Registro de linfomas cutáneos primarios (RELCP) de la AEDV: datos tras 5 años de funcionamiento / Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV): Data for the First 5 Years

Antecedentes y objetivos Los linfomas cutáneos primarios (LCP) son un conjunto de entidades poco frecuentes. En febrero del 2018 se describieron los resultados del primer año de funcionamiento del Registro de linfomas cutáneos primarios de la AEDV. En el presente trabajo actualizamos los resultados tras 5 años de funcionamiento. Pacientes y métodos Registro de enfermedad de pacientes con LCP. Se recogieron datos prospectivamente de los pacientes, incluyendo diagnóstico, tratamientos, pruebas realizadas y estado actual del paciente. Se realizó un análisis descriptivo. Resultados En diciembre del 2021 se había incluido a un total de 2020 pacientes en el Registro, pertenecientes a 33 hospitales españoles. El 59% fueron hombres, la edad media fue de 62,2 años. Se agruparon en 4grandes grupos diagnósticos: micosis fungoide/síndrome de Sézary (1.112, 55%), LCP de células B (547, 27,1%), trastornos linfoproliferativos de células T CD30+(222, 11%) y otros linfomas T (116, 5,8%). La mayoría presentó estadio T1, encontrándose actualmente casi el 75% en remisión completa (43,5%) o enfermedad estable (EE; 27%). Los tratamientos más usados fueron corticoides tópicos (1.369, 67,8%), fototerapia (890, 44,1%), cirugía (412, 20,4%) y radioterapia (384, 19%). Conclusión Las características del paciente con LCP en España no difieren de otras series. El mayor tamaño del registro permite precisar mejor los datos con respecto a los resultados del primer año. Este registro facilita al grupo de linfomas de la AEDV realizar investigación clínica, surgiendo ya trabajos publicados de dicho registro (AU)

Background and objective Primary cutaneous lymphomas (PCL) are uncommon. Observations based on the first year of data from the Spanish Registry of Primary Cutaneous Lymphomas (RELCP, in its Spanish abbreviation) of the Spanish Academy of Dermatology and Venereology (AEDV) were published in February 2018. This report covers RELCP data for the first 5 years. Patients and methods RELCP data were collected prospectively and included diagnosis, treatments, tests, and the current status of patients. We compiled descriptive statistics of the data registered during the first 5 years. Results Information on 2020 patients treated at 33 Spanish hospitals had been included in the RELCP by December 2021. Fifty-nine percent of the patients were men; the mean age was 62.2 years. The lymphomas were grouped into 4 large diagnostic categories: mycosis fungoides/Sézary syndrome, 1112 patients (55%); primary B-cell cutaneous lymphoma, 547 patients (27.1%); primary CD30+lymphoproliferative disorders, 222 patients (11%), and other T-cell lymphomas, 116 patients (5.8%). Nearly 75% of the tumors were registered in stage I. After treatment, 43.5% achieved complete remission and 27% were stable at the time of writing. Treatments prescribed were topical corticosteroids (1369 [67.8%]), phototherapy (890 patients [44.1%]), surgery (412 patients [20.4%]), and radiotherapy (384 patients [19%]). Conclusion The characteristics of cutaneous lymphomas in Spain are similar to those reported for other series. The large size of the RELCP registry at 5 years has allowed us to give more precise descriptive statistics than in the first year. This registry facilitates the clinical research of the AEDV's lymphoma interest group, which has already published articles based on the RELCP data (AU)

[Translated article] Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV): Data for the First 5 Years / Registro de linfomas cutáneos primarios (RELCP) de la AEDV: datos tras 5 años de funcionamiento

