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Tumors of the Adrenal Tumors of the Adrenal gland gland
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Tumours of the Adrenal Gland

Jan 21, 2016

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Sonam Joshi

Adrenal glands
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Page 1: Tumours of the Adrenal Gland

Tumors of the Adrenal glandTumors of the Adrenal gland

Page 2: Tumours of the Adrenal Gland

Adrenal glands are paired endocrine organs Adrenal glands are paired endocrine organs consisting of cortex & medulla.consisting of cortex & medulla.

In the adult- normal adrenal gland weighs about 4 In the adult- normal adrenal gland weighs about 4 gm.gm.

Histologically cortex contains 3 layers—Histologically cortex contains 3 layers—

zona glomerulosa ,zona fasiculata & zona zona glomerulosa ,zona fasiculata & zona reticularis.reticularis.

Medulla is composed of neuroendocrine cells. Medulla is composed of neuroendocrine cells.

Page 3: Tumours of the Adrenal Gland

WHO Classification of adrenal tumorsWHO Classification of adrenal tumors

Adrenal cortical tumors-Adrenal cortical tumors-

1. Adrenal cortical carcinoma1. Adrenal cortical carcinoma2. Adrenal cortical adenoma2. Adrenal cortical adenoma

Adrenal medullary tumors-Adrenal medullary tumors-

1.Malignant phaeochromocytoma1.Malignant phaeochromocytoma2.Benign phaeochromocytoma2.Benign phaeochromocytoma3.Composite phaeochromocytoma / 3.Composite phaeochromocytoma /

paragangliomaparaganglioma

Page 4: Tumours of the Adrenal Gland

WHO ClassificationWHO Classification

Other adrenal tumors-Other adrenal tumors- Adenomatoid tumor Adenomatoid tumor Sex-cord stromal tumorSex-cord stromal tumor Soft tissue & germ cell tumors -Soft tissue & germ cell tumors - Myelolipoma Myelolipoma TeratomaTeratoma SchwannomaSchwannoma GanglioneuromaGanglioneuroma AngiosarcomaAngiosarcoma

Secondary tumors.Secondary tumors.

Page 5: Tumours of the Adrenal Gland

WHO ClassificationWHO Classification Extra-adrenal paragangliomaExtra-adrenal paraganglioma—— Carotid bodyCarotid body JugulotympanicJugulotympanic VagalVagal LaryngealLaryngeal Aortico-pulmonaryAortico-pulmonary GangliocyticGangliocytic Cauda equinaCauda equina Orbital NasopharyngealOrbital Nasopharyngeal Extra-adrenal sympathetic paragangliomaExtra-adrenal sympathetic paraganglioma Superior & inferior para-aortic paragangliomaSuperior & inferior para-aortic paraganglioma Urinary bladder Urinary bladder Intrathoracic & cervical paravertebralIntrathoracic & cervical paravertebral

Page 6: Tumours of the Adrenal Gland

Adrenal cortical adenomaAdrenal cortical adenoma

The true incidence of adrenal cortical adenoma is The true incidence of adrenal cortical adenoma is unknown. unknown.

Can occur at any age group including paediatric Can occur at any age group including paediatric populations & in both sexes.populations & in both sexes.

Majority are unilateral.Majority are unilateral.

Are a functionally heterogenenous group of Are a functionally heterogenenous group of benign neoplasms.benign neoplasms.

Page 7: Tumours of the Adrenal Gland

Can differentiate towards any of the cortical Can differentiate towards any of the cortical layers.layers.

Overproduction of glucocorticoids- cushing Overproduction of glucocorticoids- cushing syndrome.syndrome.

Overproduction of androgenic/estrogenic steroids-Overproduction of androgenic/estrogenic steroids-androgenital syndrome.androgenital syndrome.

Ovreproduction of mineralocorticoids– conn Ovreproduction of mineralocorticoids– conn syndrome.syndrome.

Page 8: Tumours of the Adrenal Gland

Non functional adenomasNon functional adenomas

Seen in patients without clinical or Seen in patients without clinical or biochemical evidence of steroid hormone biochemical evidence of steroid hormone hypersecretion.hypersecretion.

Commonly detected with abdominal CT or Commonly detected with abdominal CT or MRI scan.MRI scan.

