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Aggressive primary cutaneous T-cell lymphomas include advanced-stage mycosis fungoides (stage ≥ IIB mycosis fungoides), Sézary syndrome, gamma/delta cutaneous lymphoma, nasal type lymphoma, aggressive epidermotropic CD8+ T-cell lymphoma and some cutaneous lymphomas not otherwise specified. To evaluate their long-term prognosis, we conducted a retrospective cohort study of 85 patients diagnosed between 2005 and 2020 with advanced-stage mycosis fungoides (n = 48), Sézary syndrome (n = 28) or aggressive non-mycosis fungoides/Sézary syndrome subtypes (n = 9). The median survival times in these 3 groups were 118.7, 45.7 and 11.2 months, respectively, and the 5-year survival rates were 55.3%, 27.8% and 33.3%, respectively. Multivariate analyses in patients with mycosis fungoides/Sézary syndrome identified age ≥ 70 years, Eastern Cooperative Oncology Group Performance Status ≥  2, and the high-risk group according to the Cutaneous Lymphoma International Consortium prognostic model, as adverse prognostic factors. Seven patients in this mycosis fungoides/ Sézary syndrome group were in complete long-term remission after treatment with bexarotene, including 4 patients living without any treatment for 16-101 months.

Background: The prognosis of unresectable cutaneous squamous cell carcinomas is very poor., Objective: To evaluate the efficacy and safety of panitumumab alone or in association with radiotherapy in the treatment of unresectable cutaneous squamous cell carcinoma., Methods: This was a monocentre retrospective study of all consecutive patients having received at least two courses of panitumumab, alone or in association with radiotherapy, between 2016 and 2019. The primary endpoint was the rate of best overall response, evaluated according to the RECIST 1.1 criteria. The secondary endpoints were the response and disease control rates at 6 weeks and 6 months, progression-free survival, overall survival and safety., Results: A total of 25 patients were included; their median age was 86 years, and 17 (86%) had a WHO performance status over 2. The best overall response rate was 52%, including four complete responses (16%) and nine partial responses (36%). All patients with complete response and five out of nine patients with partial response had received concurrent radiotherapy, in most cases in moderate to low doses (<40 Gray, 67%). The response rates at 6 weeks and 6 months were 12% and 28%, respectively. The control rates at 6 weeks and 6 months were 84% and 32%, respectively. Median progression-free survival was 6.9 months, and median overall survival was 10.5 months. Grade 3 side-effects, mostly dermatological, occurred in 16 patients (64%)., Conclusion: These results suggest that panitumumab remains pertinent in the treatment of unresectable cutaneous squamous cell carcinoma, in particular in association with radiotherapy, despite recent advances with anti-PD-1 antibodies. It presents several advantages: it can be used in very elderly or feeble patients, it does not provoke anaphylactic or other irreversible or life-threatening side-effects, and our study observed some long-term responses. Further prospective investigation of anti-EGFR antibodies, in association with anti-PD-1 antibodies and/or chemotherapy, should be conducted., (© 2020 European Academy of Dermatology and Venereology.)

Few population-based studies on the incidence and prognosis of primary mucosal melanoma (PMM) are available. The first objective was to evaluate disease-specific survival of PMM, overall and according to specific locations, and to identify prognostic factors. The second objective was to assess the global incidence of PMM compared to cutaneous melanoma and to specify the relative frequency of each affected location. A retrospective population-based study of incident PMM diagnosed between 2004 and 2014 was conducted, relying on the regional melanoma registry of the French Champagne-Ardenne region (1.34 million inhabitants). Thirty-nine cases were identified, including 17 head and neck (13 sinonasal and four oral), 12 vulvovaginal, six conjunctival, and four anorectal PMMs. Some 76.9% of cases were revealed by late symptoms. The median disease-specific survival time was 23.9 months and the five-year disease-specific survival rate was 31.8%. Univariate and multivariate analyses led to identification of primary tumour size and the presence of nodal or visceral macrometastases at diagnosis as adverse prognostic factors, while Breslow thickness and ulceration were unreported in 41% of cases and failed to display any prognostic value. Compared to other locations, conjunctival PMMs had a smaller tumour size and better prognosis. The annual incidence rate was 0.18/100,000 and the incidence ratio between PMM and cutaneous melanoma was 1/50. This population-based study confirms the rarity, delayed diagnosis, and severity of PMM, suggesting that improving prognosis will require specific, targeted therapies.

