Adrenal Tumors

BY: SARA ALRAMADHAN.

 Classification of adrenal tumor
Cortex Medulla
Benign Malignant Benign Malignant
Adrenocortical adenoma Adrenocortical carcinoma Pheochromocytoma Neuroblastoma

Pheochromocytoma rule of 10:

 10% extra adrenal (closer to 15%).
 10% recur (more if extra- adrenal).
 10% familial (closer to 20%).
 10% occur in children.
 10% bilateral or multiple (more if familial).
 10% malignant.
 10% discovered incidentally.
Metastatic Tumors to adrenal glands
Epidemiology Signs and symptoms Gross Pathology Histopathology
Most common malignant tumors of the adrenal About 90% are May be multifocal nodular disease or Depends on primary site of
gland. asymptomatic, affect Solitary lesions mimicking primary malignancy. Lung and breast
Bilateral adrenal gland involvement is found in elderly patients, and are adrenal cortical carcinoma. metastases typically show poorly
about 50% of cases. diagnosed as part of Typically involves adrenal cortex and differentiated carcinoma; squamous
Lung is most common primary tumor site, followed multi organ metastases. often shows extension into or glandular differentiation may be
by stomach, esophagus, liver, bile ducts, adjacent adipose tissue. seen in metastatic lung carcinoma
pancreas, large intestine, kidney, melanoma and Tumors may occasionally show Adenocarcinomas, squamous cell
breast. extension into vena cava. carcinomas and melanoma
Brown or black discoloration should are the most common subtypes.
raise suspicion of a metastatic
melanoma.

MULTIPLE ENDOCRINE NEOPLASIA (MEN)

