining patients were disease-free after a median clinical follow-up of 60 months. Conclusions. Our study confirms that pTis LAMNs have an excellent prognosis and suggests that pT4a and pT4aM1a LAMNs may also have a low risk of developing progressive disease.

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.


2019 ◽  
Vol 180 (2) ◽  
pp. 127-134 ◽  
Author(s):  
S Asioli ◽  
A Righi ◽  
M Iommi ◽  
C Baldovini ◽  
F Ambrosi ◽  
...  

Objective and design A clinicopathological score has been proposed by Trouillas et al. to predict the evolution of pituitary adenomas. Aim of our study was to perform an independent external validation of this score and identify other potential predictor of post-surgical outcome. Methods The study sample included 566 patients with pituitary adenomas, specifically 253 FSH/LH-secreting, 147 GH-secreting, 85 PRL-secreting, 72 ACTH-secreting and 9 TSH-secreting tumours with at least 3-year post-surgical follow-up. Results In 437 cases, pituitary adenomas were non-invasive, with low (grade 1a: 378 cases) or high (grade 1b: 59 cases) proliferative activity. In 129 cases, tumours were invasive, with low (grade 2a: 87 cases) or high (grade 2b: 42 cases) proliferative activity. During the follow-up (mean: 5.8 years), 60 patients developed disease recurrence or progression, with a total of 130 patients with pituitary disease at last follow-up. Univariate analysis demonstrated a significantly higher risk of disease persistence and recurrence/progression in patients with PRL-, ACTH- and FSH/LH-secreting tumours as compared to those with somatotroph tumours, and in those with high proliferative activity (grade 1b and 2b) or >1 cm diameter. Multivariate analysis confirmed tumour type and grade to be independent predictors of disease-free-survival. Tumour invasion, Ki-67 and tumour type were the only independent prognostic factors of disease-free survival. Conclusions Our data confirmed the validity of Trouillas’ score, being tumour type and grade independent predictors of disease evolution. Therefore, we recommend to always consider both features, together with tumour histological subtype, in the clinical setting to early identify patients at higher risk of recurrence.


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 1883-1883
Author(s):  
Charalambos Andreadis ◽  
Elise A. Chong ◽  
Edward A. Stadtmauer ◽  
Selina M. Luger ◽  
David L. Porter ◽  
...  

Abstract Introduction: FL is generally responsive to conventional-dose chemotherapy but long term disease-free survival (DFS) is uncommon. High-dose chemo-radiotherapy followed by ASCT has the potential to induce remission in this disease but the long-term benefit of this modality remains to be determined. Methods: Between 1990 and 2003, we transplanted 52 pts originally diagnosed with low-grade FL (31 grade 1, 21 grade 2). Twenty-five (48%) had biopsy-proven large cell transformation (FL grade 3 or diffuse large cell lymphoma) before ASCT. The median number of prior therapies was 2 (range: 1 to 7). Prior to ASCT, 45 pts (87%) were responsive to salvage therapy with 20 pts (38%) in CR. Five pts (10%) had chemo-resistant disease at the time of ASCT. High-dose regimens included BCNU-cyclophosphamide-etoposide (31%), melphalan/TBI (27%), and cyclophosphamide/TBI (25%). Thirty-eight pts (73%) received peripheral stem cells (PSCT) and 14 pts (27%) received autologous bone marrow (BM) with 4-hydroxyperoxycyclophosphamide (4-hc) purging in 9 cases (17%). The median age was 49 yrs (range: 29–65). Results: There was 1 treatment-related death during the first 100 days. After ASCT, 36 pts (69%) achieved a CR, 2 (4%) had a PR, and 7 (13%) had stable disease. Among those in CR, 20 (56%) had a CR pre-ASCT, 14 (41%) had a lesser response, and 1 (3%) was chemo-resistant. Median follow-up (f/u) of survivors was 5.3 yrs (range: 1.7 months to 12.4 yrs). The median overall survival (OS) has not yet been reached. The median event-free survival (EFS) is 3.4 yrs (range: 1.7 months to 12.4 yrs). Among complete responders, more than 50% are disease free at last follow-up (range 1.7 months to 12.1 yrs). Variables favorably affecting EFS and OS are age < 60 yrs (p = 0.007, 0.015 respectively), achievement of a CR after ASCT (p = 0.002, 0.001), absence of transformation (p = 0.038, 0.017), BM vs. PSCT (p = 0.042, 0.086), and 4-hc BM purging (p = 0.044, 0.059). Number of prior regimens, response prior to ASCT, type of preparative regimen, and addition of TBI, were not significantly associated with EFS, DFS, or OS. In multivariable analysis, achievement of CR after ASCT and age < 60 yrs are the only significant predictors of EFS and OS. Adjusted for age, 53% of pts with a CR after ASCT are alive and event-free at last f/u (range: 2.4 months to 12.4 yrs) (Figure 1). In contrast, the median EFS among pts without a CR is 0.5 yrs (range: 1.7 months to 5.3 yrs). Conclusion: ASCT is a reasonable therapeutic approach to FL, resulting in long term EFS for some pts, even with relapsed, refractory and/or transformed disease. In our experience, significant predictors of EFS and OS after ASCT are complete response and age <60. The appropriate application and timing of ASCT in the management of pts with FL needs to be further evaluated in randomized, controlled clinical trials. Figure Figure