Background and objective Primary cutaneous lymphomas (PCL) are uncommon. Observations based on the first year of data from the Spanish Registry of Primary Cutaneous Lymphomas (RELCP, in its Spanish abbreviation) of the Spanish Academy of Dermatology and Venereology (AEDV) were published in February 2018. This report covers RELCP data for the first 5 years. Patients and methods RELCP data were collected prospectively and included diagnosis, treatments, tests, and the current status of patients. We compiled descriptive statistics of the data registered during the first 5 years. Results Information on 2020 patients treated at 33 Spanish hospitals had been included in the RELCP by December 2021. Fifty-nine percent of the patients were men; the mean age was 62.2 years. The lymphomas were grouped into 4 large diagnostic categories: mycosis fungoides/Sézary syndrome, 1112 patients (55%); primary B-cell cutaneous lymphoma, 547 patients (27.1%); primary CD30+lymphoproliferative disorders, 222 patients (11%), and other T-cell lymphomas, 116 patients (5.8%). Nearly 75% of the tumors were registered in stage I. After treatment, 43.5% achieved complete remission and 27% were stable at the time of writing. Treatments prescribed were topical corticosteroids (1369 [67.8%]), phototherapy (890 patients [44.1%]), surgery (412 patients [20.4%]), and radiotherapy (384 patients [19%]). Conclusion The characteristics of cutaneous lymphomas in Spain are similar to those reported for other series. The large size of the RELCP registry at 5 years has allowed us to give more precise descriptive statistics than in the first year. This registry facilitates the clinical research of the AEDV's lymphoma interest group, which has already published articles based on the RELCP data (AU)

Antecedentes y objetivos Los linfomas cutáneos primarios (LCP) son un conjunto de entidades poco frecuentes. En febrero del 2018 se describieron los resultados del primer año de funcionamiento del Registro de linfomas cutáneos primarios de la AEDV. En el presente trabajo actualizamos los resultados tras 5 años de funcionamiento. Pacientes y métodos Registro de enfermedad de pacientes con LCP. Se recogieron datos prospectivamente de los pacientes, incluyendo diagnóstico, tratamientos, pruebas realizadas y estado actual del paciente. Se realizó un análisis descriptivo. Resultados En diciembre del 2021 se había incluido a un total de 2020 pacientes en el Registro, pertenecientes a 33 hospitales españoles. El 59% fueron hombres, la edad media fue de 62,2 años. Se agruparon en 4grandes grupos diagnósticos: micosis fungoide/síndrome de Sézary (1.112, 55%), LCP de células B (547, 27,1%), trastornos linfoproliferativos de células T CD30+(222, 11%) y otros linfomas T (116, 5,8%). La mayoría presentó estadio T1, encontrándose actualmente casi el 75% en remisión completa (43,5%) o enfermedad estable (EE; 27%). Los tratamientos más usados fueron corticoides tópicos (1.369, 67,8%), fototerapia (890, 44,1%), cirugía (412, 20,4%) y radioterapia (384, 19%). Conclusión Las características del paciente con LCP en España no difieren de otras series. El mayor tamaño del registro permite precisar mejor los datos con respecto a los resultados del primer año. Este registro facilita al grupo de linfomas de la AEDV realizar investigación clínica, surgiendo ya trabajos publicados de dicho registro (AU)

Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV): Data for the First 5 Years. / Registro de linfomas cutáneos primarios (RELCP) de la AEDV: datos tras 5 años de funcionamiento.

BACKGROUND AND OBJECTIVE: Primary cutaneous lymphomas (PCL) are uncommon. Observations based on the first year of data from the Spanish Registry of Primary Cutaneous Lymphomas (RELCP, in its Spanish abbreviation) of the Spanish Academy of Dermatology and Venereology (AEDV) were published in February 2018. This report covers RELCP data for the first 5 years. PATIENTS AND METHODS: RELCP data were collected prospectively and included diagnosis, treatments, tests, and the current status of patients. We compiled descriptive statistics of the data registered during the first 5 years. RESULTS: Information on 2020 patients treated at 33 Spanish hospitals had been included in the RELCP by December 2021. Fifty-nine percent of the patients were men; the mean age was 62.2 years. The lymphomas were grouped into 4 large diagnostic categories: mycosis fungoides/Sézary syndrome, 1112 patients (55%); primary B-cell cutaneous lymphoma, 547 patients (27.1%); primary CD30+lymphoproliferative disorders, 222 patients (11%), and other T-cell lymphomas, 116 patients (5.8%). Nearly 75% of the tumors were registered in stage I. After treatment, 43.5% achieved complete remission and 27% were stable at the time of writing. Treatments prescribed were topical corticosteroids (1369 [67.8%]), phototherapy (890 patients [44.1%]), surgery (412 patients [20.4%]), and radiotherapy (384 patients [19%]). CONCLUSION: The characteristics of cutaneous lymphomas in Spain are similar to those reported for other series. The large size of the RELCP registry at 5 years has allowed us to give more precise descriptive statistics than in the first year. This registry facilitates the clinical research of the AEDV's lymphoma interest group, which has already published articles based on the RELCP data.

