notes by dr Claudio Italiano
The
adrenal cortex is the outermost part of the adrenal glands, which are located
near the top of each kidney. It has the function of producing hormones (especially
cortisol, androgens, aldosterone). Benign tumors (adenomas) and malignant tumors
(adrenocortical carcinomas) may take place. I approx. adrenocorticals are rare
malignant tumors, which represent 0.3-0.5% of malignant pediatric neoplasms,
with two incidence peaks, the first under 3 years of age (over 50% of cases) and
the second during adolescence. The cause is not known. Associations with other
diseases are described (Gardner's syndrome and Li-Fraumeni's family syndrome).
We distinguish benign tumors that are randomly diagnosed during instrumental
investigations, e.g. in the course of CT abdomen, as incidentalomas and
secondary tumors to metastases, starting from other organs or apparatuses, for
ex. the lung, the breast. In fact, rarely does a malignant tumor arise primarily
in the adrenal gland.
Adrenal tumors can affect the cortical part, for example, secretory adenomas
that determine Coon's syndrome and are distinguished in secreting tumors (ie,
hormones), and not secreting, or they can affect the medullary portion and this
tumor is called >> pheochromocytoma. In other cases Cushing's syndrome is
manifested by excessive cortisol hormone production by the adrenal cortex
following the presence of an adrenocorticotropic hormone-secreting adenoma
adenoma or ACTH, as a secondary or primitive condition for adrenal tumors or
nodular hyperplasia.
The
distinction between carcinoma and adrenocortical adenoma is difficult. The
presence of cellular pleomorphism, of numerous mitoses, of areas of necrosis and
of vascular or capsular invasion do not seem to be indicative of malignancy in
the child, as happens instead in the adult patient.
Staging and prognostic factors
The chances of recovery are linked to the extent of the disease. The majority of
authors consider the following staging: St.1 localized tumor, small in size /
volume, completely removed with subsequent normalization of hormonal values;
St.2 tumor removed completely but large, or removed with microscopic residues,
or fissured, or with lymph node invasion, or without normalization of hormonal
values; St.3 removal with macroscopic residuals or initial biopsy; St.4
metastasis at diagnosis. The chances of recovery vary from 15 to 70% in relation
mainly to the radicality of the surgical exeresis and the presence of metastases.
Conn's syndrome, or primary hyperaldosteronism, is a disease due to excess production of aldosterone by the adrenal cortex. Many adrenal adenomas, ie benign tumors, are found randomly during the execution of ultrasound, CT or MRI performed for other reasons, and produce and often need not be removed, but only controlled over time by repeating periodic investigations (ultrasound). I can rarely be adrenocarcinomas of the adrenocortal system. If they cause symptoms due to altered hormone production (secretory adenomas) they must be removed surgically. In most cases there is an excess of the hormone aldosterone. Symptoms are resolved with the intervention, even if 30% of patients will experience a relapse of the blood pressure. Patients undergoing surgery for hypercortisolism will need to take steroid-based replacement therapy for some time to avoid a sudden drop in these substances that are important for the body. You can have low renin levels and few symptoms. It may be, however, that most patients have the same high blood pressure that can cause damage to eyesight (hypertensive retinopathy) and / or migraines. Occasionally there may be muscle weakness, muscle spasms, tingling sensations or excessive urination. Complications include cardiovascular diseases such as stroke, myocardial infarction, renal failure and arrhythmiasIl feocromocitoma
Pheochromocytoma forms in the medullary portion; it is a generally benign tumor
but it can also be malignant, which originates from the chromaffin cells of the
neuroectoderm, which is part of the sympathetic system. Although
phaeochromocytomas can affect every sympathetic ganglion present in the body,
more than 90% originate from the adrenal medulla. The majority of
ex-transurrenal tumors are observed in the mediastinum or abdomen. Bilateral
adrenal pheochromocytomas are observed in about 5% of cases and may be part of
familial syndromes. The feo-chromocitoma is part of multiple endocrine neoplasms
of type 2A or 2B.
It is characterized by an excess of catecholamines (adrenaline, noradrenaline),
is manifested by increases in blood pressure, often in paroxysmal crisis, which
may be accompanied by other symptoms including tachycardia, sweating, flushing,
intense headache; high urinary excretion of catecholamines or their metabolites,
metanephrines and vanilmandelic acid is used for diagnosis purposes.