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Endocrine > 171. Adrenal Pathology Cortex-Medulla > Flashcards

171. Adrenal Pathology Cortex-Medulla Flashcards

1
Q

Inner most zone

Cells produce androgens (15% of cortical volume)

Cells are more haphazardly arranged, appear smaller than cells of the other zones, have granular, eosinophilic cytoplasm

A

Zona reticularis

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2
Q

Gross:

  • solitary, unilateral masses
  • Well circumscribed gray white mass, often large

Microscopically:

  • Zellballen
  • nested in a ball of cells
  • Rich vascular network surrounding zellballens
  • salt and pepper nuclei
  • may have scattered bizzare (pleomorphic) nuclei
A

Pheochromocytoma

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3
Q

10% tumor:

  • 10% are bilateral
  • 10% are paragangliomas
  • 10% in children
  • 10% are malignant
  • 10% are familial (MEN2A or 2B or NF-1)

Combination of adrenal mass on imaging with elevated urine and/or serum catechomalines or their metabolites

  • VMA support the diagnosis
A

Pheochromocytoma

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4
Q

Gross:

  • unilateral and solitary
  • classically bright yellow in color

Microscopically:

  • Often unencapsulated, mixture of growth patterns (nests, cords, solid)
  • Most cells have vesicular/vacuolated clear cytoplasm (contains lipid) with scattered cells exhibiting eosinophilic cytoplasm
  • DO NOT see mitoses or necrosis
A

Adrenal cortical adenoma

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5
Q

Gross:

  • brown to gray color
  • very thin
  • 2 mm thick

Microscopically composed of:

  • pheochromocytes
  • sustentacular cells
  • nerves and ganglion cells
A

Adrenal medulla

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6
Q

Tumor of the adrenal medulla pheochromocytes that has metastasized 10% of pheochromocytomas

A

malignant pheochromocytoma

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7
Q

Benign tumor of adults

Two components:

  • myelo: bone marrow elements
  • lipoma: mature adipose

Grossly:

  • red (marrow)
  • yellow (fat)
A

Adrenal myelolipoma

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8
Q

Can be epithelial, endothelial, or parasitic

Involve the cortex and/or medulla

A

Cystic lesions

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9
Q

> 100 g and > 6.5 cm

A

Adrenal cortical carcinoma in adult

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10
Q

Prototypical example of primary (acute) adrenal insufficiency

Occurs in the setting of an overwhelming bacterial infection

  • Neisseria meningitidis –> sepsis

Characterized by hypotension, shock, and widespread purpura of the skin

Patients have rapidly developing adrenal insufficiency associated w/ massive bilateral adrenal hemorrhage

A

Waterhouse-Friderichsen Syndrome

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11
Q

Gross:

  • large, firm, gray to white
  • hemorrhage, necrosis, cystic changes

Microscopically:

  • small cells with hyperchromatic (dark) nuclei
  • very little cytoplasm
  • high mitotic rate and abundant apoptotic nuclear debris *** small round blue cell tumor
  • stroma made up of pink fibrillary material
  • neuropil
  • Homer-Wright pseudorosettes
A

Neuroblastoma

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12
Q

> 400 g and > 10.5 cm

A

Adrenal cortical carcinoma in child

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13
Q

Normal adrenal cortical tissue in an abnormal location

A

Adrenal cortical heterotopia

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14
Q

Most ___ are sporadic and are not associated with inherited syndromes

A

Adrenal cortical neoplasms

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15
Q

Catecholamine secreting tumor arising from the adrenal medulla composed of neoplastic chromaffin cells

Most common in 5th decade of life

Symptoms:

  • HTN
  • HA
  • Anxiety
  • Palpitations
  • Nausea
  • Diaphoresis
A

Pheochromocytoma

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16
Q

Malignant tumor composed of neural crest derived neuroblastic cells

Relatively common malignancy in CHILDREN - 6% of childhood malignancies

Present as an abdominal pass with pain and catecholamine metabolites in urine

  • VMA and HVA
A

Neuroblastoma

17
Q

Zone closest to the adrenal capsule (outer most zone)

Cells produce mineralcorticoids (15% of cortical volume)

Cells are arranged in clusters, high nuclear to cytoplasm ratio versus other zones

  • cytoplasm is pink to purple
  • cytoplasm is finely vaculated
A

Zona glomerulosa

18
Q

Stroma in a classic neuroblastoma is composed of pink fibrillary material called ___

Composed of neurotic processes of the primitive neuroblasts

Surrounded by Homer-Wright pseudorosettes

A

Neuropil

19
Q

Is it possible to tell whether a tumor is functional or nonfunctional from microscopic morphology?