Registro de linfomas cutáneos primarios de la AEDV: primer año de funcionamiento / The First Year of the AEVD Primary Cutaneous Lymphoma Registry

ANTECEDENTES Y OBJETIVO: Los linfomas primarios cutáneos son enfermedades poco frecuentes. Este artículo describe el Registro de linfomas cutáneos primarios de la AEDV y sus primeros resultados. PACIENTES Y MÉTODOS: Registro de enfermedad de pacientes con linfomas cutáneos primarios. Los centros participantes recogieron datos prospectivamente de todos los pacientes, incluyendo datos del diagnóstico, de los tratamientos, de las pruebas realizadas y del estado actual del paciente. Se realizó un análisis descriptivo. RESULTADOS: En diciembre del 2017 el registro tenía datos de 639 pacientes pertenecientes a 16 hospitales universitarios. Un 60% eran hombres y los diagnósticos, por orden de frecuencia, fueron: micosis fungoide/síndrome de Sézary (MF/SS) (348 casos; 55%), linfoma cutáneo primario de células B (LCCB) (184; 29%), trastorno linfoproliferativo de células T CD30+ (LTCD30+) (70; 11%) y otro tipo de linfoma T (OLT) (37; 5%). El número de casos incidentes recogidos durante el primer año fue de 105 (16,5%). En los pacientes con MF/SS, el diagnóstico más frecuente fue MF clásica (77,3%). La mitad de estos casos se diagnosticaron en estadio IA. La mayoría de los pacientes estaban en remisión parcial (32,5%) o enfermedad estable (33,1%). Los tratamientos más usados fueron los corticoides tópicos (90,8%) seguidos de fototerapia. En los pacientes con LCCB el diagnóstico más frecuente fue el linfoma de la zona marginal (50%). Casi todos los pacientes tuvieron afectación exclusivamente cutánea y casi la mitad fue T1a. La mayoría (76,1%) estaba en remisión completa. Los tratamientos más utilizados fueron la cirugía (55,4%) y la radioterapia (41,9%). En los pacientes con LTCD30+, el diagnóstico más frecuente fue la papulosis linfomatoide (68,6%). La mayoría fueron clasificados T3b (31,4%). La mitad de los casos estaban en remisión completa. Los tratamientos más frecuentes fueron los esteroides tópicos (68,6%), seguidos de la quimioterapia sistémica (32,9%). CONCLUSIÓN: Las características del paciente con linfoma cutáneo primario en España no difieren de otras series descritas en la literatura. El registro facilitará al grupo de linfomas de la AEDV realizar investigación clínica

