Brentuximab vedotin in the treatment of cutaneous T-cell lymphomas: Data from the Spanish Primary Cutaneous Lymphoma Registry.

BACKGROUND: Brentuximab vedotin (BV) has been approved for CD30-expressing cutaneous T-cell lymphoma (CTCL) after at least one previous systemic treatment. However, real clinical practice is still limited. OBJECTIVES: To evaluate the response and tolerance of BV in a cohort of patients with CTCL. METHODS: We analysed CTCL patients treated with BV from the Spanish Primary Cutaneous Lymphoma Registry (RELCP). RESULTS: Sixty-seven patients were included. There were 26 females and the mean age at diagnosis was 59 years. Forty-eight were mycosis fungoides (MF), 7 Sézary syndrome (SS) and 12 CD30+ lymphoproliferative disorders (CD30 LPD). Mean follow-up was 18 months. Thirty patients (45%) showed at least 10% of CD30+ cells among the total lymphocytic infiltrate. The median number of BV infusions received was 7. The overall response rate (ORR) was 67% (63% in MF, 71% in SS and 84% in CD30 LPD). Ten of 14 patients with folliculotropic MF (FMF) achieved complete or partial response (ORR 71%). The median time to response was 2.8 months. During follow-up, 36 cases (54%) experienced cutaneous relapse or progression. The median progression free survival (PFS) was 10.3 months. The most frequent adverse event was peripheral neuropathy (PN) (57%), in most patients (85%), grades 1 or 2. CONCLUSIONS: These results confirm the efficacy and safety of BV in patients with advanced-stage MF, and CD30 LPD. In addition, patients with FMF and SS also showed a favourable response. Our data suggest that BV retreatment is effective in a proportion of cases.

Primary cutaneous lymphomas in children: A prospective study from the Spanish Academy of Dermatology and Venereology (AEDV) Primary Cutaneous Lymphoma Registry.

BACKGROUND/OBJECTIVES: Primary cutaneous lymphomas are rare in pediatric patients. The clinical and histopathological manifestations may differ from those in adults. Due to their low frequency and the insidious clinical picture, the diagnosis is usually delayed. The Spanish Primary Cutaneous Lymphoma Registry was initiated in 2016 as a multicenter registry that would allow better insight into the epidemiological, clinical, histopathological, and treatment response characteristics of patients with primary cutaneous lymphomas. METHODS: We conducted a prospective observational cohort study of primary cutaneous lymphomas in pediatric patients participating in the Spanish Academy of Dermatology and Venereology (AEDV) Primary Cutaneous Lymphoma Registry. RESULTS: At the time of the analysis, 10 patients under 18 years of age out of 799 all-age cases (1.25%) had been included in the registry (7 males, 3 females). The mean age at diagnosis was 9.7 years (SD: 4.8). Seven (70%) had mycosis fungoides, 2 of them had the folliculotropic variant; and 3 (30%) had primary cutaneous marginal zone B-cell lymphoma. CONCLUSIONS: Primary cutaneous lymphomas are extremely rare in pediatric patients and usually have a good prognosis. Therefore, a high level of suspicion is necessary for the diagnosis. We suggest management by experienced physicians and follow-up into adulthood.

Congenital hemangioma: a report of evolution from rapidly involuting to noninvoluting congenital hemangioma with aberrant Mongolian spots.

Congenital hemangiomas are unusual vascular tumors that are fully developed at the time of delivery. We report a case of an infant with an exophytic congenital hemangioma with features of a rapidly involuting hemangioma on the arm which over 3 years decreased in volume but continued to persist with features of a noninvoluting congenital hemangioma. He also had aberrant, persistent Mongolian spots on both legs. To our knowledge, this association has not been reported to date.

Clinicopathologic study and DNA analysis of 37 cardiac myxomas: a 28-year experience.

