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El Síndrome de Nelson es una enfermedad endocrina que consiste en la aparición de un tumor productor de ACTH en la hipófisis tras practicar una

adrenectomía bilateral.
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1 Etiología 2 Clínica 3 Tratamiento 4 Referencias

[editar]Etiología Al extirpar las glándulas suprarrenales1 (a causa de un síndrome de Cushing2 u otras causas) se crea una importante depleción decortisol en sangre, por lo que se elimina el mecanismo que inhibe las células productoras de ACTH de la hipófisis, que a su vez proliferan y crecen, llegando a formar un verdadero tumor productor de ACTH. Aunque son tumores benignos, su crecimiento es rápido y pueden alcanzar un gran tamaño. [editar]Clínica La gran hipersecreción de ACTH puede ocasionar intensa hiperpigmentación cutánea y mucosa. La clínica por compresión puede incluir cefalea, defectos campimétricos y, en ocasiones afectación, de pares craneales (en especial el II, III, IV y VI).3 4 [editar]Tratamiento

La primera opción terapéutica es la cirugía seguida de radioterapia. Si no es efectiva puede ofrecerse tratamiento médico paliativo con distintos fármacos.5

Nelson's syndrome
From Wikipedia, the free encyclopedia

Nelson's syndrome
Classification and external resources ICD-10 DiseasesDB eMedicine E24.1 8863 ped/1558

Nelson's syndrome is the rapid enlargement of a pituitary adenoma that occurs after the removal of both adrenal glands.[1]

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1 Pathophysiology 2 Presentation 3 Treatment 4 History 5 References

[edit]Pathophysiology

who first reported on the phenomenon in 1960. some will define Nelson's syndrome according to the classical description with an evolving pituitary mass. The risk can also be minimized by pituitary irradiation. [2] [edit]Treatment Pituitary surgery is performed in some cases. even in the absence of a clear pituitary mass lesion . Continued growth can cause mass effects due to physical compression of brain tissue. Of all of the features of Nelson's syndrome. Since that time there have been numerous similar case reports and series (2-8). Moreover. known as proopiomelanocortin (POMC). whereas historically patients presented with masses large enough to be found with CT. and the lack of cortisol's negative feedback can allow any preexisting pituitary adenoma to grow unchecked. Despite numerous studies and reports. This feature is especially pertinent given the increasing use in Cushing's disease of laparoscopic bilateral adrenal surgery as a highly effective treatment modality to control cortisol-excess (9. simple sella tomography.[3] HISTORY AND DEFINITION NELSON AND COLLEAGUES made the original description of a pituitary macroadenoma and high plasma ACTH. Removal of both adrenals eliminates production of cortisol. the one that causes most concern is the development of a locally aggressive pituitary tumour. in 1958 (1). may occasionally cause death from the tumour itself. whereas others will rely on increasing plasma ACTH levels. in a patient with Cushing's syndrome who had undergone bilateral adrenalectomy. there is no formal consensus of what defines Nelson's syndrome. Nelson's syndrome remains one of the major worries facing clinicians when considering managing patients with Cushing's disease by bilateral adrenalectomy. is an operation for Cushing's syndrome. or bilateral adrenalectomy. widespread access to reliable plasma ACTH assays has greatly facilitated monitoring of patients. or with neuroopthalmic complications (11). The accompanying increase in MSH is due to ACTH and MSH being derived from a common precursor peptide. which. [edit]Presentation The common signs and symptoms include muscle weakness and skin hyperpigmentation due to excess MSH. Thus. Nelson's syndrome is now rare because bilateral adrenalectomy is now only used in extreme circumstances. [edit]History The condition is named for the American endocrinologist Dr Don Nelson. along with increased production ofadrenocorticotrophic hormone (ACTH) and melanocyte stimulating hormone (MSH). unusually for pituitary disease. This is especially true in recent years since imaging with MRI allows precise documentation of any tumour progression.Removal of both adrenal glands.10).

