Primary Follicular Lymphoma of the Duodenum Is a Distinct Mucosal/Submucosal Variant of Follicular Lymphoma: A Retrospective Study of 63 Cases

2011 ◽  
Vol 29 (11) ◽  
pp. 1445-1451 ◽  
Author(s):  
Ana-Iris Schmatz ◽  
Berthold Streubel ◽  
Elisabeth Kretschmer-Chott ◽  
Andreas Püspök ◽  
Ulrich Jäger ◽  
...  
Keyword(s):  
Follicular Lymphoma ◽  
Stable Disease ◽  
Cell Transformation ◽  
Large Cell ◽  
Low Grade ◽  
Complete Regression ◽  
Watch And Wait ◽  
Small Series ◽  
Anti Cd20

Purpose Small series with limited follow-up have suggested primary follicular lymphoma of the duodenum (FL-D) to be an indolent disease. We report our experience on a large series of patients followed for a median time period of longer than 6 years. Patients and Methods The study comprised 63 patients with primary FL-D defined as stage I disease. Endoscopy and detailed pathologic work-up was performed at diagnosis and at restaging to monitor the behavior of the neoplastic process. Results Histologically, all 63 patients had FL, low grade (1 to 2). Duodenal endosonography demonstrated lesions confined to mucosa/submucosa in 19 of 20 patients. At an overall median follow-up of 77 months (range, 12 to 177 months), only two untreated patients had developed nodal disease, the remaining 61 patients never experienced extrasmall intestinal disease and large cell transformation did not occur at all. Among 24 patients followed by watch and wait strategy, seven showed spontaneous complete regression and 17 had stable disease; radiotherapy resulted in complete regression in all 19 patients; anti-CD20 antibody monotherapy achieved complete regression in four patients and stable disease in one patient. Various chemotherapy protocols in eight patients caused complete regression in all of them, but local relapses occurred in three. No patients required surgery or died of disease. Conclusion These findings characterize primary FL-D as a remarkably indolent FL variant, which, even left untreated, does not develop tumorous growth, very rarely disseminates (two of 63 patients) and does not transform to high grade disease. A watch and wait approach appears to be the most sensible strategy.

Combination Biologic Therapy as Initial Treatment for Follicular Lymphoma: Initial Results From CALGB 50701 - a Phase II Trial of Extended Induction Epratuzumab (anti-CD22) and Rituximab (anti-CD20)

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 427-427 ◽  
Author(s):  
Barbara Grant ◽  
John P. Leonard ◽  
Jeffrey L Johnson ◽  
Lale Kostakoglu ◽  
Eric Hsi ◽  
...  
Keyword(s):  
Monoclonal Antibody ◽  
Follicular Lymphoma ◽  
Phase Ii ◽  
Stable Disease ◽  
Phase Ii Trial ◽  
Single Agent ◽  
Good Tolerability ◽  
Grade 3 ◽  
Anti Cd20

Abstract Abstract 427 Rituximab is effective as single agent therapy in the treatment of follicular lymphoma (FL), and when combined with chemotherapy has extended remissions and survival. Epratuzumab (Immunomedics), a humanized anti-CD22 monoclonal antibody, also has single agent activity in FL, and in combination with rituximab led to durable complete responses in the treatment of patients (pts) with relapsed and refractory indolent NHL. To evaluate the hypothesis that combining a second biological agent with rituximab might improve efficacy with good tolerability, the CALGB treated 60 previously untreated pts with epratuzumab and rituximab in a multicenter phase II trial and we report here the preliminary response and toxicity findings. Rituximab was administered at 375 mg/m2 iv weekly for four weeks, then every 8 weeks for four additional doses for a total of 8 doses over 9 months. Epratuzumab, was given at 360 mg/m2 two days before the first rituximab dose to assess toxicity. From week 2 on, epratuzumab was given before the rituximab on the same day for a total of 8 doses over 9 months. Fifty-seven evaluable pts were enrolled between May 2008 and September 2009. FLIPI scores at study entry were 13 (22%) low; 28 (47.5%) intermediate; and 18 (30.5%) high. Fifty-three pts completed all therapy through month 9. One pt was taken off therapy due to progression after month 5. One pt died during induction from line sepsis. Two pts were taken off study due to adverse events, 1 during induction (grade 4 thrombosis and MI), 1 following month 5 (dyspnea, hypoxia and pulmonary NOS). All other toxicities were grade 3 or lower, including fatigue (grade 3 3%, grade 2 17%), nodal pain (grade 3 5%, grade 2 8%), and cytokine release and pruritis (grade 2, 5% each). To date, there have been 19 CRs (33.3%), 29 PRs (50.9%)(ORR 84.2%); 9 (15.8%) had stable disease. All 19 CR patients completed all treatment. The mean time to CR was 9 months. Two patients progressed after a period of stable disease, and 25 of the 29 patients who achieved PR remain in response. All 19 CRs also remain in remission at this point with a median follow-up of 0.82 years (range 0.52 to 2.0). FLIPI score was not predictive of response. The CR rate in low risk pts was 31%, 44% in intermediate risk and 18% in high risk pts. There was a trend toward higher CR rate among patients with FcgR2A His (n=10, CR 60%) and to a lower CR rate among those with FcgR2A Arg (n=14, CR 14.3%). Correlations with PET scan at week 3, with tissue biomarkers and to statin use are being analyzed. Rituximab and epratuzumab is an effective and very well tolerated regimen with an ORR of 84% in previously untreated patients with follicular lymphoma. Disclosures: Off Label Use: Use of Epratuzumab, a humanized antiCD22 monoclonal antibody in treatment of follicular lymphoma. Leonard:BiogenIDEC: Consultancy; Genentech: Consultancy; Immunomedics: Consultancy. Jones:Glaxo Smith-Kline: Consultancy; Abbott: Research Funding. Cheson:Genentech: Consultancy.

