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

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.

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

Blood ◽

10.1182/blood-2019-131991 ◽

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.

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Primary Cutaneous CD30+ Anaplastic Large Cell Lymphoma: Analysis of the Stanford Series Reveals Two Clinical Subsets of Patients.

Blood ◽

10.1182/blood.v104.11.4568.4568 ◽

2004 ◽

Vol 104 (11) ◽

pp. 4568-4568

Author(s):

Sunil A. Reddy ◽

Howard Liu ◽

Sabine Kohler ◽

Richard T. Hoppe ◽

Youn H. Kim

Keyword(s):

Anaplastic Large Cell Lymphoma ◽

Cell Lymphoma ◽

Large Cell ◽

Body Regions ◽

Local Disease ◽

Kaplan Meier ◽

Large Cell Lymphoma ◽

Regional Disease ◽

Multifocal Disease

Abstract Primary cutaneous CD30+ anaplastic large cell lymphoma (PCALCL) represents the malignant end of the spectrum of CD30+ lymphoproliferative disorders. The management of PCALCL is markedly different than that of its primary nodal counterpart. The basis of this difference is due to the less aggressive nature of PCALCL. There is no evidence that chemotherapy is necessary or superior to the excellent results of local therapies such as radiation and/or excision for unilesional or local disease. Even patients with generalized disease have done well in some series. Here we review the characteristics and management of 40 cases of this rare cutaneous lymphoma. We have excluded Lymphomatoid Papulosis (LyP), the benign counterpart of PCALCL. In our series PCALCL was defined as CD30+ ALCL with disease limited to the skin as defined by a negative CT scan. Bone marrow biopsy was not necessary although it was always negative when done. Unilesional, local and extensive regional patients were generally treated with radiotherapy and/or excision, while chemotherapy and biologics were reserved for extensive regional and multifocal patients. The study included 30 men and 10 women with a mean age of 57.5 years (range 24–86). The estimated Kaplan-Meier disease specific survival (DSS) at 4 years for the entire group is 75.6%, with a median follow-up of 27.5 months (1–213). Twenty-six patients presented with unilesional or local disease, while there were 8 multifocal and 6 extensive regional patients (defined as 2 or more lesions beyond a 15 cm2 area apart but limited to 1 or 2 contiguous body regions). The unilesional/local patients had an estimated 4 year DSS of 93%. All but 2 of these patients are alive. The estimated Kaplan-Meier 4 year DSS of patients with either multifocal or extensive regional disease is 30%. Estimated 4 year Kaplan-Meir survival of PCALCL by extent of skin involvement unilesional/local 93% multifocal/extensive regional 30% P-value<0.001 Seven deaths occurred in this series of which 6 were either directly or indirectly attributable to PCALCL. Of the deaths 3 were among the 6 patients with extensive regional disease and 2 were among the 8 patients with multifocal disease. Furthermore, 4 multifocal and 2 extensive regional patients have active disease at the time of last follow-up. Further followup will determine their eventual outcome. The 2 patients with unilesional disease who died, both developed rapid progression to extensive regional disease. Although most patients with PCALCL present with local disease and do quite well with local therapy, it appears that some PCALCL patients with multifocal disease, extensive regional disease, and rapidly progressive unilesional disease behave as high grade lymphomas refractory to standard chemotherapy.

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Primary Cutaneous Anaplastic Large‐Cell Lymphoma (PC‐ALCL): A review of the SEER database from 2005‐2016

Clinical and Experimental Dermatology ◽

10.1111/ced.14777 ◽

2021 ◽

Author(s):

H. Sarfraz ◽

C. Gentille ◽

J. Ensor ◽

L. Wang ◽

S. Wong ◽

...

Keyword(s):

Anaplastic Large Cell Lymphoma ◽

Cell Lymphoma ◽

Large Cell ◽

Seer Database ◽

Large Cell Lymphoma

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Incidence and Outcomes of Primary Cutaneous Anaplastic Large Cell Lymphoma: a SEER Population Based Study (Preprint)

10.2196/preprints.34759 ◽

2021 ◽

Author(s):

Pratik Q. Deb ◽

Harry J. Hurley ◽

Donghong Cai ◽

Chinmoy Bhate

Keyword(s):

United States ◽

Anaplastic Large Cell Lymphoma ◽

Primary Tumor ◽

Cell Lymphoma ◽

White Male ◽

Large Cell ◽

The United States ◽

Molecular Evidence ◽

Large Cell Lymphoma ◽

Highly Correlated

BACKGROUND Primary cutaneous anaplastic large cell lymphoma (PC-ALCL) is the second most common type of cutaneous lymphoma of T cell origin. OBJECTIVE To examine epidemiologic features and outcomes in PC-ALCL in the population of the United States. METHODS We identified patients with PC-ALCL from the Surveillance Epidemiology and End Results 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 Of all 569 cases documented as PC-ALCL, 93 patients fulfilled the inclusion criteria. We found the incidence of PC-ALCL to be highly correlated with increased age. There is a predominance of PC-ALCL in white male population. The survival analysis did not signify age of diagnosis, sex, or race as factors affecting the outcome for the patients. Although the site of primary tumor trends towards affecting survival, it does not meet statistical significance. CONCLUSIONS PC-ALCL is a rare malignancy predominantly affecting older white male in the United States. The increased age is highly correlated with disease development, however, neither the demographic characteristics nor the site of the primary tumor affects the outcome for the patients. CLINICALTRIAL -

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