the reduced feedback from adrenal cortisol secretion cannot be the only factor. It is unclear whether the adrenalectomy and the lowering of the cortisol feedback at the pituitary itself stimulates tumour growth. CLINICAL FEATURES Patients may present with the physical and hormonal consequences of an expanding pituitary mass and. especially if there are . numerous studies have attempted to identify factors that predict its onset. Deep cutaneous pigmentation is frequently present. PATHOPHYSIOLOGY Despite increased knowledge of the pathophysiology of corticotroph tumours. ranging from a few months to 24 years. or more. or whether Nelson's syndrome develops in tumours that were programmed to behave in an aggressive fashion from the outset. Given that not all patients with Cushing's disease develop Nelson's syndrome following bilateral adrenalectomy. and in the buccal mucosa and tongue. Pigmentation is generalised.11). INCIDENCE AND NATURAL HISTORY The reported incidence of Nelson's syndrome after bilateral adrenalectomy for Cushing's disease is 8–38% (11). and is due to ACTH stimulation of the melanocortin 1 receptors on melanocytes in the skin. little is known of the pathology of the corticotroph tumours that progress to Nelson's syndrome (13). emphasising the need for careful life-long followup (10. The presence of tumour either at surgery or on imaging has been shown to predict onset. the effects of ACTH hyper-secretion. but may also occur in cutaneous scars and sites of abrasion. Mass effects include compression of the optic apparatus and visual field defects. and hypopituitarism (11). These factors need to be borne in mind when considering the reports of Nelson's syndrome. and indeed it is likely that the tumours that show progression with clinical consequence are a sub-set with an aggressive phenotype. headache.on MRI (12). PREDICTIVE FEATURES Given the concern over the onset of Nelson's syndrome after adrenalectomy. or. This wide variation in incidence is as likely to be due to differences in definition as much as bias of case mix in different centres. external ophthalmoplegias. and it is likely that overall the modern 'Nelson's syndrome' represents a different disease entity from that of the last century. as there is great heterogeneity. There is also great variation in the latency of onset from adrenalectomy to the diagnosis of Nelson's syndrome.

Some of the difficulty in establishing the influence of these various factors is the heterogeneity of the series in question. MONITORING Whilst the latency of onset from the time of bilateral adrenalectomy to pituitary tumour progression is usually within 3 years (19).7. One area that has attracted considerable interest is whether pituitary radiotherapy either diminishes the chance of developing Nelson's syndrome. As indicated above measurement of plasma ACTH is essential in monitoring. 20h after the last dose of glucocorticoid and before the morning dose of glucocorticoid. with a median follow up of 4. but this is not a uniform finding in all (11). Young age at the time of adrenalectomy has been suggested to be predictive in some studies (14-17). those with Nelson's syndrome fail to show incomplete suppression. France (19). In this single centre series 53 patients treated by bilateral adrenalectomy for Cushing's disease were followed. Paris. and the improvements in recent years in pituitary imaging. measured at 0800h. not least of which because of the wide time-frame over which they have been variously reported. An even longer-term follow up of these data are needed to give further reassurance. but this suppresses rapidly 120 minutes after morning glucocorticoid (20. some of these areas of controversy have been addressed by a modern. The independent factors that predicted progression of corticotroph tumours included short duration of Cushing's disease and a high plasma ACTH. In contrast to other reports. In patients with Addison's disease plasma ACTH measured before morning glucocorticoid is frequently very elevated. Whilst no formal guidelines can be set out. was reported. but this was not an independent variable when these other two variables are taken into account. but again not in all. Interestingly. These are important data as they indicate that whilst corticotroph tumour progression on MRI is common. and a cut-point of 200 pg/ml appears to be useful to define Nelson's syndrome (12). All patients were assessed on follow up after adrenalectomy by clinical assessment. large and carefully reported series from Cochin Hospital. whereas other data suggest that prophylactic pituitary radiotherapy at the time of adrenalectomy reduces the risk of progression to Nelson's syndrome (12).aggressive characteristics or macroadenoma (7.16).21). and then yearly.5 years) there was evidence of tumour progression on MRI in 50% of the patients followed. since the patient is attending . Pituitary tumour on MRI at the time of adrenalectomy was also a factor predicting progression. 20h after the last dose of glucocorticoid. Thus. More recently. In the year following adrenalectomy an absolute increment of 100 pg/ml (22 pmol/l) was predictive of progression of the corticotroph tumour. as determined by pituitary MRI. The duration of Cushing's disease is predictive in some series (18). but in only one patient was there a complication related to the pituitary tumour itself: a short-lived oculomotor nerve palsy.12). As indicated above this may be done by measurement of the 0800h plasma ACTH. A potential drawback of this type of monitoring is that high plasma ACTH levels are found in patients with primary adrenal insufficiency without pituitary pathology.6 years (maximum follow up of 13. and it is crucial to continue to carefully assess for tumour progression life-long. or at least delays its onset: it has not been found to be of benefit in several series (6. In contrast. the clinical consequence of this appears to be less worrisome than in some older series. it is reasonable to perform pituitary MRI scans before adrenalectomy and then 6–12 months after adrenalectomy. at least with this duration of follow-up. progression may occur much later. anycorticotroph tumour progression. pituitary MRI and plasma ACTH. with an increment in the value compared to a previous value of 100 pg/ml being significant (19).

