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Pheochromocytoma


Adrenal glands are triangular-shaped organs located on top of each kidney. They are primarily responsible for the release of hormones that have a stimulating effect on the heart, blood flow and dilation of arteries.

Each adrenal gland is divided into an outer cortex and an inner medulla. The adrenal cortex produces steroid hormones that maintain blood pressure and salt balance. The adrenal medulla is derived from nerve cells and is a source of catecholamines (adrenaline and noradrenaline), which are “stress hormones” that increase alertness, strength and speed in an emergency.

A pheochromocytoma is a tumor that occurs in the adrenal medulla, which produces catecholamines (adrenaline and noradrenaline), the so-called “stress hormones” that increase alertness, strength and speed in an emergency situation. In people with pheochromocytoma, the medulla produces excess catecholamines.

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Clinical trials
Ensayo de fase 1a/2a, abierto y multicéntrico, para investigar la seguridad, tolerabilidad y actividad antitumoral de dosis repetidas de Sym015, una mezcla de anticuerpos monoclonales dirigida frente al receptor MET, en pacientes con tumores malignos sólidos en fase avanzada
Estudio fase IIIB, prospectivo, randomizado, abierto que evalúa la eficacia y seguridad de Heparina/Edoxaban versus Dalteparina en tromboembolismo venoso asociado con cáncer.
Tumores sólidos. Antiemesis Estudio fase III, multicéntrico, aleatorizado, doble ciego, con control activo para evaluar la seguridad y eficacia de Rolapitant en la prevención de náuseas y vómitos por la quimioterapia (NVIQ) en pacientes que reciben quimioterapia altamente emética (QAE). A phase III, multicenter, randomized, double blind, placebo controlled study of the safety and efficacy of Rolapitant for the treatment of Chemotherapy-induced nausea and vomiting in subjects receiving highly Emetogenic Chemotherapy (HEC)
Ensayo clínico en fase I de determinación de dosis del antiangiogénico multidiana Dovitinib (TKI258) más paclitaxel en pacientes con tumores sólidos.

A pheochromocytoma may have no symptoms at all, or present with classic symptoms that resemble what one experiences in a “fight or flight” situation:

  • High blood pressure, either persistent or intermittent
  • Sweating
  • Headache
  • Rapid or irregular heartbeats
  • Feelings of anxiety, panic, fear
  • Pallor (paleness)
  • Dizziness/lightheadedness with standing
  • Tremor
  • Weight loss

Approximately 15% of pheochromocytomas occur as part of a genetic syndrome. Pheochromocytoma can be related to Multiple Endocrine Neoplasia type 2 (MEN2), von Hippel-Lindau Syndrome (VHL) and Neurofibromatosis type 1(NF1) and familial pheochromocytoma/ paraganglioma. Most pheochromocytomas are sporadic, meaning they have no known genetic basis.

Pheochromocytoma is usually treated with surgery, which may require removal of the entire affected adrenal gland. In some cases, the surgeon may be able to spare the cortex (outer layer) by removing only the tumorand the adrenal medulla. A laparoscopic approach using smaller incisions and miniature instruments may be possible with some carefully selected patients. If both adrenal glands are removed (bilateral pheochromocytoma), lifelong treatment with hormones is required. Screening for pheochromocytoma should be done prior to any elective surgery, pregnancy and childbirth.