[Neuronal tumors: gangliocytoma]. / Tumores neuronales: gangliocitoma.

INTRODUCTION: Gangliocytomas are neuronal tumors of the central nervous system. They tend to appear in children and young adults. These tumors usually appear in the supratentorial compartment in the temporal lobe. Their clinical presentation is frequently as refractory epilepsy. CASE REPORTS: Three gangliocytoma cases in different locations are presented and a review is made. CONCLUSIONS: Immunochemistry is of great value in the pathological study of these lesions, using neuronal markers for the diagnosis. They are usually benign lesions. Therefore, surgical complete removal is the goal to pursue.

Tumores neuronales: gangliocitoma / Neuronal tumors: gangliocytoma

Los gangliocitomas son tumores de estirpe neuronal que se presentan en individuos jóvenes y niños.Su localización más habitual es supratentorial (lóbulo temporal...). La presentación más habitual es en forma de epilepsia refractaria. Casos clínicos. Se presentan tres casos clínicos en diferentes localizaciones. Conclusiones. La anatomía patológica se ayuda de la inmunohistoquímica para enfocar el diagnóstico, utilizando marcadores neuronales. Son lesiones de naturaleza habitualmente benigna que requieren extirpación lo más completa posible

Gangliocytomas are neuronal tumors of the central nervous system. They tend to appear in childrenand young adults. These tumors usually appear in the supratentorial compartment in the temporal lobe. Their clinical presentation is frequently as refractory epilepsy. Case reports. Three gangliocytoma cases in different locations are presentedand a review is made. Conclusions. Immunochemistry is of great value in the pathological study of these lesions, using neuronal markers for the diagnosis. They are usually benign lesions. Therefore, surgical complete removal is the goal to pursue

[The medulloblastoma]. / El meduloblastoma.

INTRODUCTION AND DEVELOPMENT: Medulloblastoma is a cerebellar small cell tumor, whose ancestor cell has not been yet identified in the human normal embriology: its exact origin is, in fact, still unknown. Nevertheless, one of the most acceptable possibilities facing the origin of the tumor is the remaining rests of cerebellar outer granular sheet. It is a predominantly infantile tumor, less frequent in young adults, and World Health Organization (WHO) classification has assignated grade IV of malignancy. In this publication of the WHO, medulloblastomas have been subclassified into: classic, desmoplastic, medulloblastomas with extensive nodularity and advanced neuronal differentiation and large cell medulloblastomas. Real differences dealing with survival and prognosis amidst these subvarieties have been noted in extensive series. CONCLUSION: The most frequently genetic alteration is the presence of isochromosome 17q in most of 50% of the cases.

El meduloblastoma / The meduloblastoma

Introducción y desarrollo. El meduloblastoma es un tumorde células pequeñas de asiento cerebeloso, cuya célula progenitorano se ha identificado aún en la embriología humana. Se especulasobre su origen exacto; pero no obstante, una de las posibilidadeses que derive de remanentes de la capa granular externa cerebelosa.Es un tumor predominantemente infantil, menos frecuenteen adultos jóvenes, y la Organización Mundial de la Salud le asignael grado IV de malignidad. En la última clasificación de este organismose ha subclasificado en: meduloblastoma ‘clásico’, meduloblastomadesmoplásico, meduloblastoma con extensa nodularidad ymanifiesta diferenciación neuronal y, por último, el meduloblastomade células grandes. Entre estas subvariedades se han visto, endistintas y extensas series, diferencias en cuanto a pronóstico ysupervivencia. Conclusión. En los últimos años ha habido avancesen el conocimiento de sus alteraciones genéticas, y la demostracióndel isocromosoma 17q es la que se halla con más frecuencia (másdel 50% de los casos)

