Síndrome del dedo azul como manifestación de feocromocitoma / Blue toe syndrome as a clinical finding of pheochromocytoma

Los feocromocitomas son tumores que proceden de las células cromafines del sistema nervioso simpático y actúan sintetizando y liberando catecolaminas. Suelen presentarse entre la cuarta y quinta década de la vida y tienen presentaciones clínicas muy diversas. Ocurren solamente en 0.1-0.2% de la población hipertensa, constituyen una causa tratable y curable de hipertensión arterial, así como de otras manifestaciones derivadas de la liberación incontrolada de catecolaminas. La isquemia arterial periférica secundaria a la liberación masiva de aminas por un feocromocitoma es muy infrecuente. Aquí se presenta un caso clínico de feocromocitoma manifestado como síndrome del dedo azul en un paciente con pulsos distales conservados y el antecedente de mal control tensional a pesar de tratamiento con dos fármacos.

Pheochromocytomas are tumors that arise from chromaffin cells of the sympathetic nervous system and act by synthesizing and releasing catecholamines. They usually occur between the fourth and fifth decade of life and have a very wide clinical presentation. They occur only in 0.1-0.2% of the hypertensive population and represent a treatable and curable cause of arterial hypertension, as well as other symptoms derived from the uncontrolled secretion of catecholamines. Peripheral arterial ischemia secondary to massive amines release by a pheochromocytoma is a very uncommon condition. Here we report a case of pheochromocytoma manifested as blue finger syndrome in a patient with palpable distal pulses and history of poor blood pressure control despite treatment with two drugs.

Primary malignant tumors of the adrenal glands

Malignancy must be considered in the management of adrenal lesions, including those incidentally identified on imaging studies. Adrenocortical carcinomas (ACCs) are rare tumors with an estimated annual incidence of 0.7-2 cases per year and a worldwide prevalence of 4-12 cases per million/year. However, a much higher incidence of these tumors (>15 times) has been demonstrated in south and southeastern Brazil. Most ACCs cause hypersecretion of steroids including glucocorticoids and androgens. ACC patients have a very poor prognosis with a 5-year overall survival (OS) below 30% in most series. Pheochromocytoma or paraganglioma (PPGL) is a metabolically active tumor originating from the chromaffin cells of the adrenal medulla. The incidence of PPGL is 0.2 to 0.9 cases per 100,000 individuals per year. Pheochromocytomas are present in approximately 4-7% of patients with adrenal incidentalomas. Classically, PPGL manifests as paroxysmal attacks of the following 4 symptoms: headaches, diaphoresis, palpitations, and severe hypertensive episodes. The diagnosis of malignant PPGL relies on the presence of local invasion or metastasis. In this review, we present the clinical and biochemical characteristics and pathogenesis of malignant primary lesions that affect the cortex and medulla of human adrenal glands.

Feocromocitoma quístico gigante: reporte de un caso / Pheochromocytoma giant cystic: a case report

El feocromocitoma quístico gigante es tumor adrenal raro en el que predomina el curso asintomático; por lo que muchos de los casos no son diagnosticados hasta el momento de la cirugía. La simple movilización del tumor se asocia con el paso a la sangre de grandes cantidades de catecolaminas y a una elevada morbimortalidad.; por esta razón la cirugía per se y su manejo perioperatorio constituyen un enorme desafío. En este artículo se presenta el caso de un feocromocitoma gigante maligno (35 cm) que ocupaba todo el hemiabdomen derecho. Aun con el diagnóstico preoperatorio de feocromocitoma, el bloqueo farmacológico preoperatorio y las medidas intraoperatorias, el paciente falleció poco antes de que finalizara la cirugía.

The giant cystic pheochromocytoma is a rare adrenal tumor in the predominantly asymptomatic course; so many cases are not diagnosed until the time of surgery. The simple mobilization of the tumor is associated with the passage to the blood of large amounts of catecholamines and high morbidity and mortality. So the surgery itself and perioperative management are a huge challenge. This article describes the case of a malignant giant pheochromocytoma (35 cm) which occupied the entire right abdomen. Even with the preoperative diagnosis of pheochromocytoma, pharmacological blockade preoperative and intraoperative measures, the patient died shortly before the end of surgery.