Background: Head and neck melanomas (HNMs) account for an increasing proportion of melanomas and have a poor prognosis., Objectives: To compare the clinical and histological characteristics of HNMs with those of melanomas at other sites (MOS), and to identify pertinent clinicopathological subgroups of HNM., Methods: A retrospective population-based study of incident in situ and invasive melanomas in the period 2004-2011 was performed., Results: HNMs represented 26·7% of 1548 melanomas, corresponding to a density ratio of 3·7 between HNMs and MOS. HNMs occurred later than MOS (71·2 vs. 58·4 years; P < 0·01), included a higher proportion of in situ cases (49·6% vs. 13·5%; P < 0·01) and were mainly lentigo malignant melanomas (73·0% vs. 2·6%; P < 0·01). Invasive HNMs included a higher proportion of thick (> 2 mm) tumours [33·7% vs. 24·1% (P = 0·01); mean Breslow thickness: 2·18 vs. 1·77 mm (P = 0·03)] and nodular melanomas (20·1% vs. 12%; P < 0·01). HNMs in the peripheral area of the head and neck differed from those of central location by a younger age of onset (65·2 vs. 72·4 years; P < 0·01), male predominance (64·4% vs. 33·8%; P < 0·01), and higher proportions of invasive (67·2% vs. 42%; P < 0·01) and nodular (15·1% vs. 7·5%; P = 0·01) melanomas., Conclusions: HNMs highly differ from MOS, and are clinically and histologically heterogeneous, possibly as a consequence of different patterns of sun exposure. These data could help to improve primary and secondary prevention messages for patients and doctors., (© 2014 British Association of Dermatologists.)

Importance: The activating mutation of MYD88 L265P is a frequent feature of primary cutaneous diffuse large B-cell lymphoma, leg-type (PCLBCL-LT), reported in up to 69% of the cases. Whether patients with MYD88 mutation display specific clinical and evolutive features has not been evaluated., Objective: To identify the clinical characteristics associated with MYD88 mutation, confirm its high prevalence, and evaluate its effect on prognosis in patients with PCLBCL-LT., Design, Setting, and Participants: A retrospective multicenter study was conducted using the medical records of patients from dermatology departments belonging to the French Study Group for Cutaneous Lymphomas. Sixty-one patients with a diagnosis of PCLBCL-LT made between 1988 and 2010 who were available for molecular study were included. Of these, 58 patients displaying interpretable results constituted the study group. Median follow-up was 33 months, and 39 patients (67%) were monitored until death., Main Outcomes and Measures: Clinical features (age, sex, number of skin lesions, tumor stage, and location as leg vs elsewhere), MYD88 mutation (allele-specific TaqMan polymerase chain reaction assay), treatment regimen, and outcome were recorded. Baseline characteristics and outcome were compared according to the status of MYD88., Results: The median age of the patients was 79 years, and 59% were female. Skin lesions were located on the leg in 76% of the cases. Thirty-four of 58 patients (59%) harbored the MYD88 L265P mutation. Patients had similar clinical characteristics at presentation regardless of their MYD88 status, except that those harboring the MYD88 mutation were older (P = .006) and had more frequent involvement of the leg (P = .008). Patients harboring the MYD88 mutation had 3- and 5-year-specific survival rates of 65.7% and 60.2% vs 85.4% and 71.7% in patients with the wild-type allele. The MYD88 mutation was significantly associated with shorter disease-specific survival in univariate (P = .03) and multivariate (odds ratio, 3.01; 95% CI, 1.03-8.78; P = .04) analysis. There was no significant difference between the groups in their treatment regimens. Considering overall survival, in univariate (P = .002) and multivariate (odds ratio, 2.94; 95% CI, 1.18-7.30; P = .02) analysis, MYD88 L265P mutation was an independent adverse prognostic factor., Conclusions and Relevance: This study confirms the high prevalence of MYD88 L265P mutation in PCLBCL-LT and shows its association with shorter survival. The clinical effect of MYD88 mutation activating the nuclear factor-κB pathway supports the use of targeted therapies at the time of relapse after conventional therapies.

