scholarly journals Primary Cutaneous Anaplastic Large-Cell Lymphoma: A Surveillance, Epidemiology, and End Results Database Analysis from 2005-2016

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4022-4022
Author(s):  
Cesar Gentille Sanchez ◽  
Joe Ensor ◽  
Akshjot Puri ◽  
Jasleen K. Randhawa ◽  
Shilpan S. Shah ◽  
...  
Keyword(s):  
Overall Survival ◽  
Head And Neck ◽  
Anaplastic Large Cell Lymphoma ◽  
Cell Lymphoma ◽  
Survival Rates ◽  
Large Cell ◽  
Seer Database ◽  
Skin Site ◽  
Large Cell Lymphoma ◽  
Overall Survival Rates

Introduction Primary cutaneous anaplastic large-cell lymphoma (PCALCL) is a rare T-cell lymphoma that presents as a solitary or grouped nodules. It is characterized by anaplastic-appearing cells that are usually ALK negative but have high expression of CD30. There is paucity of epidemiologic data on PCALCL. A prior analysis of the Surveillance, Epidemiology, and End Results (SEER) database by Yu et al. reported only 157 cases from 1973 to 2004. We are presenting an analysis of the patients diagnosed with PCALCL after 2004. Methods We used the SEER database to retrospectively identify patients diagnosed with PCALCL from 2005 to 2016. The database collects data from cancer registries covering approximately 26% of the US population and was used to estimate frequencies and overall incidence rate. Survival was analyzed using the Kaplan-Meier method and log-rank tests were used to compare survival distributions. We assessed the effect of primary skin site (head and neck) and increasing age on survival as they were suggestive of decreased overall survival on multivariate analysis of the 1973-2004 cohort. P < 0.05 was considered statistically significant for all analysis. Results There were 501 cases of PCALCL recorded from 2005 to 2016. Median follow-up was 52 months. The overall incidence rate was found to be 0.12/1,000,000 age adjusted to the 2000 US standard population. More than 50% of the cases were diagnosed after 2010. The median age at diagnosis was 61 years (2-97 years). It was seen most frequently in White (72.9%) patients followed by Hispanic (10.2%) and Black (9.4%) patients. The male to female ratio was 1.42. The most common primary sites affected were the skin of the lower limbs and hip (26.4%) and head and neck (21.3%). A 33.4% of patients required treatment which was mainly excisional (1 patient required amputation). Notably, PCALCL was diagnosed as a second or third malignancy in 19.2% of cases. Overall survival rates at 5 years and 10 years were found to be 80.6% (95% CI: 76.3%, 84.3%) and 61.5% (95% CI: 54.1%, 68.1%) respectively. Age greater than 60 years old was significantly associated with a lower survival (89.7% vs 54.4%, p<0.0001). Survival was not significantly different if head and neck was the site of the primary lesion (64.2% vs 60.8%, p = 0.4371). Conclusion Our analysis of the SEER database for PCALCL is the largest done to our knowledge. Although the number of cases has almost tripled since 2005, it is still a rare type of cutaneous T-cell lymphoma. Lower extremities and hips are the most frequent primary skin site. Only a third of the patients required treatment with overall survival rates of more than 80% by 5 years. Older age (more than 60 years old) is associated with a worse outcome. Head and neck as the primary skin site does not appear to be associated to lower survival as previously thought. Disclosures No relevant conflicts of interest to declare.