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 1882-1882
Author(s):  
Stephane Vignot ◽  
Nicolas Mounier ◽  
Guillaume Sergent ◽  
Pauline Brice ◽  
Jean-Pierre Marolleau ◽  
...  

Abstract Low grade lymphoma patients (pts) have an indolent evolution with median survival ranging between 8–10 years. During disease’s course, high dose therapy (HDT) and autologous stem-cell transplantation (ASCT) can be considered as an alternative to sequential chemotherapies. However, efficacy of this strategy remains controversial. The purpose of our study is to evaluate ASCT efficacy by comparing retrospectively for each pts disease free survival (DFS) after ASCT with DFS observed with pts’ last chemotherapy regimen (LCR) just before intensification. Between apr 1988 and feb 2002, 109 low grade lymphoma pts were treated with HDT and ASCT in our department, 61 were male, the median age was 49 yrs [range 28–65]. Histological subtypes were mostly follicular small cell (86 %). At time of diagnosis, LDH were normal for 85 pts; 60 pts had high tumor burden. IPI was 0 for 16 %, 1 for 70 % and 2 for 14 %. Prior to ASCT, pts had experienced a median of 2 progressions (range 1 to 5). At time of graft, 102 pts present complete or partial response and 7 pts present stable disease. Two principal intensification chemo regimens were used before ASCT: VP16/cyclophosphamide in 84 pts and BEAM in 12. TBI was associated for 86 pts. At June 2002, the median follow up was 6.4 yrs from diagnosis and 4.5 yrs from ASCT. 3 years after ASCT, survival rate was 72 % and DFS rate was 50 %. Median DFS decreased with nb of progression (p=0.02): Median DFS according to nb of progression Nb of progression 1 2 3 > 3 Nb pts (%) 17 (16) 57 (52) 28 (26) 7 (6) Median DFS in yrs 6.4 5.1 1.8 1.0 Considering pt with more than 1 progression (n=92) as his own control, DFS was longer after ASCT than after LCR for 61 % of pts. Median DFS was 2.5 yrs after ASCT and 2.0 yrs after LCR. At 3 yrs, DFS rate was 48 % after ASCT and 37 % after LCR (p<0,001): Figure Figure This study demonstrates that HDT and ASCT significantly increase DFS in comparison with the LCR for low grade lymphoma patients. Such methodology could be useful to evaluate new strategy incorporating monoclonal antibody.


Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4665-4665
Author(s):  
Severiano Baltazar-Arellano ◽  
Patricia Pimentel ◽  
Luis Vera ◽  
Fernando Bezares ◽  
Jose Málaga ◽  
...  