PURPOSE: This study was performed to identify morphologic features of cardiac myxomas related to embolism and to provide a better understanding of the biology of these tumors, mainly in relation to their interleukin (IL)-6 expression and DNA content. PATIENTS AND METHODS: A total of 37 cardiac myxomas were reviewed retrospectively in a clinicopathologic study that included the correlation of echocardiographic and pathologic findings in 25 cases, together with immunohistochemical evaluation of IL-6 expression and flow cytometric DNA analysis of 35 tumors. RESULTS: There were 24 female patients and 13 male patients. The mean (+/- SD) age was 52 +/- 15 years. Fifty-four percent of patients presented with dyspnea, 51% presented with increased erythrocyte sedimentation rate (ESR), and 27% presented with embolic episodes, which were significantly associated with villous surface tumors. Atrial fibrillation was registered in 19% of patients and was significantly associated with large left atrial myxomas. Echocardiography proved to be a reliable method for preoperative diagnosis and for predicting tumor size and morphology. There was no perioperative mortality or long-term recurrences. The frequency of early surgical complications was associated with a longer mean ischemic time. Seventeen percent of tumors had abnormal DNA content, and 74% of tumors showed immunohistochemical expression of IL-6. Neither of these factors showed a significant association with embolism or constitutional illness. CONCLUSIONS: Villous surface myxomas are related to embolism, and large left atrial tumors are related to atrial fibrillation. Echocardiography is a reliable method with which to predict tumor size and morphology. Myxoma cells usually express IL-6, and some tumors have abnormal cellular DNA content. Surgical excision of the tumor is a safe and effective treatment.

[Necrobiosis lipoidica over appendectomy scar in a patient with morphea of the breast]. / Necrobiosis lipoídica sobre cicatriz de apendicectomía en una paciente con morfea de mama.

Morphea is a sclerosing skin disease that may be clinically confused with a carcinoma when it involves the breast. It has been described in association with earlier radiotherapy and with silicone breast implants. We describe the case of a woman with morphea of the breast who developed necrobiosis lipoidica over the scar of an appendectomy performed 38 years earlier. Necrobiosis lipoidica over surgical scars is infrequently found in the literature. We review the published cases and discuss the association of necrobiosis lipoidica with morphea.

[Acneiform eruption from epidermal growth factor receptor inhibitors]. / Erupción acneiforme por inhibidores del receptor de crecimiento epidérmico.

One of the fundamental aims of oncological research is the search for molecules with greater efficacy against tumors and less toxicity than the usual chemotherapeutic agents. Epidermal growth factor receptor inhibitors are a new group of drugs which, because of their more specific effect against neoplastic cells, seem to meet these characteristics. Skin eruptions are one of the most frequent adverse effects associated with their use, secondary to the drug's direct inhibitory effect on homeostasis of the epidermis and of the pilosebaceous follicle. Several cases of cutaneous toxicity in patients treated with epidermal growth factor receptor inhibitors have recently been published. We present three cases of acneiform eruptions attributable to different drugs in this family (cetuximab, gefitinib and erlotinib).

[Treatment of cutaneous T-cell lymphomas with bexarotene]. / Tratamiento de linfomas cutáneos de células T con bexaroteno.

INTRODUCTION: The choice of treatment in cutaneous T-cell lymphomas (CTCLs) depends on the clinical stage of the disease and the patient's general condition. To date, there is no curative treatment for this disease, and the objective is to control the symptoms and prevent the disease from progressing. Bexarotene is an X receptor-specific retinoid with anti-tumor activity. Its use as treatment for CTCLs refractory to at least one prior systemic therapy has been approved by the FDA. PATIENTS AND METHODS: We carried out a descriptive study of 9 patients treated with bexarotene in the Lymphoma Unit of our department. We analyzed the clinical characteristics of the patients and the efficacy of the treatment, and we collected data on the side effects that appeared. RESULTS: The overall response to the treatment was 44.4% (4/9). 2 patients had full remission and 2 had partial remission. Tolerance to the treatment was good, and the most frequent side effects were hypertriglyceridemia, hypercholesterolemia and central hypothyroidism. CONCLUSIONS: Even though this is a series of only 9 patients, the results that we obtained are similar to ones previously described. Bexarotene is an effective therapeutic option in this heterogeneous group of diseases.

