Sonidegib as a Locally Advanced Basal Cell Carcinoma Therapy in Real-life Clinical Setting: A National Multicentre Study. / [Artículo traducido] Sonidegib para el tratamiento del carcinoma basocelular avanzado en práctica clínica real: estudio nacional multicéntrico.

BACKGROUND: Basal cell carcinoma (BCC) is the most prevalent cancer. A minority of BCCs have an aggressive behaviour (laBCC) and may require hedgehog pathway inhibitors such as sonidegib as its treatment. OBJECTIVE: To describe the use of sonidegib in a large number of patients and provide more data on its real-life efficacy and safety profile. METHODS: We conducted a retrospective and multicentric study that included patients treated with sonidegib. Epidemiological, effectiveness and safety data were collected. RESULTS: A total of 82 patients with a mean age of 73.9 years were included. Ten patients had Gorlin syndrome. Median treatment duration was 6 months. Median follow-up duration was 34.2 months. Globally, 81.7% of the patients showed clinical improvement (52.4% partial response and 29.3% complete response), 12.2% clinical stability and 6.1% disease progression. There was no statistically significant difference in clinical improvement between the 24 h and 48 h sonidegib posology. After 6 months of treatment, 48.8% of the patients discontinued sonidegib. Prior vismodegib treatment and recurrent primary BCC were associated with a poorer response to sonidegib. At 6 months of treatment, 68.3% of the patients experienced at least one adverse effect. CONCLUSION: Sonidegib shows good effectiveness and acceptable safety profile in usual clinical practice.

Sonidegib as a Locally Advanced Basal Cell Carcinoma Therapy in Real-life Clinical Setting: A National Multicentre Study.

BACKGROUND: Basal cell carcinoma (BCC) is the most prevalent cancer. A minority of BCCs have an aggressive behaviour (laBCC) and may require hedgehog pathway inhibitors such as sonidegib as its treatment. OBJECTIVE: To describe the use of sonidegib in a large number of patients and provide more data on its real-life efficacy and safety profile. METHODS: We conducted a retrospective and multicentric study that included patients treated with sonidegib. Epidemiological, effectiveness and safety data were collected. RESULTS: A total of 82 patients with a mean age of 73.9 years were included. Ten patients had Gorlin syndrome. Median treatment duration was 6 months. Median follow-up duration was 34.2 months. Globally, 81.7% of the patients showed clinical improvement (52.4% partial response and 29.3% complete response), 12.2% clinical stability and 6.1% disease progression. There was no statistically significant difference in clinical improvement between the 24h and 48h sonidegib posology. After 6 months of treatment, 48.8% of the patients discontinued sonidegib. Prior vismodegib treatment and recurrent primary BCC were associated with a poorer response to sonidegib. At 6 months of treatment, 68.3% of the patients experienced at least one adverse effect. CONCLUSION: Sonidegib shows good effectiveness and acceptable safety profile in usual clinical practice.

Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV): Data for the First 5 Years. / Registro de linfomas cutáneos primarios (RELCP) de la AEDV: datos tras 5 años de funcionamiento.

BACKGROUND AND OBJECTIVE: Primary cutaneous lymphomas (PCL) are uncommon. Observations based on the first year of data from the Spanish Registry of Primary Cutaneous Lymphomas (RELCP, in its Spanish abbreviation) of the Spanish Academy of Dermatology and Venereology (AEDV) were published in February 2018. This report covers RELCP data for the first 5 years. PATIENTS AND METHODS: RELCP data were collected prospectively and included diagnosis, treatments, tests, and the current status of patients. We compiled descriptive statistics of the data registered during the first 5 years. RESULTS: Information on 2020 patients treated at 33 Spanish hospitals had been included in the RELCP by December 2021. Fifty-nine percent of the patients were men; the mean age was 62.2 years. The lymphomas were grouped into 4 large diagnostic categories: mycosis fungoides/Sézary syndrome, 1112 patients (55%); primary B-cell cutaneous lymphoma, 547 patients (27.1%); primary CD30+lymphoproliferative disorders, 222 patients (11%), and other T-cell lymphomas, 116 patients (5.8%). Nearly 75% of the tumors were registered in stage I. After treatment, 43.5% achieved complete remission and 27% were stable at the time of writing. Treatments prescribed were topical corticosteroids (1369 [67.8%]), phototherapy (890 patients [44.1%]), surgery (412 patients [20.4%]), and radiotherapy (384 patients [19%]). CONCLUSION: The characteristics of cutaneous lymphomas in Spain are similar to those reported for other series. The large size of the RELCP registry at 5 years has allowed us to give more precise descriptive statistics than in the first year. This registry facilitates the clinical research of the AEDV's lymphoma interest group, which has already published articles based on the RELCP data.