Incidence and outcomes of primary cutaneous anaplastic large cell lymphoma: a SEER population-based study

2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S49-S49
Author(s):  
P Q Deb ◽  
H J Hurley ◽  
C Bhate ◽  
D Cai
Keyword(s):  
Anaplastic Large Cell Lymphoma ◽  
Statistical Power ◽  
Cell Lymphoma ◽  
Statistical Significance ◽  
Large Cell ◽  
Cox Proportional Hazards ◽  
Molecular Evidence ◽  
Seer Database ◽  
Kaplan Meier ◽  
Large Cell Lymphoma

Abstract Introduction/Objective Primary cutaneous anaplastic large cell lymphoma (PC-ALCL) is the second most type of cutaneous lymphoma of T cell origin. Although PC-ALCL is considered clinically indolent with an excellent overall survival rate, very few studies have examined the epidemiology and survival of this disease in significant depth. Here we have used the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) database to identify the cases of PC-ALCL with immunohistologic or molecular confirmation and analyzed disease-specific survival across various epidemiological factors. Methods/Case Report We identified patients with PC-ALCL from the SEER database from 1975 - 2017. Cases with only histological and immunohistochemical or molecular evidence of diagnosis were included in the study. Age, sex, and race-standardized incidence rates (IR) were calculated. Survival was assessed using Kaplan-Meier curves and Cox proportional hazards models. Results (if a Case Study enter NA) 93 individuals were identified with a diagnosis of PC-ALCL. Increased age correlated significantly with the total number of cases within age groups, suggesting an associated risk. Males were significantly overrepresented in the disease population at 62%. The racial distribution of patients with PC-ALCL did not significantly differ from the racial distribution in the US. Kaplan-Meier DSS analysis was performed to assess possible influence of race, sex, age, or primary site location on the prognosis in patients with PC-ALCL. Age appears to be one of the greatest factors influencing survival, and while this trended strongly, it was not a statistically significant observation. Although males were disproportionately represented among PC-ALCL patients, sex did not affect survival. Similarly, the patient’s race had no significant effect on the length of survival. The location of the primary tumor trends towards affecting survival, and while this is quantitatively the most impressive observation, it does not reach the threshold of statistical significance. Conclusion We performed a comprehensive review of the clinical and epidemiological features present in PC-ALCL. We found age to be the most significant factor in the risk of development of PC-ALCL, and while our study lacks the statistical power to demonstrate it, age may also be the most significant factor in survival.

scholarly journals Epidemiology and Survivorship of Anaplastic Large Cell Lymphoma

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 2905-2905
Author(s):  
Janani Baskaran ◽  
Jonathan Gootee ◽  
Peter T. Silberstein
Keyword(s):  
Anaplastic Large Cell Lymphoma ◽  
Cell Lymphoma ◽  
Private Insurance ◽  
Survival Rates ◽  
Large Cell ◽  
Primary Site ◽  
Treatment Modalities ◽  
Anatomical Site ◽  
Survival Probabilities ◽  
Large Cell Lymphoma