Called as “incidentalomas.”Called as “incidentalomas.”

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Clinical featuresClinical features——

Depends on the hormonal secretory status of the tumour.Depends on the hormonal secretory status of the tumour.

Imaging Imaging ––

well-defined, rounded homogenous mass.well-defined, rounded homogenous mass.

intratumoral calcification,hemorrhage, necrosis is intratumoral calcification,hemorrhage, necrosis is rare. rare.

Page 10: Tumours of the Adrenal Gland

GROSS GROSS

Most adenomas are well circumscribed,nodular Most adenomas are well circumscribed,nodular lesion upto 2.5cm.lesion upto 2.5cm.

Some are encapsulated.Some are encapsulated.

Are usually yellow to yellow brown.Are usually yellow to yellow brown.

Hormonally active or inactive tumors can’t be Hormonally active or inactive tumors can’t be differentiated.differentiated.

Majority weigh less than 100gms.Majority weigh less than 100gms.

Haemorrhage & necrosis are rarely seen. Haemorrhage & necrosis are rarely seen.

Page 11: Tumours of the Adrenal Gland

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MicroscopyMicroscopy Adenomas are composed of cells similar to Adenomas are composed of cells similar to

those of normal cortex.those of normal cortex.

Tumor cells are arranged in small nests,cords.Tumor cells are arranged in small nests,cords.

Nuclei are small with vaculoated to eosinophilic Nuclei are small with vaculoated to eosinophilic cytoplasm.cytoplasm.

Some degree of pleomorphism may be seen.Some degree of pleomorphism may be seen.

Mitotic activity- rare.Mitotic activity- rare.

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Adrenal cortical carcinomaAdrenal cortical carcinoma Rare tumor with an incidence of 1 or 2 Rare tumor with an incidence of 1 or 2

cases/million population.cases/million population.

Bimodal age distribution.Bimodal age distribution.

More common in females.More common in females.

Tumor weighs more than 100 gms.Tumor weighs more than 100 gms.

Common presentation is associated with Common presentation is associated with glucocorticoid & androgen oversecretion. glucocorticoid & androgen oversecretion.

Page 15: Tumours of the Adrenal Gland

Gross examinationGross examination

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

C/S –varigated, poorly demarcated lesion with C/S –varigated, poorly demarcated lesion with areas of haemorrhage, necrosis & cystic areas of haemorrhage, necrosis & cystic change.change.

Invasion of contiguous structures like adrenal Invasion of contiguous structures like adrenal vein or IVC is common.vein or IVC is common.

Page 16: Tumours of the Adrenal Gland

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MicroscopyMicroscopy

Tumor cells are arranged in alveolar, trabecular Tumor cells are arranged in alveolar, trabecular or solid patterns of growth interrupted by fine or solid patterns of growth interrupted by fine sinusoidal network.sinusoidal network.

Abundant necrosis is present.Abundant necrosis is present.

Invasion of capsule, sinusoids,or large veins is Invasion of capsule, sinusoids,or large veins is common. common.

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Histopathological criteria of malignancy in an Histopathological criteria of malignancy in an adrenal cortical tumoradrenal cortical tumor

1.High nuclear grade1.High nuclear grade 2.>5 mitoses per 50 HPF2.>5 mitoses per 50 HPF 3.Atypical mitotic figures3.Atypical mitotic figures 4.<25% of tumor cells are clear cells.4.<25% of tumor cells are clear cells. 5.Diffuse architecture ( >33% of tumor)5.Diffuse architecture ( >33% of tumor) 6.Necrosis6.Necrosis 7.Venous invasion7.Venous invasion 8.Sinusoidal invasion 8.Sinusoidal invasion 9.Capsular invasion. 9.Capsular invasion.

Weiss & Aubert, 1989.Weiss & Aubert, 1989.

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Differential diagnosisDifferential diagnosis

Adrenal cortical carcinoma must be distinguished Adrenal cortical carcinoma must be distinguished from secondary tumors involving the adrenal from secondary tumors involving the adrenal gland.gland.