23). Medical There has been a long interest in medical therapy to attempt to control plasma ACTH and tumour growth. Over time radiotherapy lowers plasma ACTH levels. including diabetes insipidus (22). but at a cost of increased hypopituitarism. Unfortunately. There are only occasional responses found with dopamine agonists such as cabergoline (39-42). or failure to suppress plasma ACTH to less than 200 pg/ml 2 hours after morning glucocorticoid should prompt further assessment with imaging. and by inference tumour growth (45) (figure 1). Radiotherapy The use of fractionated external radiotherapy is well documented in the treatment of established Nelson's syndrome (24). Treatment Observation If there is MRI evidence of limited tumour progression but with no anatomical compromise. observation and repeat imaging (3–6 months) is an acceptable strategy. Given that the risk is of further tumour expansion an attempt at complete hypophysectomy has been recommended. some tumours may continue to expand. delivered by the gamma knife have been reported (25-27). Despite pituitary surgery some patients will have further progression alternative or adjuvant therapy is indicated (22. although the multi-ligand analogue SOM230 (pasireotide) may prove of benefit (38). Whilst radiotherapy may control tumour growth in many instances. Disappointing or variable results have been seen with sodium valproate (28-33). More recently. Such an assessment can be performed. and may also be used after fractionated radiotherapy. We have shown recently that even higher than licensed doses of rosiglitazone (12 mg/day) are not effective in reducing plasma ACTH levels.for assessment anyway. as long as the dose to the optic apparatus can be kept within safe limits. Surgical Pituitary surgery may be performed for corticotroph tumour progression if the anatomy is favourable for such an approach (22. In cases of a clear anatomical target this too is an effective modality of therapy. if needed. large series treated by stereotactic radiotherapy. One report showed that two out of three patients responded to rosiglitazone with lowering of plasma ACTH levels.23). apart from isolated case reports there is no medical therapy that has been shown to consistently achieve these goals. The orally active alkylating agent temozolomide has been shown to be highly effective in . but one of these subsequently escaped (43). and currently licensed somatostatin analogues (34-37). Recent interest has focused on the use of PPAR gamma agonists in Nelson's syndrome. on a more frequent basis than pituitary MRI. but is associated with increases in long-term hypopituitarism. and is associated with a good long-term control of local tumour growth and lowering of plasma ACTH levels. 20h after the last dose of glucocorticoid and before that morning's dose and then 120 minutes after the morning glucocorticoid may give more consistent results when monitoring for Nelson's syndrome: an increment in the pre-morning glucocorticoid plasma ACTH of 100 pg/ml compared to a previous measurement. Rosiglitazone at maximum licensed doses has not been found to be effective (44). measurement of plasma ACTH at 0800h.

