Survival Outcomes and Prognostic Factors in Mycosis Fungoides/Sézary Syndrome: Validation of the Revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer Staging Proposal

Purpose We have analyzed the outcome of mycosis fungoides (MF) and Sézary syndrome (SS) patients using the recent International Society for Cutaneous Lymphomas (ISCL)/European Organisation for Research and Treatment of Cancer (EORTC) revised staging proposal. Patients and Methods Overall survival (OS), disease-specific survival (DSS), and risk of disease progression (RDP) were calculated for a cohort of 1,502 patients using univariate and multivariate models. Results The mean age at diagnosis was 54 years, and 71% of patients presented with early-stage disease. Disease progression occurred in 34%, and 26% of patients died due to MF/SS. A significant difference in survival and progression was noted for patients with early-stage disease having patches alone (T1a/T2a) compared with those having patches and plaques (T1b/T2b). Univariate analysis established that (1) advanced skin and overall clinical stage, increased age, male sex, increased lactate dehydrogenase (LDH), and large-cell transformation were associated with reduced survival and increased RDP; (2) hypopigmented MF, MF with lymphomatoid papulosis, and poikilodermatous MF were associated with improved survival and reduced RDP; and (3) folliculotropic MF was associated with an increased RDP. Multivariate analysis established that (1) advanced skin (T) stage, the presence in peripheral blood of the tumor clone without Sézary cells (B0b), increased LDH, and folliculotropic MF were independent predictors of poor survival and increased RDP; (2) large-cell transformation and tumor distribution were independent predictors of increased RDP only; and (3) N, M, and B stages; age; male sex; and poikilodermatous MF were only significant for survival. Conclusion This study has validated the recently proposed ISCL/EORTC staging system and identified new prognostic factors.

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Long-term outcomes of patients with advanced-stage cutaneous T-cell lymphoma and large cell transformation

Blood ◽

10.1182/blood-2008-05-154609 ◽

2008 ◽

Vol 112 (8) ◽

pp. 3082-3087 ◽

Cited By ~ 103

Author(s):

Suzanne O. Arulogun ◽

H. Miles Prince ◽

Jonathan Ng ◽

Stephen Lade ◽

Gail F. Ryan ◽

...

Keyword(s):

De Novo ◽

Cell Transformation ◽

Early Stage ◽

Large Cell ◽

Tumor Stage ◽

Advanced Stage ◽

Long Term Outcomes ◽

Stage Disease ◽

Large Cell Transformation

Abstract Although mycosis fungoides (MF) is typically an indolent disease, patients with advanced-stage disease (stages IIB-IVB), including Sézary syndrome (SS), often have a poor outcome. A 31-year, retrospective analysis of our cutaneous lymphoma database, of 297 patients with MF and SS, was undertaken to study long-term outcomes and identify clinical predictors of outcome in patients with advanced-stage disease (ASD, n = 92) and large cell transformation (LCT, n = 22). Two-thirds of patients with ASD presented with de novo ASD. The median overall survival (OS) for ASD was 5 years with a 10-year predicted OS of 32%. Age at initial diagnosis (P = .01), tumor stage (P = .01), and clinical stage (P = .001) were found to be significant predictors of outcome. Patients who presented with de novo ASD demonstrated better outcomes that were not statistically significant than those with a prior diagnosis of early-stage MF (P = .25). Transformation developed in 22 of the 297 MF/SS patients (7.4%), with a transformation rate of only 1.4% in patients with early-stage disease, compared with stage IIB (27%) and stage IV (56%-67%) disease. The median OS from diagnosis of LCT was 2 years. We confirm that the incidence of LCT is strongly dependent on tumor stage at diagnosis, and we demonstrate a much lower overall risk of LCT than previously reported.

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Clinical outcomes and impact of prognostic factors following adjuvant radiotherapy in oral tongue cancer patients

The Egyptian Journal of Otolaryngology ◽

10.1186/s43163-021-00168-9 ◽

2021 ◽

Vol 37 (1) ◽

Author(s):

Sidharth Pant ◽

Punita Lal ◽

Shagun Misra ◽

Piyush Gupta ◽

K. J. Maria Das ◽

...