Anticipated diagnosis of left atrial myxoma following histological investigation of limb embolectomy specimens: a report of two cases.

Nonfamilial myxoma occurs as a random event. The tumor is rare and can mimic other diseases. Cardiac myxomas should always be considered as a source of embolization, which need meticulous investigation and prompt indication of surgical resection. Tumors with a villous surface are prone to embolize. We report two cases of cardiac myxoma presenting as acute ischemia of one or two limbs due to embolic phenomena. The patients were females aged 55 and 37 years. Histological study of emboli taken from obstructed limb arteries in the two patients showed a picture indicating systemic embolization of a cardiac myxoma. The embolic tissue fragments showed the gross characteristics (i.e. villous surface) of the cardiac tumor. Further echocardiography and surgical removal confirmed the cardiac myxoma. Immunohistochemical study of embolectomy material disclosed strong reactivity of the tumor cells for calretinin. The histological examination of the embolectomy material can anticipate the cardiac lesion and its gross features. Calretinin is a useful marker in the differential diagnosis of cardiac myxoma with a myxoid thrombus. The necessity of histological examination of the embolectomy material is stressed.

Necrobiosis lipoídica sobre cicatriz de apendicectomía en una paciente con morfea de mama / Necrobiosis lipoidica over appendectomy scar in a patient with morphea of the breast

La morfea es una enfermedad esclerosante de la piel que cuando afecta a la mama puede confundirse clínicamente con un carcinoma. Se ha descrito asociada con radioterapia previa y con implantes mamarios de silicona. Se describe el caso de una mujer con morfea de mama que desarrolló una necrobiosis lipoídica sobre una cicatriz de apendicectomía realizada 38 años antes. La necrobiosis lipoídica sobre cicatrices quirúrgicas es un hallazgo poco frecuente en la literatura médica. En este artículo se revisan los casos publicados y se discute la asociación de la necrobiosis lipoídica con la morfea

Morphea is a sclerosing skin disease that may be clinically confused with a carcinoma when it involves the breast. It has been described in association with earlier radiotherapy and with silicone breast implants. We describe the case of a woman with morphea of the breast who developed necrobiosis lipoidica over the scar of an appendectomy performed 38 years earlier. Necrobiosis lipoidica over surgical scars is infrequently found in the literature. We review the published cases and discuss the association of necrobiosis lipoidica with morphea

Tratamiento de linfomas cutáneos de células T con bexaroteno / Treatment of cutaneous T-cell lymphomas with bexarotene

Introducción. La elección del tratamiento en los linfomas cutáneos de células T (LCCT) depende del estadio clínico de la enfermedad y del estado general del paciente. Hasta hoy no existe ningún tratamiento curativo para esta enfermedad y el objetivo es controlar los síntomas y prevenir la progresión de la enfermedad. El bexaroteno es un retinoide agonista específico de los receptores X de los retinoides con actividad antitumoral. Su uso fue aprobado por la Food and Drug Administration (FDA) como tratamiento de LCCT refractarios a al menos una terapia sistémica previa. Pacientes y métodos. Realizamos un estudio descriptivo de 9 pacientes que han sido tratados con bexaroteno en la Unidad de Linfomas de nuestro servicio. Analizamos las características clínicas de los pacientes y eficacia del tratamiento y recogemos los efectos secundarios presentados. Resultados. La respuesta global al tratamiento fue del 44,4 % (4/9). Un total de 2 pacientes presentaron una remisión completa y los otros dos, una remisión parcial. La tolencia al tratamiento fue buena y los efectos secundarios más frecuentes fueron la hipertrigliceridemia, la hipercolesterolemia y el hipotiroidismo central. Conclusiones. Aunque se trata de una serie de únicamente 9 pacientes los resultados que obtuvimos son similares a los descritos previamente. El bexaroteno es una opción terapéutica eficaz en este grupo heterogéneo de enfermedades