This is likely to be especially true if the duration of Cushing's syndrome is relatively long and no tumour is visible on pituitary MRI before adrenalectomy. with modern follow-up including careful pituitary MRI and plasma ACTH. . as detected by MRI. can be expected in up to half of patients undergoing bilateral adrenalectomy for Cushing's disease. the actual numbers of patients that come to harm appears to be low. temozolomide could be considered in patients with Nelson's syndrome not responding to other modalities of therapy. Thus. Whilst this is a worrying statistic. and Nelson's syndrome should not be underestimated. and in many cases may be acceptable. One major drawback of this agent is high cost. Although there are no reports of its use in Nelson's syndrome. the risks associated with Nelson's syndrome need to be balanced against the risk of uncontrolled Cushing's disease.a few aggressive pituitary tumours and in lowering prolactin and controlling tumour growth in highly aggressive prolactinomas (46-48). CONCLUSIONS Progression of corticotroph tumours.

Nelson’s syndrome is the name given to the enlargement of a pituitary gland tumour associated with excess secretion of adrenocorticotropic hormone following the surgical removal of both adrenal glands. It can also cause an increase in skin pigmentation. Large tumours can sometimes also invade areas around the pituitary gland which may cause a reduction in levels of other hormones. usually to treat Cushing’s disease. The syndrome is the name given to the effects of the enlarging tumour in the pituitary gland. What is Nelson’s syndrome? Nelson’s syndrome is the term used to describe an enlargement of an adrenocorticotropic hormone -producing tumour in the pituitary gland. following surgical removal of both adrenal glands in a patient with Cushing’s disease. The tumour produces an excess of a hormone called adrenocorticotropic hormone which is involved in the production of other steroid hormones (such as cortisol). In healthy individuals. These steroid hormones then act on the pituitary gland to stop it producing more adrenocorticotropic hormone. so patients may look as if they have a suntan. the pituitary gland stimulates the adrenal glands to produce steroid hormones (such as cortisol) through production of the hormone called adrenocorticotropic hormone. What causes Nelson’s syndrome? Nelson’s syndrome results from a reduction in feedback of steroid hormones and is a consequence of removal of the adrenal glands (an adrenalectomy). This feedback system . visual problems or it may affect nerves and blood vessels.

complete blindness. This results in darkened or tanned skin. a lack of growth hormone (growth hormone deficiency) and deficiencies of the gonadotrophic hormones. This can result in difficulty moving the eyes. double vision or numbness on the face.means that levels of the hormones are kept within the normal range. The tumour may grow to each side of the pituitary gland and affect important nerves that are found in this area. especially in skin creases. this negative feedback system does not work and the pituitary tumour may expand and produce more adrenocorticotropic hormone. This may result in blind spots in the vision or. occasionally. In patients who have had an adrenalectomy. The tanned complexion is not confined to areas exposed to the sun and frequently affects the inside of the mouth and old scars. . This can result in poor functioning of thethyroid gland (hypothyroidism). luteinising hormone and follicle stimulating hormone. The tumour can also grow upwards from the pituitary gland and compress vital nerves involved in vision. Occasionally patients will also experience a headache as a result of the enlarging tumour. What are the signs and symptoms of Nelson’s syndrome? The signs and symptoms of Nelson’s syndrome result from the enlarged tumour and the increased adrenocorticotropic hormone production. The enlarged tumour compresses the rest of the pituitary gland and therefore reduces the amount of other hormones produced. The raised levels of adrenocorticotropic hormone act on cells in the skin to increase pigmentation.

Frequently these tests will be performed as an outpatient. A scan of the pituitary gland will be organised to assess the growth of the tumour and to look for effects on other areas in the brain around the pituitary gland. Nelson’s syndrome develops in 20% to 40% of cases. Most cases of Cushing’s disease are not inherited. rarely. these tumours can be part of a genetic condition such as multiple endocrine neoplasia. in patients who do require this operation. visual disturbance. but. but occasionally the doctor may advise an admission to hospital if treatment is required urgently. The doctor will request blood tests to look for a high adrenocorticotropic hormone level along with possible low levels of other pituitary hormones. Often a visual field test will be organised to check for loss of vision. skin pigmentation or other symptoms resulting from pituitary hormone deficiencies. However.How common is Nelson’s syndrome? Surgical removal of both adrenal glands to treat Cushing’s disease is rarely performed nowadays due to improvements in other treatment options for this condition. How is Nelson’s syndrome diagnosed? Nelson’s syndrome is suspected in patients who have previously undergone surgical removal of the adrenal glands when they develop symptoms suggestive of pituitary enlargement such as headache. Is Nelson’s syndrome inherited? Nelson's syndrome only occurs in patients with Cushing’s disease if they have had both adrenal glands removed. .