Primary splenic low-grade follicular lymphoma presenting with leukaemia and large cell transformation in the marrow

Pathology ◽  
2017 ◽  
Vol 49 (6) ◽  
pp. 649-652
Author(s):  
Jie-Yang Jhuang ◽  
Yen-Chuan Hsieh ◽  
Chun-Chi Kuo ◽  
Ying-Zhen Su ◽  
Shih-Sung Chuang
Keyword(s):  
Follicular Lymphoma ◽  
Cell Transformation ◽  
Large Cell ◽  
Low Grade ◽  
Large Cell Transformation

The Impact of Rituximab Resistance on Overall Survival Rate in Low-Grade Follicular Lymphoma

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 3783-3783 ◽  
Author(s):  
Shahrzad Abdollahi ◽  
Elise A Chong ◽  
Rebecca L. Olin ◽  
Sunita D. Nasta ◽  
Charalambos Andreadis ◽  
...  
Keyword(s):  
Overall Survival ◽  
Cell Transformation ◽  
Acquired Resistance ◽  
Large Cell ◽  
Median Number ◽  
Low Grade ◽  
Overall Survival Rate ◽  
Kaplan Meier ◽  
Large Cell Transformation

Abstract Background: Recent studies have prospectively established that concurrent or sequential addition of rituximab (R) to conventional therapies improves the overall survival rate (OS) for patients with low-grade follicular lymphoma (FL). However, there is little information regarding the prognosis of patients considered R-resistant. Subjects/Methods: We examined the records of 305 subjects with a diagnosis of FL seen at our institution between 1995 and 2007. To better define the prognosis for R-resistant subjects, we identified 133 subjects (grade 1, N=75; grade 2, N=41; grade 1 or 2, N=17) who completed an R-containing treatment (R alone, N=61; R combination, N=72) and had at least six months of follow-up from start of R-containing therapy. For the purposes of our study, we define R-resistance as progression of lymphoma within 6 months of the first R dose (i.e., the dose defining R-resistance) of the R-containing regimen followed by progression. Overall survival rates were evaluated for all subjects from first dose of R to last follow-up or death and, for R-resistant subjects, from the dose defining R-resistance to last follow-up or death. R-resistant subjects (N=62 [47%]) were subdivided into primary refractory (R-resistant after first R-containing treatment; N=30) or acquired resistant (R-resistant after at least one prior R-containing treatment without progression within 6 months; N=32). Median age at first treatment with R was 55 years (range: 21–87) for all subjects and 54 years (range: 21–87) for R-resistant subjects (for primary refractory, median=54 years [range: 28–87]; for acquired resistant, median=55 years [range 30–81]). The median number of prior non-R-containing treatment regimens was 1 (range: 0–4) for resistant subjects, and 0 (range: 0–3) for non resistant subjects. The median number of R-containing regimens for subjects with acquired resistance was 2 (range: 2–4). The frequency of large cell transformation did not differ between R-resistant and non R-resistant cohorts (N=7/71 [10%] and N=8/62 [13%], respectively). Results: Of 20 deaths observed for all subjects with FL receiving R or R containing regimens, 19 deaths occurred after R-resistance. At a median follow-up of 56 months (range 6–115) for all subjects receiving R, the median OS was not reached with 5-year Kaplan-Meier OS estimate = 81%. For R-resistant subjects, median OS from first dose of R defining R-resistance was 64 months (range: 6–100) with 5-year Kaplan-Meier OS estimate = 58%. Median rates of OS from R dose defining R-resistance were not significantly different between primary refractory and acquired resistant subjects. For subjects with acquired R-resistance, median time from first dose of R to first dose of R defining R-resistance was 10.4 months (range: 9–83). Conclusion: The OS estimate for subjects with R-resistant FL (5-year estimate OS = 58%) appears worse than survival estimates reported for unselected subjects with FL (5 year estimate OS = 80% for grade 1; 76% for grade 2 [SEER Survival Monograph, Non-Hodgkin Lymphoma]). This inferior prognosis seems unrelated to large cell transformation. Survival is similar for subjects with primary refractory and acquired resistance when survival is measured from the R dose used to define R-resistance.