Miocardiopatía dilatada y feocromocitoma / Dilated myocardiopathy and pheochromocytoma

El feocromocitoma, un tumor de baja incidencia con un comportamiento benigno en la mayoría de los casos, puede ser el responsable de extensas manifestaciones cardiovasculares. Las manifestaciones sobre el miocardio pueden ser de profundas consecuencias, pues llegan a producir una miocardiopatía dilatada con insuficiencia cardiaca severa. La exéresis del tumor suele ser curativa en el 90 por ciento de los casos y el manejo perioperatorio muy complejo. Se presenta un paciente joven portador de un feocromocitoma, que sometido a la larga acción de las catecolaminas llegó a desarrollar una miocardiopatía dilatada con criterio de trasplante cardiaco. La cirugía adrenal fue curativa en nuestro paciente(AU)

Pheochromocytoma, a low incidence tumor of benign behavior in most of cases, can cause extensive cardiovascular manifestations. These manifestations on the myocardium can have deep consequences since they may lead to dilated myocardiopathy with severe heart failure. The excision of tumor may be the curative solution for 90 percent of cases but the perioperative management is very complex. This is the case of a young patient who carries pheochromocytoma and after a long-acting catecholemine-based treatment, he developed dilated myocardiopathy, and the medical criterion was that he required heart transplantation. The adrenal surgery was successfully healing in our patient(AU)

Adrenalectomía laparoscópica bilateral sincrónica en feocromocitoma bilateral / Bilateral laparoscopic synchronous adrenalectomy in bilateral pheochromocytoma: experience in 8 patients

Aim: To evaluate 8 cases of bilateral pheochromocytoma and laparoscopic synchronous treatment. Patients and Methods: From May 1999 to May 2010, 8 patients with bilateral pheochromocytoma were found. A complete preoperative workup was done. Results: We perform 8 transperitoneal laparoscopic bilateral synchronous adrenalectomy. There was no open converted procedure. Mean operative time was 184.37 min. (range 95 to 300 min). Mean hospital stay was 3.8 days (range 3 to 5 days). Mean blood loss was 191.25 ml (range 0 to 500 min). In 7 cases the histopathological result was of pheochromocytoma and 1 as a malignant pheochomocytoma based on its histology. Conclusions: With very well establish diagnostic disease, an appropriate anesthetic strategy and as surgical skills, laparoscopic bilateral synchronic adrenalectomy is a feasible and safe surgical technique for bilateral pheochromocytomas.

Introducción: La incidencia del feocromocitoma varía entre un 0,005 y un 0,1 por ciento y de estos sólo un 10 por ciento se presenta en forma bilateral. El objetivo de este trabajo es presentar nuestra experiencia en adrenalectomía laparoscópica bilateral sincrónica en pacientes con diagnóstico de feocromocitoma bilateral. Se revisa la literatura y se analizan nuestros resultados. Material y Métodos: Se realiza una revisión retrospectiva de todos los pacientes sometidos a una adrenelectomía laparoscópica bilateral sincrónica entre mayo de 1999 y mayo de 2010, con diagnóstico de un feocromocitoma bilateral. Todos los pacientes fueron estudiados con medición de catecolaminas plasmáticas y urinarias y estudio por imágenes. Resultados: Se realizaron 8 adrenelectomías laparoscópicas bilaterales sincrónicas. El tiempo promedio de cirugía fue de 184 min (rango 95-300 min). La estadía intrahospitalaria media fue de 3,8 días (rango de 3-5 días). El promedio de sangrado fue de 83 ml (rango 0-500 ml). El diagnóstico histopatológico fue de feocromocitoma en todas las muestras, en un caso se diagnosticó un feocromocitoma maligno. No hubo conversión a cirugía abierta. Conclusiones: La adrenelectomía laparoscópica bilateral sincrónica para feocromocitomas bilaterales es un procedimiento seguro y de baja morbilidad, realizada por un cirujano con experiencia en cirugía laparoscópica. Es necesario tener previamente el diagnóstico de certeza para el manejo anestésico y postquirúrgico adecuado para estos pacientes.