but these will be treated if symptoms persist. although a surgeon will go through the particular risks of the operation with a patient. growth hormone replacement and testosterone oroestrogen replacement. This may involve thyroid hormone replacement. and this is usually the case if there is visual impairment or other evidence of significant growth of the tumour outside the pituitary gland. especially if surgery is not felt to be safe. Medications that are currently used to induce tumour shrinkage include dopamine agonists and these can be associated with headaches. Occasionally. dizziness and gastrointestinal symptoms such as constipation.How is Nelson’s syndrome treated? Nelson’s syndrome is most frequently treated by surgical removal of the pituitary tumour. Most patients with Nelson’s syndrome will already be taking steroid replacement therapy. Are there any side-effects to the treatment? Surgery is generally safe and well tolerated. medical treatment aimed at shrinking the tumour may be suggested and radiotherapy may also be used. Hormone deficiencies may resolve after removal of the tumour. Radiotherapy can be associated with damage to areas around the pituitary gland. . The risks and benefits of this treatment will need to be discussed with a specialist in radiation treatment.

and therefore patients underwent removal of the adrenal glands in order to lower the blood levels of cortisol. It is very important that any hormone deficiencies are diagnosed and treated properly so as to avoid long-term complications such as osteoporosis. a radiotherapist. the tumor enlarged and excessive production of ACTH . and this is one of the reasons for long-term follow-up of patients who have had surgical removal of their adrenal glands.  In Cushing’s disease.What are the longer-term implications of Nelson’s syndrome? The successful treatment of Nelson’s syndrome depends on the involvement of specialists including an endocrinologist. ACTH signals the adrenal gland to produce cortisol. blood cortisol is elevated due to excessive secretion of the hormone ACTH by a pituitary adenoma. Because the pituitary tumor was not removed. The outcome is good if the condition is detected early. In the past. heart disease and depression Nelson's Syndrome General Information  Nelson’s syndrome is an iatrogenic condition (caused by a physician) that occurs as a result of treating Cushing's diseasepatients by removal of both adrenal glands. the identification of a pituitary adenoma was more difficult. a specialist pituitary surgeon and occasionally.

We work in close cooperation with your surgeon or medical doctor . Radiotherapy.continued. The Neuro-ICU cares for patients with all types of neurosurgical and neurological injuries. in excessive amounts. controls tumor growth in the majority of patients with residual tumor growth after surgery. patients may develop large tumors resulting in visual loss. Hormonal tests generally show elevated ACTH levels in the blood but low or normal levels of cortisol. can stimulate pigment production in the skin. The hormone ACTH. including stroke. Because ACTH-secreting tumors are typically aggressive and invasive. Nelson’ syndrome develops within one to four years of surgery in about 15 percent to 25 percent of patients. trauma and tumors. Symptoms  A key finding is darkening of skin color and increased pigmentation of nevi. Magnetic Resonance Imaging (MRI) or computed tomography (CT) scan is important for the identification of a pituitary adenoma. preferably with stereotactic radiation. brain hemorrhage.  Diagnosis  The clinical history and skin pigmentation findings are usually obvious.   Treatment  Transsphenoidal surgery is the primary option but not always effective. pituitary failure and headache.

