Adrenal tumors by Dr. Gehan Mohamed

Cross Sectional Anatomy Normal size (Lt or Rt adrenal) : 3 cm × 6 mm,weighs 4 -6 grams. It is formed of golden yellow outer cortex and greyish brown inner medulla. Gerota’s fascia : connect the gland to upper pole of the kidney

Adrenal Gland Normal Histology G = salt F = sugar R = sex Medulla = epinephrine,norepinephrine Medulla Zona reticularis Zona fasciculata Zona glomerulosa

Two important questions for any adrenal mass: - Is it malignancy ? - Is it functioning ?

Age Incidence of adrenal tumors - both adrenal adenoma and carcinoma are equally occuring in adults. - in children adrenal carcinoma predominate.

Are adrenal tumors functioning? - not all adrenal tumors are functioning. - functional adrenal adenoma is usually associated with hyperaldosteronism,and cushing syndrome. - adrenal carcinoma is usually associated with virilizing manifestations secondary to secretion of sex hormones.

Adrenal gland tumors A- benign :adrenal cortical adenoma. B- malignant 1- primary malignant adrenal gland tumors a- adrenal cortical carcinoma b- malignant tumors arising from adrenal medulla as pheochromocytoma,neuroblastoma. 2- metastasis : specially from lung reaching adrenal gland.

Benign cortical adenoma Generally, adrenal adenomas are small homogeneous adrenal mass <4 cm in diameter, with a smooth border, well circumscribed ,yellow color as the tumor cells rich in lipid. Microscopically : adenoma formed of cells simulating normal cortical cells in appearance.

Adrenal Cortical Adenoma capsule cortex

Malignant adrenal tumors

Adrenocortical carcinomas -It may be sporadic or inherited cases. - inherited adrenal cortical carcinomas as : Li-Fraumeni syndrome - autosomal dominant - have predisposition to develop other cancers due to mutations in p53 adrenal carcinomas are large ( 90% > 4 cm), invasive lesions - If the tumor is functioning can lead to appearance of cushing syndrome, or more commonly virilization. a) adrenal carcinomas metastasize via lymphatics and inferior vena cava i) mean survival is ~2 yrs.

Adrenal Cortical Carcinoma:charachterised by large mass (usually more than 4cm),areas of hemorrhage and necrosis ACC Kidney

Development of adrenal medulla and tumors arising from it ?

Pheochromocytoma a) neoplasm composed of chromaffin cells i) synthesize and release catecholamines b) familial syndromes (~10%) It is symptomatic up to 15% of case. Functioning pheochromocytoma should be excluded by measuring 24-hour urinary metanephrines and catecholamines.

clinical picture of pheochromocytoma i) hypertension! - chronic, elevated BP (~70% of cases) ii) other hormones can secret - ACTH - somatostatin iii) diagnosis   urinary excretion of catecholamines and metabolites - vanillylmandelic acid - metanephrines

Role of 10 % in pheochromocytoma 1- 1o % of pheochromocytoma are extra adrenal. 2- 10 % are bilateral. 3- 10 % are malignant. 4- 10% are not associated with hypertension.

Pheochromocytoma is composed of large chief cells that have pink cytoplasm arranged in nests and separated by sustentacular cells.

Immunohistochemistry of pheochromocytoma Positive staining for chromogranin and synaptophysin is present in the chief cells, whereas the sustentacular cells are positive for S100 protein

neuroblastoma Neuroblastoma most commonly arises in and around the adrenal glands, (adrenal medulla have similar origins to nerve cells ). However, neuroblastoma can also develop in other areas of the abdomen and in the chest, neck and pelvis, where groups of nerve cells exist.

neuroblastoma Symptoms: Abdominal pain A mass under the skin that isn't tender when touched Changes in bowel habits, such as diarrhea Swelling in the legs Signs and symptoms caused by tumor secretions. Neuroblastomas may secrete certain chemicals that irritate other normal tissues, causing signs and symptoms called paraneoplastic syndromes. One paraneoplastic syndrome that occurs rarely in people with neuroblastoma causes rapid eye movements and difficulty with coordination. Another rare syndrome causes abdominal swelling and diarrhea. Complications : Spread of the cancer (metastasis). Neuroblastoma may spread (metastasize) to other parts of the body, such as the lymph nodes, bone marrow, liver, skin and bones. Spinal cord compression. Tumors may grow and press on the spinal cord, causing spinal cord compression. Spinal cord compression may cause pain and paralysis.

neuroblastoma:small round blue cells arranged in rosette manner in a fibrillary background

neuroblastoma immunohistochemical staining of CD56, chromogranin A, and synaptophysin

Positive chromogranin in neuroblastoma

investigations Fine-needle aspiration biopsy Computed tomography,MRI suggest malignancy if irregular shape,hetrogeneous density. Fine-needle aspiration biopsy

Differential diagnoses for adrenal mass

Unilateral adrenal masses Functional leisons Non Functional leisons Adrenal adenoma Adrenal cortical carcinoma. Pheochromocytoma Adrenal adenoma Adrenal cortical carcinoma. Ganglioneuroma Myelolipoma Hemorrhage in adrenal gland. Metastases to the adrenal gland.

Bilateral adrenal masses Functional leisons Non Functional leisons ACTH dependant cushing syndrome Congenital adrenal hyperplasia. pheochromocytoma Infection(T.B,Fungus). Infiltrative (amyloidosis ,leukemia,lymphoma) Hemorrhage.