Incidentaloma en evaluación pretrasplante de donante vivo / Incidentalloma in evaluation of pre transplant from live donate

Los incidentalomas adrenales, oscilan alrededor del 2 por ciento-13 por ciento. Afecta a ambos sexos, con discreta preponderancia femenina, especialmente hacia el final de la tercera y cuarta décadas. La etiología incluye: tumores benignos y malignos de todas las zonas de la corteza y de médula suprarrenal, las metástasis a las glándulas suprarrenales, así como enfermedades infiltrativas. Paciente de sexo femenino, 47 años de edad, asintomática, sin antecedentes patológicos conocidos, en evaluación preoperatoria para trasplante renal donante vivo relacionado, la angiografía por tomografía evidencia tumoración en glándula suprarrenal izquierda. Se concluye evaluación pretrasplante sin ninguna contraindicación por lo cual se decide realizar adrenalectomía laparoscópica, biopsia extemporánea y posteriormente nefrectomía laparoscópica para trasplante renal donante vivo relacionado. Se extrae glándula suprarrenal izquierda de 4 cm x 3 cm x 2 cm de consistencia firme, al corte se observan áreas hemorrágicas y de necrosis. En vista de no ser concluyente el informe de la biopsia extemporánea se difiere nefrectomía. El resultado definitivo de la biopsia reportó feocromocitoma. El 5 por ciento de los incidentalomas son feocromocitomas y el 25 por ciento de los feocromocitomas son hallazgos de algún estudio. La clínica puede ser variada, y no guarda una clara relación con el tamaño, localización o aspecto histológico del tumor. Los síntomas más comunes son: cefalea, diaforesis y palpitaciones. También pueden tener poca expresividad clínica o ser asintomáticos, circunstancia que ocurre en un 20 por ciento de los casos aproximadamente.

The adrenal carcinoma accounts for 0.02. percent of cancers. In adrenal incidentalomas, the figures range around 2 percent-13 percent. It affects both sexes, with a slight female preponderance, especially towards to the end of the third and the fourth decades. The etiology of incidentalomas includes the benign and the malignant tumors of all parts of the cortex and medulla, the adrenal metastasis, and the infiltrative diseases. Describe a female patient, 47 years old, asymptomatic, with no known pathological conditions in preoperative evaluation for living-related donor kidney transplantation, evidenced angio tomography left adrenal gland tumor. Pre transplant evaluation is concluded with no contraindication for which it was decided to perform laparoscopic adrenelectomy, extemporaneous biopsy and then laparoscopic nephrectomy for livingrelated donor renal transplantation. The left adrenal gland is removed from 4 cm x 3 cm x 2 cm firm consistence, the court observed hemorrhagic areas and necrosis. In view of the report not be conclusive of extemporaneous biopsy is deferred nephrectomy. The final results of the biopsy reported pheochromocytoma. The 5 percent of the incidentalomas are pheochromocytomas and in 25 percent of pheochromocytomas are findings of a study. The clinic can be varied, and bear no clear relation to the size, location or histology of the tumor. The most common symptoms are: Headache, diaphoresis and palpitations. They may also have little clinical expression or be asymptomatic, a circumstance that occurs in 20 percent of all cases.

Giant cystic malignant pheochromocytoma invading right hepatic lobe: report on two cases / Feocromocitoma maligno cístico gigante com invasão do lobo hepático direito: relato de dois casos

CONTEXT: Cystic pheochromocytomas are uncommon neuroendocrine tumors that originate from the adrenal medulla. Differing from the more frequent solid pheochromocytomas, which produce catecholamines and present adrenergic syndrome, cystic pheochromocytomas may not produce these. Their symptoms are generally associated with an abdominal mass or even pain, particularly if the mass attains large dimensions. Similarly, radiological diagnosis may also be difficult. Right-side lesions may be confounded with cystic hepatic tumors or even retroperitoneal sarcomas with cystic areas, using radiological methods. Sometimes, there may be a preoperative diagnosis of malignancy. Invasion of organs in this region (i.e. liver or kidney), or even the presence of a large retroperitoneal mass (of uncertain origin) with which multiple organs are involved, may be indicative of malignant origin. CASE REPORT: Two cases of giant cystic pheochromocytoma that invaded the right hepatic lobe are described. These presented as abdominal masses. Both cases were malignant. They were treated by radical right nephrectomy plus right hepatectomy.