Prognostic factors in patients with primary cutaneous anaplastic large cell lymphoma: a multicentric, retrospective analysis of the Spanish Group of Cutaneous Lymphoma.

BACKGROUND: Reliable prognostic factors for patients with primary cutaneous anaplastic large cell lymphoma (PCALCL) are lacking. OBJECTIVE: To identify prognostic factors for specific survival in patients with PCALCL. METHODS: Using the convenience sampling method, patients with PCALCL diagnosed from May 1986 to August 2017 in 16 University Departments were retrospectively reviewed. RESULTS: One hundred eight patients were included (57 males). Median age at diagnosis was 58 years. All of them showed T1-3N0M0 stages. Seventy per cent of the cases presented with a solitary lesion, mostly at the limbs. Complete response rate after first-line treatment was 87%, and no advantage was observed for any of them (surgery, radiotherapy, chemotherapy or other approaches). Nodal and visceral progression rate was 11% and 2%, respectively. 5-year specific survival (SSV) reached 93%; 97% for T1 patients and 84% for T2/T3 patients (P = 0.031). Five-year SSV for patients developing early cutaneous relapse was 64%; for those with late or no relapse, 96% (P = 0.001). Estimated median SSV for patients showing nodal progression was 103 months (95% CI: 51-155 months); for patients without nodal progression, estimated SSV did not reach the median (P < 0.001). Nodal progression was an independent predictive parameter for shorter survival (P = 0.011). CONCLUSION: Multiple cutaneous lesions at presentation, early skin relapse and nodal progression portrait worse prognosis in patients with PCALCL.

The First Year of the AEVD Primary Cutaneous Lymphoma Registry. / Registro de linfomas cutáneos primarios de la AEDV: primer año de funcionamiento.

BACKGROUND AND OBJECTIVE: Primary cutaneous lymphomas are uncommon. This article describes the Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV) and reports on the results from the first year. PATIENTS AND METHODS: Disease registry for patients with primary cutaneous lymphoma. The participating hospitals prospectively recorded data on diagnosis, treatment, tests, and disease stage for all patients with primary cutaneous lymphoma. A descriptive analysis was performed. RESULTS: In December 2017, the registry contained data on 639 patients (60% male) from 16 university hospitals. The most common diagnoses, in order of frequency, were mycosis fungoides/Sézary syndrome (MF/SS) (348 cases, 55%), primary cutaneous B-cell lymphoma (CBCL) (184 cases, 29%), primary cutaneous CD30+ T-cell lymphoproliferative disorder (CD30+ CLPD) (70 cases, 11%), and other types of T-cell lymphoma (37 cases, 5%). In total, 105 (16.5%) of the cases recorded were incident cases. The most common diagnosis in the MF/SS group was classic MF (77.3%). Half of the patients with MF had stage IA disease when diagnosed, and the majority were either in partial remission (32.5%) or had stable disease (33.1%). The most widely used treatments were topical corticosteroids (90.8%) and phototherapy. The most common form of primary CBCL was marginal zone lymphoma (50%). Almost all of the patients had cutaneous involvement only and nearly half had stage T1a disease. Most (76.1%) were in complete remission. The main treatments were surgery (55.4%) and radiotherapy (41.9%). The most common diagnosis in patients with CD30+ CLPD was lymphomatoid papulosis (68.8%). Most of the patients (31.4%) had stage T3b disease and half were in complete remission. The most common treatments were topical corticosteroids (68.8%) and systemic chemotherapy (32.9%). CONCLUSION: The characteristics of patients with primary cutaneous lymphoma in Spain do not differ from those described in other series in the literature. The registry will facilitate clinical research by the AEDV's lymphoma group.

First-line treatment in lymphomatoid papulosis: a retrospective multicentre study.

