Nuevas aportaciones a la estadificación del carcinoma epidermoide cutáneo

[ES]En el presente trabajo doctoral nos hemos planteado como objetivo global definir mejor el pronóstico del carcinoma epidermoide cutáneo. Para ello hemos establecido tres objetivos específicos: 2.1.1 OBJETIVO 1: definir y estratificar subgrupos de pronóstico homogéneo dentro del estadio T3 de la octava edición de la AJCC (AJCC8). 2.1.2 OBJETIVO 2: evaluar los diferentes patrones de infiltración perineural incidental y determinar su influencia en el pronóstico del CEC. 2.1.3 OBJETIVO 3: categorizar de forma más precisa el pronóstico de los CECs acantolíticos

Cutaneous collagenous vasculopathy: papular form

Cutaneous collagenous vasculopathy is a rare clinicopathological entity, first described in 2000. Cutaneous collagenous vasculopathy has been considered a form of microangiopathy of superficial dermal vessels and produce lesions that appear as telangiectasia. We present a patient with histopathologic features of cutaneous collagenous vasculopathy and scattered erythematous papules on the trunk with a striking dermatoscopic finding. We propose the term of 'cutaneous papular collagenous vasculopathy' as a new clinical manifestation of this disease

Patterns of incidental perineural invasion and prognosis in cutaneous squamous cell carcinoma: A multicenter, retrospective cohort study

To the Editor: Perineural invasion (PNI) is rare and usually incidental in cutaneous squamous cell carcinoma (SCC), with an incidence of 2.5% to 14%.1 Incidental PNI is associated with poor prognosis in cutaneous SCC,2 and some evidence suggests its outcome differs, depending on the PNI pattern. We evaluated patterns of incidental PNI, using a multicenter retrospective cohort of 140 cutaneous SCCs with incidental PNI to determine the influence of nerve involvement on cutaneous SCC prognosis.Dr Canueto is partially supported ~ by grants PI18/000587 (Instituto de Salud Carlos III, cofinanced by Fondo Europeo de Desarrollo Regional) and GRS 1835/A/18 (Gerencia Regional de Salud de Castilla y Leon)

A New Case of Schindler Disease

Lysosomal storage disorders (LSDs) are a group of genetic disorders caused by mutations in genes encoding enzymes involved in lysosomal function. Schindler disease is an autosomal recessive, inherited LSD caused by defective or non-existent activity of the enzyme α-N-acetylgalactosaminidase (α-NAGA). To date, three main phenotypes of Schindler disease have been described. We report the case of a 68-year-old man presenting with axonal and demyelinating polyneuropathy, sensorineural hearing loss, chronic lymphoedema, angiokeratoma corporis diffusum and bilateral carpal tunnel syndrome. Genetic testing (PCR) for α-galactosidase revealed the c.577G>T (p.Glu193*) mutation in the NAGA gene, confirming Schindler disease, which is clinically compatible with Kanzaki disease and Schindler disease type II

Cutaneous collagenous vasculopathy: papular form

Cutaneous collagenous vasculopathy is a rare clinicopathological entity, first described in 2000. Cutaneous collagenous vasculopathy has been considered a form of microangiopathy of superficial dermal vessels and produce lesions that appear as telangiectasia. We present a patient with histopathologic features of cutaneous collagenous vasculopathy and scattered erythematous papules on the trunk with a striking dermatoscopic finding. We propose the term of 'cutaneous papular collagenous vasculopathy' as a new clinical manifestation of this disease

Clinical and histopathological evaluation of 50 acantholytic cutaneous squamous cell carcinomas: Analysis outcome in a retrospective case-control study

[Background]: Acantholytic cutaneous squamous cell carcinomas (aCSCCs) have been classically considered as a high-risk variant of CSCC. However, more recent studies show that aCSCC does not confer more aggressiveness. This study aims to establish whether the prognosis of the aCSCC is worse than that of the non-acantholytic (naCSCC) or not. [Methods]: Retrospective case-control study with 50 aCSCCs and 50 naCSCCs. For each aCSCC, an naCSCC with similar high-risk features to the aCSCC but with no acantholysis was selected. Prognosis between both groups was compared. [Results]: The mean age was 86 years (SD 9.61). Sixty-one patients were men. Thirty-nine CSCCs were located in high-risk head and neck areas. Twenty CSCCs exhibited a poor degree of differentiation, and 36 showed an infiltrative growth pattern. The tumor diameter was 18.71 mm (interquartile range, IQR 35), and the tumor thickness was 6.72 mm (IQR 15.50). Twelve CSCCs exhibited perineural infiltration, and eight CSCCs exhibited invasion beyond the subcutaneous fat. Positive margins after excision of the tumor in 22 aCSCCs vs eight naCSCCs (P < 0.02). Nineteen poor-prognosis events were observed (local recurrence, lymph node metastasis, and death from CSCC). However, no differences were observed between both groups when comparing poor-prognosis events. [Conclusion]: The proportion of unfavorable events is similar in aCSCC and naCSCC. The acantholytic histopathological subtype is not associated with a poorer prognosis than the non-acantholytic CSCC in our cohort.Instituto de Salud Carlos III, Grant/Award Number: PI18/00058

Performance of Salamanca refinement of the T3-AJCC8 versus the Brigham and Women's Hospital and Tübingen alternative staging systems for high-risk cutaneous squamous cell carcinoma

