Operational classification of cutaneous squamous cell carcinomas based on unsupervised clustering of real cases by experts.

BACKGROUND: There is currently no staging system for cutaneous squamous cell carcinoma (cSCC) that is adapted to decision-making and universally used. Experts have unconscious ability to simplify the heterogeneity of clinical situations into a few relevant groups to drive their therapeutic decisions. Therefore, we have used unsupervised clustering of real cases by experts to generate an operational classification of cSCCs, an approach that was successful for basal cell carcinomas. OBJECTIVE: To generate a consensual and operational classification of cSCCs. METHOD: Unsupervised independent clustering of 248 cases of cSCCs considered difficult-to-treat. Eighteen international experts from different specialties classified these cases into what they considered homogeneous clusters useful for management, each with freedom regarding clustering criteria. Convergences and divergences between clustering were analysed using a similarity matrix, the K-mean approach and the average silhouette method. Mathematical modelling was used to look for the best consensual clustering. The operability of the derived classification was validated on 23 new practitioners. RESULTS: Despite the high heterogeneity of the clinical cases, a mathematical consensus was observed. It was best represented by a partition into five clusters, which appeared a posteriori to describe different clinical scenarios. Applicability of this classification was shown by a good concordance (94%) in the allocation of cases between the new practitioners and the 18 experts. An additional group of easy-to-treat cSCC was included, resulting in a six-group final classification: easy-to-treat/complex to treat due to tumour and/or patient characteristics/multiple/locally advanced/regional disease/visceral metastases. CONCLUSION: Given the methodology based on the convergence of unguided intuitive clustering of cases by experts, this new classification is relevant for clinical practice. It does not compete with staging systems, but they may complement each other, whether the objective is to select the best therapeutic approach in tumour boards or to design homogeneous groups for trials.

Longitudinal Study of Different Progression Patterns in High-Risk Cutaneous Squamous Cell Carcinoma. / Estudio longitudinal de los diferentes patrones de progresión en el carcinoma cutáneo de células escamosas de alto riesgo.

BACKGROUND AND OBJECTIVE: Cutaneous squamous cell carcinoma (cSCC) is the second leading cause of skin cancer mortality in Europe. Few studies have analyzed the different pathways of this tumor progression in its natural history. The main objective of this study was to analyze the different metastatic and progression pathways and their temporal occurrence in the evolution of cSCC. MATERIAL AND METHOD: We conducted a multicenter, retrospective, and observational study of consecutive high-risk sSCCs included in the SQUAMATA project. RESULTS: A total of 222 out of the 1346 patients included relapsed. The most frequent route of progression was the lymphatic one (62.6%). A total of 20.2% of the cases with lymphatic progression developed distant metastases. Only 1 case (3.1%) of distant metastasis followed local recurrence without previous lymphatic metastasis. The median time to disease-related mortality was longer in patients who developed systemic metastases than in those who died of locoregional progression. CONCLUSIONS: The mortality of patients with cSCC is mostly due to the regional progression of their lymphatic metastases. The appearance of distant metastases is practically always (96.9%) associated with previous lymphatic metastatic progression. Therefore, in the future, new studies will be needed to assess the regional management of cSCC in both surgical and adjuvant therapies.

Imatinib: una nueva herramienta para el manejo de la enfermedad del injerto contra el hospedador crónica esclerodermiforme / Imatinib: A New Tool for the Management of Chronic Sclerodermatous Graft-Vs-Host Disease

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Prevalence of Actinic Keratosis in Different Regions of Spain: The EPIQA Study / Prevalencia de la queratosis actínica en diferentes regiones españolas: estudio EPIQA

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Precáncer cutáneo / Precancerous skin lesions

Bajo el término de precáncer cutáneo se han englobado tradicionalmente distintas entidades, clínica e histológicamente reconocibles, asociadas a un cierto riesgo de evolución a carcinoma escamoso cutáneo invasivo aunque en la actualidad se tiende a interpretarlas como carcinomas in situ. En este documento de consenso se abordan distintos aspectos de estas lesiones como son su evaluación a través de las características clínicas e, histopatológicas de las mismas, la evaluación inicial del paciente afecto, la identificación de los factores de riesgo para su desarrollo, los distintos métodos hoy día existentes para su estudio y diagnóstico así como las diferentes estrategias terapéuticas

Certain clinically and histologically recognizable skin lesions with a degree of risk of progression to squamous cell carcinoma have been traditionally grouped as precancerous skin conditions but now tend to be classified as in situ carcinomas. This consensus statement discusses various aspects of these lesions: their evaluation by means of clinical and histopathologic features, the initial evaluation of the patient, the identification of risk factors for progression, and the diagnostic and treatment strategies available today

Tumoración cutánea pulsátil supraclavicular / Pulsatile Supraclavicular Skin Tumor

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Carcinoma escamoso de pene / Penile Squamous Cell Carcinoma