Abstract Background: fludarabine (F) is licensed for the management of indolent non Hodgkin’s lymphoma in countries such as Canada and Switzerland. Clinical evidence suggests that fludarabine monotherapy is as least as effective, than conventional therapies such as cyclophosphamide, vincristine, prednisone (CVP) for the first and second line treatment of B-cell low grade NHL achieving objective response rates. Better response rates can be achieved combining F with Mitoxantrone (M) in low grade NHL even in refractory relapsed (RR) patients (pts). The Latin American Cooperative Oncology Hematology Group (LACOHG) proposed a multicenter study in Latin American countries in 2002 to use FM in RR B-cell low grade NHL. Aims: to assess the response rate, safety, disease free survival (DFS) and overall survival (OS) of FM in RR B-cell low grade NHL during (2003–2006). Methods: Forty-eight patients in the period of January 2003 to February 2006 were evaluated. Forty-four pts. had follicular lymphoma and 4 small lymphocytic lymphoma. Median age 63.5 years old (range: 24–83). Gender: female 56% and male 44%. Inclusion criteria for low grade NHL-LG was: any previous treatment excluding autologous transplantation, Ann Arbor stage II to IV, age &gt; 18 years old, ECOG performance status 0–2 and written informed consent. ECOG performance status 0: 2%, 1: 71% and 2: 27%. Ann Arbor staging: II: 2%, III: 29% and IV: 69%. International Prognostic Index (IPI): 0–1: 19%, 2–3: 71% and 4–5: 10%. Median previous treatment was 1 (range: 1–3). FM treatment consisted of F 25 mg/m2 i.v. (day 1–3) and M 10 mg/m m2 i.v. (day 1) each 28 days for 6–8 cycles. Results: on this low grade NHL cohort the overall response rate (ORR was 81%; progressive disease and non-response 19%. With a median follow up of 17 months, OS at 24 months was 86% (DE 5.2%) and DFS at 24 months 57.1% (DE 11.3%). LDH in serum was not an adverse prognostic factor for DFS and OS. Safety: on the 286 cycles in 48 pts, the toxicity was: 18 episodes of grade 3-4 neutropenias, 15 episodes of grade 3-4 thrombocytopenia, 7 episodes of grade 1–2 nausea/vomiting, grade 1–2 diarrhea in 4 pts, 8 pts were admitted to the clinic, 11 fever episodes, 2 allopecia, 4 pts developed grade 1–2 peripheral neuropathy and infections 7%: one case herpes zoster. Mortality rate: 12,5% (6/48 patients), 5 of them because progressive disease. No cardiac toxicity was reported. Conclusions: FM is an effective and safe treatment for RR low grade NHL. A longer follow up and a larger trial, might be needed to confirm these results in a multicenter, randomized study. DFS with FM in RR low grade NHL : LACOHG DFS with FM in RR low grade NHL : LACOHG


2016 ◽  
Vol 32 (2) ◽  
pp. 194-203 ◽  
Author(s):  
Santhosh A. Upadhyaya ◽  
Carl Koschmann ◽  
Karin Muraszko ◽  
Sriram Venneti ◽  
Hugh J. Garton ◽  
...  

Safe maximal surgical resection is the initial treatment of choice for pediatric brainstem low-grade gliomas. Optimal therapy for incompletely resected tumors or that progress after surgery is uncertain. We reviewed the clinical characteristics, therapy, and outcomes of all children with nontectal brainstem low-grade gliomas treated at the University of Michigan between 1993 and 2013. Median age at diagnosis was 6 years; histology was confirmed in 23 of 25 tumors, 64% were pilocytic astrocytoma. Nineteen patients underwent initial tumor resection; 14/19 received no upfront adjuvant therapy. Eight patients in the study had progressive disease; 5 initially resected tumors received chemotherapy at tumor relapse, all with partial or complete radiographic responses. Ten-year progression-free survival is 71% and overall survival, 100%. This single-institution retrospective study demonstrates excellent survival rates for children with brainstem low-grade gliomas. The efficacy of the well-tolerated chemotherapy in this series supports its role in the treatment of unresectable or progressive brainstem low-grade gliomas.


2021 ◽  
Vol 108 (Supplement_6) ◽  
Author(s):  
D Goodman ◽  
R Taggart ◽  
J Salmond ◽  
E H Day