scholarly journals NCOG-42. INITIAL PRESENTATION AND OUTPATIENT VISIT COMPLIANCE OF INMATES WITH BRAIN TUMORS

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii138-ii138
Author(s):  
Iyad Alnahhas ◽  
Appaji Rayi ◽  
Yasmeen Rauf ◽  
Shirley Ong ◽  
Pierre Giglio ◽  
...  
Keyword(s):  
Brain Tumors ◽  
Civil Liberties ◽  
Delayed Diagnosis ◽  
Low Grade ◽  
Small Series ◽  
Initial Symptoms ◽  
The Ohio State University ◽  
American Civil Liberties Union ◽  
Lost To Follow Up

Abstract INTRODUCTION While advocacy for inmates with cancer has recently gained momentum, little is known about management of brain tumors in inmates. Delays in acknowledging or recognizing nonspecific initial symptoms can lead to delayed diagnosis and treatment. Inmates with cancer are reported to either be ignored or receive substandard care due in part to cost or logistics (American Civil Liberties Union; ASCO Post 2018). METHODS In this retrospective study, we identified inmates with gliomas seen in the Ohio State University Neuro-oncology Center between 1/1/2010-4/20/2019. RESULTS Twelve patients were identified. Median age at presentation was 39.5 years (range 28-62). Eleven patients were Caucasian and one was African American. Diagnoses included glioblastoma (GBM) (n=6), anaplastic astrocytoma (n=1), anaplastic oligodendroglioma (n=1), low-grade astrocytoma (n=3) and anaplastic pleomorphic xanthroastrocytoma (n=1). Patients were more likely to present early after seizures or focal neurologic deficits (9/12) than after headaches alone. Patients with GBM started RT 12-71 days after surgery (median 34.5). One patient’s post-RT MRI was delayed by a month and another with GBM had treatment held after 4 cycles of adjuvant temozolomide (TMZ) due to “incarceration issues”. For one patient who received adjuvant TMZ, the facility failed to communicate with the primary team throughout treatment. Two patients suffered significant nausea while on chemotherapy due to inability to obtain ondansetron in prison, or due to wrong timing. 7/12 (58%) patients were lost to follow-up for periods of 3-15 months during treatment. Three patients refused adjuvant treatment. CONCLUSIONS Although this is a small series, our results highlight the inequities and challenges faced by inmates with gliomas who are more likely to forego treatments or whose incarceration prevents them from keeping appropriate treatment and follow-up schedules. Additional studies are needed to define and address these deficiencies in the care of inmates with brain tumors and other cancers.

Secretory Carcinoma in Children and Young Adults: A Case Series

2021 ◽  
pp. 109352662110469
Author(s):  
Caroline T Simon ◽  
Jonathan B McHugh ◽  
Raja Rabah ◽  
Amer Heider
Keyword(s):  
Case Series ◽  
Salivary Gland Neoplasm ◽  
Low Grade ◽  
Secretory Carcinoma ◽  
Patients Âgés ◽  
Male Predominance ◽  
Small Series ◽  
Children And Young Adults ◽  
Grade Malignancy

Secretory carcinoma (SC), previously known as mammary analogue secretory carcinoma, is a rare salivary gland neoplasm that typically presents as a slow-growing painless lesion in the head and neck. SC occurs mainly in adults but has been described in children with the youngest reported patient diagnosed at five years of age. In children the gender distribution has been reported as female to male ratio of 1:1.2. SC is generally considered a low-grade malignancy with characteristic morphological features and immunological profile. SC also harbors ETV6-NTRK3 fusion (t(12;15)(p13:q25)). Surgical resection with or without lymph node dissection is the standard treatment, with generally favorable clinical outcomes. Here we present a single institution case series of six patients (ages 9-21) with SC and a review of the previously described pediatric cases. Our small series showed male predominance in pediatric patients with predominantly low-grade and stage tumors. All cases underwent complete surgical resections and when follow up is available there was no evidence of recurrences or metastases. To the best of our knowledge, this is the only SC case series comprised exclusively of pediatric and youth patients.