Abstract Background: Anaplastic Large Cell Carcinoma (ALCL) is a rare form of Non-Hodgkin Lymphoma. The incidence of ALCL, especially of breast implants, has been increasing over the years. We utilized the NCDB database to study prognostic variables for ALCL, to create the largest and most comprehensive cohort of patients with primary ALCL, to analyze primary anatomical site and other prognostic variables and how they affect survival so as to help improve clinical outcomes across the world. Methods: Utilizing the National Cancer Database, 3,298 patients with primary T-cell anaplastic large cell lymphoma were identified by the histology code 9714 and grade code 5. Kaplan-Meier curves and survival tables were created to calculate 1-, 5-, and 10-year survival probabilities for variables including stage, race, sex, facility type, age, insurance, primary site, year of diagnosis, and adjuvant therapy. Log-rank analysis was used to compare the groups within each variable. Results: The cohort consisted of 61.2% male patients and 77.9% Caucasian patients with a median age of diagnosis of 51 years old. Approximately 67% of the patients were diagnosed with ALCL between the years of 2010 and 2015. The most common type of insurance was private insurance with 49.4% of the cohort followed by Medicare with 27%, Medicaid with 12.8% and not insured with 6.3%. Most patients (27.3%) were treated at academic or research facilities, closely followed by comprehensive community cancer programs with 26.4% of the cohort. The most common primary anatomical site was lymph nodes of multiple sites consisting of 34.4% of the cohort. Stage IV was the most common stage consisting of 31.8% of the cohort. Overall 1-, 5-, and 10-year survival probabilities for patients with ALCL were 71.5%, 59.7%, and 51.8%, respectively. As age increased, overall survival decreased, with the ≥75 age group having the worst 1- and 5-year survival probability of 43.6% and 23.5%, respectively. Patients diagnosed between the years of 2004-2009 (1-year:66.4% and 5-year:52.7%) had much worse survival probabilities when compared to patients diagnosed between the years of 2010-2015 (1-year:74.5% and 5-year:68.1%). Caucasian patients had better 1-,5- and 10-year survival outcomes when compared to African Americans with 1-year survival probabilities of 72.2% and 64.9%, respectively. Facilities other than research or academic centers and Medicare or Medicaid insurance had worse 1-,5-, and 10-year survivals when compared to research or academic centers and private insurance. As stage increased, overall survival probabilities decreased. The lower limb and hip anatomical site had the best survival outcomes with 1- and 5-year survival probabilities of 84.7% and 78.3% respectively. Adjuvant hematologic transplant patients did best with a 1- and 5-year survival probabilities of 94.8% and 87.4%, respectively, followed by an adjuvant combination of both radiation and hematologic transplant (1-year:85.2% and 5-year:81.5%), adjuvant radiation (1-year:83.3% and 5-year:74.2%), and chemotherapy alone (1-year:73.7% and 5-year:65.0%). DISCUSSION: This is the largest and most comprehensive study to date on the epidemiology and prognostic factors of Anaplastic Large Cell Lymphoma. The results of our analysis bring up several interesting trends, the most interesting one being the marked increase in survival rates between 2010- 2015 compared to 2004-2009. This is likely attributed to better treatment modalities. With regards to treatment modalities, patients who underwent chemotherapy followed by adjuvant transplant had better 1, 3, 5 and 10 year survival rates compared to all other treatment modalities. As previously established, younger age at diagnosis is a good prognosis factor. Other major factors influencing survival, previously unstudied, are facility type, race, insurance status, stage, adjuvant therapy, and primary site. Asians and those with private insurance had better survival rates. Primary site involving multiple lymph nodes carried the worst prognosis, while lower limb and hip as primary sites carried the best prognosis. Patients treated at academic and research center had better prognosis compared to those treated at non- academic centers. Performing a multi-variate analysis and further studies to elucidate the causes of improved survival associated with some of these factors will help improve quality of care for patients with ALCL. Table. Table. Disclosures No relevant conflicts of interest to declare.

Primary Systemic Anaplastic Large Cell Lymphoma in Korean Adults; Retrospective Analysis of 36 Patients.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4633-4633
Author(s):  
Soo Jung Park ◽  
Shin Kim ◽  
Dae Ho Lee ◽  
Yong Pil Jeong ◽  
Yunjeong Bae ◽  
...  
Keyword(s):  
Overall Survival ◽  
Prognostic Factor ◽  
Retrospective Analysis ◽  
Anaplastic Large Cell Lymphoma ◽  
Median Overall Survival ◽  
Cell Lymphoma ◽  
Large Cell ◽  
Overall Response ◽  
Large Cell Lymphoma