Introduction. The choice of treatment in cutaneous T-cell lymphomas (CTCLs) depends on the clinical stage of the disease and the patient's general condition. To date, there is no curative treatment for this disease, and the objective is to control the symptoms and prevent the disease from progressing. Bexarotene is an X receptor-specific retinoid with anti-tumor activity. Its use as treatment for CTCLs refractory to at least one prior systemic therapy has been approved by the FDA. Patients and methods. We carried out a descriptive study of 9 patients treated with bexarotene in the Lymphoma Unit of our department. We analyzed the clinical characteristics of the patients and the efficacy of the treatment, and we collected data on the side effects that appeared. Results. The overall response to the treatment was 44.4 % (4/9). 2 patients had full remission and 2 had partial remission. Tolerance to the treatment was good, and the most frequent side effects were hypertriglyceridemia, hypercholesterolemia and central hypothyroidism. Conclusions. Even though this is a series of only 9 patients, the results that we obtained are similar to ones previously described. Bexarotene is an effective therapeutic option in this heterogeneous group of diseases

Placas amarillentas en tronco y flexuras axilares / Yellowish plaques on the trunk and the axillary folds

No disponible

Erupción acneiforme por inhibidores del receptor de crecimiento epidérmico / Acneiform eruption from epidermal growth factor receptor inhibitors

Uno de los objetivos fundamentales de la investigación oncológica es la búsqueda de moléculas con mayor eficacia antitumoral y menor toxicidad que los agentes quimioterápicos habituales. Los inhibidores del receptor de crecimiento epidérmico son un nuevo grupo de fármacos que, por su efecto más específico frente a las células neoplásicas, parecen cumplir estas características. Entre los efectos adversos asociados a su uso, destacan por su frecuencia las erupciones cutáneas, secundarias a un efecto inhibitorio directo del fármaco sobre la homeostasis de la epidermis y del folículo pilosebáceo 1,2. Recientemente se han publicado varios casos de toxicidad cutánea en pacientes tratados con inhibidores del receptor de crecimiento epidérmico. Presentamos 3 casos de erupciones acneiformes atribuibles a diferentes fármacos de esta familia (cetuximab, gefitinib y erlotinib)

One of the fundamental aims of oncological research is the search for molecules with greater efficacy against tumors and less toxicity than the usual chemotherapeutic agents. Epidermal growth factor receptor inhibitors are a new group of drugs which, because of their more specific effect against neoplastic cells, seem to meet these characteristics. Skin eruptions are one of the most frequent adverse effects associated with their use, secondary to the drug's direct inhibitory effect on homeostasis of the epidermis and of the pilosebaceous follicle. Several cases of cutaneous toxicity in patients treated with epidermal growth factor receptor inhibitors have recently been published. We present three cases of acneiform eruptions attributable to different drugs in this family (cetuximab, gefitinib and erlotinib)

Histiocitosis de células de Langerhans nodular hepática / Nodular Langerhans cell histiocytosis of the liver

Planteamiento: La histiocitosis de células de Langerhans es una enfermedad caracterizada por la acumulación, uni o multifocal, de células de Langerhans que rara vez afecta al hígado. Los hallazgos histopatológicos más comunes son la infiltración y destrucción de pequeños conductos biliares, la colangitis destructiva de grandes conductos o masas de células de Langerhans en el parénquima hepático. Los pacientes con manifestaciones hepáticas suelen tener enfermedad diseminada. Material y método: Se presenta el caso de un paciente con histiocitosis de células de Langerhans nodular hepática asociada a un adenocarcinoma rectal. La lesión se detectó de manera incidental durante la resección del tumor rectal y se interpretó macroscópicamente como un nódulo metastásico. Resultados: El nódulo estaba constituido por células de Langerhans positivas para CD1a y proteína S100, con numerosos eosinófilos. El paciente se encuentra sin evidencia de enfermedad después de 14 meses de seguimiento. Conclusiones: La histiocitosis de células de Langerhans nodular hepática sin afectación extrahepática es poco frecuente. En nuestro caso se puede interpretar como un proceso reactivo frente al adenocarcinoma rectal. Lesiones semejantes se deberían tener en cuenta en el diagnóstico intraoperatorio de nódulos hepáticos en pacientes con tumores gastrointestinales