CONTEXTO: Feocromocitomas císticos são tumores neuroendócrinos raros oriundos da glândula adrenal. Ao contrário dos feocromocitomas sólidos (mais comuns), que usualmente produzem catecolaminas e se manifestam com uma síndrome adrenérgica, os feocromocitomas císticos podem não produzi-las. Por conseguinte, seus sintomas habituais estão relacionados à presença de massa abdominal ou dor, mormente quando atingem grandes dimensões. Devido a essa singularidade, o seu diagnóstico, inclusive radiológico, pode ser complexo. Lesões à direita podem ser confundidas com tumores hepáticos císticos ou mesmo sarcomas de retroperitônio com áreas císticas pelos exames de imagem. Invasão de órgãos contíguos (fígado, rim) ou mesmo massa retroperitoneal de grandes dimensões envolvendo múltiplos órgãos (sem uma origem identificada) podem ser indicativos de sua natureza maligna. RELATO DE CASOS:Os autores relatam dois casos de feocromocitoma cístico gigante com invasão do lobo hepático direito. Esses tumores apresentavam quadro clínico de massa abdominal. Ambos os casos eram de feocromocitomas malignos e foram tratados com nefrectomia radical direita e hepatectomia direita em monobloco.

Extremes of hyper and hypotensions in pheochromocytoma-a perioperative management

We report a case of 30 years old female who presented with history of frequent headaches, palpitation, sweating along with mass abdomen. Hypertension was accelerated as ECG reflected sinus rhythm with left ventricular hypertrophy and no papilloedema. Ultrasound abdomen revealed right sided, well defined solid mass posterolateral to the inferior vena cava. Urine analysis revealed raised metabolite vanillylmandalic acid. Patient was diagnosed as a case of pheochrojnocytoma of the right suprarenal gland. After control of blood pressure, laparotomy under general anaesthesia was performed. A big tumour was excised. Histopathology confirmed the diagnosis of pheochromocytoma

Feocromocitoma suprarrenal derecho: reporte de un caso y revisión de la literatura / Right adrenal pheochromocytoma: a case report and literature review

Presentar el caso clínico de una patología poco frecuente su manejo y su resolución quirúrgica, tratado en el Servico de Cirugía General IAHULA Mérida-Venezuela. Revisión de la literatura y descripción del caso clínico. El feocromocitoma es una enfermedad inusual y es causa de hipertensión arterial secundaria. Se presenta más frecuentemente como un tumor adrenal unilateral y en los mayores de 60 años. Las manifestaciones clínicas más comunes son cefalea paroxística, palpitaciones, diaforesis e hipertensión arterial paroxística o persistente. El diagnóstico se apoya en la clínica y en la determinación bioquímica, siendo el métdodo de elección la cuantificación de metanefrinas plasmáticas. Planteado el diagnósticio, es fundamental conocer la localización del tumor, lo que debe ser realizado mediante tomografía axial computada o resonancia nuclear magnética. El tratamiento consiste en el empleo de drogas antihipertensivas y la resección quirúrgica del tumor. Las drogas de elección son los antagonistas de los receptores alfa 2 adrenérgicos y/o bloqueadores de los canales de calcio, la resección puede ser realizada por laparotomía clásica como lo fue nuestro caso.

