Ángel Pizarro

Nodular lesions arising in a large congenital melanocytic naevus in a newborn with eruptive disseminated Spitz naevi.

Congenital malignant melanoma within a pre‐existing large congenital melanocytic naevus (CMN) is exceedingly rare. Its incidence is difficult to determine due to the small number of reported cases and because of problems associated with diagnosis. Some benign nodular proliferations (called proliferative nodules) arising in CMN, while rare, are significantly more common and can mimic malignant melanoma clinically or histologically. There are no reported cases of congenital melanoma or benign proliferative nodules in CMN in patients who also had eruptive disseminated Spitz naevi. We describe a girl who was noted to have a dark‐brown plaque with several large erythematous nodules affecting the scalp at delivery, in addition to multiple erythematous dome‐shaped papules that developed in a disseminated manner over several months, beginning at 10 days of age. It was difficult, not only clinically but also histologically, to determine the benign or malignant nature of all of these lesions. As primary cutaneous melanoma, atypical proliferative nodules in CMN, bland CMN or CMN with foci of increased cellularity and Spitz naevi show clear differences in the genetic aberration patterns, comparative genomic hybridization (CGH) could be a diagnostic help in ambiguous cases such as this. CGH performed on this patient showed multiple DNA copy number changes in the most atypical nodule, but such alterations could not be found in the remainder of the lesions. CGH showed differences between the nodular lesions that occurred in the CMN and helped us in supporting the diagnosis of this unique case of benign proliferative nodules and a possible congenital melanoma arising in a large CMN, associated with multiple widespread eruptive Spitz naevi.

Dermatoscopic characteristics of acrochordon-like basal cell carcinomas in Gorlin-Goltz syndrome

BACKGROUND Gorlin-Goltz syndrome (GGS) is an autosomal-dominant disease characterized by the early onset of multiple basal cell carcinomas (BCCs), among other findings. Clinically, the BCCs may appear as soft pedunculated neoplasms that can be mistaken for true acrochordons. OBJECTIVE We sought to describe the dermatoscopic characteristics of small acrochordon-like or polypoid BCCs in a child with GGS, and to perform histopathologic correlation. METHODS Acrochordon-like growths from a child with GGS were studied. Clinical records and digital dermatoscopic images were collected, and excision and histopathologic examination of the most representative lesions were performed. RESULTS Some acrochordon-like lesions showed specific dermatoscopic criteria for BCC, including multiple blue-gray globules and arborizing telangiectasia. Other polypoid lesions, especially the smaller ones, exhibited characteristics that suggested BCC, such as isolated blue-gray globules, small blue-gray ovoid nests, and fine elongated telangiectases. LIMITATIONS Conclusions are limited by the small sample size. CONCLUSION Dermatoscopy may be a useful diagnostic tool to analyze acrochordon-like lesions in children and to facilitate early diagnosis and treatment of BCCs in patients with GGS.

Tratamiento tópico de las metástasis cutáneas de melanoma con imiquimod

Resumen El tratamiento de las metastasis cutaneas de melanoma puede ser dificil en muchos casos debido a la edad de los pacientes, asi como al numero, tamano y localizacion de las lesiones. Se presenta el caso de un varon de 82 anos con metastasis cutaneas de melanoma en el cuero cabelludo que respondieron satisfactoriamente al tratamiento con imiquimod al 5% en crema. El imiquimod es un inmunomodulador de uso topico con actividad antiviral y antineoplasica. El presente caso, junto con otros recientemente publicados, apoya la utilidad de este tratamiento en casos seleccionados de metastasis cutaneas de melanoma, al menos con un fin paliativo.The treatment of skin metastases of melanoma can be difficult in many cases because of the patients age, as well as the number, size and location of the lesions. We present the case of an 82-year-old male with melanoma skin metastases on the scalp, which responded satisfactorily to treatment with 5 % imiquimod cream. Imiquimod is a topical immunomodulator with antiviral and antineoplastic action. This case, along with others that have recently been published, supports the usefulness of this treatment in selected cases of melanoma skin metastases, at least for palliative purposes.

