Liponeurocitoma: un raro ejemplo de neoplasia cerebelosa / Liponeurocytoma: a unusual cerebellar tumour

Presentamos en nuestro trabajo, un caso de liponeurocitomacerebeloso, tumor poco frecuente, observado en unamujer de 62 años de edad. Dicho tumor mostró una imagenhistológica compatible con un tumor de células pequeñas,de escasa agresividad, positivo para marcadores neuronales(sinaptofisina y enolasa neuronal específica) con áreasfocales de diferenciación lipídica. Tras establecer un diagnósticodiferencial con otras neoplasias, comentamos brevementelas características de esta rara entidad(AU)

We report a case of cerebellar liponeurocytoma in a 62year old woman. Histologically, the tumour was of lowaggressiveness, composed of small cells with areas of lipidisedcells and was positive for neuronal markers (synaptophysinand neuron specific enolase). The principal featuresof this unusual entity are reviewed and the pathologicalaspects of its differential diagnosis are discussed(AU)

Traumatic neuroma of extrahepatic bile ducts after orthotopic liver transplantation.

Traumatic neuromas (TN) of the biliary tree causing strictures have only occasionally been described after liver transplantation. Herein, we have reported 15 cases of TN that were detected between 1 and 17 months after transplantation (median: 4 months) during surgery for obstructive jaundice (12 cases), after alterations of liver function tests (two cases), or incidentally discovered after retransplantation (n = 1) we resected the lesion and the biliary anastomosis. Pathological examination and immunostaining for S-100 protein were performed to study the nerve fascicles. After a median follow-up time of 64 months (range = 0-127), 10 patients are alive without any complication related to the previous biliary TN. We propose the following classification: type I: TN originating from and located in the main biliary tract wall, and type II: TN arising from the surrounding tissues next to the main biliary tract. We conclude that TN are not uncommon after liver transplantation and that they are sometimes symptomatic, causing a biliary stricture that requires surgical treatment. We propose a classification to help patient selection for surgery. In our opinion, resection of the TN is the operation of choice, together with resection of the involved biliary tract in type I TN.

Cirugía endovascular de la disección aguda tipo B / Endovascular surgery for acute type B dissection

Introducción. La disección aguda tipo B de la aorta torácica es una entidad compleja y grave, asociada a una elevada morbilidad y mortalidad. Desarrollo. Tradicionalmente, el tratamiento médico se ha preferido para las disecciones no complicadas, y se ha reservado la reparación quirúrgica para las que presentan complicaciones isquémicas o rotura. El tratamiento endovascular con endoprótesis ha aportado mayores esperanzas para los casos complicados, a la vez que se ha postulado su aplicación en casos asintomáticos. Estos aspectos se revisan y se discuten a fondo en el presente documento. Asimismo, se analizan aspectos técnicos del tratamiento endovascular pertenecientes al pre, peri y posprocedimiento. Conclusión. Por el momento, el uso de endoprótesis sólo está justificado en el tratamiento de disecciones agudas complicadas o inestables, o en las crónicas con expansión aneurismática

Introduction. Acute type B dissection of the thoracic aorta is a complex, severe condition associated to a high rate of morbidity and mortality. Development. Medical treatment has traditionally been preferred for non complicated dissections while surgical repair has been reserved for those with ischaemic complications or rupture. Endovascular treatment with a stent has brought greater hope for complicated cases, and its application in asymptomatic cases has also been suggested. These issues are reviewed and discussed at length in this article. Likewise, technical aspects of the endovascular treatment belonging to the pre-, peri- and post-operative periods are also analysed. Conclusions. For the time being, the use of stents is only justified in the treatment of complicated or unstable acute dissections, or in chronic cases with aneurysmal expansion

Remodelación aórtica en la disección tipo B crónica tras la reparación con stents recubiertos / Aortic wall remodeling of the chronic type B dissection after stent graft repair