El carcinoma escamoso de pene (CEP) es una neoplasia infrecuente en Europa, suponiendo un 0,7% de los tumores en varones. La mala higiene, no estar circuncidado, la infección por el virus del papiloma humano (VPH) y algunas dermatosis inflamatorias crónicas son los principales factores de riesgo. El VPH se detecta en un 70-100% de los CEP in situ y en un 30-60% de las formas invasivas, sobre todo en los tumores basaloides y condilomatosos. Los tumores in situ pueden tratarse de forma conservadora, pero requieren un seguimiento estricto, puesto que del 1 al 30% evolucionan a formas invasivas. En los CEP invasivos el tratamiento de elección es la cirugía. La irradiación profiláctica de los ganglios inguinales está actualmente desaconsejada. Parece que el uso de la biopsia selectiva de ganglio centinela podría ser útil para disminuir la morbilidad asociada a la linfadenectomía inguinal profiláctica. La supervivencia se relaciona directamente con la presencia de metástasis ganglionares. El conocimiento de las alteraciones moleculares y genotípicas subyacentes abrirá nuevas vías terapéuticas (AU)

Penile squamous cell carcinoma (SCC) is uncommon in Europe, where it accounts for approximately 0.7% of all malignant tumors in men. The main risk factors are poor hygiene, lack of circumcision, human papillomavirus (HPV) infection, and certain chronic inflammatory skin diseases. HPV infection is detected in 70% to 100% of all penile in situ SCCs and in 30% to 50% of invasive forms of the disease, mainly basaloid and warty SCCs. In situ tumors can be treated conservatively, but close monitoring is essential as they become invasive in between 1% and 30% of cases. The treatment of choice for penile SCC is surgery. Inguinal lymph node irradiation is no longer recommended as a prophylactic measure, and it appears that selective lymph node biopsy might be useful for reducing the morbidity associated with prophylactic inguinal lymph node dissection. Survival is directly related to lymph node involvement. Improving our knowledge of underlying molecular changes and their associated genotypes will open up new therapeutic pathways (AU)

Nódulo hipertricótico en la pierna de un niño de 3 años / Hypertrichotic nodule on the leg of a 3-year-old child

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Pitiriasis rubra pilaris aguda postinfecciosa: una dermatosis mediada por superantígenos / Acute Postinfectious Pityriasis Rubra Pilaris: A Superantigen-Mediated Dermatosis

La pitiriasis rubra pilaris (PRP) aguda post infecciosa es una variante de la forma juvenil de PRP (tipo III de Griffiths) caracterizada por la ausencia de antecedentes familiares, curso agudo relacionado con un episodio febril previo y buen pronóstico. Clínicamente puede simular otras enfermedades mediadas por superantígenos, como los exantemas escarlatiniformes o el síndrome de la escaldadura estafilocócica; sin embargo, su histología y tratamiento son distintos. Presentamos 4 casos de PRP aguda post infecciosa que ilustran las características clínicas de este proceso infrecuente y revisamos los posibles mecanismos fisiopatogénicos subyacentes (AU)

Acute postinfectious pityriasis rubra pilaris (PRP) is a variant of juvenile PRP (Griffiths type III) characterized by no family history, an acute course associated with a prior fever, and good prognosis. Clinical features may resemble other superantigen-mediated diseases, such as scarlatiniform rash or staphylococcal scalded skin syndrome, but its histology and treatment are different. We present 4 cases of acute postinfectious PRP that illustrate the clinical features of this uncommon disease and we review possible underlying pathogenic mechanisms (AU)

Xantomatosis cerebrotendinosa: descripción de 4 casos / Cerebrotendunous Xanthomatosis: report of 4 patients

La xantomatosis cerebrotendinosa (XCT) es una enfermedad hereditaria infrecuente causada por la mutación del gen CYP27A1. Es característica la aparición de xantomas en diferentes tejidos, principalmente en el cerebro y los tendones, secundarios al depósito de -colestanol. El diagnóstico se confirma mediante la determinación de -colestanol en suero, y de los alcoholes biliares en orina. El ácido quenodesoxicólico es la terapia más eficaz, pudiendo llegar a frenar la progresión de la enfermedad. Presentamos 4 pacientes con alteraciones neurológicas desde la infancia que fueron diagnosticados de XCT tras el desarrollo de xantomas tendinosos. El reconocimiento de los xantomas tendinosos es fundamental para orientar el diagnóstico de XCT, pero estos no son un signo inicial de la enfermedad, que debuta con alteraciones neurológicas, cataratas o diarrea crónica. Por lo tanto, el diagnóstico temprano de la XCT requiere la determinación del -colestanol sérico en estos pacientes, aun en ausencia de xantomas (AU)

Cerebrotendinous xanthomatosis (CTX) is an uncommon autosomal recessive disease caused by mutation of the CYP27A1 gene. It is characterized by the presence of xanthomas in different tissues, principally brain and tendon, due to the accumulation of -cholestanol. Diagnosis is confirmed by measurement of serum -cholestanol and urinary bile alcohol levels. Therapy with chenodeoxycholic acid has been shown to be the most effective treatment and can halt progression of the disease. We present 4 patients with a history of neurological disorders since childhood and who were diagnosed with CTX after developing tendon xanthomas. Although diagnostic suspicion depends to a large extent on recognition of tendon xanthomas, these are not an early sign of the disease, which can present with neurological disorders, cataracts, and chronic diarrhea. Early diagnosis of CTX therefore rests on measurement of serum -cholestanol levels, even in absence of tendon xanthomas (AU)