Clinical-Pathological Features of Cutaneous Melanomas Diagnosed in a Mediterranean Tertiary Hospital Between 1990 and 2004: A Comparison Between Sexes and Age Groups and Analysis of Long-Term Outcomes

BACKGROUND The incidence of malignant melanoma has increased over recent decades. Early diagnosis continues to be essential for effective treatment. Our objective was to analyze cutaneous malignant melanomas diagnosed over a 15-year period in a tertiary hospital for trends towards earlier diagnosis and to identify subgroups with poorer prognosis. MATERIAL AND METHODS Retrospective analysis of primary cutaneous melanomas analyzed in the pathology department of Hospital La Paz, Madrid, Spain, between 1990 and 2004. RESULTS In total, 526 melanomas were diagnosed. The mean (SD) Breslow thickness was 2.63 (4.84) mm and the median thickness was 0.98 mm (range, 0-65 mm). The mean size (widest point) was 16.59 (12.11) mm. The most common histological type was surface-spreading melanoma and the most common site was the trunk. Melanomas detected in men were generally larger and thicker than in women (P=0.05). Individuals aged over 60 years consulted for significantly thicker and larger tumors than younger individuals. The incidence of malignant melanomas has increased steadily over the years whereas the mean Breslow thickness and size have decreased. CONCLUSIONS Diagnosis of melanoma in Spain is made increasingly earlier, although locally advanced tumors are still sometimes seen in men and in individuals aged over 60 years.

Retículo pigmentado negativo en un nevus melanocítico en crecimiento

A pigmented lesion with focal growth was detected during follow-up of a patient with multiple melanocytic nevi. Dermoscopy examination revealed a slightly blue zone as well as a zone with negative pigment network in the growing area. A possible diagnosis of early melanoma was considered. Histopathology revealed a compound nevus with architectural atypia but without cytologic atypia in the growing area.

Why Does Sentinel Lymph Node Biopsy Not Increase Survival in Patients With Melanoma

demonstrate. The findings of this study—published recently—indicate that this is not the case, since survival in the group of patients who initially underwent SLNB was similar to that of patients who were initially allocated to observation alone. 5 Here, we should emphasize a second point: long-term survival in patients with stage III melanoma is approximately 30%, 2 suggesting that delayed or therapeutic lymphadenectomy can be curative in a significant percentage Before trying to answer the question posed by the title of this article, we should perhaps ask another question: why might we expect sentinel lymph node biopsy (SLNB) to increase survival in patients with melanoma at all? If we think of SLNB as an essentially diagnostic technique, we would not expect the technique to provide therapeutic benefit or to directly (as opposed to indirectly) prolong survival in patients with melanoma. SLNB offers prognostic information of undoubted value, as reflected in the extensive review of the subject by Mangas et al 1 published in this same issue of Actas Dermo-Sifiliograficas. The prognostic value of detecting the presence of melanoma micrometastases in sentinel lymph nodes has led to SLNB being incorporated into the most recent tumor staging system published by the American Joint Committee on Cancer. 2 In my personal opinion, this inclusion is fully supported by the available data. It is at this point, however, that I shall place special emphasis on a first observation essential to following the line of argument in this article: positive SLNB is indicative of poor prognosis even though lymph node micrometastases are detected, in theory, at a very early stage in the process of melanoma spread. This poor prognosis associated with positive SLNB is a strong indication in itself that the technique detects a problem at a very early stage but that, in most cases, we are unable to resolve it (or at least, not to a greater extent than when the problem is detected later). However, a recent study by Essner 3 affirmed that debate about whether SLNB is a diagnostic or therapeutic intervention persists and Ferrandiz and Mangas, 4 in another recent article, concluded that we might indeed expect some direct therapeutic benefit from SLNB in patients with melanoma. Moreover, the design of the Multicenter Selective Lymphadenectomy Trial-I (MSLT-I) 5 was based on the belief that SLNB would improve overall survival in patients with melanoma, and that was also what the trial aimed to

