Diagnosis Of Primary Cutaneous Lymphoma Research Articles

Epidemiology of cutaneous T-cell lymphomas: state of the art and a focus on the Italian Marche region.

Among primary cutaneous T-cell lymphomas (CTCL), mycosis fungoides (MF) is the most frequent and, along with Sézary syndrome (SS), the best-studied subtype. Most available studies on epidemiology of MF and SS are based on small cohorts or different inclusion criteria. Moreover, although this has become a hot topic, most studies show limitations, such as selection bias and lack of clinical information or follow-up data. Therefore, no reliable conclusions can be drawn. This paper reviews the current data underpinning our understanding of the epidemiology of MF and SS, and presents some original findings based on data retrieved from the cutaneous lymphoma registry of the Italian Marche region. The Marche Regional Cutaneous Lymphoma Registry is a multidisciplinary team founded 27 years ago to share the management of these rare disorders. All patients with a clinical and histologically confirmed diagnosis of primary cutaneous lymphoma are centralized in Ancona (Italy) at the Haematology Clinic, Polytechnic University of Marche, for clinical evaluation, staging, treatment, and follow-up. This paper emphasizes the need for a national registry of pCLs in Italy, as no detailed epidemiological information is available in the country except for the Marche Regional Cutaneous Lymphoma Registry. A national registry would allow for more comprehensive data collection from all over Italy and could provide more accurate information on incidence and epidemiology. This would be beneficial for understanding the pathogenesis and diagnostic procedures of these diseases and could improve patient outcomes. Therefore, we advise the creation of a national registry of pCLs in Italy.

Epi-P-03 - Association between primary cutaneous lymphomas and other primary malignancies in patients’ personal or family history in Greek population; a retrospective single-center study

<h3>Background:</h3> Primary cutaneous lymphomas (PCLs) consist of an heterogenous group of rare malignancies. Cutaneous T-cell lymphomas are the most common among them, with mycosis fungoides being the main representative. The etiology of PCLs is rather unknown; however, there are indications that genetic factors may play a role in CTCL developments [1]. Several studies have examined the incidence of second malignancies in patients with PCLs, as well as their family history of malignancies [2–4]. In our study we examine the association between PCLs and other primary malignancies in patients' personal or family history in Greek population. <h3>Methods:</h3> This retrospective study included 144 patients with histopathologically confirmed diagnosis of PCL and 130 controls matched by gender and age (±4 years). All patients and controls completed a detailed questionnaire regarding sun exposure habits-including personal history of sunburns, use of photo-protective measures, use of sunbeds- exposure to ionizing radiation, smoking and alcohol consumption habits, detailed medical personal history (diseases, medication, other chronic skin conditions, personal history of malignancies, etc.) and family history (chronic skin conditions, malignancies). Statistical analysis of the data was performed using PASW Statistics v25 software. <h3>Results:</h3> Of the 144 patients included, 78 (54.2%) were males and 66 (45.8%) females (median age 57 years old) and among the 130 control subjects, 70 (53.8%) were males and 60 (46.2%) females (median age 61 years old). Analysis showed that family history of solid organ cancer is related to higher relative risk (OR 1.815) of CL development (p<0.05, 95% CI 1.11–2.95). The two types of solid organ tumors more highlighted were liver cancer (p=0.046) and stomach cancer (p=0.002). Family history of skin cancer as well as hematologic malignancies doesn't seem to affect the risk of CL in statistically significant way (p=0.247 and p=0.093 relatively) in our group. As far as the personal history of other primary malignancies is concerned, statistical analysis showed that there might be a correlation between other malignancies and primary cutaneous lymphomas (p<0.05). At this point we should notice the limitation that the number of patients and controls having a personal history of any type of cancer was small, but it consists a predictor. <h3>Conclusion:</h3> There are several studies analyzing the correlation of PCLs and second primary cancers. The results of our study showed an association of personal history of cancer with PCLs, as well as a correlation of family history of solid organ malignancy with a higher relative risk of cutaneous lymphoma development. No higher relative risk was detected in subjects with family history of hematologic malignancies. We believe that it is important to be aware of the personal and the family history of patients with PCL, as far as the malignancies are concerned, and try to early recognize possible second malignancies in these patients.