Keyword(s):

Prognostic Factors ◽

Cancer Patients ◽

Advanced Disease ◽

Tongue Cancer ◽

Early Stage ◽

Locally Advanced ◽

Oral Tongue ◽

Early Stage Disease ◽

Stage Disease ◽

Oral Tongue Cancer

Abstract Background The purpose of the study was to evaluate survival outcomes in post-operative oral tongue cancer patients undergoing adjuvant radiotherapy (RT) at a tertiary cancer care center and to critically review the impact of various clinical-pathological factors on recurrence and survival. Demographic factors, stage of all the histology proven oral tongue cancer, and treatment details were documented. Overall survival (OS) and recurrence-free survival (RFS) were analyzed along with the potential prognostic factors affecting outcome. Results One hundred forty-four post-operative oral tongue cancer patients referred to our department for adjuvant treatment were evaluated. Median age at presentation was 45 years. Forty-seven patients had pathological early stage disease (stages I and II) and 95 had locally advanced (stages III and IV) disease while post-op details were not present in 2 patients. At a median follow-up of 87 months (60–124) of alive patients, the median RFS for entire cohort was 62 months while median OS was 74 months respectively. Age, perineural invasion (PNI), and grade of the tumor emerged as independent prognostic factors for OS and RFS. Among patients with early stage disease, depth of invasion (DOI), age, and PNI were found as independent prognostic factors for RFS and OS. In locally advanced disease, higher grade, age, and PNI independently impacted the respective survival end points. Conclusions Age (> 45 years), higher grade, and presence of PNI showed inferior survival outcomes across the sub-groups (early versus locally advanced disease). This may warrant adjuvant treatment intensification. DOI > 10 mm was particularly found to worsen survival in early node negative SCC oral tongue patients.

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Response Rate of 16% and High Incidence of Adverse Events In Placebo-Treated Patients with CD25 Assay-Positive Cutaneous T-Cell Lymphoma (CTCL): An Analysis of Patient Characteristics and Predictors of Response

Blood ◽

10.1182/blood.v116.21.4158.4158 ◽

2010 ◽

Vol 116 (21) ◽

pp. 4158-4158

Author(s):

Miles Prince ◽

Madeleine Duvic ◽

Ann Martin ◽

Wolfram Sterry ◽

Chalid Assaf ◽

...

Keyword(s):

Prognostic Factors ◽

Adverse Events ◽

Active Treatment ◽

Response Rate ◽

Early Stage ◽

Disease Stage ◽

Early Stage Disease ◽

Stage Disease ◽

Hodgkin's Lymphomas ◽

Systemic Antibiotics

Abstract Abstract 4158 Background: CTCLs are rare, extranodal non-Hodgkin's lymphomas characterized by the accumulation of malignant T lymphocytes in the skin; mycosis fungoides (MF) and Sézary syndrome (SS) are the most common variants. Their clinical course is typically chronic and progressive, despite multiple therapeutic interventions. Observational studies of patients with CTCL, although limited by rarity, are necessary to improve understanding of prognostic factors. Aims: The goals of this analysis were to describe the initial disease characteristics, prognostic factors, and natural history of disease observed in 44 patients with CD25-positive, recurrent/persistent CTCL included in the placebo arm of Study L4389-11. We were particularly interested to determine if there were any characteristics peculiar to the 16% of patients treated with placebo who were observed to have a response in this study. Methods: This multicenter, randomized, double-blind, placebo-controlled phase III trial compared 2 different doses of denileukin diftitox (9 or 18 μ g/kg/day) with placebo in 144 patients with histopathologically confirmed stage IA to III MF/SS who had received ≤3 previous therapies. Medications essential for patient welfare (cardiac medications, narcotics, anti-nausea agents, vitamins, or laxatives) and transfusions were also allowed. The primary efficacy endpoint was overall response rate (ORR); progression-free survival (PFS) was a secondary endpoint. Relationships between baseline covariates and clinical outcomes were assessed by multivariate regression analyses. Results: Placebo-treated patients had a median age of 59.0 years, and the majority were white (77.3%), had early-stage disease (stage <IIA; 68.2%), had a diagnosis of MF (84.1%), and had <20% circulating abnormal lymphocytes (85.4%) at baseline. The ORR was 15.9% (95% exact confidence interval [CI]: 6.6%-30.1%) for patients receiving placebo (complete response: 2.3%; partial response: 13.6%), reflecting a possible baseline rate of spontaneous disease remission expected in untreated CTCL. Of the 7 responding patients, 3 were treated during the trial with systemic anti-staphylococcal antibiotics (2 pts with IIB, 1pt with erythroderma). Of the remaining 4 pts the stages were 1A (1), IB (2), IIB (1). Median PFS according to Kaplan-Meier analysis was 124 days (4.1 months) (95% exact CI: 92–176 days (3.0-5.8 months)), and the median values for patients with stage ≤IIA disease and stage ≥IIB disease at baseline were 107 days (3.5 months) and 211 days (6.9 months), respectively. Multivariate analyses identified no significant effects of any baseline factors on either ORR or PFS, although there was a trend toward poorer PFS associated with advanced age (hazard ratio: 1.21; 95% CI: 0.97–1.52; P=.0947). These analyses were likely limited by small sample size. A total of 95.5% of patients in the placebo arm used rescue medications (skin moisturizers etc.) at least once during the treatment period. Adverse events (AEs) and treatment-related AEs occurred in 90.9% and 59.1% of placebo-treated patients, respectively. General disorders and administration site conditions were the most common types of treatment-related AEs (52.3%), which included fatigue (22.7%), rigors (15.9%), peripheral edema (15.9%), pyrexia (13.6%), and pain (2.3%). Sepsis occurred significantly more often with placebo versus active treatment (6.8% vs. 0%; P < .05). Three of the 7 (43%) responders had received systemic antibiotics and 14/37 non-responders had received systemic antibiotics (38%). Conclusion: The findings from this study demonstrate that responses can be observed in patients with MF/SS in the absence of anti-tumor treatment. Likely contributing factors to these ‘spontaneous responses' are the use of systemic antibiotics, aggressive ‘rescue medication' use and the waxing and waning nature of skin lesions, particularly in early-stage disease. All future studies should carefully document the use of systemic antibiotics during therapy. These results can serve as standard comparators for other active-treatment studies that lack a placebo control arm. Disclosures: Prince: Eisai: Consultancy, Honoraria. Duvic:Eisai: Consultancy, Research Funding, Speakers Bureau. Assaf:Eisai: Consultancy, Honoraria. Straus:Eisai: Consultancy, Honoraria.

