Adrenal Tumors

Adrenal Cortical Adenoma

* Etiology: Most cases are sporadic. Association with MEN I syndrome can occur. * Signs and symptoms: Most adrenal cortical adenomas are asymptomatic (nonfunctional) and found incidentally. Patients may present with Cushing syndrome or hyperaldosteronism (Conn syndrome). Adenomas associated with Cushing syndrome and Conn syndrome are usually small and solitary.

* Gross Pathology: Adenomas associated with Cushing syndrome or conn syndrome are usually solitary and unilateral and weigh less than 50 gms. Well-defined tumors appear capsulated. Adenomas associated with Conn syndrome have a characteristic bright-yellow or golden-yellow color. Adenomas associated with Cushing syndrome may be bright yellow or tan. All adenomas may show focal hemorrhage or necrosis (typically in larger lesions).

Adrenocortical adenoma

* Histopathology: Circumscribed tumor. Typically has trabecular or alveolar (nesting) architecture. Tumor cells are large and have round, regular nuclei with small, dot-like nucleoli; focal pleomorphism and large prominent nucleoli may be seen. Absent or rare mitotic activity; never atypical mitoses. Cytoplasm is abundant and “clear” or “compact”

Adrenocortiocal adenoma

Adrenocortical adenoma

Adrenal Cortical Carcinoma

* Etiology: Sporadic adrenal cortical carcinoma is most common; however, it also occurs in MEN I syndrome.

* Epidemiology: Rare tumor; occurs in about 1 per 1 million population. Typically presents in fourth and fifth decades of life; less common in pediatric population. Equal incidence in males and females * Signs and symptoms: Usually presents as incidental finding or associated with abdominal or flank pain; may present with a palpable abdominal mass or with evidence of distant metastasis. About 79% of carcinomas secrete hormones, and most functional tumors secrete cortisol with marked virilization owing to co-secretion of 17- ketosteroids and dehydroepiandrosterone (DHEA).

* Gross Pathology: Usually large tumors weighing between 100 and 1000 gms; may measure more than 20 cm (average, 14 to 15 cm). Irregular, variegated, tan-yellow mass with infiltrative borders. Extension into adjacent soft tissue or surrounding organs is common. Cut surface often shows extensive hemorrhage and necrosis.

Adrenocortical carcinoma

* Histopathology: The tumor cells are arranged in broad trabeculae with anastomosing architecture. Other common patterns include solid or alveolar architecture. The tumor cells may resemble normal adrenal cortical cells; however, there is marked nuclear atypia, atypical and frequent mitoses vascular and extra-adrenal invasion, and necrosis.

Adrenocortical carcinoma

Broad fibrous bands are a characteristic feature. Diagnostic features of malignancy include; 1.Size. 2.Weight >100 g. 3.Vascular invasion and metastasis

Pheochromocytoma

* Epidemiology: Sporadic tumors are usually diagnosed in patients aged 40 to 50 years, whereas hereditary forms are most often detected before age 40 years. * Signs and symptoms: Clinical presentation is paroxysmal and results from the direct actions of secreted catecholamines, including hypertension, tachycardia, pallor, headache, and anxiety. Up to 25% of pheochromocytomas are asymptomatic Anesthesia and tumor manipulation most often elicit a catecholamine crisis.

* Gross Pathology: Variable size and weight (from few grams to >2000 gms). Round to oval, sharply circumscribed mass. Cut surface shows a soft, variegated appearance with a dusky red-brown color. Marked hemorrhage and necrosis may be seen.

Pheochromocytoma

* Histopathology: Tumor cells are arranged in well-defined nests, Zellballen appearance; distinct nests of tumor cells surrounded by thin strands of fibrovascular stroma Tumor cells have varying size and shape with round nuclei, prominent nucleoli, and granular amphophilic to basophilic cytoplasm.

Pheochromocytoma

Marked pleomorphism with bizarre tumor giant cells and numerous mitotic figures may be seen; these features are not diagnostic of malignant behavior

Malignant behavior cannot be determined based on morphologic findings; only presence of distant metastases proves malignancy. Metastatic spread through lymphatic or hematogenous pathways most commonly involves lymph nodes, bones (particularly ribs and spine), lung, and liver

Neuroblastoma

* Clinical Features: Malignant tumor of sympathetic nervous system. Affects children younger than 4 years. Can occur in a variety of locations, with adrenal gland being most common site. Other common sites include posterior mediastinum.

* Signs and symptoms: About 40% of patients present with localized disease ranging from an incidental adrenal mass discovered on prenatal ultrasound to large and invasive tumors. Classic signs of disseminated neuroblastoma include periorbital ecchymoses, proptosis, or both, due to metastasis to the bony orbit

* Gross Pathology: Typically circumscribed, round to oval mass, often with a variegated, lobular cut surface; usually tan to gray-white May be variable in size ranging from less than 1 cm to greater than 10 cm. Typically solid mass. Often shows marked hemorrhage, necrosis, or calcification Invasion into adjacent organs and soft tissue may be seen.

* Histopathology: Cellular, small round blue cell tumor. Nodular aggregates of tumor cells separated by delicate fibrovascular septa. Prominent Homer-Wright pseudorosettes (round spaces surrounded by palisading peripheral nuclei and filled with a faintly eosinophilic fibrillary matrix) may be seen. Hemorrhage and microcalcifications are common

Neuroblastoma

Neuroblastoma; rosettes

Metastatic Tumors to adrenal glands

* Epidemiology: Most common tumors of the adrenal gland Bilateral adrenal gland involvement is found in about 50% of cases. Lung is most common primary tumor site, followed by stomach, esophagus, liver, bile ducts, pancreas, large intestine, kidney, and breast * Signs and symptoms: About 90% are asymptomatic, affect elderly patients, and are diagnosed as part of multiorgan metastases.

* Gross Pathology: Often multifocal nodular disease Solitary lesions may mimic primary adrenal cortical carcinoma. Typically involves adrenal cortex and often shows extension into adjacent adipose tissue. Tumors may occasionally show extension into vena cava. Brown or black discoloration should raise suspicion of a metastatic melanoma.

* Histopathology: Depends on primary site of malignancy. Adenocarcinomas and squamous cell carcinomas are the most common subtypes. Lung and breast metastases typically show poorly differentiated carcinoma; squamous or glandular differentiation may be seen in metastatic lung carcinoma Metastatic melanoma composed of large, polygonal cells with pleomorphic nuclei and prominent nucleoli; melanin pigment may be seen