BACKGROUND: Data regarding response to treatment in lymphomatoid papulosis (LyP) are scarce. AIM: To assess the daily clinical practice approach to LyP and the response to first-line treatments. METHODS: This was a retrospective study enrolling 252 patients with LyP. RESULTS: Topical steroids, methotrexate and phototherapy were the most common first-line treatments, prescribed for 35%, 20% and 14% of the patients, respectively. Complete response (CR) was achieved in 48% of treated patients. Eczematous lesions significantly increased relative risk (RR) of not achieving CR (RR = 1.76; 95% CI 1.16-2.11). Overall median time to CR was 10 months (95% CI 6-13 months), and 78% of complete responders showed cutaneous relapse; both results were similar for all treatment groups (P > 0.05). Overall estimated median disease-free survival (DFS) was 11 months (95% CI 9-13 months) but DFS for patients treated with phototherapy was 23 months (95% CI 10-36 months; P < 0.03). Having the Type A LyP variant (RR = 2.04; 95% CI 0.96-4.30) and receiving a first-line treatment other than phototherapy (RR = 5.33; 95% CI 0.84-33.89) were significantly associated with cutaneous early relapse. Of the 252 patients, 31 (13%) had associated mycosis fungoides unrelated to therapeutic approach, type of LyP or T-cell receptor clonality. CONCLUSIONS: Current epidemiological, clinical and pathological data support previous results. Topical steroids, phototherapy and methotrexate are the most frequently prescribed first-line treatments. Although CR and cutaneous relapse rates do not differ between them, phototherapy achieves a longer DFS. Presence of Type A LyP and use of topical steroid or methotrexate were associated with an increased risk of early relapse.

Rituximab en el tratamiento de los linfomas cutáneos B primarios: revisión / Rituximab in the Treatment of Primary Cutaneous B-Cell Lymphoma: A Review

Rituximab es un anticuerpo quimérico murino-humano dirigido contra el antígeno CD20 presente en los linfocitos B normales y neoplásicos. Su uso en los linfomas cutáneos de células B ha ido en creciente desarrollo en los últimos años. Así se plantea como una alternativa a los tratamientos clásicos de radioterapia y cirugía en los linfomas de curso indolente, el linfoma folicular y el linfoma de la zona marginal. También se utiliza en el tratamiento del linfoma cutáneo primario de células grandes tipo piernas como alternativa a la poliquimioterapia. Su desarrollo como alternativa terapéutica ha llevado a su uso intralesional también con buenos resultados. En este artículo se revisa la literatura publicada del uso de rituximab en los linfomas cutáneos primarios de células B

Rituximab is a chimeric mouse-human antibody that targets the CD20 antigen, which is found in both normal and neoplastic B cells. In recent years, it has been increasingly used to treat cutaneous B-cell lymphoma and is now considered an alternative to classic treatment (radiotherapy and surgery) of 2 types of indolent lymphoma, namely, primary cutaneous follicle center lymphoma and primary cutaneous marginal zone B-cell lymphoma. Rituximab is also administered as an alternative to polychemotherapy in the treatment of primary cutaneous large B-cell lymphoma, leg type. Its use as an alternative drug led to it being administered intralesionally, with beneficial effects. In the present article, we review the literature published on the use of rituximab to treat primary cutaneous B-cell lymphoma

Xantomas planos diseminados normolipémicos asociados a gammapatía monoclonal de significado incierto / Diffuse normolipemic plane xanthomas associated with monoclonal gammopathy of undetermined significance

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Rituximab in the treatment of primary cutaneous B-cell lymphoma: a review.

Rituximab is a chimeric mouse-human antibody that targets the CD20 antigen, which is found in both normal and neoplastic B cells. In recent years, it has been increasingly used to treat cutaneous B-cell lymphoma and is now considered an alternative to classic treatment (radiotherapy and surgery) of 2 types of indolent lymphoma, namely, primary cutaneous follicle center lymphoma and primary cutaneous marginal zone B-cell lymphoma. Rituximab is also administered as an alternative to polychemotherapy in the treatment of primary cutaneous large B-cell lymphoma, leg type. Its use as an alternative drug led to it being administered intralesionally, with beneficial effects. In the present article, we review the literature published on the use of rituximab to treat primary cutaneous B-cell lymphoma.