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Prognostic Factors for Small Cell Neuroendocrine Carcinoma of the Uterine Cervix: An Institutional Experience

International Journal of Gynecological Cancer ◽

10.1097/igc.0000000000000059 ◽

2014 ◽

Vol 24 (2) ◽

pp. 272-279 ◽

Cited By ~ 15

Author(s):

Suthida Intaraphet ◽

Nongyao Kasatpibal ◽

Sumalee Siriaunkgul ◽

Anchalee Chandacham ◽

Kornkanok Sukpan ◽

...

Keyword(s):

Prognostic Factors ◽

Early Stage ◽

Small Cell ◽

International Federation ◽

Advanced Stage ◽

Stromal Invasion ◽

Small Cell Neuroendocrine Carcinoma ◽

Early Stage Disease ◽

Gynecology And Obstetrics ◽

Stage Disease

ObjectiveThis study aimed to determine the clinicopathologic characteristics that affected the survival in patients with small cell neuroendocrine carcinoma of the uterine cervix (SNEC).Materials and MethodsAll patients with SNEC treated at Chiang Mai University Hospital between January 1995 and October 2011 were retrospectively reviewed with histologic confirmation of SNEC diagnosis. The prognostic predictors for survival were assessed using competing risk regression analysis concerning the probabilities of competing events.ResultsOne hundred thirty histologically confirmed patients with SNEC met the study criteria. The median overall survival and median cancer-specific survival (CSS) for entire group were 47.8 and 58.1 months, respectively. Five-year CSS for patients with early-stage disease was 62.6% and for patients with advanced-stage disease was 18.1% (P< 0.001). Among the patients with surgically treated early-stage disease, those with adjuvant chemotherapy had a better 5-year survival rate than those with surgery alone, those with adjuvant radiotherapy, and those with adjuvant chemoradiation therapy (P= 0.041). In multivariable analyses, decreased survival in patients with early-stage disease was associated with age older than 60 years at diagnosis (hazards ratio [HR], 4.9;P= 0.007) and deep stromal invasion (HR, 2.9;P= 0.011). Among the patients with advanced-stage disease, decreased survival was associated with age at diagnosis (older than 60 years: HR, 9.9;P< 0.001 and younger than 45 years: HR, 3.4;P= 0.035) and International Federation of Gynecology and Obstetrics stage IV (HR, 7.4;P= 0.024).ConclusionsInternational Federation of Gynecology and Obstetrics stage, age at diagnosis, and deep stromal invasion were important prognostic factors for CSS in patients with SNEC. Adjuvant chemotherapy may provide survival benefits in surgically treated patients with early-stage SNEC.

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Is breast implant-associated anaplastic large cell lymphoma a hazard of breast implant surgery?