BACKGROUND AND OBJECTIVE: Primary cutaneous lymphomas are uncommon. This article describes the Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV) and reports on the results from the first year. PATIENTS AND METHODS: Disease registry for patients with primary cutaneous lymphoma. The participating hospitals prospectively recorded data on diagnosis, treatment, tests, and disease stage for all patients with primary cutaneous lymphoma. A descriptive analysis was performed. RESULTS: In December 2017, the registry contained data on 639 patients (60% male) from 16 university hospitals. The most common diagnoses, in order of frequency, were mycosis fungoides/Sézary syndrome (MF/SS) (348 cases, 55%), primary cutaneous B-cell lymphoma (CBCL) (184 cases, 29%), primary cutaneous CD30+ T-cell lymphoproliferative disorder (CD30+ CLPD) (70 cases, 11%), and other types of T-cell lymphoma (37 cases, 5%). In total, 105 (16.5%) of the cases recorded were incident cases. The most common diagnosis in the MF/SS group was classic MF (77.3%). Half of the patients with MF had stage IA disease when diagnosed, and the majority were either in partial remission (32.5%) or had stable disease (33.1%). The most widely used treatments were topical coricosteroids (90.8%) and phototherapy. The most common form of primary CBCL was marginal zone lymphoma (50%). Almost all of the patients had cutaneous involvement only and nearly half had stage T1a disease. Most (76.1%) were in complete remission. The main treatments were surgery (55.4%) and radiotherapy (41.9%). The most common diagnosis in patients with CD30+ CLPD was lymphomatoid papulosis (68.8%). Most of the patients (31.4%) had stage T3b disease and half were in complete remission. The most common treatments were topical corticosteroids (68.8%) and systemic chemotherapy (32.9%). CONCLUSION: The characteristics of patients with primary cutaneous lymphoma in Spain do not differ from those described in other series in the literature. The registry will facilitate clinical research by the AEDV's lymphoma group

The First Year of the AEVD Primary Cutaneous Lymphoma Registry. / Registro de linfomas cutáneos primarios de la AEDV: primer año de funcionamiento.

BACKGROUND AND OBJECTIVE: Primary cutaneous lymphomas are uncommon. This article describes the Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV) and reports on the results from the first year. PATIENTS AND METHODS: Disease registry for patients with primary cutaneous lymphoma. The participating hospitals prospectively recorded data on diagnosis, treatment, tests, and disease stage for all patients with primary cutaneous lymphoma. A descriptive analysis was performed. RESULTS: In December 2017, the registry contained data on 639 patients (60% male) from 16 university hospitals. The most common diagnoses, in order of frequency, were mycosis fungoides/Sézary syndrome (MF/SS) (348 cases, 55%), primary cutaneous B-cell lymphoma (CBCL) (184 cases, 29%), primary cutaneous CD30+ T-cell lymphoproliferative disorder (CD30+ CLPD) (70 cases, 11%), and other types of T-cell lymphoma (37 cases, 5%). In total, 105 (16.5%) of the cases recorded were incident cases. The most common diagnosis in the MF/SS group was classic MF (77.3%). Half of the patients with MF had stage IA disease when diagnosed, and the majority were either in partial remission (32.5%) or had stable disease (33.1%). The most widely used treatments were topical corticosteroids (90.8%) and phototherapy. The most common form of primary CBCL was marginal zone lymphoma (50%). Almost all of the patients had cutaneous involvement only and nearly half had stage T1a disease. Most (76.1%) were in complete remission. The main treatments were surgery (55.4%) and radiotherapy (41.9%). The most common diagnosis in patients with CD30+ CLPD was lymphomatoid papulosis (68.8%). Most of the patients (31.4%) had stage T3b disease and half were in complete remission. The most common treatments were topical corticosteroids (68.8%) and systemic chemotherapy (32.9%). CONCLUSION: The characteristics of patients with primary cutaneous lymphoma in Spain do not differ from those described in other series in the literature. The registry will facilitate clinical research by the AEDV's lymphoma group.

Intralesional rituximab in the treatment of indolent primary cutaneous B-cell lymphomas: an epidemiological observational multicentre study. The Spanish Working Group on Cutaneous Lymphoma.