Abstract Anaplastic large cell lymphoma (ALCL) is a CD30+ T-cell non-Hodgikin’s lymphoma and represents only 2–8% of lymphoma overall. Information on clinical finding and response to treatment of primary systemic ALCL is still limited in Korea. The aim of this study was to report our experience thirty-six cases with focusing on the clinical feature, treatment response, survival, and prognosis. We retrospectively reviewed the medical records of the patients who were diagnosed as primary systemic ALCL at the Asan Medical Center from February 1995 through December 2005. The median follow up period was 47 months. The median age was 39 years (range, 17–67). The ratio of male to female was 4.14. Patients with primary systemic ALCL had high frequency of advanced-stage disease and extranodal involvement. Seventy-two percent of patients showed Ann Arbor stage III and IV. The most common involved extranodal sites were bone (19%) and soft tissue (17%). Thirty-two (89%) patients were treated by anthracycline-based regimen (CHOP) as induction chemotherapy. Sixteen patients (44%) obtained CR and 14 patients (39%) attained PR, with overall response rate of 83%. The overall survival and disease-free survival was 49 and 17 months. In ALK-positive patients, median overall survival was not reached in follow up period and 3-year overall survival was 61%. In ALK-negative patients, median overall survival was 53 months and 1-year and 3-year overall survival was 75% and 40% respectively. On univariate analysis, international prognostic index (IPI) was significant prognostic factor for overall survival (p = .03). But ALK expression did not affect overall survival in Korea (p = .78). There was no independent prognostic factor for overall survival in multivariate analysis. This retrospective analysis contributes to our understanding of clinical aspects for patients with primary systemic ALCL in Korea. Overall survival is 49 months and overall response to CHOP chemotherapy is 83%. IPI is important predictor of overall survival, but ALK expression may not provide prognostic information for primary systemic ALCL in Korea.

scholarly journals Primary Anaplastic Large-Cell Lymphoma in Adults: Clinical Presentation, Immunophenotype, and Outcome

Blood ◽  
1997 ◽  
Vol 90 (9) ◽  
pp. 3727-3734 ◽  
Author(s):  
Hervé Tilly ◽  
Philippe Gaulard ◽  
Eric Lepage ◽  
Charles Dumontet ◽  
Jacques Diebold ◽  
...  
Keyword(s):  
Overall Survival ◽  
Tumor Cell ◽  
Anaplastic Large Cell Lymphoma ◽  
Cell Lymphoma ◽  
Large Cell ◽  
Cell Phenotype ◽  
Event Free Survival ◽  
Free Survival ◽  
Response To Chemotherapy ◽  
Large Cell Lymphoma

Abstract Anaplastic, CD30+, large-cell lymphoma is now a well-recognized pathologic entity that accounts for 2% to 8% of all lymphomas. Recent progress has been made in the understanding of certain biologic features found in anaplastic large-cell lymphoma, but information about its clinical behavior, in comparison to other large-cell lymphomas, is limited. The pathologic review of a large multicenter study of the treatment of aggressive lymphoma identified 146 cases of anaplastic large-cell lymphoma (ALCL) on the basis of morphology and CD30 expression. We compared initial presentation, immunophenotype, and clinical outcome of these cases with those of the 1,695 nonanaplastic diffuse large-cell lymphomas (non-ALCL) included in the same trial. Patients with ALCL were more likely to be male (P = .018) and were younger (P < .0001) than those with non-ALCL. B symptoms were more frequent in ALCL (P = .006). Skin (P < .0001) and lung (P < .05) involvement was also more frequent in ALCL, but frequency of bone marrow involvement was identical (P = .5). Tumor cell phenotype was B in 56 cases (38%), T in 49 cases (34%), and null in 33 cases (22%). Response to chemotherapy (P = .001), event-free survival (P = .006), and overall survival (P = .0004) were better for ALCL than for non-ALCL. Multivariate analyses identified anaplastic character as an independent factor that predicted a longer survival. Tumor cell phenotype did not influence event-free survival (P = .72) or overall survival (P = .83). ALCL in adults is a clinicopathologic entity which, independent of its phenotypic characteristics, has a better outcome than other diffuse large-cell lymphomas.