Introduction and development. Medulloblastoma is a cerebellar small cell tumor, whose ancestor cell has not beenyet identified in the human normal embriology: its exact origin is, in fact, still unknown. Nevertheless, one of the mostacceptable possibilities facing the origin of the tumor is the remaining rests of cerebellar outer granular sheet. It is apredominantly infantile tumor, less frequent in young adults, and World Health Organization (WHO) classification hasassignated grade IV of malignancy. In this publication of the WHO, medulloblastomas have been subclassified into: classic,desmoplastic, medulloblastomas with extensive nodularity and advanced neuronal differentiation and large cell medulloblastomas.Real differences dealing with survival and prognosis amidst these subvarieties have been noted in extensive series.Conclusion. The most frequently genetic alteration is the presence of isochromosome 17q in most of 50% of the cases

[Femoral osteolytic lesions with soft tissue tumors and hypercalcemia as presentation form of a B-cell lymphoma]. / Lesiones osteolíticas femorales con tumoración de partes blandas e hipercalcemia como forma de presentación de un linfoma de estirpe B.

Hypercalcemia associated with haematological neoplasms account for 15 to 20% of hipercalcemia in malignancy, and occurs usually in patients with multiple myeloma. However, its incidence in patients with linfoma is low, and it is observed usually in T-cell linfomas. Bone affectation is also uncommon in patients with non-Hodgkin linfoma. It usually is seen as a late manifestation of the disease, and its occurrence as the form of presentation is exceptional. We hereby report a patient with a B-cell non-Hodgkin linfoma presenting with hypercalcemia and femoral osteolytic lesions.

Lesiones osteolíticas femorales con tumoración de partes blandas e hipercalcemia como formade presentación de un linfoma de estirpe B / Femoral osteolytic lesions with hipercalcemia and soft tissue tumors as presentation of Non-hodgkin lymphoma

La hipercalcemia de los tumores hematológicos supone del 15 al 20 porciento de las hipercalcemias tumorales y ocurre sobre todo en pacientes con mieloma múltiple. Sin embargo, su incidencia en pacientes con linfoma es baja y se observa principalmente en linfomas de células T. Tampoco es habitual la afectación esquelética en los pacientes con linfoma no hodgkiniano. Suele ser una manifestación tardía de la enfermedad y constituye la forma de presentación sólo en raras ocasiones. Se describe el caso de un paciente con lesiones osteolíticas femorales e hipercalcemia como forma de presentación de un linfoma no hodgkiniano de estirpe B (AU)

[Jadassohn's nevus sebaceus and nevus unius lateris. Report of an observation with multiple dysplasia (author's transl)]. / Nevus sebáceo de Jadassohn y nevus unius lateris: a propósito de una observación con manifestaciones displásicas múltiples.

The case of a female patient with multiple ectodermal and mesodermal malformations present since birth is reported. The cutaneous lesions were of two types: Jadassohn's nevus sebaceus and nevus unius lateris. These entities have been described in the literature as congenital dermatologic alterations of nevoid character and organoid structure. They can be considered as congenital epidermal nevi. In many cases, including this one, there are various associated disorders especially of the nervous system, eyes, and skeleton. Both syndromes are cutaneous hamartomas which can be differentiated histologically but not by the anomalies accompanying them. Their dermatologic aspects are very similar. The histopathologic characteristics of the skin lesions of nevus unius lateris consist of hyperkeratosis, acanthosis, and epidermal papillomatosis. In Jadassohn's nevus sebaceus there are also alterations of the skin adnexa, namely the absence of hair follicles and the presence of numerous mature sebaceus and hyperplastic glands. In general, the presence of organoid nevus may be a sign of multiple ectodermal and mesodermal malformations. Both syndromes are often present in the same patient, as in the case described here, and their etiology is the same. It is based on an alteration in embryogenic development affecting primarily, though not exclusively, the formations of ectodermal origin. Thus Jadassohn's nevus sebaceus and nevus unius lateris are both forms of phacomatosis. Clinical cases have in common the cutaneous cited above, either in combination or singly. The other possible signs and symptoms are variable, depending on which stage of embryogenic development is affected. There may be defects in the structures of both ectodermal and mesodermal origin.