Epidemiological changes in cutaneous lymphomas: an analysis of 8593 patients from the French Cutaneous Lymphoma Registry.

Primary cutaneous lymphomas (PCLs) are a heterogeneous group of T-cell (CTCL) and B-cell (CBCL) malignancies. Little is known about their epidemiology at initial presentation in Europe and about potential changes over time. The aim of this retrospective study was to analyse the frequency of PCLs in the French Cutaneous Lymphoma Registry (GFELC) and to describe the demography of patients. Patients with a centrally validated diagnosis of primary PCL, diagnosed between 2005 and 2019, were included. The calculated incidence was unprecedently high at 1·06 per 100000 person-years. The number of included patients increased yearly. Most PCL subtypes were more frequent in male patients, diagnosed at a median age of 60years. The relative frequency of rare CTCL remained stable, the proportion of classical mycosis fungoides (MF) decreased, and the frequency of its variants (e.g. folliculotropic MF) increased. Similar patterns were observed for CBCL; for example, the proportion of marginal-zone CBCL increased over time. Changes in PCL frequencies may be explained by the emergence of new diagnostic criteria and better description of the entities in the most recent PCL classification. Moreover, we propose that an algorithm should be developed to confirm the diagnosis of PCL by central validation of the cases.

The clinical value of imaging in primary cutaneous lymphomas: Role of high resolution ultrasound and PET-CT.

Primary cutaneous lymphoma is a rare extranodal non-Hodgkin's lymphoma confined to the skin. The data on the imaging findings of primary cutaneous lymphomas are largely lacking and the current diagnosis is based on clinical and histopathological examination. With the advances in dermatological ultrasound and molecular imaging, newer perspectives in the evaluation of cutaneous lymphomas are available. To review and describe the imaging findings in patient's with the diagnosis of primary cutaneous lymphoma. A multicentric, retrospective observational study was undertaken in four countries to review the high resolution ultrasonography (HRUS) and fluorine 18-fludeoxyglucose positron emission tomography-computed tomography (PET-CT) imaging findings. We had 41 patients, Female:Male 1:4.1; mean age, 57 years; range, 13-94 years. High resolution ultrasonography of the primary cutaneous lesions revealed thickening of the dermis in all the cases and the lesions were hypoechoic without any calcifications or central necrosis. The sonographic appearances of the lesions were categorised into focal infiltrative, nodular, pseudonodular, and diffusely infiltrative patterns. Nodular and pseudonodular lesions were predominant in B cell lymphomas, while diffusely infiltrative lesions were more common in T-cell lymphomas. On colour Doppler imaging, the lesions were hypervascular. Whole body 18F-fludeoxyglucose PET-CT imaging of the patients revealed increased uptake of the metabolite in the lesions. Sonographic patterns based on high resolution ultrasonography provide early clues to the non-invasive diagnosis of primary cutaneous lymphomas and PET-CT is the recommended modality of imaging for staging and follow-up. High resolution ultrasound with colour Doppler and PET-CT imaging are complimentary to the clinical diagnosis of primary cutaneous lymphomas.

Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Cutaneous Lymphoma—Inpatient Considerations

Purpose of review This is an overview of the important considerations for hospitalized patients with primary cutaneous lymphoma (PCL). The evaluation and management of inpatients with a suspected or confirmed diagnosis of PCL are discussed. Additionally, attention is paid to the complications associated with therapy for PCL that might warrant inpatient care.

Primary cutaneous lymphoma in southern Brazil: a 12-year single-center experience.