BACKGROUND: Intravenous rituximab is a safe and effective option for the treatment of systemic non-Hodgkin B-cell lymphoma. The effectiveness of intralesional rituximab (ILR) in primary cutaneous B-cell lymphomas (PCBL) has been described in a small number of patients. OBJECTIVES: To evaluate the effectiveness, tolerance and adverse effects of ILR in patients with follicle centre (FCL) and marginal zone (MZL) PCBL. METHODS: This was an epidemiological observational multicentre study of patients with PCBL treated with ILR. RESULTS: Seventeen patients with MZL and 18 with FCL PCBL were included. The median number of lesions treated was two per patient. The treatment regimen used in 74% of the patients was a course of three injections in a single week at 1-month intervals. The dose per lesion and day of treatment was 10 mg in 71% of the patients. The median cumulative dose of rituximab per lesion was 60 mg (range 13-270) and per patient was 150 mg (range 20-360 mg). Complete response (CR) and partial response were achieved in 71% and 23% of patients, respectively. The median time to CR in patients who received 10 mg of ILR per lesion was 8 weeks. Similar response rates were observed in MZL and FCL. Median disease-free survival was 114·1 weeks. No parameters that significantly predicted CR were identified. Adverse reactions were recorded in 19 patients; the most frequent was localized pain at the injection site. Median follow-up was 21 months. CONCLUSIONS: Intralesional rituximab is a well-tolerated and effective treatment for FCL and MZL PCBL. It should be considered a useful alternative in patients with recurrent lesions and in which the sequelae of radiotherapy or surgery would be significant.

Cutaneous metastases of hepatocellular carcinoma.

Cutaneous metastases are an unusual finding that may present as the first sign of an internal neoplasia. A case of cutaneous metastases of hepatocellular carcinoma, which may often involve other organs but very rarely metastases to the skin, is reported.

Trasplante autólogo de progenitores hematopoyéticos seguido de bexaroteno oral en paciente con micosis fungoide avanzada / Autologous hematopoietic stem cell transplantation folowed by oral bexarotene in a patient with advanced mycosis fungoides

Exponemos el caso de una paciente de 17 años que presentó una micosis fungoide de evolución rápida y agresiva, con múltiples tumores cutáneos y transformación fenotípica celular a linfoma de células grandes. Fue tratada inicialmente con 3 ciclos de quimioterapia en dosis altas (MEGA-CHOP) sin respuesta, por lo que se decidió realizar un trasplante autólogo de progenitores hematopoyéticos. Este tratamiento consiguió una remisión parcial de la enfermedad, y con la posterior introducción del bexaroteno oral se logró la remisión completa, que se ha mantenido durante más de 3 años con muy buena tolerancia al bexaroteno oral. Analizamos las ventajas y desventajas de la utilización del trasplante autólogo de progenitores hematopoyéticos y del uso de bexaroteno oral (AU)

We describe the case of a 17-year-old patient with rapidly progressing and aggressive mycosis fungoides, with multiple cutaneous tumors and large cell transformation. She was initially treated with 3 cycles of high-dose chemotherapy with mega-CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) without response, leading to the decision to undertake autologous hematopoietic stem cell transplantation. Partial remission of the disease was achieved with this treatment and subsequent introduction of oral bexarotene led to complete remission, which has been maintained for more than 3 years with good tolerance of oral therapy. We discuss the advantages and disadvantages of autologous hematopoietic stem cell transplantation and the use of oral bexarotene (AU)

[Autologous hematopoietic stem cell transplantation followed by oral bexarotene in a patient with advanced mycosis fungoides]. / Trasplante autólogo de progenitores hematopoyéticos seguido de bexaroteno oral en paciente con micosis fungoide avanzada.

We describe the case of a 17-year-old patient with rapidly progressing and aggressive mycosis fungoides, with multiple cutaneous tumors and large cell transformation. She was initially treated with 3 cycles of high-dose chemotherapy with mega-CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) without response, leading to the decision to undertake autologous hematopoietic stem cell transplantation. Partial remission of the disease was achieved with this treatment and subsequent introduction of oral bexarotene led to complete remission, which has been maintained for more than 3 years with good tolerance of oral therapy. We discuss the advantages and disadvantages of autologous hematopoietic stem cell transplantation and the use of oral bexarotene.

Poroma ecrino pigmentado / Pigmented Eccrine Poroma

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[Eruptive pseudoangiomatosis: study of 7 cases]. / Pseudoangiomatosis eruptiva: 7 casos clínicos.