Open Biology ◽

10.1098/rsob.190006 ◽

2019 ◽

Vol 9 (4) ◽

pp. 190006 ◽

Cited By ~ 11

Author(s):

Florian Fitzal ◽

Suzanne D. Turner ◽

Lukas Kenner

Keyword(s):

Anaplastic Large Cell Lymphoma ◽

Local Governments ◽

Cell Lymphoma ◽

Early Stage ◽

Breast Implant ◽

Large Cell ◽

Early Stage Disease ◽

True Incidence ◽

Stage Disease ◽

Large Cell Lymphoma

Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) may occur after reconstructive or aesthetic breast surgery. Worldwide, approximately 1.7 million breast implant surgeries are performed each year. To date, over 500 cases of BIA-ALCL have been reported around the world, with 16 women having died. This review highlights the most important facts surrounding BIA-ALCL. There is no consensus regarding the true incidence rate of BIA-ALCL as it varies between countries, is probably significantly under-reported and is difficult to estimate due to the true number of breast prostheses used largely being unknown. BIA-ALCL develops in the breast mostly as a seroma surrounding the implant, but contained within the fibrous capsule, or more rarely as a solid mass that can become invasive infiltrating the chest wall and muscle, in some instances spreading to adjacent lymph nodes, in these cases having a far worse prognosis. The causation of BIA-ALCL remains to be established, but it has been proposed that chronic infection and/or implant toxins may be involved. What is clear is that complete capsulectomy is required for treatment of BIA-ALCL, which for early-stage disease leads to cure, whereas chemotherapy is needed for advanced-stage disease, whereby improved results have been reported with the use of brentuximab. A worldwide database for BIA-ALCL and implants should be supported by local governments.

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Mycosis Fungoides in the Pediatric Population: Report from an International Childhood Registry of Cutaneous Lymphoma

Journal of Cutaneous Medicine and Surgery ◽

10.2310/7750.2009.08091 ◽

2010 ◽

Vol 14 (1) ◽

pp. 1-6 ◽

Cited By ~ 57

Author(s):

Elena Pope ◽

Sheila Weitzman ◽

Bo Ngan ◽

Scott Walsh ◽

Kimberly Morel ◽

...

Keyword(s):

Mycosis Fungoides ◽

Clinical Characteristics ◽

Pediatric Patients ◽

Early Stage ◽

Cutaneous Lymphoma ◽

Topical Steroids ◽

Early Stage Disease ◽

Stage Disease ◽

Cutaneous Lymphomas ◽

Pathologic Features

Background/Objectives: There are limited data on the clinical presentation and progression of pediatric cutaneous lymphoma. This study focuses on the clinical characteristics of pediatric patients with mycosis fungoides (MF). Materials and Methods: This descriptive study presents clinical characteristics of 22 pediatric patients with MF, enrolled in the international Childhood Registry for Cutaneous Lymphomas (CRCL). Results: The mean ages at onset and at diagnosis were 7.5 (SD 3.8 years) years and 9.9 (SD 3.4) years, respectively. The most common MF presentation was patch stage (68%), followed by hypopigmentation (59%) and plaque stage disease (50%). Epidermotropism and lymphocytic atypia were the most common pathologic features, found in 89% and 85%, respectively. Cerebriform nuclei were noted in 42%, and Pautrier microabscesses were seen in 16% of cases. A cytotoxic pattern was more commonly seen (67% vs 33%), and clonality was detected in 21% (3 of 14) of patients. All patients presented with early-stage disease and received skin-directed therapy (topical steroids, 73%; light therapy, 54%; or combination therapy, 35%). Conclusions: Pediatric patients with MF present in the first decade of life, with early-stage disease and unusual forms such as hypopigmented variant. Further patient enrolment will provide information regarding natural history, treatment response, and overall prognosis of pediatric cutaneous T-cell lymphoma (CTCL).

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NCCN Guidelines Insights: Primary Cutaneous Lymphomas, Version 2.2020

Journal of the National Comprehensive Cancer Network ◽

10.6004/jnccn.2020.0022 ◽

2020 ◽

Vol 18 (5) ◽

pp. 522-536 ◽

Cited By ~ 3

Author(s):

Neha Mehta-Shah ◽

Steven M. Horwitz ◽

Stephen Ansell ◽

Weiyun Z. Ai ◽

Jeffrey Barnes ◽

...

Keyword(s):

Systemic Therapy ◽

Cell Lymphoma ◽

Early Stage ◽

Brentuximab Vedotin ◽

Cutaneous T Cell Lymphoma ◽

Early Stage Disease ◽

Therapy Options ◽

Nccn Guidelines ◽

Stage Disease ◽

Cutaneous Lymphomas

Mycosis fungoides (MF) is the most common subtype of cutaneous T-cell lymphoma (CTCL), and Sézary syndrome (SS) is a rare erythrodermic and leukemic subtype of CTCL characterized by significant blood involvement. Although early-stage disease can be effectively treated predominantly with skin-directed therapies, systemic therapy is often necessary for the treatment of advanced-stage disease. Systemic therapy options have evolved in recent years with the approval of novel agents such as romidepsin, brentuximab vedotin, and mogamulizumab. These NCCN Guidelines Insights discuss the diagnosis and management of MF and SS (with a focus on systemic therapy).

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