Background: Langerhans cell histiocytosis is a disease whose hallmark is the accumulation, uni or multifocal, of Langerhans cells. Liver is rarely affected. Small bile duct infiltration and destruction, destructive cholangitis of larger bile ducts or masses of Langerhans cells are common findings. Patients with liver manifestations usually have disseminated disease. Material and methods: A nodular Langerhans cells histiocytosis of the liver in a patient with concomitant rectal adenocarcinoma is presented. The lesion was incidentally found during the resection of a rectal tumour and it was macroscopically thought to be a metastatic nodule. Results: The nodule was made of Langerhans cells that reacted with CD1a and S100 protein, with numerous eosinophils. The patient is well and no evidence of disease exists after 14 months of follow-up. Conclusions: Nodular Langerhans cell histiocytosis of the liver without extrahepatic involvement is not usual. In this case the nodule is probably a reactive response to the rectal carcinoma. Similar lesions must be kept in mind in the intraoperative diagnosis of liver nodules in patients with gastrointestinal tumors

Histiocitosis de células de Langerhans nodular hepática / Nodular Langerhans cell histiocytosis of the liver

Planteamiento: La histiocitosis de células de Langerhans es una enfermedad caracterizadapor la acumulación, uni o multifocal, de células de Langerhans que rara vez afecta al hígado. Loshallazgos histopatológicos más comunes son la infiltración y destrucción de pequeños conductosbiliares, la colangitis destructiva de grandes conductos o masas de células de Langerhansen el parénquima hepático. Los pacientes con manifestaciones hepáticas suelen tener enfermedaddiseminada. Material y método: Se presenta el caso de un paciente con histiocitosis decélulas de Langerhans nodular hepática asociada a un adenocarcinoma rectal. La lesión sedetectó de manera incidental durante la resección del tumor rectal y se interpretó macroscópicamentecomo un nódulo metastásico. Resultados: El nódulo estaba constituido por células deLangerhans positivas para CD1a y proteína S100, con numerosos eosinófilos. El paciente seencuentra sin evidencia de enfermedad después de 14 meses de seguimiento. Conclusiones:La histiocitosis de células de Langerhans nodular hepática sin afectación extrahepática es pocofrecuente. En nuestro caso se puede interpretar como un proceso reactivo frente al adenocarcinomarectal. Lesiones semejantes se deberían tener en cuenta en el diagnóstico intraoperatoriode nódulos hepáticos en pacientes con tumores gastrointestinales

Background: Langerhans cell histiocytosis is a disease whose hallmark is the accumulation, unior multifocal, of Langerhans cells. Liver is rarely affected. Small bile duct infiltration and destruction,destructive cholangitis of larger bile ducts or masses of Langerhans cells are common findings.Patients with liver manifestations usually have disseminated disease. Material and methods: Anodular Langerhans cells histiocytosis of the liver in a patient with concomitant rectal adenocarcinomais presented. The lesion was incidentally found during the resection of a rectal tumour and it wasmacroscopically thought to be a metastatic nodule. Results: The nodule was made of Langerhanscells that reacted with CD1a and S100 protein, with numerous eosinophils. The patient is well andno evidence of disease exists after 14 months of follow-up. Conclusions: Nodular Langerhans cellhistiocytosis of the liver without extrahepatic involvement is not usual. In this case the nodule is probablya reactive response to the rectal carcinoma. Similar lesions must be kept in mind in the intraoperativediagnosis of liver nodules in patients with gastrointestinal tumors

Neurocristopatía / Neurocristopathy

Las neurocristopatías son las enfermedades secundarias al desarrollo embriológico anómalo de la cresta neural. Al contribuir en la formación de varios tipos de células, las manifestaciones clínicas pueden afectar a una gran variedad de estructuras, entre ellas la piel. Se presenta una niña con un trastorno pigmentario congénito y estudio anatomopatológico compatible con una neurocristopatía (AU)