A

No

20
Q

Gross:

  • large bulk tumors are typical
  • hemorrhage and necrosis are common –> invade liver, kidney, pancreas

Microscopic:

  • Highly cellular tumors
  • Arranged in variable patterns (solid, gland-like, nested)
  • Increased nuclear to cytoplasmic ratio
  • clear to pink cytoplasm
  • mitotic activity present
  • atypical mitoses may be present
A

Adrenal cortical carcinoma

21
Q

Cell types are prominent in the adrenal medulla

Presence is critical in the process of stimulating release of catecholamines by the pheochromocytes

A

Nerves and ganglion cells

22
Q

Spindle shaped support cells

Use immunoperoxidase staining with Abs to S100 to highlight these cells

Exact function unknown

A

Sustentacular cells

23
Q

Also known as medullary or chromaffin cells

Main cell type of the mature adrenal medulla

Synthesize and secrete the main product

  • catecholamines

Microscopically:

  • large size
  • abundant blue cytoplasm
  • salt and pepper nuclei with occasional prominent nucleoli
  • arranged in clusters and supported by a rich fibrovascular network
A

Pheochromocytes

24
Q

Gross:

  • solitary, unilateral mass
  • May be calcified, well-circumscribed, firm, tan-yellow, often large

Microscopically:

  • Mixture of mature schwannian stroma with interspersed ganglion cells
A

Ganglioneuroma

25
Q

Pattern of hyperplasia

Glands appear enlarged but the enlargement is characterized by multiple nodules < 1.0 cm in greatest dimension

Nodules lack a capsule 30-40% of Cushing syndrome cases

A

Micronodular pattern of hyperplasia

26
Q

Most common cause of primary chronic adrenal insufficiency

Produce antibodies to cortical cells

  • most commonly cytochrome p450 enzymes

Adrenal glands appear atrophic

Microscopically:

  • cortex is remarkable for atrophy
  • prominent infiltrate of lymphocytes and plasma cells
A

Addison Disease (autoimmune adrenalitis)

27
Q

Fairly common, 1-5% of the general population

80% are non-functional, 20% are functional

  • aldosterone-producing (Conn Syndrome)
  • glucocorticoid-producing (Cushing Syndrome)
  • w/ mixed endocrine syndrome or associated virilizing/feminizing (rare)

Benign neoplasms with excellent prognosis after surgical removal

A

Adrenal cortical adenoma

28
Q

Uncommon pattern of hyperplasia

Glands appear enlarged but the enlargement is characterized by multiple nodules some of which measure > 1.0 cm in greatest dimension

A

Macronodular pattern of hyperplasia

29
Q

Histopathologic criteria to determine if an adrenal cortical neoplasm is benign or malignant

  1. Pink cytoplasm
  2. Difference pattern
  3. Necrosis
  4. High nuclear grade
  5. >5 mitoses/50 HPF
  6. Atypical mitosis
  7. Venous invasion
  8. Capsular invasion
  9. Extraadrenal invasion

>3 criteria –> malignancy

A

Weiss Criteria

30
Q

Benign tumor of neural crest derivation composed of mature ganglion cells and schwannian (neural derived) stroma

Embryonal tumors (neuroblastic) of the sympathetic nervous system

  • most common site is posterior mediastinum 20% of cases occur in the adrenal medulla
A

Ganglioneuroma

31
Q

Pattern of hyperplasia

Glands appear symmetrically enlarged grossly

Most common pattern seen in congenital adrenal hyperplasia and Cushing Syndrome

A

Diffuse pattern of hyperplasia

32
Q

Li Fraumeni Syndrome

Carney complex

MEN1 (Wermer Syndrome)

Beckwith-Wiedemann

A

Inherited syndromes that predispose patients to adrenal cortical neoplasms

33
Q

Middle zone

Cells produce glucocorticoids (70% of cortical volume)

Cells are arranged in columns that are perpendicular to the capsule

A

Zona fasciculata

34
Q

Bilateral increase in adrenal cortical mass d/t a documented endocrine abnormality

Diagnosis cannot be made on pathological exam alone, one must correlate with clinical presentation and other lab tests

A

Adrenal cortical hyperplasia

35
Q

Renal cell carcinoma, lung carcinoma

  • most common
  • renal: CA9
  • lung: TTF1 HCC, colon, breast cancer
A

Common primary sites of metastasis

36
Q

Important cause of secondary adrenal insufficiency

Adrenal glands are frequently involved d/t cancer of the lung, breast, and kidney

A

Metastatic carcinoma

37
Q

Histologically identical to pheochromocytomas

Secrete catechomaines at other sites

  • sympathetic ganglia
A

Paragangliomas

Endocrine (26 decks)

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