Extra-adrenal composite pheochromocytoma-ganglioneuroma

Composite tumors containing pheochromocytoma and ganglioneuroma are rare tumors typically arising in the adrenal glands. Very few were reported outside of the adrenals. We present the case of a middle-aged woman with a retroperitoneal mass that was discovered when she complained of local signs of compression and systemic signs of catecholamine hypersecretion. On pathology the mass was composed of large polygonal and pleomorphic cells arranged in nests characteristic of pheochromocytoma that were mixed with clusters of mature ganglion cells and bundles of spindle cells characteristic of ganglioneuroma. The histological features were benign, but due to its invasion of the inferior vena cava and of the right renal artery, we had concerns about the malignant potential of the pheochromocytoma component, which is extremely rare in these composite tumors

Pheochromocytoma of the organ of Zuckerkandl. Unusually small lesion detected with magnetic resonance imaging

A case report of a 40-year-old female with an unusually small pheochromocytoma of the organ of Zuckerkandl is discussed. The tumor is diagnosed by magnetic resonance imaging MRI examination, which was requested to evaluate her as a potential renal donor. There is a family history of Carney s triad gastric leiomyosarcoma, extra-adrenal pheochromocytoma and pulmonary chondroma. The MRI technique and findings are discussed

Asymptomatic giant pheochromocytoma.

An interesting case of pheochromocytoma is being presented which remained asymptomatic despite attaining a size of 16 x 11 cm. On intensive investigations, it was found to be a dopamine secreting tumour with certain features in favour of malignancy. The case was successfully operated and advised regular follow up.

Multicentric pheochromocytoma and involvement of the inferior vena cava

CONTEXT: Extension of pheochromocytomas to the inferior vena cava is rare. Multicentric tumors are rare as well, being present in up to 10 percent of cases. Surgery is the treatment of choice because of the long-term survival free of disease. DESIGN: Case report. CASE REPORT: We report on a case of right adrenal pheochromocytoma with extension to the supra-diaphragmatic vena cava, which underwent surgical excision through thoracophrenic laparotomy without the need for cardiopulmonary bypass. In a 6-year follow-up, another pheochromocytoma was found in the infra-renal Zuckerkandl's organ. Complete surgical excision of the tumor was performed by a median laparotomy and complete retroperitoneal dissection. In both cases, the total removal of the pheochromocytoma has been guaranteed by having margins free of tumor and a normal post-operative level of catecholamines. The pathological study revealed a malignant pheochromocytoma with margins free of neoplasia in both specimens

Feocromocitoma bilateral: cirugía laparoscópica en dos casos / Bilateral pheochromocytoma: laparoscopic adrenalectomy in 2 cases

Laparoscopic adrenalectomy, if done by skilled surgeons, is now the first choice for treating most adrenal tumors, including bilateral pheochromocytoma. We report two women, aged 35 and 34 years old, with bilateral adrenal pheochromocytoma successfully excised by laparoscopic surgery. Both had severe hypertension, high urinary catecholamine values (epinephrine + norepinephrine: 528 and 1083 ug/24h) and bilateral adrenal tumors at CT scan. After 4 weeks of doxazosin treatment, a laparoscopic transperitoneal adrenalectomy was done (Gugner's technique), with surgical times of 7 and 5 hours respectively. Both patients received hydrocortisone and only the second one required one unit of packed cells. Postoperative evolution was uneventful and both patients were discharged at the fifth postoperative day. At two months of follow up, both patients are asymptomatic and normotensive

Neoplasia endócrina múltipla tipo 2b: relato de três casos e revisão de literatura / Multiple endocrine neoplasm type 2b: report of three cases and review of the literature

Neoplasia endocrina múltipla tipo 2B (NEM-2B) é uma síndrome incomum, herdada de forma autossômica dominante e com penetrância variável. Múltiplos órgãos são envolvidos, mas com uma tríade predominante de carcinoma medular de tireóide, feocromocitoma e ganglioneuromas do trato gastrointestinal. A maioria dos pacientes tem fácies característica, composta por eversão e alargamento dos lábios e eversão das pálpebras por neuromas mucosos. Os autores relatam três casos de NEM-2B, sendo dois do sexo masculino, com idade média de 15,9 anos. A manifestação clínica inicial em todos os casos foi crescimento de massa cervical em topografia de tireóide e hábito marfanóide. Foram submetidos a tireoidectomia total, com esvaziamento cervical bilateral por carcinoma medular de tireóide. Um paciente apresentou feocromocitoma quatro anos após o diagnóstico de tumor de tireóide, sendo submetido a laparotomia com excisão do tumor. Um paciente evoluiu a óbito 2,5 anos após o diagnóstico e os outros dois encontram-se livres de doença, com uma média de acompanhamento ambulatorial de um ano, apesar do teste de calcitonina positivo em um dos casos.