Primary cutaneous lymphomas (PCL) are a group of extranodal non-Hodgkin lymphomas presenting with no evidence of extracutaneous disease at the time of diagnosis. Few longitudinal studies of PCL have been conducted, particularly in South American populations. Our objective was to describe the behavior of PCL and evaluate patient survival in a cohort of patients from southern Brazil. We conducted a retrospective cohort study of all patients with a histopathological diagnosis of PCL receiving care at a tertiary referral center in southern Brazil from 2000 to 2012. Clinical, laboratory, and histopathological data, treatment variables, and survival curves were analyzed. Eighty-nine patients with PCL were included, with a mean age at diagnosis of 58.9 years. Cutaneous T-cell and natural killer (NK) cell lymphomas (CTCL/NKCL) accounted for 78.4% of cases and cutaneous B-cell lymphomas (CBCL) for 21.6%; 57.4% of patients with CTCL/NKCL and 50% of patients with CBCL were male. Half of all cases were mycosis fungoides (MF). The 5-year survival rate was 74.8% for MF, 61% for Sézary syndrome, 87.5% for primary cutaneous CD30+ lymphoproliferative disorders, and 88.9% for primary cutaneous follicle center lymphoma. In this cohort of patients with PCL from southern Brazil, CTCL/NKCL was more prevalent than CBCL, and the MF subtype was predominant. Survival curves were similar to those reported in the literature, except for Sézary syndrome, which had a better prognosis in this sample.

Best practices in diagnostic immunohistochemistry: workup of cutaneous lymphoid lesions in the diagnosis of primary cutaneous lymphoma.

Primary cutaneous lymphoma is a common extranodal non-Hodgkin lymphoma. These lesions share common features with their nodal counterparts, but also have differences in morphology, unique clinical presentations, and immunohistochemical features. To review the 2008 World Health Organization (WHO) and 2005 consensus WHO-EORTC (European Organisation for Research and Treatment of Cancer) classifications, and address the immunohistochemical findings in the most common primary cutaneous T- and B-cell lymphomas. Since clonality testing is commonly used as an ancillary test in the evaluation of cutaneous lymphoma, a brief section in the use and pitfalls of clonality testing is included. The WHO and EORTC classification publications and the relevant recent literature were used to compile appropriate and practical guidelines in this review. The practice of dermatopathology and hematopathology varies widely. Thus, while this review provides an overview and guideline for the workup of lymphoid lesions of the skin, the practitioner should understand the importance of clinical correlation as well as appropriate utility of available resources (such as clonality testing) in arriving at a diagnosis in cutaneous lymphoid lesions.

Primary cutaneous lymphomas: A clinical and histological study of 99 cases in Isfahan, Iran.

Background:Primary cutaneous lymphomas (PCLs) represent a heterogeneous group of T- and B-cell lymphomas that present in the skin with no evidence of extracutaneous disease at the time of diagnosis. The aim of this study was to assess and report the epidemiological characteristics of PCLs in Isfahan, Isfahan Province, Iran – as a main province of Iran.Materials and Methods:A total of 99 patients were recruited over a recent 10-year period (2003-2013) with diagnosis of PCLs; the patients were classified according to the The World Health Organization/European Organization for Research and Treatment of Cancer (WHO-EORTC) criteria. Mean and standard deviations (SDs) were used to describe continuous data, numbers, and percentages for categorical data. Statistical significance was defined as P < 0.05.Results:The patients comprised 45 men and 54 women aged 5-80 years (median 36) at diagnosis. The male-to-female ratio was 1:1.2. Histological examination showed features of primary cutaneous B-cell lymphomas (PCBCLs) in four cases. The mean ± SD age in primary cutaneous T-cell lymphomas (PCTCLs) and PCBCLs was 37.9 ± 16.5 years and 39.7 ± 9.1 years, respectively (P = 0.72). The mean ± SD latent period between the time of diagnosis and initiation of skin lesions in men and women was 2.3 ± 4.1 years and 5.9 ± 10.1 years, respectively (P = 0.02). The most frequent subtypes were mycosis fungoides (MFs) (86.9%) followed by Sιzary syndrome (SS) (4%). Five patients died from PCL-related deaths.Conclusion:The distinguishing epidemiologic characteristics of PCL in Iran are the absence of a male predominance and a lower age of diagnosis. The study highlights the ethnic or regional variations in the clinicoepidemiological characteristics of PCLs.