1.RCC1.RCC 2.HCC2.HCC 3.Metastatic adenocarcinoma.3.Metastatic adenocarcinoma. 4.Liposarcoma4.Liposarcoma 5.Pheochromocytoma5.Pheochromocytoma

Page 21: Tumours of the Adrenal Gland

IHCIHCTYPETYPE CKCK VIMVIM NFNF EMAEMA CEACEA CGCG SYNSYN MEL-MEL-

AAINHINH

CortiCortical cal caca

+/-+/- ++ +/-+/- -- -- -- +/-+/- ++ ++

RCCRCC ++ ++ -- ++ -- -- -- -- -/+-/+

HCCHCC ++ +/-+/- -- +/-+/- ++ -- -- -- -/+-/+

Met.aMet.adenodenocaca

++ +/-+/- -- ++ ++ -- -- -- --

LipoLiposa.sa.

-- ++ -- -- -- -- -- -/+-/+ --

PheoPheochrochro

-- +/-+/- ++ -- -- ++ ++ -- --

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PheochromocytomaPheochromocytoma

Uncommon neoplasm composed of Uncommon neoplasm composed of neuroendocrine cells.neuroendocrine cells.

Can occur at any age but are most frequent in the Can occur at any age but are most frequent in the 44thth & 5 & 5thth decade. decade.

Familial tumors – present at younger age.Familial tumors – present at younger age.

Sporadic lesions are solitary.Sporadic lesions are solitary.

Familial cases– bilateral involvement.Familial cases– bilateral involvement.

Page 25: Tumours of the Adrenal Gland

10% of pheochromocytoma arise in association 10% of pheochromocytoma arise in association with one of several familial syndromes.with one of several familial syndromes.

10% are extra-adrenal.10% are extra-adrenal.

10% of nonfamilial pheochromocytomas are 10% of nonfamilial pheochromocytomas are bilateral.bilateral.

10% are biologically malignant.10% are biologically malignant.

10% arise in childhood, usually the familial 10% arise in childhood, usually the familial subtype.subtype.

Rule of 10

Page 26: Tumours of the Adrenal Gland

GROSSGROSS

Range from small,circumscribed lesion Range from small,circumscribed lesion confined to adrenal to large haemorrhagic confined to adrenal to large haemorrhagic masses.masses.

Weigh from <5 to 3500gm Weigh from <5 to 3500gm

C/S – grey white to tan & darkens on C/S – grey white to tan & darkens on exposure to air.exposure to air.

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

Page 27: Tumours of the Adrenal Gland

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MicroscopyMicroscopy

Tumor cells are composed of polygonal to spindle Tumor cells are composed of polygonal to spindle shaped chromaffin cells.shaped chromaffin cells.

Cells are clustered into small nests or alveoli Cells are clustered into small nests or alveoli (zellballen)(zellballen)

Nuclei are round to ovoid with stippled Nuclei are round to ovoid with stippled

chromatin.chromatin.

Cytoplasm has finely granular appearance.Cytoplasm has finely granular appearance.

Capsular & vascular invasion may be encoutered Capsular & vascular invasion may be encoutered in benign lesion. in benign lesion.

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Malignant pheochromocytomaMalignant pheochromocytoma

Are currently defined by the presence of Are currently defined by the presence of metastases.metastases.

Metastases must be present at a site Metastases must be present at a site where chromaffin tissue is not otherwise where chromaffin tissue is not otherwise found.found.

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Histologic criteria of maligancy in adrenal Histologic criteria of maligancy in adrenal medullary tumormedullary tumor

1.vascular invasion—11.vascular invasion—1

2.capsular invasion—12.capsular invasion—1

3.profound pleomorphism—13.profound pleomorphism—1

4.nuclear hyperchromasia—14.nuclear hyperchromasia—1

5.Extension into periadrenal adipose tissue—25.Extension into periadrenal adipose tissue—2

6.expanded,large,confluent nests—26.expanded,large,confluent nests—2

7.Diffuse growth--27.Diffuse growth--2

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8.necrosis—28.necrosis—2

9.Tumor cell spindling—29.Tumor cell spindling—2

10.Atypical mitotic figures—210.Atypical mitotic figures—2

11.macronucleoli—211.macronucleoli—2

12.Absence of hyaline globules—212.Absence of hyaline globules—2

Interpretation of scoringInterpretation of scoring Score=/> 4; malignancy.Score=/> 4; malignancy.

Page 34: Tumours of the Adrenal Gland

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