Popular Science Public Health Knowledge: Etiology, Classification, and Clinical Manifestations of Adrenal Tumors (Part 1)

Popular Science Public Health Knowledge: Etiology, Classification, and Clinical Manifestations of Adrenal Tumors (Part 1)
Adrenal tumors refer to malignant tumors of adrenal glands, including neuroblastoma, adrenocortical carcinoma, and several adrenal pheochromocytomas. Popular Science Public Health Knowledge: Etiology, Classification, and Clinical Manifestations of Adrenal Tumors (Part 1)

morbidity

  • Adrenal cortex carcinoma is a rare tumor that affects only 2 people in a million.
  • Adrenal carcinoma accounts for 0.2% of all cancer-related deaths.
  • Gender

  • The ratio of men to women is approximately 1:3.
  • Female patients are more likely than male patients to have a concomitant endocrine syndrome.
  • Non-functioning adrenocortical carcinoma was evenly distributed between sexes.
  • Male patients are older than female patients and have a worse overall prognosis.
  • Age

  • It occurs in two main peaks: in first decade of life and in fourth and fifth years.
  • Mean age at diagnosis was 44 years, but tumors occurred at any age. The average age of children was 4 years.
  • Race and ethnicity

    There is no racial or ethnic preference for adrenocortical carcinoma.

    Geography

    Several reports suggest that, for unknown reasons, incidence is disproportionately high among children in southern Brazil.

    Popular Science Public Health Knowledge: Etiology, Classification, and Clinical Manifestations of Adrenal Tumors (Part 1)
Causes and risk factors

    Risk factors for adrenal cancer have not been established. This is because they are very rare, making epidemiological studies fruitless and difficult.

    Chromosomal abnormalities

    At genetic level, adrenal tumors exhibit following abnormalities.

  • Mutation or loss of p53 The tumor suppressor gene is located on chromosome 17 (17p). Adrenocortical carcinoma, a common form of Li-Fraumeni syndrome, contains a germline mutation in TP 53.
  • Frequent loss of heterozygosity between chromosomes 11p and 13q... Adrenocortical carcinoma is seen in Becker-Wiedemann syndrome, which includes mutations in 11p15.
  • Smoking

    Men who smoked more than 25 cigarettes per day had an increased risk (odds ratio 2:0; 95% confidence interval [CI] 1.0–4.4).

    Pill

    Women who used oral contraceptives, especially those under 25 years of age, had an increased risk of developing adrenocortical carcinoma (odds ratio 1:8; 95% CI 1.03.2).

    Popular Science Public Health Knowledge: Etiology, Classification, and Clinical Manifestations of Adrenal Tumors (Part 1)
Symptoms and signs

    Functional tumor

    Approximately 60% of patients experience symptoms associated with excessive production of hormones (cortisol, aldosterone, androgens and estrogens). Functional tumors can present with any of following hormonal syndromes:

  • Hypercortisolism (Cushing's syndrome) is most common symptom (30%). It is characterized by rapidly progressive symptomatic development of Cushing's syndrome (3 months). Symptoms include: weight gain, muscle weakness, easy bruising, irritability, and insomnia. Other symptoms may include those related to androgenic effects of cortisol.
  • Men (30%): female hirsutism, acne, menstrual irregularities or amenorrhea. They may be more noticeable and include male pattern baldness, deep voice, breast reduction, clitoral enlargement, and decreased libido. In men, manifestations of androgen excess are less pronounced. It causes premature puberty in men.
  • Feminization (10%): Tumors in women that cause breast tenderness and dysfunctional uterine bleeding. It causes premature puberty in women. These tumors are associated with obstetrics, breast tenderness, testicular atrophy, and decreased libido in men.
  • Hyperaldosteronism (2.5%): Aldosterone-producing adrenocortical carcinoma is very rare. Hypertension and hypokalemia are typical clinical manifestations of primary aldosteronism. Cancer patients had large tumors, high aldosterone levels, and severe hypokalemia compared to benign aldosterone adenomas. Evaluation should include measurement of serum electrolytes, aldosterone and plasma renin levels. Results included severe hypokalemia, potassium levels below 2.5 mEq/L, hypernatremia, and metabolic alkalosis. Serum aldosterone levels are high and plasma renin levels are suppressed.
  • Excess hormones (35%).
  • Non-functioning tumor

    40% of cases are non-functioning carcinomas and thus may be a sign of local tumor invasion or metastasis. The main symptoms are a large abdominal mass, abdominal pain, or weight loss.