Anaplastic Large Cell Lymphoma of the Breast: Incidence, Patterns of Care, and Outcome in the US Population

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 5035-5035 ◽  
Author(s):  
Pratima Chalasani ◽  
Ronald S. Go ◽  
Benjamin Parsons ◽  
Mohammed Al-Hamadani
Keyword(s):  
Overall Survival ◽  
Anaplastic Large Cell Lymphoma ◽  
Initial Treatment ◽  
Cell Lymphoma ◽  
Large Cell ◽  
Patterns Of Care ◽  
Cell Transplant ◽  
Anaplastic Lymphoma ◽  
The Us ◽  
Large Cell Lymphoma

Abstract Background: Anaplastic large cell lymphoma (ALCL) of the breast is a recently described clinicopathologic entity, almost always associated with breast implants, and does not overexpress the anaplastic lymphoma kinase (ALK) protein. Using the two largest US cancer databases, we describe its incidence, patterns of care, and clinical outcome. Methods: Incidence rate was calculated using 2000-2011 data from the Surveillance, Epidemiology, and End Results (SEER) Program and age-adjusted to the US 2000 standard population. For patient level data, we used the National Cancer Data Base (NCDB) Participant User File. We included all adult patients diagnosed from 1998-2011 with ALCL (ICD-O-3: 99714) who had breast as the primary site. Results: The incidence of breast ALCL was 0.001 per million. We identified 61 cases from NCDB. All of the patients were females and they were predominantly White (88.5%). The median age at diagnosis was 64 years (range, 18-90). The stage distribution was I (45.9%), II (21.3%), III (0%), IV (9.8%), and unknown (21.3%). The pattern of initial treatment was surgery alone (13.1%), chemotherapy alone (32.7%), chemotherapy and radiation (19.7%), surgery and chemotherapy (4.9%), surgery and radiation (4.9%), and tri-modality treatment (13.1%). In 7 patients (11.5%), no treatment was given. Two patients (1.6%) received stem cell transplant (1 autologous, 1 allogeneic) as part of initial treatment, one after chemotherapy and another one after surgery and chemotherapy. Overall survival data was available for patients diagnosed in 1998-2006 (29 patients). The median overall survival was 149.8 months (range, 2.6-157.9). Conclusions: ALCL of the breast is very rare. It presents mainly as early stage disease with very good prognosis. The patterns of care varied, although most patients received chemotherapy as part of the initial treatment. Table 1. Treatment type received Frequency Percent Cumulative Frequency Cumulative Percent Chemo only 20 32.79 20 32.79 Surgery only 8 13.11 28 45.90 Chemo, Radiation, No surgery 12 19.67 40 65.57 Chemo, Surgery, No radiation 3 4.92 43 70.49 Radiation, Surgery, No Chemo 3 4.92 46 75.41 All three combined 8 13.11 54 88.52 Nothing 7 11.48 61 100.00 Disclosures Parsons: Celgene: Speakers Bureau.

scholarly journals The biology and management of systemic anaplastic large cell lymphoma

Blood ◽  
2015 ◽  
Vol 126 (1) ◽  
pp. 17-25 ◽  
Author(s):  
Greg Hapgood ◽  
Kerry J. Savage
Keyword(s):  
Anaplastic Large Cell Lymphoma ◽  
Cell Lymphoma ◽  
Survival Rates ◽  
Large Cell ◽  
High Response Rate ◽  
First Line ◽  
Antibody Drug Conjugate ◽  
Anaplastic Lymphoma ◽  
Favorable Prognosis ◽  
Large Cell Lymphoma

Abstract Systemic anaplastic large cell lymphoma (ALCL) is an aggressive CD30+ non-Hodgkin lymphoma. Anaplastic lymphoma kinase–positive (ALK+) ALCL is associated with the NPM-ALK t(2;5) translocation, which is highly correlated with the identification of the ALK protein by immunohistochemistry. ALK+ ALCL typically occurs in younger patients and has a more favorable prognosis with 5-year survival rates of 70% to 90% in comparison with 40% to 60% for ALK-negative (ALK−) ALCL. Studies support young age as a strong component of the favorable prognosis of ALK+ ALCL. Until recently, no recurrent translocations were identified in ALK− ALCL. However, emerging data now highlight that ALK− ALCL is genetically and clinically heterogeneous with a subset having either a DUSP22 translocation and a survival rate similar to ALK+ ALCL or a less common P63 translocation, the latter associated with an aggressive course. Anthracycline-based regimens such as cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) remain the standard first-line treatment choice for systemic ALCL, but in many patients with ALK− ALCL, it is ineffective, and thus it is often followed by consolidative autologous stem cell transplantation. However, selection of appropriate patients for intensified therapy remains challenging, particularly in light of genetic and clinical heterogeneity in addition to the emergence of new, effective therapies. The antibody drug conjugate brentuximab vedotin is associated with a high response rate (86%) and durable remissions in relapsed/refractory ALCL and is under investigation in the first-line setting. In the future, combining clinical and genetic biomarkers may aid in risk stratification and help guide initial patient management.

