Facial Angiofibroma Severity Index (FASI): reliability assessment of a new tool developed to measure severity and responsiveness to therapy in tuberous sclerosis-associated facial angiofibroma.

BACKGROUND: Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous disorder characterized by the development of multisystem hamartomatous tumours. Topical sirolimus has recently been suggested as a potential treatment for TSC-associated facial angiofibroma (FA). AIM: To validate a reproducible scale created for the assessment of clinical severity and treatment response in these patients. METHODS: We developed a new tool, the Facial Angiofibroma Severity Index (FASI) to evaluate the grade of erythema and the size and extent of FAs. In total, 30 different photographs of patients with TSC were shown to 56 dermatologists at each evaluation. Three evaluations using the same photographs but in a different random order were performed 1 week apart. Test and retest reliability and interobserver reproducibility were determined. RESULTS: There was good agreement between the investigators. Inter-rater reliability showed strong correlations (> 0.98; range 0.97-0.99) with inter-rater correlation coefficients (ICCs) for the FASI. The global estimated kappa coefficient for the degree of intra-rater agreement (test-retest) was 0.94 (range 0.91-0.97). CONCLUSIONS: The FASI is a valid and reliable tool for measuring the clinical severity of TSC-associated FAs, which can be applied in clinical practice to evaluate the response to treatment in these patients.

Positive margins in excised dermatofibrosarcoma protuberans: a study of 58 cases treated with slow-Mohs surgery.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is characterized by unpredictable subclinical extension, meaning that positive margins are frequently detected following conventional surgical excision. OBJECTIVE: To study the presence or absence of residual tumour in DFSP with positive margins after conventional surgery and identify possible predictors of residual tumour or clear margins following a single Mohs micrographic surgery (MMS) stage. METHODS: A retrospective study of patients with DFSP and positive margins following conventional excision referred for MMS was performed. We studied gender, age, tumour site, time from presentation to diagnosis, and affected margins. RESULTS: We studied 58 cases, 35 (60.3%) of which had histological evidence of residual tumour. Tumours of the head and neck were significantly associated with the persistence of tumour. A single MMS stage was sufficient to achieve clearance in the majority of cases (n = 46). All tumours with lateral involvement only were resolved with a single Mohs stage. CONCLUSIONS: DFSPs with positive margins after conventional surgical excision should undergo re-excision because the majority have histologic evidence of residual tumour. Re-excision with 1-cm margins beyond the scar could be an option in certain tumour sites, particularly when it is known which margins are involved.

Conventional surgery compared with slow Mohs micrographic surgery in the treatment of lentigo maligna: a retrospective study of 62 cases.

INTRODUCTION: Surgical excision with margins of 0.5cm is the standard treatment for lentigo maligna (LM). Excision, however, is often incomplete as many of these tumors have indistinct borders. OBJECTIVE: To identify clinical predictors of subclinical extension in primary and recurrent LM of the head and thereby determine which lesions might require wider surgical margins. MATERIAL AND METHODS: We reviewed the clinical records of patients with LM of the head treated definitively with conventional surgical excision or slow micrographic Mohs surgery (MMS) at the dermatology department of Instituto Valenciano de Oncología between January 1993 and April 2011. RESULTS: Surgical margins larger than 0.5cm were required in 69.2% of recurrent LM and 26.5% of primary LM. Factors associated with the need for wider margins were prior treatment that might have interfered with the clinical delineation of the border, lesions in the center of the face, and skin phototypes III to V. CONCLUSIONS: Surgical margins of 0.5cm are inadequate for the treatment of a considerable number of LM lesions located on the head, particularly if these are recurrent. Slow MMS using paraffin-embedded sections appears to be the treatment of choice in such cases, particularly for recurrent lesions or lesions with poorly defined borders or possible subclinical extension.

Tratamiento quirúrgico del lentigo maligno: cirugía convencional vs. Mohs diferida. Estudio retrospectivo de 62 casos / Conventional Surgery Compared With Slow Mohs Micrographic Surgery in the Treatment of Lentigo Maligna: A Retrospective Study of 62 Cases

Introducción: El tratamiento estándar del lentigo maligno (LM) es la escisión quirúrgica con márgenes de 0,5 cm. Sin embargo, dada la mala delimitación de muchos tumores, es frecuente que esta exéresis sea incompleta. Objetivo: identificar parámetros clínicos que puedan predecir qué LM localizados en la cabeza, extirpados de forma primaria o tras recidivar, se extienden más allá de los límites visibles y por tanto, puedan requerir márgenes quirúrgicos más amplios. Material y métodos: se revisó retrospectivamente la información clínica de los pacientes con LM localizado en la cabeza cuyo tratamiento quirúrgico definitivo, mediante cirugía convencional o cirugía de Mohs diferida, fue realizado en el Servicio de Dermatología del Instituto Valenciano de Oncología (IVO) entre enero de 1993 y abril de 2011. Resultados: un 69,2% de los LM recidivados y un 26,5% de los tumores primarios requirieron márgenes de más de 0,5 cm. La administración previa de tratamientos que puedan interferir en la delimitación clínica, la localización centro facial y las lesiones que se presentan en pacientes con fototipos altos (III-V) fueron los factores asociados a la necesidad de márgenes quirúrgicos más amplios. Conclusiones: la utilización de márgenes de 0,5 cm para el tratamiento del LM es insuficiente para un número importante de casos localizados en la cabeza, especialmente los recidivados. La cirugía de Mohs diferida, con el estudio de todos los márgenes en parafina, parece el tratamiento de elección en particular para los casos recidivados o en los que la delimitación clínica pueda verse dificultada (AU)

Introduction: Surgical excision with margins of 0.5 cm is the standard treatment for lentigo maligna (LM). Excision, however, is often incomplete as many of these tumors have indistinct borders. Objective: To identify clinical predictors of subclinical extension in primary and recurrent LM of the head and thereby determine which lesions might require wider surgical margins. Material and methods: We reviewed the clinical records of patients with LM of the head treated definitively with conventional surgical excision or slow micrographic Mohs surgery (MMS) at the dermatology department of Instituto Valenciano de Oncología between January 1993 and April2011. Results: Surgical margins larger than 0.5 cm were required in 69.2% of recurrent LM and 26.5% of primary LM. Factors associated with the need for wider margins were prior treatment that might have interfered with the clinical delineation of the border, lesions in the center of the face, and skin phototypes III to V. Conclusions: Surgical margins of 0.5 cm are inadequate for the treatment of a considerable number of LM lesions located on the head, particularly if these are recurrent. Slow MMS using paraffin-embedded sections appears to be the treatment of choice in such cases, particularly for recurrent lesions or lesions with poorly defined borders or possible subclinical extension (AU)