with whom you have had initial contact. Este es un derivado del péptido precursor de ACTH que es derived. que causa el síndrome de Nelson. los niveles de cortisol bajan a la normalidad y la aumenta la producción de CRH. The NeuroICU team consists of the bedside nurses. physicians in specialty training (Fellows) and attending physicians. El nivel alfa de hormona estimulante de melanocitos (a-MSH) es también muy alto. Mutaciones reguladoras de genes y las mutaciones en el receptor de glucocorticoides también pueden desempeñar un papel. La mayoría de los casos ocurren en pacientes con síndrome de Cushing que tienen una hormona adrenocorticotropa (ACTH) macroadenoma hipofisario secretor. Tales pacientes tienen niveles elevados de cortisol . El mecanismo de retroalimentación cortisol normal del hipotálamo-pituitario-adrenal (HPA) El eje está perturbado por lo tanto. the NeuroICU attending physician and team members direct your family member's care while in the ICU. nurse practioners.The signos y síntomas se derivan de la presión del tumor en las estructuras circundantes y la pérdida secundaria de otras hormonas pituitarias. Together with the surgeon or medical doctor. que suprimen la producción de hormona liberadora de corticotropina (CRH) de la glándula pituitaria. con pérdida del ritmo circadiano y la producción excesiva de cortisol. Fisiopatología [ 1 ] Esto puede considerarse como la enfermedad iatrogénica. . [ 2 ] Una vez que el paciente ha tenido una bilateral adrenalectomía . compresión por el tumor puede inhibir la liberación de otras hormonas pituitarias. Esto a su vez da lugar a veces en el crecimiento descontrolado de la adenoma pituitario .

Los factores de riesgo El embarazo es un factor predisponente. Presentación [ 1 ] Historia   En por lo menos 99% de los casos existe una historia de la adrenalectomía. . Las tasas de los estudios posteriores varían desde 8 hasta 44%. Incluso en las primeras series. pero todavía hay un lugar definido para suprarrenalectomía.Epidemiología [ 1 ] El síndrome de Nelson es una enfermedad rara que parece estar disminuyendo aún más. ya que requieren un tumor lo suficientemente grande como para obstruir el flujo del líquido cefalorraquídeo (LCR). Cirugía de la hipófisis se utiliza más a menudo. sólo el 2040% de los pacientes con un adenoma hipofisario que tenía adrenalectomía bilateral desarrollado el síndrome de Nelson. tumores productores de ACTH son más comunes en mujeres jóvenes o de mediana edad y por lo tanto son el grupo con mayor riesgo de síndrome de Nelson. Los dolores de cabeza son comunes con los tumores hipofisarios y son probablemente el resultado del estiramiento de la sellae diaphragma.Esto puede estar relacionado con una mejor gestión de síndrome de Cushing y adenomas pituitarios. Características del aumento de la presión intracraneal ocurren de forma tardía y son poco frecuentes.

El síndrome de Cushing con frecuencia retrasa el crecimiento en los niños. A menudo deficiencia de la hormona es incompleta. pero la hiperprolactinemia suele causar disfunción eréctil . la presencia degalactorrea y poliuria y polidipsia. o o o o o o  Hipopituitarismo se produce cuando el sistema portal hipotálamopituitaria es interrumpido o tejido pituitario normal es destruido por el tumor: Puede ser parcial en lugar de total. Si no. La hiperpigmentación suele ser evidente. En las mujeres la amenorrea puede ser el primer signo de la enfermedad de la hipófisis. En niños y adolescentes. preguntar por los síntomas de hipotiroidismo . pero la operación debe devolverlo. Los dos últimos se deben a la diabetes insípida . Una linea nigra es a menudo aparente. tenga en cuenta la altura y el peso. el crecimiento de la nota y la edad de la pubertad. Esta es una línea oscura desde el pubis hasta el Examen   . Galactorrea es poco común en los hombres. La hipófisis anterior es más complicado que a menudo la hipófisis posterior. En todos los pacientes. se requiere una investigación. En niños y adolescentes. Defectos del campo visual debe ser consultado acerca. El tumor también puede causar diplopía y lesiones craneales con la participación de la oculomotor. troclear y abducens nervios y también la rama oftálmica del trigémino. Pueden ser demasiado insidioso que se ha dado cuenta y la prueba formal puede ser requerida.