Aggressive Behavior and Elevated Lactate Dehydrogenase at Baseline Confer Inferior Prognosis in Patients With Primary Cutaneous Lymphoma

Primary cutaneous lymphoma (PCL) comprises a heterogeneous group of diseases in regard to clinical presentation, histologic appearance, and biological behavior. Its rare occurrence limits analysis of disease features and survival of patients. All patients with PCL treated in our hospital during January 2006 to October 2012 were included in this retrospective study. Their histologic and clinical data were analyzed. The diagnosis of PCL and the evaluation of clinical behavior were based on the 2005 World Health Organization-European Organisation for Research and Treatment of Cancer (WHO-EORTC) classification. Fifty-four cases of PCL were included in the study. The median age was 52.5 years. Thirteen (24.1%) patients had B-cell lymphoma and 41 (75.9%) had T-cell lymphoma. Twenty-nine (53.7%) patients exhibited disease having indolent clinical behavior, 14 (25.9%) presented with B symptoms, and 16 (29.6%) had elevated lactate dehydrogenase (LDH) levels at baseline. Within a median follow-up of 47.8 months, the expected 5-year progression-free survival (PFS) rate and overall survival (OS) rate were 6% and 14%, respectively. Using multivariate analysis, aggressive behavior (hazard ratio [HR], 2.92; P = .01] and elevated LDH levels at baseline (HR, 2.88; P = .01) were identified as independent risk factors for PFS. In addition, aggressive behavior (HR, 4.09; P = .01) and elevated LDH levels at baseline (HR, 3.69; P = .01) were also identified as independent risk factors for OS. The study data suggest that aggressive behavior and elevated LDH levels at baseline were predictive factors for poor PFS and OS, which supports the need for immediate treatment of those patients.

AN UNUSUAL CASE OF PRIMARY CUTANEOUS LYMPHOMA PRESENTING IN THE UPPER EXTREMITY: A CASE REPORT

Primary cutaneous lymphomas rarely present to the hand surgeon. Diagnosis of primary cutaneous lymphoma in the upper extremity can be difficult to make, as they are rare, and may be mistaken for other lesions. We report an unusual case of primary cutaneous lymphoma presenting in the upper extremity. This rare differential should be considered when encountered with a lump of similar appearance in the upper extremity.

Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Other Tumours in Primary Cutaneous Lymphomas.