Background: Identification of differences in melanoma location between the sexes could lead to sex-specific preventive measures., Objectives: To compare precise melanoma location and side in men and women., Methods: The location of 1542 incident melanomas diagnosed during the period 2004-2011 in the French Champagne-Ardenne region (1·3 million inhabitants) was recorded using a regional registry and questionnaires sent to physicians. Men and women were compared for frequency of tumours on the head and neck; trunk; upper limb; lower limb; hand and foot. For each location, more precise sublocations were recorded. The laterality (right vs. left) was studied for head/neck and limb tumours., Results: Melanomas predominated on the lower limb in women (32·2% vs. 9·3% in men; P < 0·01) and on the trunk in men (41·8% vs. 14·9%; P < 0·01), while the proportion of upper limb and head/neck tumours was similar in both sexes. Hand and foot melanomas predominated in women (10·3% vs. 6·3%; P < 0·01), with a sex-related distribution between sublocations. Within the head and neck location, 75·1% of tumours in women were located in the central area vs. 53·7% in the peripheral area in men (P < 0·01). Head and neck melanomas were more frequently right-sided in women and left-sided in men (P = 0·04), with the left/right ratio reaching 1·58 in men vs. 0·61 in women for peripheral tumours (P < 0·01). No difference in laterality was observed for other locations., Conclusions: Sex differences in occupational and leisure time ultraviolet exposure, clothing (including shoes), hairstyle, and side and photo exposure in cars could explain these results. General preventive messages could be completed by sex-specific advice for melanoma prevention., (© 2014 British Association of Dermatologists.)

Importance: Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT), occurs in elderly patients and has been considered as a lymphoma with a poor prognosis, with estimated 5-year specific survival rates of approximately 50%. The hypothesis of an improvement in prognosis over time has not been studied., Objectives: To evaluate this hypothesis in a large series of patients and investigate factors associated with prognosis as well as improvement in the prognosis., Design, Setting, and Participants: A retrospective multicenter study was conducted including dermatology departments belonging to the French Study Group on Cutaneous Lymphoma. Participants were 115 patients with PCDLBCL-LT diagnosed between 1988 and 2003 (period 1) or between 2004 and 2010 (period 2)., Main Outcomes and Measures: Age, sex, period of diagnosis, number of skin lesions, tumor stage, tumor location (leg vs nonleg), lactate dehydrogenase level, type of therapy (with or without a combination of rituximab and polychemotherapy [PCT]), and outcome were recorded. Baseline characteristics and outcome were compared according to period of diagnosis and type of therapy. Prognosis factors were identified by univariate and multivariate survival analyses., Results: The mean age of the patients was 76.9 years, and 47% of the patients were older than 80 years. The 3- and 5-year specific survival rates improved between period 1 and period 2, from 55% to 74% and from 46% to 66%, respectively (P = .01). Patients had similar baseline characteristics during both periods, but rituximab-PCT regimens were administered to 88.5% of the patients in period 2 vs 16.7% in period 1 (P < .001). The 3- and 5-year specific survival rates were 80% and 74%, respectively, in patients who received a rituximab-PCT regimen compared with 48% and 38% in those who received less-intensive therapies. No significant difference was observed between both groups in age and baseline prognostic factors. In multivariate analysis, treatment without rituximab-PCT was the only adverse prognostic factor (odds ratio, 4.6 [95% CI, 2.4-9.1]; P < .001), whereas the number of skin lesions (P = .06) and location on the leg (P = .07) had only borderline significance., Conclusions and Relevance: A major improvement in the survival of patients with PCDLBCL-LT has occurred over time in France, mainly as a result of the use of intensive rituximab-PCT regimens in most patients, including very elderly ones. Until further prospective clinical trials are conducted, such regimens should be considered as the standard of care in these patients.

Background: To date, no strategy for improving early diagnosis of melanoma has been evaluated on a population basis in France., Objective: To evaluate the efficacy of a general practitioner (GP) awareness and training campaign in a pilot French geographical region (Champagne-Ardenne), including 1.34 million inhabitants, 1241 GPs, 56 dermatologists and a population-based melanoma registry., Methods: All GPs received repeated awareness postal mailings in 2008 and 398 (32.1%) attended training sessions organized by 27 dermatologists. The pre- (2005-7) and post-campaign (2009-11) periods were compared for the following: primary endpoint - the world-standardized incidence of very thick melanomas (VTM) (Breslow thickness ≥ 3 mm); secondary endpoints--the mean Breslow thickness; the proportions of VTM and of thin (< 1 mm) melanomas among invasive cases; and the ratio of in situ/all melanoma cases. Similar measures were performed in the control area of Doubs/Belfort territory (655,000 ha), where no similar campaign was carried out., Results: The incidence of VTM decreased from 1.07 to 0.71 per 100 000 habitants per year (P = 0.01), the mean Breslow thickness from 1.95 to 1.68 mm (P = 0.06) and the proportion of VTM from 19.2% to 12.8% (P = 0.01). The proportion of thin and in situ melanomas increased from 50.9% to 57.4% (P = 0.05) and from 20.1% to 28.2% (P = 0.001), respectively. No significant variation was observed in Doubs/Belfort territory., Conclusion: These results strongly support the efficacy of such a campaign targeting GPs and provide a rationale for a larger public health campaign in France, including training of GPs by dermatologists and encouraging patients to ask their GP for a systematic skin examination., (© 2013 British Association of Dermatologists.)