Objetivo. Estudiar los cambios morfológicos de la pared aórtica en la disección crónica tipo B de Stanford tratada mediante stents recubiertos. Pacientes y métodos. Fueron tratados 17 pacientes (edad media: 61 años; rango: 35-70 años) por disección crónica tipo B. Se implantaron endoprótesis Talent (n = 11) y Excluder (n = 6); abordaje femoral (n = 15) y retroperitoneal (n = 2); control intraoperatorio mediante fluoroscopia y ecocardiografía transesofágica; cobertura intencionada de la arteria subclavia en cinco casos. Resultados. Éxito técnico con cierre de la puerta de entrada (17/17); tiempo quirúrgico: 73 ± 22 min (rango: 55-160 min); sin conversiones ni paraplejía; estancia postoperatoria media: 48 horas; trombosis de la falsa luz torácica completa (n = 13) y parcial (n = 4). El diámetro mínimo de la luz verdadera (LV) aumentó de 18,1 ± 5,4 a 27,9 ± 5,06 mm (p < 0,01). La luz falsa (LF) se redujo de 39,22 ± 18 a 26,6 ± 12,7 mm (p < 0,01). Seguimiento medio: 18 meses (rango: 1-58 meses); sin migraciones, fugas u otras complicaciones relacionadas con la endoprótesis; tres pacientes necesitaron a posteriori cobertura de la reentrada distal. Conclusiones. El cierre de la puerta de entrada mediante stents recubiertos suele inducir la trombosis en la LF torácica, ello conlleva una remodelación aórtica: estabilización del diámetro aórtico máximo, con cambios estadísticamente significativos del índice LV/LF (reducción de la LF, aumento de la LV). La aorta abdominal suele permanecer inalterada por persistencia de reentradas paraviscerales. Los resultados preliminares sugieren que el tratamiento endovascular de la disección crónica tipo B es una opción terapéutica factible y efectiva, con excelentes resultados a medio plazo

Aim. To study the morphologic evolution in the aortic wall of the chronic aortic type B dissection treated by means of stent-graft. Patients and methods. 17 patients (average age: 61 years; range 35-70 years) were treated by chronic aortic type B dissection. The endoprosthesis implanted were Talent (n = 11) and Excluder (n = 6); femoral access (n = 15) and iliac (n = 2); intraoperative transesophageal ecocardiography; intentionally left subclavian artery coverture in five patients. Results. Closure of the entry tear in all cases; surgical time: 73 ± 22 min (range: 55-160 min); no conversions, no neurological complications; median postoperative length of stay 48 hours; thrombosis of the thoracic false lumen (FL), complete (n = 13) and partial (n = 4). The minimum diameter of the true lumen (TL) increase from 18.1 ± 5.4 to 27.9 ± 5.06 mm (p < 0.01). FL reduction from 39.22 ± 18 o 26.6 ± 12.7 mm (p < 0.01). Mean follow up: 18 months; no related graft complications. In the follow up three patients needed to seal distal thoracic re-entry. Conclusions. The clossure of the entry tear usually induces the thrombosis of the thoracic FL. It promotes a remodelling of the aortic wall consistent in the stabilization of the maximum aortic diameter with significant changes of the index TL/FL (reduction of the LF and increase of the TL). The abdominal aorta usually remains unchanged by persistence of paravisceral reentries. The preliminary results suggest that stent-graft repair of the aortic dissection B type is a feasible and effective therapeutic option with excellent mid-term results

[Buttock mass and malignant sciatic nerve tumor]. / Masa glútea y tumor maligno del nervio ciático.

Malignant peripheral nerve sheath tumors (MPNST) are rare. We report a case of a MPNST of the proximal sciatic nerve in the thigh, unassociated with pre-existing type 1 neurofibromatosis or history of radiation therapy. A 71-year-old man had a 6 month history of constant, severe, burning pain affecting the sole of the left foot. One month after the onset, the pain radiated to the left calf, posterior aspect of the thigh and buttock, and distal leg weakness followed. Three months prior to admission, the patient developed a large and painful mass in the buttock, that occupied the entire left gluteal region on examination. There was severe weakness of ankle and toe dorsiflexion and plantarflexion, decreased sensation on the lateral and posterior aspect of the left leg as well as on the dorsal and plantar surfaces of the foot, and absent ankle jerk. EMG showed denervation and motor unit loss in the short head of biceps femoris and muscles supplied by tibial and peroneal nerves on the left side. Magnetic resonance imaging revealed a 10-cm enhancing mass of the left sciatic nerve from the upper thigh to the greater sciatic notch. In surgery, a large MPNST with a high Ki67 labeling index (> 60 %) was subtotally removed from the sciatic nerve, and adjuvant radiation therapy was administered. In the ensuing months the tumor invaded the entire pelvic region. A high sciatic malignant tumor can present with a rapidly growing buttock mass and unilateral, neuropathic foot pain imitating the tarsal tunnel syndrome.

[Cauda equina meningioma in a girl: presentation of a case and review of the literature]. / Meningioma de la cauda equina en una niña: presentación de un caso y revisión de la literatura.