Registro nacional de melanoma cutáneo. Características del tumor en el momento del diagnóstico: 15 años de experiencia / The Spanish National Cutaneous Melanoma Registry. Tumour characteristics at Diagnosis: 15 Years of Experience

Introducción y objetivos: El registro nacional de melanoma cutáneo (RNMC) se creó en el año 1997 con el objetivo de conocer las características del melanoma en el momento del diagnóstico. Se muestran las características de los tumores en el momento de su diagnóstico inicial. Pacientes y métodos: Registro observacional transversal, con base poblacional. Se incluyeron casos incidentes y prevalentes de melanoma con resultados de la primera biopsia disponibles. Resultados: El RNMC contiene información de 14.039 pacientes. Se analizaron las características del melanoma en los pacientes diagnosticados en el periodo 1997-2011, sumando un total de 13.628 melanomas. El 56,5% de los pacientes eran mujeres y el 43,5% hombres. La edad media fue de 57 años (IC 95%: 56,4 a 57), con mediana de 58 años. La localización más frecuente fue en el tronco (37,1%), seguido de la extremidad inferior (27,3%). El tipo clínico-patológico más observado fue el melanoma de extensión superficial en un 62,6% (n = 7.481), seguido del melanoma nodular en un 16,8% de los casos (n = 2.014). El 86,2% (n = 10.382) tenían enfermedad localizada, el 9,9% metástasis regionales (n = 1.188) y el 3,9% (n = 479) a distancia. Se observó en los hombres, independientemente de la edad de diagnóstico, un mayor espesor del tumor y una mayor proporción de tumores ulcerados, con niveles de lactatodeshidrogenasa elevados y con enfermedad metastásica (p < 0,0001). Conclusiones: Con los resultados observados las campañas preventivas deberían orientarse al colectivo masculino mayor de 50 años, en el que se observan tumores de mayor espesor, y por lo tanto de peor pronóstico (AU)

Background and objectives: The Spanish National Cutaneous Melanoma Registry (Registro Nacional de Melanoma Cutáneo [RNMC]) was created in 1997 to record the characteristics of melanoma at diagnosis. In this article, we describe the characteristics of these tumors at diagnosis. Patients and methods: This was a cross-sectional observational study of prevalent and incident cases of melanoma for which initial biopsy results were available in the population-based RNMC. Results: The RNMC contains information on 14,039 patients. We analyzed the characteristics of 13,628 melanomas diagnosed between 1997 and 2011. In total, 56.5% of the patients studied were women and 43.5% were men. The mean age of the group was 57 years (95% CI , 56.4-57 years) while median age was 58 years. The most common tumor site was the trunk (37.1%), followed by the lower limbs (27.3%). The most frequent clinical-pathologic subtype was superficial spreading melanoma (n = 7481, 62.6%), followed by nodular melanoma (n = 2014, 16.8%). Localized disease was observed in 86.2% of cases (n = 10,382), regional metastasis in 9.9% (n = 1188), and distant metastasis in 3.9% (n = 479). Independently of age at diagnosis, men had thicker tumors, more ulceration, higher lactate dehydrogenase levels, and a higher rate of metastasis than women (P < 0.001). Conclusions: Based on our findings, melanoma prevention campaigns should primarily target men over 50 years old because they tend to develop thicker tumors and therefore have a worse prognosis (AU)

Melanoma characteristics at diagnosis from the Spanish National Cutaneous Melanoma Registry: 15 years of experience.

BACKGROUND AND OBJECTIVES: The Spanish National Cutaneous Melanoma Registry (Registro Nacional de Melanoma Cutáneo [RNMC]) was created in 1997 to record the characteristics of melanoma at diagnosis. In this article, we describe the characteristics of these tumors at diagnosis. PATIENTS AND METHODS: This was a cross-sectional observational study of prevalent and incident cases of melanoma for which initial biopsy results were available in the population-based RNMC. RESULTS: The RNMC contains information on 14,039 patients. We analyzed the characteristics of 13,628 melanomas diagnosed between 1997 and 2011. In total, 56.5% of the patients studied were women and 43.5% were men. The mean age of the group was 57 years (95% CI, 56.4-57 years) while median age was 58 years. The most common tumor site was the trunk (37.1%), followed by the lower limbs (27.3%). The most frequent clinical-pathologic subtype was superficial spreading melanoma (n=7481, 62.6%), followed by nodular melanoma (n=2014, 16.8%). Localized disease was observed in 86.2% of cases (n=10,382), regional metastasis in 9.9% (n=1188), and distant metastasis in 3.9% (n=479). Independently of age at diagnosis, men had thicker tumors, more ulceration, higher lactate dehydrogenase levels, and a higher rate of metastasis than women (P<.001). CONCLUSIONS: Based on our findings, melanoma prevention campaigns should primarily target men over 50 years old because they tend to develop thicker tumors and therefore have a worse prognosis.