Age and Response after Autologous Stem Cell Transplantation (ASCT) Define a Group of Long-Term Event-Free Survivors among Patients (Pts) with Low-Grade Follicular Lymphoma (FL): A Single Institution Experience.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 1883-1883
Author(s):  
Charalambos Andreadis ◽  
Elise A. Chong ◽  
Edward A. Stadtmauer ◽  
Selina M. Luger ◽  
David L. Porter ◽  
...  
Keyword(s):  
Multivariable Analysis ◽  
Disease Free Survival ◽  
Autologous Bone Marrow ◽  
Large Cell ◽  
High Dose ◽  
Low Grade ◽  
Free Survival ◽  
Disease Free

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

Follicular Dendritic Cell Sarcoma (FDCS): A Rare and Incurable Disease.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3892-3892
Author(s):  
Andres O. Soriano ◽  
Michael Thompson ◽  
Jorge Romaguera ◽  
Alma Rodriguez ◽  
Fredrick B. Hagemeister ◽  
...  
Keyword(s):  
Dendritic Cell ◽  
Stable Disease ◽  
Initial Treatment ◽  
Castleman’S Disease ◽  
Mitotic Rate ◽  
Cancer Center ◽  
Low Grade ◽  
High Grade ◽  
Md Anderson

Abstract Objectives: To describe the clinical characteristics, pathologic features, immunophenotype, treatment and outcome of patients with FDCS seen at MD Anderson Cancer Center in the past 10 years. Background: FDCS is grouped in the WHO classification of tumors with the histiocytic and dendritic cell neoplasms, this group also includes histiocytic sarcoma, Langerhans cell tumors and interdigitating dendritic cell tumors. Data on this disease is based on case reports and case series. Methods: After IRB approval, cases were identified from the files of the lymphoma and pathology departments at MD Anderson Cancer Center from 1995 to 2005. Results: Fourteen patients were identified. Median age was 48 years old (25–69). Three patients presented with cervical lymphadenopathy, five had abdominal lymphadenopathy, three had mediastinal adenopathy, two had nasopharyngeal disease and one had pleural involvement. Extranodal disease included liver, spleen, pancreas and pleura. Constitutional symptoms were reported in 2 patients. Median performance status was 1 (0–2). Histologically, five cases showed low grade cytology with proliferation of spindled cells, growth patterns included whorled, storiform, fasicular and nodular. Three cases showed low grade features with focal high grade cytology and a diffuse growth pattern. Five cases showed high grade cytology that also included areas of necrosis in 2 cases and a high mitotic rate (>20/10HPF) in one case. CD21, CD23 and CD35 were positive in 83%, 90% and 44% of the cases respectively. Epidermal growth factor receptor (EGFR) was strongly positive in 12/13 cases tested (92%). The case that tested negative had high grade features and high mitotic rate. One patient had coexistent Castleman’s disease. Information on initial treatment was available in 11 patients which included surgery alone in 1 patient, surgery and radiation in 2 patients, surgery and chemotherapy in 1, chemotherapy alone in 3, chemotherapy and radiation in 1, surgery followed by radiation and chemotherapy in 3 patients. The initial chemotherapy regimen was CHOP in 8 patients. Complete remission (CR) was achieved in 7/11 patients (63%), 3 patients had disease progression and 1 had stable disease. Relapse occurred in all the patients who had a CR. Salvage treatment included surgery in 2 patients and chemotherapy in 7 patients (including the 2 patients who progressed on initial treatment). Two patients underwent allogenic hematopoietic stem cell transplantation after salvage therapy. CR was achieved in 5 patients, partial remission in 2, progression in 1 and stable disease in 1. Information on disease status at last follow up was available in 13 patients. Ten patients were alive at a median follow up of 22 months, 3/13 patients (23%) had no evidence of disease and 7/13 patients (53%) were alive with disease. Conclusions: The pathologic characteristics and immunophenotype found in our series were similar to those previously reported. EGFR was strongly positive in all but one of the cases tested. Consistent with previous reports Castleman’s disease was found in one of the cases. Although most of the patients initially responded to treatment, all of them eventually relapsed, which is in contrast to previously reported relapse rates of 16–36%. A better understanding of the biology of FDCS could guide our efforts in the development of new treatment modalities for this rare disease.

