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Adrenal tumors

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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 ... – PowerPoint PPT presentation

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Title: Adrenal tumors


1
Adrenal tumors
  • by
  • Dr. Gehan Mohamed

2
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.
  • Gerotas fascia connect the gland to upper pole
    of the kidney

3
Adrenal Gland Normal Histology
G salt F sugar R sex Medulla
epinephrine,norepinephrine
Zona fasciculata
Zona glomerulosa
Zona reticularis
Medulla
4
Two important questions for any adrenal mass
  • - Is it malignancy ?
  • - Is it functioning ?

5
Age Incidence of adrenal tumors
  • - both adrenal adenoma and carcinoma are equally
    occuring in adults.
  • - in children adrenal carcinoma predominate.

6
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.

7
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.

8
  • Benign cortical adenoma
  • Generally, adrenal adenomas are small
    homogeneous adrenal mass lt4 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.

9
Adrenal Cortical Adenoma
10
Malignant adrenal tumors
11
  • 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 gt 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.

12
Adrenal Cortical Carcinomacharachterised by
large mass (usually more than 4cm),areas of
hemorrhage and necrosis
ACC
Kidney
13
Development of adrenal medulla and tumors arising
from it ?
14
  • 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.

15
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
16
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.

17
Pheochromocytoma is composed of large chief cells
that have pink cytoplasm arranged in nests and
separated by sustentacular cells.
18
Immunohistochemistry of pheochromocytoma
  • Positive staining
  • for chromogranin and synaptophysin is
  • present in the chief cells, whereas the
    sustentacular cells are positive for S100 protein

19
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.

20
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.

21
neuroblastomasmall round blue cells arranged in
rosette manner in a fibrillary background
22
neuroblastoma
  • immunohistochemical staining of CD56,
    chromogranin A, and synaptophysin

23
Positive chromogranin in neuroblastoma
24
investigations
  • Computed tomography,MRI suggest malignancy if
    irregular shape,hetrogeneous density.
  • Fine-needle aspiration biopsy

25
Differential diagnoses for adrenal mass
26
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.

27
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.
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