Intralesional rituximab in the treatment of indolent primary cutaneous B-cell lymphomas: an epidemiological observational multicentre study. The Spanish Working Group on Cutaneous Lymphoma.

BACKGROUND: Intravenous rituximab is a safe and effective option for the treatment of systemic non-Hodgkin B-cell lymphoma. The effectiveness of intralesional rituximab (ILR) in primary cutaneous B-cell lymphomas (PCBL) has been described in a small number of patients. OBJECTIVES: To evaluate the effectiveness, tolerance and adverse effects of ILR in patients with follicle centre (FCL) and marginal zone (MZL) PCBL. METHODS: This was an epidemiological observational multicentre study of patients with PCBL treated with ILR. RESULTS: Seventeen patients with MZL and 18 with FCL PCBL were included. The median number of lesions treated was two per patient. The treatment regimen used in 74% of the patients was a course of three injections in a single week at 1-month intervals. The dose per lesion and day of treatment was 10 mg in 71% of the patients. The median cumulative dose of rituximab per lesion was 60 mg (range 13-270) and per patient was 150 mg (range 20-360 mg). Complete response (CR) and partial response were achieved in 71% and 23% of patients, respectively. The median time to CR in patients who received 10 mg of ILR per lesion was 8 weeks. Similar response rates were observed in MZL and FCL. Median disease-free survival was 114·1 weeks. No parameters that significantly predicted CR were identified. Adverse reactions were recorded in 19 patients; the most frequent was localized pain at the injection site. Median follow-up was 21 months. CONCLUSIONS: Intralesional rituximab is a well-tolerated and effective treatment for FCL and MZL PCBL. It should be considered a useful alternative in patients with recurrent lesions and in which the sequelae of radiotherapy or surgery would be significant.

Solitary oral ulceration as the first appearance of lymphomatoid papulosis: a diagnostic challenge.

Lymphomatoid papulosis (LyP) may involve any cutaneous site but the oral areas seems to be an unusual location. We report a 72-year-old patient who presented with a 1-week history of a solitary oral ulcer on the lateral tongue, which had raised and indurated borders. Although squamous cell carcinoma was initially diagnosed, the morphological, phenotypical and genotypical studies confirmed diagnosis of LyP. We are not aware of previous reports of definite LyP presenting as oral lesions, which may pose a diagnostic challenge. The differential diagnosis includes several neoplastic, reactive and infectious disorders. LyP should be considered in patients showing solitary, rapidly developing ulcers with raised, indurated borders in the oral cavity.

Relaparotomía en la sepsis peritoneal. Incidencia, oportunidad y factores pronósticos / Repeated laparotomy in a case of sepsis peritoneal

Fundamento. Los pacientes con sepsis grave de origen abdominoperitoneal requieren frecuentemente reintervenciones, persistiendo con una elevada morbimortalidad. Su manejo terapéutico es dificultoso y controvertido, con estudios que consideran poblaciones poco homogéneas. Material y métodos. Se realizó un análisis retrospectivo durante un período de 5 años. Se estudiaron un total de 55 pacientes con sepsis abdominal grave que requirieron al menos una relaparotomía. Analizamos la incidencia, las manifestaciones clínicas, la mortalidad y los factores pronósticos. La ausencia de disfunción multiorgánica (DMO) constituyó un criterio de exclusión. Resultados. Se realizaron un total de 121 relaparotomías, de las cuales 17 (14 por ciento) fueron en blanco. La presencia de síntomas prolongados, la realización de anastomosis gastrointestinal y/o la presencia de DMO al momento de relaparotomizar al paciente, se asociaron menos frecuentemente a relaparotomías en blanco (p < 0,05).Los signos locales no resultaron buenos indicadores de la oportunidad de reintervenir. Al comparar la relaparotomía programada frente a la demanda no encontramos diferencias en cuanto al número de relaparotomías, estancia media de los pacientes y mortalidad. La mortalidad cruda fue del 76 por ciento y la relacionada fue del 55 por ciento. La edad mayor de 70 años, la puntuación APACHE II mayor de 17 y la presencia de 3 o más disfunciones se asociaron significativamente a una mayor mortalidad, cercana al 90 por ciento. Conclusiones. Dada la alta mortalidad de los pacientes con 3 o más disfunciones, una estrategia para mejorar la mortalidad es reoperar en forma precoz a los pacientes con 1 o 2 disfunciones, en particular los que presentan síntomas prolongados y/o anastomosis gastrointestinal (AU)