scholarly journals The clinicopathological relevance of uniform CD56 expression in anaplastic large cell lymphoma: a retrospective analysis of 18 cases

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Bao-Hua Yu ◽  
Yan Zhang ◽  
Tian Xue ◽  
Ruo-Hong Shui ◽  
Hong-Fen Lu ◽  
...  
Keyword(s):  
Overall Survival ◽  
Anaplastic Large Cell Lymphoma ◽  
Gene Rearrangement ◽  
Cell Lymphoma ◽  
Large Cell ◽  
Female Ratio ◽  
Anaplastic Lymphoma ◽  
Significant Difference ◽  
Cd56 Expression ◽  
Large Cell Lymphoma

Abstract Background Anaplastic large cell lymphoma (ALCL) with uniform CD56 expression is a rare condition, that has been described in limited literature, and its clinicopathological features have not yet been well illustrated. The aim of our study was to fully investigate the clinical, histological, immunohistochemical and molecular features of CD56+ ALCL. Methods The clinical and histological characteristics of CD56+ ALCL cases were retrospectively evaluated. The immunohistochemical phenotype, status of Epstein-Barr virus (EBV) and T-cell receptor (TCR) gene rearrangement were examined. Overall survival was also analyzed. Results Eighteen (5.8%) cases with diffuse CD56 expression were identified out of 313 archived ALCL cases with CD56 test. CD56 expression was significantly higher in ALK+ systemic ALCLs (sALCLs) (13/64, 20.3%) than in ALK- sALCLs (3/101, 3.0%) (p < 0.001) as well as primary cutaneous ALCLs (2/148, 1.4%) (p < 0.001). Regarding the CD56+ ALK+ sALCLs, the median age was 20 years (range, 8–60 years) with a male-to-female ratio of 2.3:1, and these cases more frequently affected extranodal sites (11/38, 28.9%) rather than lymph nodes (2/26, 7.7%) (p = 0.038). Eleven (84.6%) cases presented with stage I-II diseases, which was significantly more than their CD56- ALK+ counterparts (45.5%) (p = 0.015). Histologically, 2 ALK+ cases were of small cell variant and all the others displayed characteristic morphology of classic ALCL. Regarding the immunophenotype, both CD30 and CD56 were diffusely positive in all cases. CD3, CD43, anaplastic lymphoma kinase-1 (ALK1), TIA-1, EMA expression was observed in 30.8% (4/13), 90.9% (10/11), 100% (13/13), 100% (9/9), and 80.0% (8/10) cases, respectively. EBV infection was consistently absent. Monoclonal TCR gene rearrangement was found in 100% (5/5) of investigated ALK+ cases. Chemotherapy with a CHOP regimen was most frequently employed. The 3-year overall survival (OS) rate for CD56+ ALK+ patients was 92.0%, compared with 73.0% for their CD56- counterparts, but there was no significant difference in OS between the two groups (p = 0.264). Conclusions Uniform CD56 expression is an unexpected condition in ALCL. Of ALK+ ALCLs, CD56 expression correlated with a high frequency of early stage and an extranodal predominance. It is of great importance to raise awareness of this condition and familiarity with its characteristic features to avoid diagnostic and therapeutic pitfalls. Further investigations are warranted for a better understanding of this unusual phenotype and the significance of CD56 expression in ALCL.

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