    Transferability

  • Light 45%
  • Liver 42%
  • Lymph nodes - 24%
  • Bone 15%
  • Pancreas 12%
  • Spleen 6%
  • Aperture 12%
  • Miscellaneous (brain, peritoneum, skin, palate) 12%
  • Popular Science Public Health Knowledge: Etiology, Classification, and Clinical Manifestations of Adrenal Tumors (Part 1)
diagnosis

    CT

    CT allows you to reliably differentiate hyperplasia of cerebral cortex from tumors.

    By way, an adrenal tumor is found in 1-3% of patients who undergo abdominal CT. Most of these lesions were benign, and adrenocortical adenomas were 60 times larger than primary carcinomas.

    Criteria for malignancy

  • A typical malignant case is characterized by a large unilateral tumor of adrenal gland. Irregular edges.
  • A mass less than 3 cm in diameter is usually benign; conversely, when a mass is measured, probability of having a malignant mass usually increases. Greater than 6 cm... there is uncertainty at mass sizes of 3–6 cm and concerns that adrenal carcinoma may be overlooked in early stages of development.
  • As evidenced by continuous adenosis.
  • Rationale

  • Pinal CT of adrenal glands with slices of 3 to 5 mm provides best resolution and is especially useful in detecting tumors 1 cm or smaller.
  • CT has high sensitivity (over 95%) but no specificity.
  • CT can show primary tumor as well as local and distant tumor metastases.
  • Popular Science Public Health Knowledge: Etiology, Classification, and Clinical Manifestations of Adrenal Tumors (Part 1)

    Magnetic resonance imaging

    MRI can distinguish adenoma from carcinoma from pheochromocytoma. Loss of chemical shift signal in adrenocortical carcinoma.

  • MRI has a sensitivity of 89%, a specificity of 99%, and an accuracy of 94% in differentiating between benign and malignant lesions.
  • PET

    Positron emission tomography (PET) studies used fluorodeoxyglucose or C11 methionine. A better imaging indicator may be 11C-midimidate. Although this indicator cannot distinguish between benign and malignant tumors, absorption outside adrenal glands may indicate recurrence or metastasis in patients undergoing adrenocortical carcinoma resection.

  • The sensitivity is 100% and specificity is 95%.
  • Ultrasound

    The test has low diagnostic sensitivity for adrenal tumors and is highly user dependent in terms of interpretation and quality of results. However, it is especially useful in follow-up of previously discovered incidental tumors.

    The echogenic texture of malignancies is variable and heterogeneous in appearance, with lesions or scattered anechoic or echogenic areas representing areas of tumor necrosis, hemorrhage, or calcification.

    Angiography and venography of adrenal glands

    Selective angiography and adrenal venography are ineffective in diagnosing adrenal neoplasms. This can help differentiate smaller lesions and help differentiate adrenal tumors from tumors in upper pole of kidney. Vena cava contrast examination and angiography may provide additional staging information and allow for a more complete preoperative assessment.

    Visualization

    Most adrenocortical carcinomas cannot be visualized with 131 I-6β-iodomethylcholesterol. Because cortisol production suppresses ACTH secretion and contralateral adrenal function, patients with cortisol-producing adrenal tumors cannot visualize location of tumor or contralateral adrenal gland. CT and iodomethylcholesterol imaging can be used to diagnose small (less than 4 cm) normal adrenal masses. CT images (CT image and increased absorption on ipsilateral side) were benign in 100% of cases, while discordant images (CT image of tumor on one side and increased absorption on contralateral side) were malignant in 73% of cases.

    Popular Science Public Health Knowledge: Etiology, Classification, and Clinical Manifestations of Adrenal Tumors (Part 1)

    Adrenal Function Test

    Additional studies may include appropriate endocrine studies. Serum or urine steroid profiles can help differentiate benign from malignant adrenal tumors due to presence of intermediate precursors in steroid biosynthetic pathway or metabolites in patients with malignant tumors.

    Fine-needle aspiration and core-needle biopsy

    Fine needle aspiration should never be performed on any neoplasm of adrenal gland unless pheochromocytoma has been previously excluded; otherwise, procedure can be fatal.

    Because histological analysis of these lesions can be uncertain, fine-needle and/or core-needle biopsy (percutaneous approach) is generally not recommended unless metastases are suspected.

    Fine needle and/or percutaneous biopsy can be performed under CT or ultrasound guidance. Currently, only reason for this is to screen patients with known malignancy to rule out adrenal metastases.