Meningiomas are unusual in childhood, and the intraspinal location is very uncommon. Those arising from lumbar dura matter are the most rare among spinal neoplasms of meningeal origin. We present the case of a 12-years-old girl with a cauda equina meningioma. As in previously reported cases, the initial complain was back pain with radicular irradiation. The girl had few neurological findings, with pyramidal signs of both lower limbs as single neurological impairment. She underwent surgical treatment through an open door laminoplasty and the tumour was completely removed without neurological deficit. After 2-year of follow-up she remains asymptomatic.

Meningioma de la cauda equina en una niña: presentación de un caso y revisión de la literatura / Meningioma of the caudal equina in a child: case report and review of the literature

Los meningiomas son tumores raros en los niños, siendo especialmente inusual su localización raquídea y excepcional su origen en la duramadre de la teca lumbar. Presentamos el caso de una niña de 12 años con un meningioma de cauda equina. En concordancia con la literatura el síntoma inicial fue lumbalgia con irradiación radicular, siendo escasos los hallazgos de la exploración neurológica, que se limitaban a la presencia de signos piramidales en ambas extremidades inferiores. Fue intervenida mediante una laminoplastia en puerta abierta, consiguiéndose la resección completa de la tumoración sin déficit neurológico postoperatorio. La niña permanece asintomática dos años después de la cirugía (AU)

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Cardiac epithelioid angiosarcoma presenting as cutaneous metastases.

BACKGROUND: Cardiac angiosarcoma is a rare tumor that has a predilection for middle-aged males and a marked predominance in the right atrium. The tumor may present abruptly with a fulminant clinical course. Initial presentation with metastatic disease is rare. Only one case has been reported of a patient who presented with cutaneous metastases. METHODS AND RESULTS: We here report the case of a 51-year-old man who initially presented with cutaneous metastases in the absence of cardiac symptoms. The skin biopsy was diagnosed as metastatic undifferentiated sarcoma. The patient died 26 days later with widely disseminated disease. At autopsy a tumor arising in the wall of the left atrium and in the interatrial septum was found. After an immunohistochemical study including CD31 and CD34 stains the diagnosis of cardiac pure epithelioid angiosarcoma was made. To the best of our knowledge this tumor variant has not been documented in the heart until now. CONCLUSION: Although cardiac angiosarcoma is a rare neoplasm, its presence should be suspected in patients with cutaneous metastatic angiosarcoma without an evident source of the tumor, even in absence of cardiac symptoms.

Primary brain lymphoma presenting as Parkinson's disease.

Neoplasm is an uncommon cause of a parkinsonian syndrome. We report a woman with primary brain B-cell lymphoma presenting as Parkinson's disease. After 1 year of the illness, CT and MRI showed lesions without mass effect in the basal ganglia and corpus callosum. The patient did not respond to levodopa and right cerebellar and brain-stem signs appeared, which prompted further neuroimaging, showing an increase in size of the lesions and a right cerebellar and pontine mass. Stereotactic biopsy of the basal ganglia showed high-grade B-cell lymphoma. Despite the basal ganglia frequently being involved in lymphoma of the brain, presentation with typical or atypical parkinsonism is exceptional.

Perineurium contributes to axonal damage in acute inflammatory demyelinating polyneuropathy.

OBJECTIVE: To assess if axonal damage in severe acute inflammatory demyelinating polyneuropathy (AIDP) correlates with the appearance of epiperineurium in nerve trunks. BACKGROUND: Increase of endoneurial fluid pressure in nerve trunks possessing epiperineurium may be an important mechanism of axonal damage in AIDP. METHODS: A 79-year-old man had a 2-day history of acroparesthesias and ascending paralysis culminating in quadriplegia, bilateral facial palsy, and mechanical ventilation. Five intravenous immunoglobulin cycles were given without response. He died on day 60. Electrophysiologic studies (days 4, 17, and 50) initially showed normal nerve conduction velocities with further slowing, progressive attenuation of compound muscle action potentials, and profuse denervation. The authors studied the preforaminal anterior and posterior L3 and L5 spinal roots, third and fifth lumbar nerves and their branches, and femoral and sural nerves. RESULTS: Density of myelinated fibers was preserved in L5 ventral and dorsal roots and reduced in sural nerve. Mild de-remyelination was observed in lumbar roots. In both lumbar nerves and their branches, there were extensive de-remyelination and centrofascicular or wedge-shaped areas with marked loss of large myelinated fibers. Axonal degeneration was the predominant lesion in sural nerve. CONCLUSION: The presence of epiperineurium correlates with a drastic change of pathology with superimposed ischemic lesions and distally accentuated axonal loss, suggesting that endoneurial fluid pressure increase could cause axonal damage in AIDP.