Patients with primary cutaneous lymphomas (PCLs) are treated with multiple therapeutic regimens, which may increase the risk of subsequent solid and haematological neoplasms. The aim of our study was to assess the incidence of other malignancies in our series of PCLs. From March 1994 to January 2007, 272 patients with PCLs (179 M, 93 F, median age 65 yr, range 14–88) were referred to our center for staging, treatment and follow-up. The clinical charts were reviewed to detect the incidence of malignancies occurred before or after the diagnosis of PCL or concomitantly. The series was composed by 228 patients (150 M, 78 F, median age 66 yrs) with T-cell lymphomas (202 Mycosis Fungoides/MF, 10 Sézary Syndrome/SS, 9 CD30+ PCL, 7 non MF/non CD30+ T cell PCL); 43 patients (28 M, 15 F, median age 60 yrs) with B-cell lymphomas (25 Follicular/FL, 14 marginal/MZL, 3 Leg-type, 1 Lymphoblastic) and one patient with CD4+/CD56+ hematodermic neoplasm. Chemotherapy was administered to 48 patients. During follow-up 12 patients died for the disease and 24 for other causes. A second tumor was observed in 41 patients (15%): 6 of them experienced more than one neoplasms: overall we observed 48 malignancies, 38 solid and 10 haematological. The other neoplasms appeared similarly before (20) and after (21) the diagnosis of PCL; in 7 cases they were diagnosed simultaneously. Solid tumours (17 preceding, 4 concurrent, 17 subsequent) were diagnosed in: skin (11), colon (5), lung (4), breast (3), CNS (3), bladder (2), liver (2), kidney (2), uterus (2), testis (1), prostate (1), stomach (1), thyroid (1). The haematological malignancies (3 preceding, 3 concurrent, 4 subsequent) were: B-cell lymphomas (4), acute myeloid leukemias (3), plasmocytoma (1), T-cell lymphoma (1), Hodgkin's lymphoma (1). Among the six patients with more than one adjunctive neoplasms one patient had lung and kidney carcinoma preceding PCL; two patients a preceding carcinoma (skin and bladder, respectively) and subsequently a lung carcinoma; other two patients showed both a preceding and a concurrent neoplasm (skin and colon carcinoma, B-cell lymphoma and skin carcinoma, respectively). Finally a patient had a preceding skin carcinoma, a concurrent nodal Hodgkin's lymphoma and a subsequent nodal B-cell lymphoma. So we have reported 48 other neoplasms in 41 patients within 272 PCLs (15%). The occurrence of the other malignancy was not related to the B/T phenotype of PCLs, as it was observed in 35/228 (15.4%) T-cell lymphomas (32 MF, 2 SS, 1 non MF/non CD30+ T cell lymphoma) and in 6/43 (14%) B-cell lymphomas (3 FL, 3 MZL; χ2 test: P=0.88). The interval of occurrence was longer for tumors preceding (median 60 mo.s, range 8–180) than for tumors following PCL (median 45, range 6–122). The administration of chemotherapy for PCL was not associated with an increased incidence of second neoplasm(χ2 test, P=0.77). Multicentric studies might help in elucidating the role of genetic and immunitary factors in the pathogenesis of multiple neoplasms in patients with PCLs.

18 F-fluorodeoxyglucose-positron emission tomography in evaluation of primary cutaneous lymphoma

The diagnosis of primary cutaneous lymphoma (PCL) is currently based on clinical and histological findings and/or relatively invasive procedures such as bone marrow and fine-needle lymph node biopsies. Although computed tomography (CT) is a noninvasive imaging modality that is widely used for staging in patients with lymphoma, it cannot provide information about malignant cutaneous lesions. To investigate the usefulness of (18)F-fluorodeoxyglucose (FDG)-positron emission tomography (PET) in the management of PCL. We retrospectively analysed 31 FDG-PET studies in 19 patients with PCL [15 T-cell non-Hodgkin lymphoma (NHL) and four B-cell NHL]. There were 10 men and nine women (age range 23-84 years, mean +/- SD 54 +/- 16). Eleven FDG-PET studies were performed for initial staging and 20 FDG-PET studies were performed for restaging following therapy. Results of FDG-PET were compared with those of CT. Clinical parameters and/or biopsy results of lesions served as reference for the accuracy of PET and CT in evaluating local and metastatic lesions. For the initial staging, FDG-PET had a sensitivity of 82% for the evaluation of local disease and 80% for the detection of distant metastasis. The corresponding values for CT were 55% and 100%, respectively. For restaging of cutaneous lymphoma, FDG-PET had a sensitivity of 86% and specificity of 92% for local recurrence/residual disease and a sensitivity of 100% and specificity of 100% for distant metastasis. The corresponding values for CT were 50% and 83% for local recurrence/residual disease and 100% and 67% for distant metastasis. FDG-PET has a potential value for initial staging and restaging following therapy in patients with PCL. FDG-PET has higher diagnostic value than CT in the detection both of local disease and distant metastasis.

Gene Rearrangements in Cutaneous Lymphoma

Histologic diagnosis of primary cutaneous lymphoma can be very difficult. While immunophenotypic analyses are useful, the results are often