bimodal distribution with peak below age 5 and a second peak in 4-5th decades of life
genetic syndromes may predispose to increased incidence of tumors including Beckwith-Wiedenann, Li-Fraumeni, Carney and MEN type 1
f>m with females accounting for 65% of adrenal cancer
usually unilateral (left gland more common)but may be bilateral in 2-10% of cases
more than half of patients have no endocrine abnormality at presentation (54.3% in recent publication)
when tumor hormonal active may present most commonly with Cushing’s syndrome (30-40% of patients with adrenal cancer).
Virilization due to hypersecretion of adrogens is next most common (20-30% of patients with functioning tumors). 95% of children with functioning tumors present with virilization
other clinical syndromes at presentation include feminization, hyperaldosteronism, hypoglycemia, polycythemia and nonglucocorticoid-related insulin resistance
in patients without hormonally active tumor presentation is due to abdominal pain, fullness or palpable mass, or incidentally discovered mass on imaging study. Metastatic disease may be presentation as well incluing lung, liver and/or bone metatstases
tumor spread may be local to renal vein and IVC or distant to lungs , liver or bone. 20% have spread at time of presentation
treatment is surgical with removal of the entire tumor or if not possible by removing as much tumor as possible. Metastatic sites are also resected.
mean survival is but 18 months with children doing better than adults. Patients with aggressive surgical management tend to do best with mean survival of 48 months
Clinical presentation
in patients without hormonally active tumor presentation is due to abdominal pain, fullness or palpable mass, or incidentally discovered mass on imaging study. Metastatic disease may be presentation as well including lung, liver and/or bone metatstases
in hormonally active tumors presentation may range from Cushings to virilization (see above). CT is then ordered and detects the tumor
CT Findings
mass greater than 5 cm in size at time of presentation with most nonfunctioning tumors being in the range of 10 cm or better
solid mass on noncontrast scans and will enhance variably on contrast enhanced studies
renal vein or IVC extension may be seen
tumors have variable enhancement with areas of necrosis common
calcifications in the tumor are not rare (up to 30% of cases)
may be bilateral in up to 10% of cases
metastases to lung bases, liver or nodes may be seen at time of adrenal evaluation
MR Findings
hyperintense mass compared to liver on T2 weighted images but hypointense to liver on T1 weighted images
sagitalimaging helpful for vascular IVC invasion and for relationship of right sided tumors and liver (i.e. invasion vs. adjacentcy)
Ultrasound Findings
variable appearance depending on size
functioning tumors are smaller and have echo pattern similar to renal cortex
larger tumors show mixed heterogeneous echo patterns depending on presence of necrosis and hemorrhage
lesions usually have sonographically defined echogenic border
Therapy/Management
surgical therapy usually means resection of the ipsilateral kidney, adrenal gland and adjacent structures depending on tumor extent
depending on tumor extent laparoscopic adrenalectomy is performed.
If tumor has spread chemotherapy may help including mitotane as well as cisplatin, 5-FU, and suramin
Differential Diagnosis
metastases- metastases may be unilateral although more commonly bilateral. Metastases usually under 5 cm but may be larger especially from melanoma. Clinical history is usually available to help in most cases.
ganglioneuroma – may look similar although these tumors are usually smoother than metastases
pheochromocytoma- both can have liver matastases and lung metastases but pheo’s are usually very vascular on CT and very bright on T2 weighted images. Clinical presentation of pheochromocytoma may be helpful as well.
adrenal lymphoma- usually bilateral and shape is more like classic adrenal ( triangular) than adrenal carcinoma. Non-hodgkins lymphoma is most common pathology
neuroblastoma-usually a pediatric age tumor but may occur in adults occassionally
References
Adrenal Cancer Stratakis CA, Chrousos GP Endocrin Metabolism Clinics NA Volume 29, no. 1, March 2000 pp 15-25
Primary Adrenocortical Carcinoma; CT Evaluation with Clinical Correlation Fishman EK, Deutch BM, Hartman DS et al. AJR 1987;148:531-535
State of the Art Adrenal Imaging Mayo-Smith WW, Boland GW, Noto RB, Lee MJ RadioGraphics 2001;21:995-1012