INTRODUCTION: Eruptive pseudoangiomatosis consists in the acute development of small vascular lesions in the face and extremities that resolve in several weeks without scarring. Lesions are described as 3-4 mm asymptomatic macules and papules with peripheral whitish halo that blanch upon pressure. Initially it was considered a disease limited to children but it has also been described in adults. It overlaps with the entity known in Japan as <>, possibly caused by an insect named Culex pipiens pallens. METHODS: We report a serie of 7 patients that consulted for lesions compatible with eruptive pseudoangiomatosis. We performed a detailed clinical history and histological, microbiological and serological studies. Follow-up time was up to 4 years. RESULTS: Eighty-five percent of patients were women and the mean age was 62 years. All cases appeared in spring/summer and 71 % relapsed. Lesions predominated in the face and extremities and the outbreak lasted 2-4 weeks. The anamnesis did not disclose any specific etiologic agent in any of the cases. Complete laboratory tests including serologies and cultures were negative or within normal limits. Histological study revealed vascular dilatation in all cases with endothelial cell protrusion and a peripheral lymphohistiocytic infiltrate. CONCLUSIONS: Currently, the etiology of this entity is not well established although it probably represents a reactive disorder to different etiologic processes.

Pseudoangiomatosis eruptiva: 7 casos clínicos / Eruptive pseudoangiomatosis: study of 7 cases

Introducción. La pseudoangiomatosis eruptiva consiste en la aparición aguda de lesiones de aspecto angiomatoso de pequeño tamaño en cara y extremidades y su desaparición en el curso de varias semanas sin dejar cicatriz residual. Las lesiones se describen como máculo-pápulas de 3-4 mm, con un halo blanquecino periférico, asintomáticas y que blanquean a la vitropresión. En un principio se pensaba que era un cuadro limitado a niños, pero posteriormente se ha descrito también en adultos. Se considera superponible a un cuadro conocido en Japón como erythema punctatum Higuchi y posiblemente causado por un insecto denominado Culex pipiens pallens. Métodos. Presentamos una serie de 7 pacientes que consultaron en nuestro Servicio por un cuadro compatible con la pseudoangiomatosis eruptiva. Se les realizó una historia clínica detallada, estudio histológico, microbiológico y serológico. El seguimiento fue de hasta 4 años. Resultados. El 85 % de los pacientes fueron mujeres, y la edad media fue de 62 años. Todos los casos se iniciaron en los meses de primavera/verano y el 71 % sufrieron recidivas. Las localizaciones predominantes fueron la cara y las extremidades y la duración del brote fue de 2 a 4 semanas. La historia clínica no permitió establecer un agente desencadenante en ninguno de los casos. Las analíticas, estudios histológicos, serologías y cultivos estuvieron dentro de los rangos de normalidad. En las imágenes histológicas apreciamos en todos los casos dilatación vascular, con cierta protrusión de las células endoteliales hacia la luz del vaso y un infiltrado de predominio linfohistiocitario periférico. Conclusiones. Hoy en día la etiología de este cuadro clínico sigue sin estar bien establecida, aunque probablemente suponga una manifestación clínico-patológica reactiva a distintos procesos etiológicos

Introduction. Eruptive pseudoangiomatosis consists in the acute development of small vascular lesions in the face and extremities that resolve in several weeks without scarring. Lesions are described as 3-4 mm asymptomatic macules and papules with peripheral whitish halo that blanch upon pressure. Initially it was considered a disease limited to children but it has also been described in adults. It overlaps with the entity known in Japan as «erythema punctatum Higuchi», possibly caused by an insect named Culex pipiens pallens. Methods. We report a serie of 7 patients that consulted for lesions compatible with eruptive pseudoangiomatosis. We performed a detailed clinical history and histological, microbiological and serological studies. Follow-up time was up to 4 years. Results. Eighty-five percent of patients were women and the mean age was 62 years. All cases appeared in spring/summer and 71 % relapsed. Lesions predominated in the face and extremities and the outbreak lasted 2-4 weeks. The anamnesis did not disclose any specific etiologic agent in any of the cases. Complete laboratory tests including serologies and cultures were negative or within normal limits. Histological study revealed vascular dilatation in all cases with endothelial cell protrusion and a peripheral lymphohistiocytic infiltrate. Conclusions. Currently, the etiology of this entity is not well established although it probably represents a reactive disorder to different etiologic processes

Merkel cell carcinoma: a clinicopathological study of 11 cases.

