Adrenal cancer is a rare cancer that develops in the small, triangular gland located on the top of your kidneys especially adrenal gland. Adrenal glands produce hormones that give instructions to virtually every organ and tissue in your body. Adrenal cancer is often aggressive, when found early adrenal cancer can be cured. Non cancerous adrenal tumors such as adenomas or pheochromocytoma also develop in the adrenal glands.Age/sex prevalence of Adrenal cancer
Adrenal cancer is more prevalent in males than in females.Female to male ratio is 2.5:3.1.Male patients who are affected are tending to be older and have worse overall prognosis than the female patients.Adrenal cancer is more common in individuals of first decade of life and again in fourth decade of life.
Adrenal cancer is not related to any specific race.
Adrenal malignancies are classified as follows
•Adrenocortical carcinomas Functional Nonfunctional Well differentiated Intermediate Poorly differentiated to anaplastic.
•Metastatic adrenal tumors are as follows Most common involvements are as follows Lung Breast Melanoma, Renal cell carcinoma, Extra-adrenal lymphoma Leukemias Pancreatic carcinoma Colonic carcinoma Ovarian carcinoma.
•Adrenomedullary tumors Ganglioneuroblastoma Neuroblastoma Neuroendocrine carcinoma Malignant pheochromocytoma
•Stromal malignancies Neurofibrosarcoma Angiosarcoma Liposarcoma Myxosarcoma Malignant teratoma Fibrosarcoma
•Composite or mixed tumors
•Adrenal malignancies in the setting of familial predisposing syndromes Gardner syndrome Cowden syndrome Carney complex Carney triad MEN-1 Turcot syndrome Familial polyposis coli li-fraumeni syndrome.
While the mutation-induced inactivation of tumor suppressor genes appears to be a plausible mechanism for adrenal carcinoma development, other potential mechanisms include activation of various proto oncogenes inhibition of apoptosis or changes in various adreno cortical tissue-specific factors are possible.
Non functional variants: These hormonal silent tumors account for approximately 40% of patients with AC.
These tend to be more common in older patients and appear to progress more rapidly than functional tumors.
These typically present with fever, weight loss, abdominal pain and tenderness, back pain, abdominal fullness, or symptoms related to metastases.
In other cases, the mass is found incidentally, during either examination or radiologic imaging. Endocrine syndromes: The hormonally active variants of AC constitute approximately 60% of cases.
Approximately 30-40% of adult patients present with the typical features of Cushing syndrome, while 20-30% present with virilization syndromes. In children, however, more than 80% present with virilization syndromes while isolated Cushing syndrome is much less common at approximately 6% of cases.
Virilization (in girls) or precocious puberty (in boys) is the most common endocrine presentation of a functional AC. Hirsutism, facial acne, oligo amenorrhea and increased libido all are possible presenting symptoms.
Feminization as a presentation of AC is quite rare. Other modes of presentation include profound weakness, hypertension, and/or ileus from hypokalemia related to hyperaldosteronism and hypoglycemia.
Combined endocrine systems: Some cases of adrenal insufficiency are described in association with primary adrenal lymphomas, while other cases are associated with hypercalcemia.
Endocrine syndromes associated with adrenocortical carcinoma Cushing syndrome (30%) Virilization and precocious puberty (22%) Feminization (10%) Primary hyperaldosteronism (2.5%) Combined hormone excess (35%) Polycythemia (< 1%) Hypercalcemia (< 1%) Hypoglycemia (< 1%) Adrenal insufficiency (particularly from primary adrenal lymphomas) Non–glucocorticoid-mediated insulin resistance Catecholamine excess due to rare instances of coexisting pheochromocytoma Cachexia (usually preterminal)
Laboratory studies are as follows 1-mg dexamethazone suppression test 24 hour urinary cortisol excretion test Serum testosterone 24 hour urinary 17 ketosteroids Imaging studies to be done are as follows:-
• CT scans and MRI of adrenal gland
• Intravenous contrast
• Ultrasonography of adrenal gland
• Arteriography and venographyplace in the diagnostic evaluation of adrenal masses.Other tests to be done are as follows
Fine needle aspiration and core tissue biopsy.
Medications to be helpful in adrenal carcinoma are as follows Mitotane.The goal of the medication is to reduce morbidity, prevent complications and eradicate the carcinoma if possible.Suramin Gossypol Cisplatin-based chemotherapy Radiation therapy also help to treat adrenal carcinoma.
Complications associated with adrenal carcinoma are as follows:-
• Local invasion by tumor, include the thrombus formation by tumor, which later on embolize similar to renal cell carcinoma.
• Hormone excess syndromes’ such as cushing syndrome, hyperaldosteronism, hirsuitism, virilization and hypertension.
• Paraneoplastic syndromes such as cachexia.
• Local pain in patients with bone metastases.
• Pancreatic cancer
• Renal cell carcinoma
• Adrenal adenoma