[Introduction]: The Brigham and Women's Hospital and the Tübingen cutaneous squamous cell carcinoma (SCC) stratification systems propose different criteria from the American Joint Committee on Cancer, eighth edition. Our group identified prognostic subgroups within T3 stage according to the American Joint Committee on Cancer eighth edition's classification, the most common classification for high-risk cutaneous SCCs.[Objective]: To compare the performance and prognostic accuracy of these staging systems in a subset of high-risk cutaneous SCCs.[Methods]: Homogeneity, monotonicity, and McNemar tests for pairwise comparisons were assessed. Distinctiveness and relative risk of poor outcome were calculated by stage. Prognostic accuracy was compared with respect to quality (Akaike and Bayesian information criteria), concordance (Harrell C-index and Gönen and Heller concordance probability estimate), and predictive accuracy (sensitivity, specificity, negative predictive value, positive predictive value, and global accuracy).[Results]: The Brigham and Women's Hospital and Salamanca systems were more distinctive, homogeneous, and monotonic than the Tübingen system. The Tübingen system was the most specific, whereas the Salamanca and Brigham and Women's Hospital systems were more sensitive. Negative predictive value was high in all 3 systems, but positive predictive value and accuracy were low overall.[Conclusions]: Alternative staging systems may partially overcome the heterogeneity and low prognostic accuracy of the American Joint Committee on Cancer, eighth edition and enable high-risk cutaneous SCCs to be stratified more reliably, but their prognostic accuracy is still low. Considering the accumulation of risk factors may improve high-risk cutaneous SCC risk stratification.Dr Cañueto is partially supported by grant PI18/000587 (Instituto de Salud Carlos III, cofinanced by FEDER funds)

Definition of prognostic subgroups in the T3 stage of the eighth edition of the American Joint Committee on Cancer staging system for cutaneous squamous cell carcinoma: Tentative T3 stage subclassification

[Background]: Although the eighth edition of the American Joint Committee on Cancer staging system (AJCC8) provides improved prognosis stratification of cutaneous squamous cell carcinoma (CSCC) over AJCC7, T3 has a variable prognosis.[Objective]: To define prognostic subgroups in T3-AJCC8 CSCC.[Methods]: Retrospective cohort study of 196 primary T3-AJCC8 CSCCs. We conducted multidimensional scaling analysis using the 6 risk factors that define T3 CSCCs. The prognoses of the groups obtained were analyzed by means of competing risk analysis.[Results]: Group 1 was characterized by a tumor thickness greater than 6 mm (without invasion beyond the subcutaneous fat), alone or in combination with a tumor width of at least 4 cm. Group 2 was characterized by the presence of either invasion beyond the subcutaneous fat or by the involvement of nerves (≥0.1 mm, or deeper than the dermis). Group 3 was characterized by the combination of both T3b risk factors, or of 3 or more risk factors. Group 3 (tentatively named T3c) patients had the worst prognosis for disease-specific poor outcome events and major events, Group 2 (T3b) had intermediate risk, and Group 1 (T3a) had the best prognosis (disease-specific poor outcome events: hazard ratio [HR], 1.94; P = .00009; major events: HR, 2.55; P = .00001; disease-specific death: HR, 10.25; P = .0009).[Limitations]: Retrospective study.[Conclusions]: There is statistically significant evidence that T3-AJCC8 may be classified into distinct prognostic subgroups.Funding sources: Dr Cañueto is partially supported by the grants PI18/000587 (Instituto de Salud Carlos III, cofinanciación Fondo Europeo de Desarrollo Regional [FEDER]) and GRS 1835/A/18 (Gerencia Regional de Salud de Castilla y León), and by the Programa de Intensificación de la Actividad Investigadora de la Gerencia Regional de Salud de Castilla y León (INT/M/10/19), Spain.Peer reviewe

Comparing the eighth and the seventh editions of the American Joint Committee on Cancer staging system and the Brigham and Women's Hospital alternative staging system for cutaneous squamous cell carcinoma: Implications for clinical practice.

[Background]: The new eighth edition of the American Joint Committee on Cancer staging system (AJCC-8) incorporates changes regarding cutaneous squamous cell carcinoma (CSCC).[Objectives]: We aimed to compare the AJCC-8 staging system with the previous seventh edition of the AJCC staging system (AJCC-7) and the Brigham and Women's Hospital (BWH) alternative staging system to identify their usefulness and the utility of their risk factors in defining prognostic groups in CSCC.[Methods]: A series of 186 CSCCs of the head and neck were retrospectively collected. All 3 staging systems were compared from the standpoint of their ability to predict poor prognosis. Binary logistic regression models were built to determine which risk factors were most relevant.[Results]: Poor prognosis was mainly associated with stage T2 of the AJCC-7, with stages T2b/T3 of the BWH system, and with stage T3 of the AJCC-8. The AJCC-8 and the BWH staging systems displayed overlap with each another in predicting poor prognosis, and both were superior to the AJCC-7. The new risk factors incorporated into the AJCC-8 and the poor degree of differentiation were independently associated with poor outcome.Dr Cañueto is partially supported by a grant (GRS 1342/A/16) from the Regional Health Office of Castile and Leon and by the program INT/M/16/17 from the Regional Health Office of Castile and Leon. Dr Pérez-Losada was partially supported by FEDER and the MICINN (grants SAF2014-56989-R and SAF2017-88854-R), the Instituto de Salud Carlos III (PIE14/00066), and the We Can Be Heroes Foundatio