OBJECTIVE: To report our 12-year experience with Merkel cell carcinomas (MCCs) from a clinical and pathological point of view. SUBJECTS AND SETTING: Eleven MCCs were diagnosed at our institution between 1991 and 2002. METHODS: A retrospective clinical, histopathological and immunohistochemical study was performed. Age, gender, location, size, stage, treatment and follow-up data were collected. Histopathological pattern and immunohistochemical study with CAM 5.2, cytokeratin 20 (CK20), CK7, Ber EP4, neurofilaments, synaptophysin, chromogranin, S100 protein, p53 protein, CD117, leucocyte common antigen (LCA) and Ki-67 were accomplished. RESULTS: Six females and five males with a mean age of 82 years were identified. Tumours were located on the face (n = 6), extremities (n = 3) and trunk (n = 1). At diagnosis, one patient was in stage Ia, six in stage Ib, three in stage II and one in stage III. All but one patient experienced wide surgical excision of the tumour. Additional treatment consisted of lymph node dissection in two patients, radiotherapy in four patients and systemic chemotherapy in one patient. Local recurrence developed in five patients. Three patients died because of MCC after 14 months of follow-up. Intermediate-size round cell proliferation was found in all cases. Additional small-size cell pattern and trabecular pattern were observed in seven and six cases, respectively. Eccrine and squamous cell differentiation were found in three cases. A dot-like paranuclear pattern was observed in all cases with CAM 5.2 and neurofilaments, and in 89% of cases with CK20. Seventy-five per cent of cases reacted with Ber EP4, chromogranin and synaptophysin, 70% with p53, 22% with S100 protein, 55% with CD117 and none with LCA. Ki-67 was found in 75% of tumoral cells on average. Fifty per cent of MCCs reacted with CK7 and showed eccrine differentiation areas. CONCLUSIONS: MCC is an aggressive neuroendocrine tumour of the elderly. Wide surgical excision is the recommended treatment. Lymph node dissection, adjuvant radiotherapy and chemotherapy decrease regional recurrences but have not been demonstrated to increase survival. Immunohistochemically, MCC is an epithelial tumour with neuroendocrine features.

Prichard's structures of the fossa ovalis are not histogenetically related to cardiac myxoma.

AIMS: Cardiac myxomas are neoplasms of unknown histogenesis. They are thought to arise from hypothetical subendothelial vasoformative reserve cells or from primitive cells which reside in the fossa ovalis and surrounding endocardium. In 1951 Prichard described a kind of microscopic endocardial structure with a predilection for the interatrial septum, which were suggested to be related to cardiac myxomas. To confirm the existence of Prichard's structures and to clarify their role in the genesis of cardiac myxomas, we examined histologically the fossa ovalis and we performed an immunohistochemical study of the endocardial abnormalities that were found. METHODS AND RESULTS: A prospective histological study of 100 interatrial septa and an immunohistochemical study of three out of the 12 endocardial abnormalities that were detected, as well as of four conventional cardiac myxomas were accomplished. Antibodies were used to vimentin, CD31, CD34, alpha-smooth muscle actin, S100 protein, thrombomodulin, calretinin and c-kit (CD117), a tyrosine kinase growth factor receptor for stem cell factor usually expressed by embryonic/fetal endothelium. Structures similar to the ones described by Prichard were found in 12% of septa, most of them in the left side of the fossa ovalis. The hearts with these structures were from patients 10 years older than the ones without them (72 +/- 10 versus 62 +/- 16 years, P=0.006). Immunohistochemically the cells comprising Prichard's structures were positive for vimentin, CD31, CD34 and thrombomodulin, and negative for alpha-smooth muscle actin, S100 protein, calretinin and c-kit. Therefore these cells seem to be mature endothelial cells, but not primitive multipotential mesenchymal cells. Furthermore, these cells were not found in the atrial tissue from the bases of any of the conventional cardiac myxomas. CONCLUSIONS: Our study suggests that there is no apparent relation between Prichard's structures and cardiac myxomas, and that Prichard's minute endocardial deformities are age-related phenomena.