Las cicatrices y areolas se pigmentan y. Compruebe el fondo de ojo. El hipotiroidismo puede causar bradicardia . pero la hipertensión es común si los niveles son elevados. Investigaciones [ 1 ]  . [ 3 ] En los adolescentes puede haber características de retraso de la pubertad . IV y VI pares craneales están implicados. ACTH serán muy marcadamente elevados. la pigmentación es más marcada en los pliegues de las manos. El daño a la rama oftálmica del nervio trigémino perjudicará sensación sobre la frente y tal vez reflejo corneal. el examen del pulso puede mostrar para ser rápido pero filiforme. si el reemplazo suprarrenal es insuficiente. [ 4 ] La respuesta de la ACTH a la CRH también se ha mejorado pero esto no es necesario para el diagnóstico. pero no desarrollan síndrome completo de Nelson. Algunos pacientes desarrollan hiperpigmentación después de adrenalectomía bilateral. incluyendo la búsqueda de edema de papila . En las mujeres adultas puede haber alguna regresión de los senos o galactorrea. Compruebe los movimientos oculares como los músculos oculares externos se verán afectados si los nervios III. pero. La presión arterial es baja con insuficiencia esteroides. Un estudio encontró que una concentración de ACTH en plasma por encima de 154 pmol / L se produjo sólo en los sujetos con síndrome de Nelson. como en la enfermedad de Addison .    ombligo.

puede estar asociada con hepatomegalia y esplenomegalia posiblemente). Hipoplasia suprarrenal. Diagnóstico diferencial [ 1 ]        Gestión [ 1 ] . sin embargo. Craneofaringioma . Prolactina bien puede ser elevado pero no tan alta como en un tumor productor de prolactina. La experiencia es. se requiere en la interpretación. entonces las pruebas para la diabetes insípida deben llevarse a cabo. Terapia con glucocorticoides y el síndrome de Cushing. la hormona del crecimiento se debe medir. [ 5 ] Perimetría formal es necesaria para los campos visuales. Otras causas de la pigmentación de la piel .      Thyroxine niveles puede ser baja y hormona estimulante del tiroides (TSH) también será baja. El hipopituitarismo. si es menor que 1010. Congénita hiperplasia suprarrenal . La insuficiencia suprarrenal . MRI es útil para la detección y para controlar la progresión de un microadenoma. Orina de la mañana temprana puede hacerse la prueba de la gravedad específica y. Una resonancia magnética con gadolinio de la región pituitaria y produce imágenes excelentes paraselar. Las gonadotropinas y hormonas sexuales puede ser baja. En los niños.por ejemplo ictericia (también afecta a la esclerótica) y hemocromatosis (más de un color de bronce.

radiocirugía estereotáctica parece muy prometedor. Transesfenoidal cirugía da el menor riesgo de lesión del hipotálamo . a pesar de que son más eficaces para controlar los niveles de ACTH que reducir el tamaño del tumor. Las técnicas modernas con aceleradores lineales de alta energía producen menos dispersión de radiación y el daño por lo menos garantía.ciproheptadina con y sin bromocriptina y valproato de sodio también se han utilizado con diversos grados de éxito. [ 8 ] evaluación a largo plazo de la función pituitaria es necesario con la terapia de reemplazo hormonal como sea . como la óptica aparato. cabergolina (un antagonista del receptor de la dopamina) ha sido utilizado con éxito para inducir la remisión en el síndrome de Nelson con disminución de los niveles de ACTH y resolución de los microadenomas o macroadenomas. Esto ha facilitado el tratamiento médico temprano y evita el uso de la cirugía en algunos casos. sobre todo si se presiona sobre las estructuras circundantes. La radioterapia se asocia con graves problemas a largo plazo. la irradiación adyuvante reduce la tasa de recurrencia y mejora el pronóstico. Cirugía aún ofrece la mejor probabilidad de cura para los tumores que son grandes. [ 6 ] La radioterapia puede ser la opción preferida para un adenoma invasivo que muestra la progresión. Un estudio informó el uso exitoso de la temozolomidadespués de el fracaso del tratamiento con cirugía y radioterapia. mientras que todavía están en la etapa de microadenoma. [ 7 ] El uso de un bisturí de rayos gamma para enfocar el haz de radioterapia ofrece un mayor refinamiento. daño visual y el riesgo de tumores secundarios. incluidas las dificultades de aprendizaje y memoria. El tratamiento con protones en vez de fotones (rayos X) ha mejorado la eficacia y se ha efectuado una cura en algunos pacientes en los que la cirugía no ha tenido éxito. Octreotide (a análogo de la somatostatina administrada parenteralmente). Radioterapia fraccionada de haz externo o radiocirugía estereotáctica puede ser utilizado dependiendo del tamaño y ubicación del tumor.El uso de la resonancia magnética ha permitido la detección de tumores. Si el retiro es incompleta o cuando hay invasión.