Abstract Aim This study addresses surveillance cystoscopy in patients diagnosed with bladder and upper tract cancer. Managed Clinical Network (MCN) guidelines have clear recommendations for the timetable of follow-up cystoscopy, and we conducted this audit to study regional compliance. Method Using a multisite pathology database of bladder cancer cases from 2016, we collected and analysed data on 100 non-muscle invasive bladder cancers. We took the first 10 cases from each month to ensure cross-regional representation. Each case was stratified according to MCN guidelines. Electronic medical records were examined to assess upper tract follow up. We allowed for +/- 1 month each side of the target timeframe. Results We had 64 male and 36 female subjects. In our risk categories, we had 31 low risk, 37 intermediate risk and 32 high risk bladder cancers. 67 were new cases, 33 were recurrent tumours. 10 (43.4%) of low-risk and 19 (79.2%) of intermediate-risk patients underwent surveillance cystoscopy earlier than the recommended 12-month timeframe. 18 (78.3%) of low-risk patients continued to have further surveillance cystoscopies after a 12-month disease-free period. Conclusions 43.4% of low-risk bladder cancer patients are receiving surveillance cystoscopy earlier than recommended. 78.3% of these patients are then undergoing unnecessary procedures following a 12-month disease-free period against regional guidelines and recommendations. This places an increased burden on clinic/theatre time and contributes to patient anxiety surrounding cancer follow-up. Evidence-based medicine guidelines have shown that less is more when it comes to low-grade bladder cancer surveillance. We now need to assess why we are deviating from our own guidelines.


2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 7706-7706 ◽  
Author(s):  
D. Sugarbaker ◽  
W. G. Richards ◽  
C. A. Alsup ◽  
M. T. Jaklitsch ◽  
J. M. Corson ◽  
...  

7706 Objective: There is no universally accepted staging system in malignant pleural mesothelioma (MPM). A seventeen-year single institution experience of surgically treating a large epithelial MPM cohort with extrapleural pneumonectomy (EPP) gives insight to the applications and limitations of BWH/ DFCI and AJCC staging systems. Methods: We retrospectivly reviewed 526 consecutive patients with epithelial MPM who were surgically explored at our institution since 1988 with intent to perform EPP. Pathologic diagnoses of mesothelioma were confirmed and clinicopathologic data were recorded. Kaplan-Meyer survival from diagnosis was calculated. Those who underwent EPP were staged using BWH / DFCI (J Thorac Cardiov Surg 117:5463;1999) and AJCC (6th Edition) criteria. Operative deaths were included in the analysis and patients received varied adjuvant regimens. Results: Of 526 patients explored for potential EPP, 53 (10%) underwent alternative pleurectomy and 55 (11%) were unresectable. The remaining 418 (79%) underwent EPP. Of these, 307 (73%) were male. Median age at diagnosis was 57.9 years (17–78). Operative mortality was 5%. Median follow-up was 16 months and 23% of observations were censored. Overall median, 1-yr, 3-yr and 5-yr survival was: 18.9 mo., 68.8%, 26.3%, and 13.9%. The table below presents Kaplan-Meyer survival by stage. Conclusions: 1) Both pathological staging systems stratify survival in this cohort, although each system is limited in that a majority of patients are classified as stage 3. 2) BWH / DFCI criteria identify more stage 1–2 patients with favorable prognosis, 164 (39%) vs 46 (11%). 3) AJCC criteria classify more patients to stage 4, 76 (18%) vs 4 (1%), but appear to identify some patients with relatively favorable prognosis. 4) Selected criteria from both systems might be combined to optimally stratify patients with epithelial MPM undergoing EPP. No significant financial relationships to disclose. [Table: see text]


2009 ◽  
Vol 27 (15_suppl) ◽  
pp. e19516-e19516
Author(s):  
K. A. Cadoo ◽  
M. A. Lowery ◽  
J. Cumiskey ◽  
J. McCaffrey ◽  
D. N. Carney

