A pheochromocytoma is a rare, usually noncancerous (benign) tumour that forms in the centre of one or both of the adrenal glands (adrenal medulla). The adrenal glands form an integral part of the body's hormone-producing (endocrine) system which also includes the parathyroid glands, pituitary gland, thyroid gland, pancreas, ovaries (in females) and testicles (in males). The tumour primarily ensues in a type of cell called a chromaffin cell, whose main work is to produce and release the hormones that cause the “fight or flight” response in the body.
pheochromocytoma

Also Read: Adrenal Cancer: Causes, Symptoms And Treatment

Although pheochromocytomas are mostly noncancerous, about 10% to 15% of them might be malignant (cancerous). However, even if you develop a benign pheochromocytoma tumour, it usually releases hormones that may cause headaches, sweating, high blood pressure, and symptoms of a panic attack. If a pheochromocytoma is left untreated for a long time, it may lead to severe or life-threatening damage to other body systems within the body.

Causes

The majority of scientific case studies suggest that although the exact cause of pheochromocytoma is still unknown, it primarily develops when there are certain mutations or alterations in the genetic code of the DNA of the chromaffin cells of the adrenal gland. These mutations cause the healthy chromaffin cell to proliferate and multiply out of control instead of dying off as they should. This causes aberrant cells to group together and form cancerous tumours in the adrenal tissue. However, in some cases, Pheochromocytoma can be linked to hereditary conditions such as multiple endocrine neoplasia type 2 (MEN2), von Hippel-Lindau disease, or neurofibromatosis type 1 (NF1).

 Also Read: Parathyroid Cancer: Causes, Symptoms And Treatment

Risk Factors

Certain causative factors that increase the chances of developing pheochromocytoma include:

Age: Although Pheochromocytomas can occur at any age, it is most commonly diagnosed between the ages of 30 and 50

Family History: Having a family history of Pheochromocytoma or related genetic disorders may increase the incidence of developing the tumour

Genetic Syndromes: Certain hereditary conditions, such as MEN2, Von Hippel-Lindau disease, Hereditary paraganglioma syndrome, Carney-Stratakis dyad syndrome, Carney triad syndrome, and NF1, are associated with an increased risk of developing Pheochromocytoma as well

Symptoms

The characteristic symptoms of Pheochromocytoma can vary and are often episodic, meaning they come and go when the tumour releases too much adrenaline (epinephrine) or noradrenaline (norepinephrine) into your blood. Some of the common indications of  Pheochromocytoma include:

  • Tremors
  • Headache
  • Excessive sweating 
  • Intense fear and/or anxiety
  • Chest and/or abdominal pain
  • Pale complexion
  • Diarrhoea
  • Nausea and vomiting
  • Constipation
  • orthostatic hypotension
  • Blurry vision
  • Unexplained weight loss

Since Pheochromocytoma is symptomatic, some symptoms and indicators might even appear suddenly after the following situations:

  • High-intensity exercise session
  • A severe physical injury
  • Mental anguish
  • Childbirth
  • Undergoing anaesthesia
  • Certain medications
  • Surgery
  • Consuming tyramine-rich foods, such as cheese, chocolate, red wine, dried or smoked meats

Diagnosis

If you experience any of the aforementioned symptoms, consult an oncologist straight away for an early diagnosis and treatment. Following a thorough physical examination, the doctor would typically acknowledge the patient's medical history and genetic history as well as any prior history of carcinogenic exposure. The following diagnostics are usually performed by the doctor:

  • 24-hour urine test
  • Blood profile including Blood catecholamine tests
  • Imaging tests including X-rays, CT scans, MRI scans, PET scans and MIBG scan
  • Genetic testing

Staging Of Pheochromocytoma

The staging system for malignant pheochromocytoma is as follows:

Localised pheochromocytoma: Only one or both adrenal glands are affected by the tumour

Regional pheochromocytoma: The cancerous cells have spread to the tissues close to the adrenal glands, such as the lymph nodes

Metastatic pheochromocytoma: Tumour that has spread to distant lymph nodes or other organs such as the liver or lungs 

Recurrent pheochromocytoma: Recurrence of the cancerous cells in the same or a different location of the body following full treatment

Treatment

The team of treating doctors usually chooses the best course of action to deal with the malignant tumour to ensure that the patient has a long and healthy life after accurately staging the small cell lung cancer, knowing the size, location, and patient's condition. The following are some of the available therapies for the management and treatment of Pheochromocytoma:

  • Surgery
  • Radiation therapy
  • Chemotherapy
  • Ablation therapy
  • Embolization therapy
  • Targeted therapy
  • Clinical trials