Importance: Life expectancy is increasing in most developed countries, and elderly people have the highest incidence of melanoma., Objective: To identify characteristics of melanoma and its management in the elderly compared with younger patients., Design, Setting, and Participants: Retrospective population-based study of incident cases of primary melanoma in 1621 patients with stage I or II melanoma in 2004 and 2008. Questionnaires administered to physicians and a survey of cancer registries and pathology laboratories were used to obtain data. The study was conducted in 5 regions in northeastern France., Main Outcomes and Measures: Characteristics of patients and tumors, circumstances of diagnosis, and further management in older patients (≥70 years, 487 patients [30.0%]) compared with younger ones (<70 years, 1134 [70.0%])., Results: Older patients had more frequent melanomas of the head and neck (29.4% vs 8.7%; P < .001) and of the nodular, lentigo maligna, or acral lentiginous histologic subtypes. They had thicker and more frequently ulcerated tumors, categorized as T3 or T4 in 36.7% of cases vs 20.1% in younger patients. Diagnosis of melanoma occurred more frequently in a general practice setting and less frequently in direct consultation with a dermatologist or regular screening for skin cancer. Time to definitive excision was longer in older patients, and 16.8% of them compared with 5.0% of the younger population had insufficient excision margins (P < .001). A sentinel lymph node biopsy was performed in 23.3% of the older patients with melanoma thicker than 1 mm vs 41.4% in the younger patients (P < .001). Adjuvant therapy was less frequently started in older patients and was prematurely stopped in a higher proportion of that population., Conclusions and Relevance: Age-related variations are observed at every step of melanoma management. The most important concerns are access of elderly people to settings for early diagnosis and excision with appropriate margins.

Background: Stage III melanoma represents a borderline situation regarding the potential curability of this potentially aggressive cancer and consequently, regional lymph node metastases (RLNM) are a major challenge for melanoma management., Objective: To describe the management of melanoma with RLNM as practised in France in 2008 and compare results with previous data from 2004, considering that new French recommendations were published in 2005., Methods: Retrospective population-based study in five regions of France totalling 8.3 million inhabitants, targeting all incident cases of RLNM diagnosed in 2008. Questionnaires were mailed to physicians to identify cases and collect data, with verification by cancer registries for cases diagnosed concomitantly with the primary tumour using sentinel lymph node biopsies (SLNB)., Results: Data were collected for 101 patients in 2008, and compared to 89 cases treated in 2004. Palpation by a dermatologist was the most common circumstance of diagnosis of RLNM in 2008 (36%), followed by SLNB (29%), self-palpation by the patient (16%) and lymph node ultrasonography (6%), without significant modification from 2004. After lymphadenectomy an adjuvant therapy was proposed in 62% of cases, mainly consisting in high-dose interferon (HD-IFN) (80%). Overall, HD-IFN was proposed in 49% of cases, but effectively started in only 40% of cases after being proposed, and prematurely withdrawn in 28%, showing major changes as compared with 2004 (33%, 77% and 67%, respectively, P < 0.05). Adjuvant chemotherapy was not proposed to any patients in 2008, compared to 29% in 2004. Surveillance procedures included medical imaging less often in 2008 (76%) than in 2004 (92%) (P = 0.004), but more often included FDG-PET (23% vs. 12%, P = 0.09)., Conclusion: Overall, actual practice was in accordance with French recommendations. The main developments from 2004 to 2008 were the disappearance of adjuvant chemotherapies and a more accurate selection of patients for adjuvant interferon., (© 2012 The Authors. Journal of the European Academy of Dermatology and Venereology © 2012 European Academy of Dermatology and Venereology.)

