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Patho 2 TOPIC: adrenal gland tumors

Surgical pathology
Topic: adrenal gland tumors

TUMOR EPIDEMIOLOGY ETIOLOGY PATHOGENESIS CLINICAL MANIFESTATION MORPHOLOGY

Adrenal myelolipoma mixture of mature Not clearely understood unknown Asymptomatic composed of macroscopic fat and
(benign) adipose tissue and When large: Symptoms include pain in mature hematopoietic tissue,
hematopoietic marrow the abdomen or flank, blood in the urine, resembling bone marrow.
and is notable for its a palpable lump or high blood pressure.
occasional large size. There is a mixture of normal
adrenal tissue, fat, and a full
trilineage maturation of the three
major blood-
forming elements: myeloid (white
blood cell forming), erythroid (red
blood cell forming),
and megakaryocytic (platelet
forming) lines
Adrenal cysts predisposing factors for adrenal When it becomes large it can impinge near by When large: significant for poorly controlled revealed bloody proteinaceous
pseudocysts that occur as a noted to occur from birth pseudocysts include trauma, structure in the retroperitoneal area such as kidney hypertension, chronic back pain, arthritis, fluid with hypocellularity and no
result of degenerative to 80 years of age, with a infection, anoxia in infants, palpitations, and anxiety disorder. malignancy.
changes in benign adrenal peak incidence in the hemorrhagic diathesis, vascular
tumors or resolution of third to sixth decades of aneurysm, or embolism with
haemorrhage they represent life; subsequent infarction.
remnants of an underlying
vascular lesion.
Pheochromocytomas more frequent in part of hereditary syndromes, Associated to medical syndromes with genetic Hypertension, sustained or episodic, is the key
women than in men. such as MEN types 2A and 2B defect and produces a tumor that secretes high symptom.
associated with von Hippel- amounts of catecholamines,  asymptomatic hypertension
They occur at any age, Lindau disease, mostly norepinephrine, plus epinephrine to a  symptomatic hypertension resistant to
including infancy, but neurofibromatosis type 1 and lesser extent antihypertensive therapy
are uncommon after 60 McCune-Albright syndrome.  malignant
years of age.  hypertension (e.g., encephalopathy,
Rare Catecholamine-
Secreting Tumors of papilledema, proteinuria),
80% are unilateral,  myocardial infarction or aortic dissection
Chromaffin Cells of the encapsulated, spongy, reddish
10% are bilateral and  paroxysms of convulsions, anxiety or
Adrenal Medulla masses, with prominent central
10% occur in extra- hyperventilation. scars, haemorrhage and foci of
adrenal locations; 10%
(malignant) cystic degeneration
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Transcribed by: John Velasco - #makingEAC-SMgreatAgain #batch2019 Taste and see that the LORD is good; blessed is the one who takes refuge in him.- Psalm 34:8
Patho 2 TOPIC: adrenal gland tumors

are malignant and 10% with severe throbbing headache, sweating,


occur in children palpitations, tachycardia, abdominal pain and circumscribed nests (zellballen)
vomiting. of neoplastic cells are present.
two thirds of tumors Tumor cells range from
occurring in the context cardiac complications are attributed to myocardial polyhedral to fusiform, with
of MEN (see below) are necrosis caused by elevated catecholamine levels granular, amphophilic or
bilateral. (catecholamine cardiomyopathy) basophilic cytoplasm and
vesicular nuclei
Tumor: Most are 5 to 6 diagnostic: Increased urinary levels of
cm in diameter and catecholamine metabolites, particularly Eosinophilic cytoplasmic globules
weigh 80 to 100 g. vanillylmandelic acid (VMA), metanephrine and are seen. Cellular pleomorphism is
unconjugated catecholamines, often prominent and may include
multinucleated tumor giant cells
associated to MEN2A: RET protooncogene
on chromosome 10q11.2 is responsible + neuron-specific enolase,
for MEN2 syndromes chromogranin and
synaptophysin.
Carotid body tumor form palpable masses germline mutation in a gene SDHD gene (11q23), which encodes a subunit of
 Hoarseness. zellballen” growth pattern
(benign) in the neck. encoding a mitochondrial cytochrome B that has been proposed to  Difficulty swallowing. well-developed nested or
Prototypical protein. participate in oxygen sensing. organoid growth pattern of the
 Partial paralysis or numbness in the tongue.
paragangliomas, arising at 10 times more frequent tumor cells with an intervening
the carotid bifurcation. in people living at high  Weakness or pain in the shoulders. stromal component of delicate
altitude than in those at  Vision changes, or a drooping eyelid. fibrovascular tissue and
sea level,  High blood pressure or heart palpitations. supporting cells or
“sustentacular” cells at the
Autosomal dominant periphery of the zellballen or cell
transmission nests.