R-FND Followed by Radioimmunotherapy for High-Risk Follicular Lymphoma

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 3056-3056 ◽  
Author(s):  
Peter McLaughlin ◽  
Sattva Neelapu ◽  
Michelle Fanale ◽  
Maria Rodriguez ◽  
Ana Ayala ◽  
...  
Keyword(s):  
High Risk ◽  
Follicular Lymphoma ◽  
International Prognostic Index ◽  
Prognostic Index ◽  
Ibritumomab Tiuxetan ◽  
Free Survival ◽  
Grade 3 ◽  
Anti Cd20 ◽  
Better Than

Abstract Follicular lymphoma (FL) patients, (pts) with high-risk features using the FL International Prognostic Index (FLIPI) have an expected 5-year survival of only about 50% with conventional therapy. With the incorporation of anti-CD20 monoclonal antibody (mAb) therapy, results are improving (e.g., Buske, Blood2006; 108: 1504). Starting in 2003, we have treated high-risk (FLIPI ≥3) FL pts with R-FND (rituximab, fludarabine, mitoxantrone, dexamethasone) for 4 cycles, followed by radioimmunotherapy (RIT) with ibritumomab tiuxetan, and subsequent rituximab maintenance. Results for the first 35 pts are: complete (CR) and partial (PR) remission 83% and 14%; 3-year overall (OS) and failure-free survival (FFS) 89% and 74% (median follow-up 24 mo.). RIT converted 5 PR pts to CR. Toxicity was mainly hematologic. Five pts did not receive RIT, one because of neutropenia after R-FND. Following RIT, platelet and neutrophil nadirs were 28 and 0.3, occurring at 4–7 weeks. 16 pts required transfusions, and 27 received growth factors. 13 pts had infections, only 2 of which were grade 3. Recovery occurred by 3 weeks in most, with prolonged cytopenias in 6. There has been 1 case of myelodysplasia. In conclusion, the additional complexity of this RIT intensification strategy is warranted in this high-risk FL population, resulting in OS and FFS outcomes that are better than non-mAb therapies, and at least as good as published chemotherapy-rituximab combination therapy.

Hormonal treatment of metastatic endometrial stromal sarcoma

2009 ◽  
Vol 27 (15_suppl) ◽  
pp. e16576-e16576
Author(s):  
K. Y. Terada ◽  
J. Davis ◽  
J. Kitayama ◽  
D. Shimizu
Keyword(s):  
Aromatase Inhibitors ◽  
Stable Disease ◽  
Progesterone Receptors ◽  
Rank Test ◽  
Low Grade ◽  
Image Analyzer ◽  
High Grade ◽  
Estrogen And Progesterone Receptors ◽  
Significant Difference

e16576 Background: Endometrial stromal sarcomas (ESS) traditionally have been classified as low grade or high grade based on mitotic activity and histologic appearance. High-grade tumors are currently referred to as undifferentiated uterine sarcomas and are not included in this series. ESS are known to have high expression of estrogen and progesterone receptors. This is a retrospective study of patients with metastatic ESS treated with hormonal therapy. Methods: Following approval by the institutional review board all patients diagnosed with ESS from 1987–2007 were identified. Clinical and demographic information were abstracted from the charts and all histologic materials were re-reviewed. Estrogen and progesterone receptor testing was performed utilizing mouse monoclonal antibodies and the Cell Analysis Systems 200 Image analyzer. Survival was calculated using the Kaplan-Meier method and comparisons utilized the log rank test. Results: Thirteen patients with ESS were identified during this period. Seven had disease confined to the uterus. Six had extrauterine disease; 5 patients presented with metastases and 1 patient presented with a pelvic recurrence 20 years following a hysterectomy. All underwent surgical resection except for 1 patient that declined surgery. All 6 patients with metastases had tumors that tested positive for estrogen and progesterone receptors; all were treated with megestrol acetate initially for a period of 1–4 years. Two patients were then changed to maintenance with medroxyprogesterone acetate. Three patients with persistent disease were changed to aromatase inhibitors; 1 to letrozole and 2 to anastrazole. One of these patients has had a complete response and 2 have had stable disease. One patient has been lost to follow-up. Follow-up for the 6 patients was 2–22 years; no known patients died of their disease. Actuarial 2- and 5-year survivals were 80% and 65%, respectively. There was no significant difference in survival between patients with metastases and without metastases. Conclusions: ESS tumors are relatively uncommon and there is an absence of studies to guide treatment of patients with metastases. This experience indicates that these tumors respond well to hormonal manipulation. Treatment with progestins or aromatase inhibitors may result in remission or stable disease. No significant financial relationships to disclose.

Sign in / Sign up

Export Citation Format

Share Document