Thrombomodulin, calretinin and c-kit (CD117) expression in cardiac myxoma.

The immunohistochemical profile of cardiac myxoma has been debated. The tumor is thought to be derived from multipotential undifferentiated mesenchymal cells. A consistent marker for this tumor has not been found. In this article an immunohistochemical study of 23 cardiac myxomas was accomplished. This study comprised the immunoreactivity of the tumors for thrombomodulin, calretinin and and c-kit (CD117). To the best of our knowledge, thrombomodulin and c-kit have not been tested in cardiac myxoma. Calretinin expression has been recently demonstrated in cardiac myxoma, although this finding has not been yet validated. Surface lining cells, tumor vascular endothelium, cells around the vascular slits and stromal cells embedded in the myxoid matrix were assessed independently. All tumors showed reactivity for thrombomodulin in the surface cells and in the endothelium of neoplastic vessels. 82.6% of cardiac myxomas expressed thrombomodulin in the stromal cells and 69.6% of the tumors were reactive in the perivascular cells. 73.9% of cardiac myxomas expressed calretinin in the stromal cells and in the perivascular cells. All myxomas were negative for c-kit. Thrombomodulin and calretinin may be important diagnostic aids for cardiac myxoma. Cardiac myxoma cells do not express embryonic/fetal endothelial antigens.

Papillary cystic tumor of the pancreas coexisting with hairy cell leukemia.

The coexistence of a pancreatic papillary cystic tumor with hairy cell leukemia is reported. To the best of our knowledge this association has never been published. A 41-year-old man diagnosed with hairy cell leukemia developed a second malignancy that corresponded to a papillary cystic pancreatic tumor. The patient underwent splenectomy and a tumoral surgical resection, and is currently well at 21 months follow-up. A pathogenetic relationship between the two malignancies was not demonstrated. Hairy cell leukemia has been reported to be associated to a great number of different second malignancies. In contrast, only two papillary cystic tumors of the pancreas have been described associated to a second neoplasm, a papillary thyroid carcinoma and a colonic carcinoma. This unusual benign or low-grade malignant pancreatic tumor more commonly occurs in the tail of the pancreas of young women. We want to stress the unusual presentation of this pancreatic tumor affecting the head of the gland in a male patient as well as its coexistence with a hairy cell leukemia.

Primary amyloid tumor (amyloidoma) of the jejunum with spheroid type of amyloid.

The present report documents an incidentally discovered, solitary amyloidoma of the jejunum in a 70 year old man with no predisposing disorder or other sites of deposition of amyloid, as was demonstrated at autopsy. Amyloid tumors of the intestine have been reported only rarely. In those reports the deposits were conventional in type, forming large masses of acellular, eosinophilic, homogeneous material. In the case described here the deposits were in the form of corpora amylacea-like structures, or spheroids. To the best of our knowledge, the corpora amylacea-like structures, or spheroid amyloid, have not been previously described in bowel amyloidoma. This rare form of amyloid deposition should be recognized so as to prevent misdiagnosis.

Solitary neurofibroma of the gallbladder. A case report and review of the literature.

Benign non-epithelial neoplasms of the gallbladder are rare. A case of incidental neurofibroma of the gallbladder in a 44-year-old man without neurofibromatosis 1 is reported. The intramural tumor, located in the neck of the gallbladder, was associated with lithiasis, chronic cholecystitis and localized fundic adenomyomatosis. Seven cases including the present one are reviewed. The seven patients ranged in age from 44 to 77 years, with an average age of 61.6 years. Four of the seven patients were women. Five tumors appeared as mural nodules, and two showed intraluminal growth. The most common location was the body of the gallbladder. The size of the lesion varied from 0.3 to 5.3 cm, with a mean size of 1.3 cm. Five of the seven cases were incidentally found in a gallbladder resected for cholelithiasis. Using ultrasonography, two cases with no lithiasis were detected preoperatively, presenting as intracavitary growing lesions by ultrasonography. It is possible that neurofibroma of the gallbladder may be more common than the small number of known cases indicates.