About 10% of these


tumors can be malignant
and metastasize to
distant organs
such as lung and bone.
Neuroblastoma DNA ploidy: N-myc short arm of chromosome 16 appears Neuroblastoma (Schwannian stroma poor)
embryonal malignancy of It originates in the amplification occurs in 20% to to be the affected locus. NBs may occur with ■ Ganglioneuroblastoma, intermixed
neural crest origin adrenal medulla, 25% of cases, deletion of neurofibromatosis type 1, Beckwith-Wiedemann (Schwannian stroma
composed of neoplastic paravertebral chromosome 1p Allelic gain of syndrome and rich)
neuroblasts sympathetic ganglia and 17q is associated with more Hirschsprung disease ■ Ganglioneuroma (Schwannian stroma
sympathetic aggressive tumors. dominant)
paraganglia. Deletions on chromosome 1 (1p35- ■ Ganglioneuroblastoma, nodular (composite
36) are frequent, with unbalanced translocation Schwannian
arise from primitive with 17q. stroma rich/stroma dominant and stroma poor) Dense sheets of small, round to
sympathogonia fusiform cells with dark nuclei and
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Transcribed by: John Velasco - #makingEAC-SMgreatAgain #batch2019 Taste and see that the LORD is good; blessed is the one who takes refuge in him.- Psalm 34:8
Patho 2 TOPIC: adrenal gland tumors

The HSRs represent amplification of N-myc; this scant cytoplasm, discloses a firm,
Neuroblastomas are abnormality is key in determining the Spinal cord compression may lead to gait irregular, nontender mass.
the most common solid aggressiveness of neuroblastoma. disturbance and sphincter dysfunction.
extracranial neoplasms The cut surface is soft and friable,
of childhood, accounting Severe diarrhea may be caused in tumors with a variegated maroon color.
for up to 10% of all secreting vasoactive intestinal peptide Areas of necrosis, hemorrhage,
childhood cancers and calcification and cystic change are
15% of cancer deaths in The urine contains increased amounts of often present.
children. norepinephrine, VMA,
homovanillic acid (HVA) and dopamine. Homer Wright rosettes

Survival is 90% in stage I Low-grade (better-differentiated)


decreases to less than 3% in stage IV tumors have better prognoses
than high-grade
(undifferentiated)
tumors.

If the VMA/HVA ratio is less than


1, the tumor is deficient in
dopamine _-hydroxylase and
likely to be more aggressive.
Ganglioneuromas older children and genetic problems, such as Unknown No symptoms, only noticed with other underlying Mature ganglion cells (arrow)
Mature Variants of young adults neurofibromatosis type 1 condition interspersed among wavy spindle
Neuroblastic Tumors cells embedded in a myxoid
neural crest tumor 30% occur in the adrenal matrix.
medulla.
Ganglioneuromas are Ganglioneuromas do not well encapsulated with myxoid,
benign and arise in show the chromosomal glistening, cut surfaces.
sympathetic ganglia, abnormalities neuron-specific enolase and
typically in the posterior characteristic of NB. certain peptide hormones, are
mediastinum. detectable

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Transcribed by: John Velasco - #makingEAC-SMgreatAgain #batch2019 Taste and see that the LORD is good; blessed is the one who takes refuge in him.- Psalm 34:8