Los síntomas visuales o signos dependen de donde el tumor presiona. Cambio maligno en tumores productores de ACTH es raro. las células corticales suprarrenales pueden migrar a lo largo de la línea de descendencia gonadal e incluso puede ser secuestrado en el hilo de los testículos. produciendo tejido adrenal resto. Estos incluyen el oculomotor. especialmente si el tumor era grande. En el síndrome de Nelson. Superior extensión del tumor puede comprimir o invadir el aparato óptico o el hipotálamo. La extensión lateral del tumor puede invadir los senos cavernosos con atrapamiento o compresión de los nervios craneales que lo atraviesan.necesario. Durante la embriogénesis. este . troclear y abducens nervios y la división oftálmica del trigémino. Complicaciones [ 1 ]     Hipopituitarismo es muy común después del tratamiento. La presión arterial también deben ser monitoreados.

que puede causar el agrandamiento doloroso testicular y oligospermia. La coordinación entre los cirujanos y radioterapeutas es importante. el tejido resto suprarrenal puede producir suficiente cortisol para producir niveles normales o incluso causar la recurrencia del síndrome de Cushing. Cirugía pituitaria anterior. pero el seguimiento debe incluir la conciencia del síndrome de Nelson. A nivel de ACTH alta un año después de la adrenalectomía se piensa que es predictivo de la progresión tumoral corticotropos. [ 2 ] Irradiación de la hipófisis de rutina después de la adrenalectomía bilateral ya no se recomienda. incluso si no tiene éxito. La información epidemiológica es escasa. es vital. siempre y cuando haya un reconocimiento temprano. con un adecuado monitoreo y reemplazo de hormonas. [ 10 ] [ 11 ] Control de nivel de ACTH pituitaria y la RM se recomienda 3-6 meses después de la cirugía y después con regularidad. Postoperatorio postratamiento. parece proteger contra la . El pronóstico [ 1 ] El pronóstico es bueno. En raras ocasiones. pero los datos disponibles sugieren que la incidencia está cayendo y que los tratamientos modernos están ayudando a reducir la morbilidad y la mortalidad. si en los testículos. [ 2 ] Un estudio realizado en Italia encontró que la incidencia de síndrome de Nelson se relacionó significativamente con el nivel de cortisol en orina pretratamiento y la presencia de adenoma hipofisario en la cirugía pituitaria anterior.tejido adrenal resto puede llegar a ser estimulado y. [ 1 ] [ 9 ] Prevención    Adrenalectomía bilateral se dice que tienen un riesgo aceptable.

recaída.com'. [ 12 ] Historia Aunque el síndrome de Cushing se remonta al papel Harvey Cushing en 1932. [ 13 ] el síndrome de Nelson se remonta sólo a 1960 [ 14 ] y el autor no ha logrado llegar a las páginas de la web de la 'whonamedit. Los hallazgos sugieren la importancia de la presentación clínica de los pacientes y subgrupos indican que están en alto riesgo de recaída después de la cirugía pituitaria o la irradiación y para el desarrollo de síndrome de Nelson después de una adrenalectomía bilateral. . Los resultados indican que las recaídas después de un curado por cirugía pituitaria o irradiación es un problema clínico considerable que aumenta con el tiempo.