Tratamiento del lentigo maligno con interferón alfa-2b intralesional

Resumen —Las limitaciones de la cirugia oncologica cutanea han llevado a buscar nuevas modalidades terapeuticas que, con una seguridad y eficacia similares a las de la cirugia, la superen en resultado estetico y funcional. En el tratamiento de lesiones cutaneas malignas y premalignas es cada vez mas frecuente el uso de la laserterapia, la criocirugia y los inmunomoduladores topicos o intralesionales. En la mayoria de los casos se recurre a estos tratamientos cuando la cirugia va a ser excesivamente mutilante, desfiguradora, o tecnicamente dificil, lo cual suele ser relativamente frecuente en localizaciones como la cara o las zonas acrales. La respuesta favorable observada en el tratamiento con interferon (IFN) intralesional de metastasis cutaneas inoperables de melanoma ha llevado a probar su utilidad en casos seleccionados de melanoma primario, y existen ya varios trabajos que sugieren la eficacia y seguridad de este farmaco en el lentigo maligno. Se describe un caso de lentigo maligno localizado en el canto externo del ojo izquierdo que fue tratado con interferon alfa-2b (IFN-α2b) intralesional. Se objetivo la desaparicion clinica e histologica de la lesion sin efectos adversos destacables y con un resultado estetico excelente. El IFN intralesional puede ser una opcion terapeutica valida para casos seleccionados de lentigo maligno.

THE RHYTHMIC PARADOXICAL ERUPTION SPECTRUM IN THE AIDS ERA

We read with interest the case report by Diekman et a1 (1 992 j entitled ‘Spontaneous regression of Ki-1 positive T-cell non Hodgkin lymphoma in a patient with HIV infection’. They reported the case of an HIV-1 antibody seropositive male who developed cutaneous lesions and tumours in the respiratory tract, with an autoresolutive course. Both cutaneous and bronchial biopsies showed an identical anaplastic Ki-1 positive non-Hodgkin’s lymphoma. In the differential diagnosis, Diekman et a1 (1992) considered lymphomatoid papulosis (LyP). We think that another suggestive differential diagnosis for this case could be a regressing cutaneous phase of Ki-l positive anaplastic large cell lymphoma, also called regressing atypical histiocytosis (RAH) (Matley et al, 1992). Clinically, this lymphoma is characterized by large spontaneously remitting nodule-ulcerative lesions, confined to, or initially arising from, the skin. Although LyP is also characterized by self-healing skin lesions, their lesions are smaller and more widespread than those of RAH. Originally described by Flynn Pt a1 (1982). 19 cases of this particular type of Ki-1 positive lymphomas have been reported to the best of our knowledge (Matley et al. 1982: Cerio &Black, 1990). To date, systemic involvement in RAH and fatal outcome have been reported in four cases (Matley et al, 1992). The differential diagnosis between certain cutaneous Ki-1 positive anaplastic large cell lymphoma, RAH and LyP are controversial, as well as the ultimate nature of these diseases, included in the ‘rhythmic paradoxical eruption spectrum’ (Macaulay, 1989). Thus, recently, both LyP and RAH were diagnosed in the same patient (Cerio et al, 1990). The authors suggested that these diseases are variants of the same disorder, and proposed that RAH should be more accurately renamed ‘pleomorphic giant lymphomatoid papulosis’. An identical rearrangement of the T-cell-receptor alpha chain gene in a patient who developed consecutively a LyP, a Hodgkin disease and a cutaneous T-cell lymphoma is of interest. It suggests a common T-cell clone in the origin of these diseases in this patient (Davis et a!, 1992). It supports the idea that diseases included in the ‘rhythmic paradoxical eruption spectrum’ are a common disease type with differing manifestation (Matley ct nl, 1992). The case reported by Diekman et a1 (1 992) which could represent a regressing cutaneous phase of Ki-1 positive anaplastic large cell lymphoma (or RAH), with systemic involvement from the initial phase of the disease, suggests that HIV infection and subsequently immune disregulations. could favour a more aggressive course of the diseases included in the ‘rhythmic paradoxical eruption spectrum’. Thus, in the AIDS era, the study of clinical, pathological, immunological and molecular events in HIV seropositive patients with any of these diseases (LyP, Hodgkin disease, RAH, Ki-1 positive lymphomas, mycosis fungoides) may aid in understanding their intricate nature.