Fatal familial insomnia: clinical, neuropathological, and genetic description of a Spanish family.

The clinical presentation and evolution, neuropathological findings, and genotyping of three members of a Spanish family affected with fatal familial insomnia are reported. The mother and two of her offspring developed a rapidly evolving disease with insomnia and behavioural disorders as the initial symptoms and died between 5 and 10 months after the onset of the illness. Frontal brain biopsy in the mother disclosed only non-significant spongiosis, and full neuropathological examination of her offspring showed thalamic and olivary degeneration with isolated focal cortical spongiosis. Genetic examination could only be performed in the contemporary patients and both harboured the prion protein (PrP) 178Asn mutation and homozygous 129 Met/Met genotype.

Loss of dopamine uptake sites and dopamine D2 receptors in striatonigral degeneration.

To explore the mechanisms underlying L-dopa response, we studied, by postmortem autoradiography, selective makers of dopamine presynaptic terminals, [3H]WIN 35428, and dopamine D2 receptors, [3H]nemonapride, in the putamen of four Parkinson's disease (PD) and one striatonigral degeneration (SND) neuropathologically confirmed brains as compared with six matched control brains. Dopamine uptake transporter was dramatically decreased (> 90%) both in PD and SND striatum. Dopamine D2 receptors were preserved in PD, but clearly reduced (> 76%) in the SND putamen. These data confirm that L-dopa response is closely associated with the preservation of striatal dopamine D2 receptors.

Biologic parameters that correlate with the prognosis of human gliomas.

Much clinical and biologic data have been processed in the search for useful objective parameters to predict brain tumor behavior. Seventy cases of astrocytic glioma collected by a single clinical team were studied using a full complement of clinical procedures: follow up (7 years), histologic analysis, DNA content estimation, and cell kinetics by flow cytometry. Proliferating cell nuclear antigen (PCNA) was determined by immunocytochemical-coupling flow cytometry (PFC) and also by counting under light microscopy (PIHC). A statistical evaluation was carried out to establish the usefulness of several parameters for glioma prognosis. The cases were histologically classified as 14 low-grade astrocytomas, 20 anaplastic astrocytomas, and 36 glioblastomas multiforme. The survival curve showed significant differences between histologic groups. Diploid populations were more frequent in low-grade astrocytomas, and aneuploid tumors often had increased S-phase and proliferative fractions. The PCNA-labeled index (PCNA-LI) increased with malignancy and correlated with histologic grading (P = 0.01). The PCNA-LI and age segregated low- from high-grade astrocytomas (including anaplastic astrocytoma and glioblastoma multiforme), but none of the variables considered differentiated anaplastic astrocytoma from glioblastoma multiforme. The Cox regression test displayed significant values for age, histologic diagnosis, and PCNA determinations when considered in tandem. Discriminant analysis obtained a function integrating age and specifically PIHC-LI to help in the prognosis of doubtful cases. The results emphasize the importance of parameters integrating different variables in an attempt to provide an accurate prognosis, the most significant being age, histopathologic diagnosis, and the proliferative fraction determined by PCNA.

5-HT1B receptor binding in degenerative movement disorders.

Using [3H]sumatriptan as a radioligand, 5-hydroxytryptamine (5-HT)1B receptors were examined in posterior striatum and midbrain post-mortem tissue sections of 12 patients who had died from representative degenerative movement disorders as compared to nine controls. In the control human basal ganglia, the highest densities of [3H]sumatriptan binding were observed in the globus pallidus and substantia nigra. No significant change in the density of [3H]sumatriptan binding sites was found in the striatum and substantia nigra of the six Parkinson's disease brains. In the two brains from patients with progressive supranuclear palsy an increase was found in the densities of [3H]sumatriptan binding sites, most marked in the substantia nigra. In contrast, [3H]sumatriptan labelling was almost absent in the striatonigral degeneration brain and was markedly reduced in the three Huntington's disease brains. This study indicates that the status of 5-HT1B receptors is different in each degenerative movement disorder and suggests that human 5-HT1B receptors are located somatodendritically on GABAergic and peptidergic caudate-putamen neurons which project to the substantia nigra and globus pallidus, where these receptors are presynaptic.

Fulminant Guillain-Barré syndrome with universal inexcitability of peripheral nerves: a clinicopathological study.