e19516 Background: Anthracycline based chemotherapy is the treatment of choice for aggressive primary lymphomas of the GI tract, with surgery reserved for management of complications. We report long term follow up of 71 cases of primary GI NHL treated with chemotherapy and/or surgery. Methods: Cases were identified from the institutional histology database & records reviewed. Results: 71 patients over an 24 year period were identified; 43 (61%) male, 28 (39%) female. Median age at diagnosis was 60 (15–83). 52 (73%) were DLBCL, 11 (16%) were T-cell, 8 (11%) were MALT. The 8 patients with MALT were treated with single agent chemotherapy; 7 (88%) are alive at median follow up of 8.5 years (2–16). Of the aggressive lymphomas (63), all patients with T cell lymphoma had small bowel as primary site and histological evidence of celiac associated enteropathy, even in the absence of known celiac disease. Primary sites of DLBCL were stomach 35 (67%), small bowel 11 (21%) & colon 6 (12%). 39 (62%) patients underwent surgery at diagnosis due to acute presentation with perforation, bleeding or obstruction, or to obtain histology. Following confirmed diagnosis, 61 patients received anthracycline based chemotherapy. 2 patients with T cell lymphoma presented with perforation, were treated with surgery only and died of rapid disease progression. Of the 63 patients with aggressive NHL, 37 (59%) remain alive & disease free at median follow up of 13 years (1–24). 35 (67%) patients with DLBCL are alive & disease free. Only 2 (18%) of the T cell lymphomas are alive & disease free. 5 deaths in the DLBCL group were not related to cancer or treatment. All deaths in the T cell group were due to progressive disease. There was no difference in survival between patients treated with chemotherapy only and those who also underwent surgery. Conclusions: Patients with aggressive primary B cell GI NHL have almost 70 % survival following anthracycline based chemotherapy. However, in contrast, coeliac enteropathy associated T-cell lymphomas present with rapidly progressive disease & have a survival of < 20% with chemotherapy and/or surgery. A novel therapeutic approach is required to improve outcome in this group. No significant financial relationships to disclose.


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 4601-4601 ◽  
Author(s):  
Sagun Shrestha ◽  
Christina Johnson ◽  
Sanjeev Jain ◽  
Dilip V. Patel ◽  
Bhoomi Mehrotra

Abstract OBJECTIVE: High dose chemotherapy with stem cell transplantation (ABMT) has become the standard treatment for relapsed aggressive non-Hodgkins and Hodgkins Lymphomas. However, there is no one salvage therapy accepted as a standard regimen prior to ABMT. ESHAP, as a combination chemotherapy regimen consisting of Etoposide, Methylprednisolone, Cytosine arabinoside, and infusional Cisplatin, has been shown to be an active regimen for the treatment of refractory Non-Hodgkins Lymphoma in previously published trials. The objective of this study was to determine the efficacy of ESHAP with and without Rituximab as a cytoreductive regimen in relapsed lymphomas prior to stem cell transplant. METHODS: This retrospective study was approved by the Institutional Review Board. Patients with relapsed Hodgkins and Non-Hodgkins lymphoma who were treated at our institution between March 1997 and July 2004, with the intent of proceeding to ABMT after obtaining a complete remission and were treated with ESHAP containing salvage regimen, were included in this study. Data collection was done by review of all pertinent medical records. RESULTS: Twenty four patients with relapsed lymphomas were identified that were treated with ESHAP +/− Rituximab during this period, with the intent to proceed with ABMT after achieving a maximal response. Histology included: large cell lymphoma (n=15), mantle cell lymphoma (n=3), and Hodgkin’s disease (n=6). Median age was 54 years and male-to-female ratio was 1.4:1. Staging of disease at the time of initial diagnosis was done by PET scan, CT scans, and bone marrow examination. Staging at time of salvage treatment for relapsed disease: stage I (n=1) stage II (n=2)), stage III (n=12), stage IV (n=9). Prior treatment consisted of CHOP, ABVD, TVC, MOPP/ABVD, CNOP, CVP, and CAV. The median disease free interval prior to salvage therapy was 12 months. Of 24 patients, 17 received ESHAP and 7 received ESHAP+ Rituximab as salvage therapy. The median time to transplant after initiation of salvage therapy was four months. Nineteen of twenty four patients (79%) proceeded to receive stem cell transplantation. Five patients did not proceed to ABMT secondary to failure to achieve a complete remission or had progressive disease. Patients treated with ESHAP + rituximab compared to ESHAP alone had no evidence of altered transplant related morbidity, as measured by time to engraftment, duration of hospitalization and the number of episodes of febrile neutropenia. No treatment related mortality occurred in all patients. Of the patients who underwent ABMT, 53% are disease free at a median follow up of 48 months. Among those who did not receive stem cell transplant, 4 died of progressive disease, and one has no evidence of disease in 12 months of follow up. CONCLUSIONS: ESHAP with and without Rituximab is an effective and a feasible salvage therapy for patients with relapsed lymphomas when used as a cytoreductive regimen prior to stem cell transplantation and does not negatively impact on transplant treatment related morbidity and mortality. Further studies are necessary to compare its efficacy with other standard salvage regimens prior to ABMT.


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