MEN-1/ Wermer syndrome MEN-2A/Sipple syndrome MEN-2B
1. Parathyroid gland: hyperplasia or
adenomas. 1. Parathyroid hyperplasia. 1. Medullary carcinoma of thyroid.
2. Pancreas: Endocrine tumors of the 2. Medullary carcinoma of thyroid. 2. Pheochromocytoma.
pancreas. 3. Pheochromocytoma. 3. Neuromas or ganglioneuromas.
3. Pituitary: Prolactinoma.
 Adrenocortical adenoma Adrenocortical carcinoma
Etiology: Epidemiology:
Most cases are sporadic; some cases may be familial. Rare tumor; occurs in about 1 per 1 million populations.
Signs and symptoms: Typically presents in fourth and fifth decades of life; less common in
Most adrenal cortical adenomas are asymptomatic nonfunctional pediatric population.
(incidentloma) and found incidentally. Equal incidence in males and females.
Patients may present with Cushing syndrome or hyperaldosteronism Prognosis:
(Conn syndrome). is poor with Median survival is about 2 years.
Gross Pathology: Signs and symptoms:
Adenomas associated with Cushing syndrome or conn syndrome are Usually presents as incidental finding or associated with abdominal or
usually small, solitary and unilateral and weigh less than 50 gms. flank pain; palpable abdominal mass or with evidence of distant
Well-defined Circumscribed tumor appear capsulated. Adenomas metastasis. About 79% of carcinomas secrete hormones, and most
may have bright-yellow or golden-yellow color. functional tumors secrete cortisol with marked virilization owing to co-
adenomas may show focal hemorrhage or necrosis (typically in secretion of 17- ketosteroids and dehydroepiandrosterone (DHEA).
larger lesions). Gross Pathology:
Histopathology: Usually large tumors weighing between 100 and 1000 gms; may
Architecture: measure more than 20 cm (average, 14 to 15 cm).
Typically has trabecular or alveolar (nesting). Irregular, variegated, tan-yellow mass with infiltrative borders,
cells: extension into adjacent soft tissue or surrounding organs is common.
are large with focal pleomorphism Cut surface
have round, regular nuclei with small, dot-like nucleoli, Absent often shows extensive hemorrhage and necrosis.
mitosis and necrosis. Broad fibrous bands are a characteristic feature.
Cytoplasm: Diagnostic features of malignancy include:
is abundant clear due to presence of intra cytoplasmic lipid. Size.
Weight >100 g.
Vascular invasion.
Metastasis by lymphatic and blood.
Histopathology of adrenocortical carcinoma:
Architecture:
tumor cells are arranged in broad trabeculae with anastomosing
architecture, other common patterns include solid or alveolar.
cells:
resemble normal adrenal cortical cells; however, there is marked
nuclear atypia, atypical and frequent mitoses, vascular and extra-
adrenal invasion, necrosis.
Immunohistochemistry:
positive to inhibin tumor marker.
 Pheochromocytoma Neuroblastoma
Cell of origin: Cell of origin: malignant tumor arising from Primitive neural crest
chromaffin cells, which synthesize and release catecholamines cells.
Epidemiology: Sites:
Sporadic tumors are usually diagnosed in patients aged 40 to 50 years, whereas Adrenal medulla 40%.
hereditary forms are most often detected before age 40 years. It can associate Sympathetic ganglia in Paravertebral region of the abdomen,
MEN type II (pheochromocytoma+ medullary carcinoma of Thyroid +primary Posterior mediastinum.
hyperparathyroidism) Age:
Signs and symptoms: Asymptomatic Up to 25% of pheochromocytomas are. Prevalence is about one case in 7000 live births.
Clinical presentation Most frequently diagnosed tumor of infancy.
is paroxysmal and results from the direct actions of secreted catecholamines, Median age at diagnosis 18 months.
including hypertension, tachycardia, pallor, headache, and anxiety. Most common extracranial solid tumor of childhood.
Triggers of paroxysm: Clinical presentation:
exercise, emotional stress, change in posture. Anesthesia and tumor A-<2 years’ age: Large abdominal mass, Fever, and Weight loss.
manipulation most often elicit a catecholamine crisis B-In older children: usually metastases produce manifestations.
(sudden release of catecholamines ). Signs and symptoms:
Gross Pathology: Localized tumor: ranging from an incidental adrenal mass
Size: variable and weigh (from few grams to>2000 gms). discovered on prenatal ultrasound.
Shape: round to oval, sharply circumscribed mass. Invasive large tumor: to adjacent organs as Kidney, Renal vein,
Cut surface: Inferior vena cava, and aorta.
soft, variegated appearance with a dusky red-brown color, marked hemorrhage Disseminated tumor: leading to periorbital ecchymoses, proptosis
and necrosis may be seen specially in large lesions. due to metastasis to the bony orbit, Liver, Lungs, Bones, and Bone
Histopathology: marrow.
Architecture: “Blueberry muffin baby”: are deep blue discoloration of the skin of
cells are arranged in well-defined nests (Zellballen appearance) neonates due to multiple cutaneous metastases of
distinct nests of tumor cells surrounded by thin strands of fibrovascular stroma neuroblastoma.
Cells: varying size and shape with round nuclei, prominent nucleoli, and granular Gross Pathology:
cytoplasm (granules containing catecholamines). Shape: circumscribed, round to oval mass.
Nuclei: Cut surface: variegated, lobular; usually tan to gray- white, solid
“salt and pepper” chromatin. Marked pleomorphism with bizarre tumor giant mass with marked hemorrhage, necrosis, or calcification.
cells and numerous mitotic figures may be seen; these features are not Size: variable in ranging from less than 1 cm to greater than 10 cm.
diagnostic of malignant behavior. Prognosis:
Laboratory diagnosis of pheochromocytoma: Age:<18 months - excellent prognosis.
Demonstration of increased urinary excretion of Free catecholamines, Stage:1, 2A, or 2B - excellent prognosis.
Catecholamine metabolites, such as vanillylmandelic acid and metanephrines. Histopathology:
Definitive criteria for malignancy in pheochromocytoma is presence of Architecture: Nodular aggregates of tumor cells separated by
metastases to: delicate fibrovascular septa.
so Malignant behavior cannot be determined based on morphologic findings; Cells: Small round blue cell with Prominent Homer-Wright
only presence of distant metastases proves malignancy. Metastatic spread pseudorosettes (round spaces surrounded by palisading peripheral
through lymphatic or hematogenous pathways most commonly involves lymph nuclei and filled with eosinophilic neurofibrillary matrix) .
nodes, bones (particularly ribs and spine), lung, and liver.