The pathological basis of nerve inexcitability in Guillain-Barré syndrome has not been established with certainty. We report the clinicopathological findings in a 67-year-old patient with fulminant Guillain-Barré syndrome who died 18 days after onset. Three serial electrophysiological studies revealed nerve inexcitability. Antibodies to Campylobacter jejuni were present but there was no antiganglioside reactivity. Spinal root sections revealed extensive and almost pure macrophage-associated demyelination with occasional presence of T lymphocytes and neutrophil leukocytes. Conversely, in femoral, median, and sural nerves the outstanding lesion was axonal degeneration, with some denuded axons remaining. Unmyelinated fibers, posterior root ganglia, and dorsal columns were preserved. Endoneurial postcapillary venules showed plump endothelial cells with loss of their tight junctions. We conclude that both primary demyelination and axonal degeneration secondary to inflammation account for nerve inexcitability. Our findings lend support to the hypothesis of increased endoneurial pressure as the cause of wallerian degeneration in nerve trunks.

[Osterosclerotic myeloma and polyneuropathy]. / Mieloma osteosclerótico y polineuropatía.

INTRODUCTION: The peripheral neuropathy is a common complication in patients with osteosclerotic myeloma. The association with hepatosplenomegaly, lymphadenopathy and variable endocrine disturbances and skin changes constitute the POEMS syndrome. The pathogenesis of this syndrome is unclear, but the presence of M protein and elevated levels of interleukin-6 in the serum of patients, suggests an immunologic mechanism. CASE: A 68 year old woman presented with a 2 year history of progressive weakness and paresthesias in both legs. Neurological examination revealed a sensorimotor polyneuropathy, involving predominantly the lower extremities. In addition to neurological findings, the patient had lymphadenopathy and skin hyperpigmentation. Serum protein immunoelectrophoresis revealed a peak of monoclonal IgA lambda paraprotein. Radiographic examination disclosed multiple osteosclerotic lesions. Electrophysiological evaluation showed a severe axonal and demyelinating sensorimotor polyneuropathy. Biopsy of sural nerve revealed demyelination and axonal degeneration. Axillary lymph node biopsy were consistent with giant lymph node hyperplasia. Interleukin-6 serum levels were normal. CONCLUSIONS: The POEMS syndrome or Crow-Fusake syndrome is a rare multisystem disorder often associated with osteosclerotic myeloma. A severe demyelinating sensorimotor polyneuropathy frequently constitutes the chief complaint. These patients have a polyneu-ropathy which resembles idiopathic chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and the electrophysi-ological studies are essential in the diagnosis. The pathogenesis has not been explained. Presumably, the plasma cells secrete an immunoglobulin or another substance that is toxic to peripheral nerves. Interleukin-6 is implicated in the pathogenesis of this disease.

Lectin histochemistry of ethylnitrosourea-induced oligodendrogliomas in the rat.

Oligodendrogliomas (n = 26) induced by ethylnitrosourea (ENU) in wistar rats were examined to assess the lectin specificity to oligodendroglial membranes. Two different types of oligodendrogliomas were found in our material: an isomorphous type (n = 12), and a polymorphous type (n = 14). The first one, with two variants according to its size, macro- (n = 9) and microtumors (n = 3), had predominantly a honey-comb pattern with 'clear halos' around the nuclei without anaplasia. The second type, composed mostly by macrotumours, was anaplastic, with high cellular density, necrosis and intratumoral hemorrhages. Peanut agglutinin (PNA) labelled plasma membranes of well-differentiated cellular components of the first group. The tumoral oligodendrocytes lost the property to bind PNA in the second group of tumours, while Concanavalin A (Con A) showed affinity to intracytoplasmic structures of these tumours. PNA is a reliable marker of oligodendroglial plasma membrane of well-differentiated ENU-induced oligodendrogliomas. This experimental model, using PNA and Con A, may have important clinical applications regarding the biological behaviour of this type of neoplasm.

Value of endothelin in cellular rejection after liver transplantation.

Endothelin-1 is a vasoconstrictor peptide released by the vascular endothelium by various chemical and mechanical factors. Cellular rejection is one of the most common complications following orthotopic liver transplantation, endotheliitis being its most specific and consistent histological feature. To assess the role of endothelin in cellular hepatic rejection, we studied 21 cirrhotic patients undergoing elective liver transplantation. These patients were divided in two groups: Group I comprised 10 patients without cellular rejection, and Group II comprised 11 patients with cellular rejection. Endothelin was measured on day 7 after transplantation, on the day of liver biopsy, and after treatment for rejection. We found a significant increase in the plasma endothelin levels in the early postoperative period in the patients with moderate or severe cellular rejection compared with the non-rejection group. It remained significantly elevated until the clinical diagnosis of rejection was made. This value returned to baseline with successful treatment. So, the monitorization of this peptide may be of help in the diagnosis of rejection, its severity, and the evaluation of its resolution.