Feocromocitoma y neurofibromatosis de von Recklinghausen: crisis postparto y trombosis de arteria renal / Pheochromocytoma and von Recklinghausen neurofibromatosis presenting with a crisis during the puerperal period: report of 1 case

Pheochromocytoma is observed with higher frequency in patients with von Recklinghausen neurofibromatosis. We report a 36 years old female with von Recklinghausen neurofibromatosis who developed mild hypertension during the fourth month of pregnancy. A cesarean section was performed at 37 weeks of pregnancy. Thereafter, the patient presented severe hypertensive and hypotensive crises, sinus tachycardia and fever. No evidences of an infectious disease were found. Abdominal ultrasound examination showed a right adrenal mass of 7 x 5 cm. High levels of urinary cathecolamines confirmed the diagnosis of pheochromocytoma. After three weeks of prazosin therapy, the patient was operated. During the surgical procedure, an encapsulated pheochromocytoma was found and excised. A right renal atrophy and renal artery thrombosis were also found and a nephrectomy was done. Postoperative evolution was uneventful and the patient remains with normal blood pressure levels six months after the operation

Insuficiencia autonómica en un caso de feocromocitoma con hipotensión postural / Pheochromocytome with autonomic failure and orthostatic hypotension

En un caso de feocromocitoma con crisis de hipertensión e hipotensión postural, el análisis espectral de la variabilidad de la frecuencia cardíaca indicó que en decúbito supino la actividad simpática y vagal estaba disminuída, con falta de incremento en la actividad simpática durante la bipedestación. Luego de la extirpación del tumor se observó un aumento marcado en la actividad simpática y vagal en reposo y una respuesta normal ante la bipedestación. La insuficiencia autonómica global, causal en parte de la hipotensión postural, podría deberse a inhibición de la liberación de noradrenalina y acetilcolina en las terminaciones nerviosas, resultante de la estimulación de los receptores alfa-adrenérgicos por las concentraciones elevadas de catecolaminas y de neuropéptido Y segregadas por el tumor

Hypertension secondary to adrenal tumors ten years experience

We have presented our ten years experience in the surgical management of adrenal tumors. Patients presenting with hypertension (9) are normotensive following the removal of the offending adrenal tumor. One patient had an extra-adrenal pheochromocytoma, one had a huge organized adrenal hematoma and one patient has a non-functional tumor. The diagnosis and management of endocrine tumors is a challenge to the clinician

Feocromocitoma: relato de um caso / Pheocromocytoma: report of a case

O feocromocitoma (FEO) é um tumor originário das células da medula da glândula supra-renal ou do sistema nervoso simpático liberador de catecolaminas. É causa incomum de hipertensäo, que pode ser curada com o tratamento cirúrgico do tumor. Neste trabalho, relatamos um caso desta patologia.

Neoplasia endócrina múltipla IIa e gestaçäo / Multiple endocrine neoplasia type IIa and pregnancy

Multiple endocrine neoplasia typella (Sipple Syndrome) is characterized by the presence of medullary thyroid carcinoma (MTC), pheochromocytoma and parathyroid hyperplasia. Although this is a rare tumor, its study is extremely relevant because its autosomal dominant type of inheritance requires familial screening. This is a report of a 35-year old pregnant woman with signs and symptoms involving arterial hypertyension, cephalea and palpitation. Her medical history included right adrenalectomy due to a phenochromocytoma and aspirative needle biopsy of thyroid sugesting MTC. In this pregnancy the patient received Prazosin and was later submitted to left adrenalectomy at 23 weeks of gestation. Histopathologic examination reveaaled a pheochromocytoma. The clinical picture became acute again stating on the 31 st week of gestation, requiring the use of Prazosin. Gestation was interrupted by cesarian section during yhe 34t week due to fetal sulfering. The infant's condition at birth was regular and recovery was good. During the late postpartum period, the patient was submited to toal thyroidectomy and left inferior parathyroidectomy. Histopathologic examination revealed MCT and parathyroid hyperplasia