Objective: To identify clinical and sociodemographic factors associated with very thick melanoma (VTM) (Breslow thickness, 3 mm) in France., Design: Retrospective, population-based, case-case study using a survey of cancer registries and questionnaires to practitioners., Setting: Five regions covering 19.2% of the French territory and 8.2 million inhabitants., Cases: Cases included all incident melanomas with a Breslow thickness of 3 mm or greater (ie, VTM), diagnosed between January 1 and December 31, 2008, in residents of the study area (Alsace, Bourgogne, Champagne-Ardenne, Franche-Comte', and Lorraine, France), and a randomly selected sample of melanomas thinner than 3 mm., Main Outcome Measures: Circumstances of diagnosis,clinical and pathological characteristics of melanomas,and sociodemographic characteristics of patients(age, sex, residence, home and family life conditions, educational level, and smoking habits)., Results: Among 898 melanomas, 149 (16.6%) were VTMs. Very thick melanomas were more often diagnosed in a general-practice setting than thinner melanomas.The rate of immediate clinical recognition by dermatologists was lower for VTMs than for thinner melanomas. In a multivariate logistic regression analysis,factors associated with VTM were the nodular and acrolentiginous types; the head and neck and lower limb locations; older age; male sex; and being single, separated,divorced, or widowed. When only factors related to patients were taken into account, older age, male sex,and living alone were independent risk factors for VTM.The most significant risk was observed for patients living alone., Conclusions: Intrinsic factors related to the tumor and socio demographic characteristics of patients contribute to the occurrence of VTM. These factors should be better targeted in future secondary prevention programs.

Background: Little data are available concerning the role of general practitioners (GPs) in the diagnosis of melanoma., Objectives: To evaluate the actual role of GPs in a population-based study covering five regions of France and 8·2 million inhabitants., Materials and Methods: A survey of cancer registries and pathology laboratories, and questionnaires to practitioners were used to identify incident melanomas in 2008, and evaluate characteristics of patients (age, sex, area of residence, social isolation), tumours (Breslow, ulceration, location, histological type), and GPs (training, conditions of practice), and their influence on patterns of diagnosis and Breslow thickness., Results: Among 898 melanomas, 376 (42%) were first diagnosed in a general practice setting (GP group). Breslow thickness was much higher in the GP group than in other melanomas (median: 0·95 vs. 0·61 mm, P < 0·0001). Multivariate analysis identified an older age, lower limb location, nodular subtype and Breslow thickness as factors associated with the GP group. Within this group, 52·5% of melanomas were detected by patients (median Breslow thickness: 1·30 mm) and 47·5% by GPs (median Breslow thickness: 0·80 mm, P = 0·0009), including 8% during a systematic full-body skin examination. Previous GP training on melanoma was associated with active detection by GPs. Male sex and social isolation of patients were associated with thicker melanomas, whereas active detection by GPs was associated with thinner CMs., Conclusions: GPs play a key role in melanoma diagnosis in France, but still frequently detect thick tumours. Increasing awareness and training of GPs and focusing attention on male and/or socially isolated patients should help to improve early detection of melanoma., (© 2012 The Authors. BJD © 2012 British Association of Dermatologists.)

Background: There is no consensus regarding the therapeutic utility of sentinel lymph node biopsy (SLNB) versus that of nodal observation (NO) in melanoma., Objective: To prospectively evaluate a standardized counseling procedure and its effect on patient choices to undergo SLNB or NO., Methods: In four centers, patients with melanoma eligible for SLNB or NO received a complete counseling procedure that included verbal information from dermatologists and surgeons, a detailed information sheet, and a written consent form. Data collected included patient and tumor characteristics, counseling conditions, and specialties of informing doctors. Factors influencing patients' choices were studied using multivariate analysis., Results: Of 343 consecutive patients, 309 were offered SLNB and NO and received complete verbal and written information from a dermatologist alone (62%) or in association with a surgeon (38%). Approximately half took advice from trusted persons, and half asked for additional time before making a decision; 268 (86.7%) ultimately decided to undergo SLNB. Multivariate analysis showed that older patients, those with a head and neck melanoma, and those informed without a surgeon present were more likely to prefer NO., Conclusions: This counseling procedure was easily implemented in clinical practice. Patients favored SLNB but were able to understand uncertainties and express preferences., (© 2011 by the American Society for Dermatologic Surgery, Inc.)