Tumors of the Adrenal gland

Adrenal glands are paired endocrine organs consisting of cortex & medulla. In the adult- normal adrenal gland weighs about 4 gm. Histologically cortex contains 3 layers zona glomerulosa ,zona fasiculata & zona reticularis. Medulla is composed of neuroendocrine cells.

WHO Classification of adrenal tumors

Adrenal cortical tumors-

1. Adrenal cortical carcinoma 2. Adrenal cortical adenoma

Adrenal medullary tumors-

1.Malignant phaeochromocytoma 2.Benign phaeochromocytoma 3.Composite phaeochromocytoma / paraganglioma

WHO Classification

Other adrenal tumorsAdenomatoid tumor Sex-cord stromal tumor Soft tissue & germ cell tumors Myelolipoma Teratoma Schwannoma Ganglioneuroma Angiosarcoma
Secondary tumors.

WHO Classification

Extra-adrenal paraganglioma Carotid body Jugulotympanic Vagal Laryngeal Aortico-pulmonary Gangliocytic Cauda equina Orbital Nasopharyngeal Extra-adrenal sympathetic paraganglioma Superior & inferior para-aortic paraganglioma Urinary bladder Intrathoracic & cervical paravertebral

Adrenal cortical adenoma

The true incidence of adrenal cortical adenoma is unknown. Can occur at any age group including paediatric populations & in both sexes. Majority are unilateral. Are a functionally heterogenenous group of benign neoplasms.

Can differentiate towards any of the cortical layers.

Overproduction of glucocorticoids- cushing syndrome. Overproduction of androgenic/estrogenic steroids-androgenital syndrome. Ovreproduction of mineralocorticoids conn syndrome.

Non functional adenomas

Seen in patients without clinical or biochemical evidence of steroid hormone hypersecretion. Commonly detected with abdominal CT or MRI scan. Called as incidentalomas.

Clinical features

Depends on the hormonal secretory status of the tumour.

Imaging well-defined, rounded homogenous mass. intratumoral calcification,hemorrhage, necrosis is rare.

GROSS

Most adenomas are well circumscribed,nodular lesion upto 2.5cm. Some are encapsulated. Are usually yellow to yellow brown.

Hormonally active or inactive tumors cant be differentiated.

Majority weigh less than 100gms. Haemorrhage & necrosis are rarely seen.

 F:\16FF7A.jpg

Microscopy

Adenomas are composed of cells similar to those of normal cortex.

Tumor cells are arranged in small nests,cords. Nuclei are small with vaculoated to eosinophilic cytoplasm.

Some degree of pleomorphism may be seen.

Mitotic activity- rare.

 F:\16FF9.jpg

Adrenal cortical carcinoma

Rare tumor with an incidence of 1 or 2 cases/million population. Bimodal age distribution.

More common in females.

Tumor weighs more than 100 gms. Common presentation is associated with glucocorticoid & androgen oversecretion.

Gross examination

Adrenal carcinomas are large, invasive lesions exceeding 20 cm in diameter.

C/S varigated, poorly demarcated lesion with areas of haemorrhage, necrosis & cystic change. Invasion of contiguous structures like adrenal vein or IVC is common.

 F:\16FF8A.jpg

Microscopy

Tumor cells are arranged in alveolar, trabecular or solid patterns of growth interrupted by fine sinusoidal network. Abundant necrosis is present. Invasion of capsule, sinusoids,or large veins is common.

 F:\16FF10.jpg

Histopathological criteria of malignancy in an adrenal cortical tumor

1.High nuclear grade 2.>5 mitoses per 50 HPF 3.Atypical mitotic figures 4.<25% of tumor cells are clear cells. 5.Diffuse architecture ( >33% of tumor) 6.Necrosis 7.Venous invasion 8.Sinusoidal invasion 9.Capsular invasion.
Weiss & Aubert, 1989.

Differential diagnosis

Adrenal cortical carcinoma must be distinguished from secondary tumors involving the adrenal gland. 1.RCC 2.HCC 3.Metastatic adenocarcinoma. 4.Liposarcoma 5.Pheochromocytoma

IHC
TYPE CK Corti +/cal ca RCC HCC + + VIM + NF +/EMA CEA CG SYN +/MEL- INH A + +

+ +/+/-

+ +/+

+
+

-/+ -/+
-

Met.a + deno ca

Lipo sa.

+ +/-

-/+ -

Pheo chro

 F:\16FF13A.jpg

 F:\16FF13C.jpg

Pheochromocytoma

Uncommon neoplasm composed of neuroendocrine cells.

Can occur at any age but are most frequent in the 4th & 5th decade. Familial tumors present at younger age. Sporadic lesions are solitary. Familial cases bilateral involvement.

Rule of 10

10% of pheochromocytoma arise in association with one of several familial syndromes. 10% are extra-adrenal. 10% of nonfamilial pheochromocytomas are bilateral. 10% are biologically malignant. 10% arise in childhood, usually the familial subtype.

GROSS

Range from small,circumscribed lesion confined to adrenal to large haemorrhagic masses. Weigh from <5 to 3500gm C/S grey white to tan & darkens on exposure to air.

Focal haemorrhage, degenerative change,cystic change & calcification may be present.

 F:\16FF28A.jpg

Microscopy

Tumor cells are composed of polygonal to spindle shaped chromaffin cells. Cells are clustered into small nests or alveoli (zellballen)

Nuclei are round to ovoid with stippled chromatin.

Cytoplasm has finely granular appearance. Capsular & vascular invasion may be encoutered in benign lesion.

 F:\16FF29A.jpg

 F:\16FF29B.jpg

Malignant pheochromocytoma

Are currently defined by the presence of metastases. Metastases must be present at a site where chromaffin tissue is not otherwise found.

Histologic criteria of maligancy in adrenal medullary tumor

1.vascular invasion1 2.capsular invasion1 3.profound pleomorphism1 4.nuclear hyperchromasia1 5.Extension into periadrenal adipose tissue2 6.expanded,large,confluent nests2 7.Diffuse growth--2

8.necrosis2 9.Tumor cell spindling2 10.Atypical mitotic figures2 11.macronucleoli2

12.Absence of hyaline globules2

Interpretation of scoring Score=/> 4; malignancy.

THANK YOU