Pituitary AdenomasPituitary Adenomas

SMSSMS 30233023

Dr. Mohanad r. alwanDr. Mohanad r. alwan

FunctionFunction

Anterior Lobe:Anterior Lobe: FSHFSH LHLH ACTHACTH TSHTSH ProlactinProlactin GHGH

Posterior Lobe:Posterior Lobe: ADHADH OxytocinOxytocin

Pituitary Adenomas/ Pituitary Adenomas/ EpidemiologyEpidemiology

Etiology is unknownEtiology is unknownNot associated with environmental factorsNot associated with environmental factors10-15% of all primary brain tumors10-15% of all primary brain tumors20-25% of pituitary glands at autopsy 20-25% of pituitary glands at autopsy found to have adenomasfound to have adenomas70% of adenomas are endocrinogically 70% of adenomas are endocrinogically secretingsecreting25% of those with MEN-I develop pituitary 25% of those with MEN-I develop pituitary adenomasadenomas

Natural HistoryNatural History

Pituitary adenomas have long natural Pituitary adenomas have long natural historyhistory

Vary in size and direction of spreadVary in size and direction of spread

Microadenomas < 10 mm – may cause Microadenomas < 10 mm – may cause focal bulgingfocal bulging

Macroadenomas > 10 mm – cause Macroadenomas > 10 mm – cause problems due to mass effectproblems due to mass effect

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Clinical Features of Pituitary TumorsClinical Features of Pituitary Tumors

HYPER: HYPER: Growth, lactation, thyroid, adrenal cortex.Growth, lactation, thyroid, adrenal cortex.

HYPO: HYPO: Growth, thyroid, adrenal cortex.Growth, thyroid, adrenal cortex.

MASS EFFECT: MASS EFFECT: Visual fields, brain Visual fields, brain

bitemporal hemianopsia and superior temporal defectsbitemporal hemianopsia and superior temporal defectsPituitary Apoplexy.Pituitary Apoplexy.

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Types of adenoma:Types of adenoma:

- - Non-functional 25-35%Non-functional 25-35%

--Prolactinoma Prolactinoma (Prolactin > 200 ng /dl) (N<14 ng/ml). (Prolactin > 200 ng /dl) (N<14 ng/ml). 25-30%25-30%

-FSH + LH releasing adenoma. -FSH + LH releasing adenoma.

-ACTH adenoma -ACTH adenoma 10-15%10-15%

-GH adenoma -GH adenoma 20-25% 20-25%

Mixed 5%Mixed 5%

-TSH adenoma. -TSH adenoma.

Hyperpituitarism and Adenomas of Hyperpituitarism and Adenomas of PituitaryPituitary

Endocrine-Active Pituitary Endocrine-Active Pituitary AdenomasAdenomas

Prolactin Prolactin – Amenorrhea, galactorrhea, impotence– Amenorrhea, galactorrhea, impotence

Growth hormone Growth hormone – Gigantism and acromegaly– Gigantism and acromegaly

CorticotropinCorticotropin – Cushing’s disease, Nelson’s – Cushing’s disease, Nelson’s syndrome post adrenalectomysyndrome post adrenalectomy

TSH TSH - Hyperthyroidism- Hyperthyroidism

Non-functioning AdenomasNon-functioning Adenomas

25-30 % of patients do not have classical 25-30 % of patients do not have classical hypersecretory syndromeshypersecretory syndromes

May grow to a large size before they are May grow to a large size before they are detecteddetected

Present due to mass effectPresent due to mass effect Visual deficitsVisual deficits HAHA Hormone deficiencyHormone deficiency

Pathologic ClassificationPathologic Classification

Benign or malignantBenign or malignant

Chromophobic – Non-functioningChromophobic – Non-functioning

Basophilic – Cushing’s Basophilic – Cushing’s

Acidophilic - AcromegalyAcidophilic - Acromegaly

MixedMixed

Pituitary adenomasPituitary adenomas

Microadenomas: Microadenomas: benign neoplasms < 1 benign neoplasms < 1 cm, found in 25% of autopsies, 50% are cm, found in 25% of autopsies, 50% are PRL secreting.PRL secreting.

Histo: Histo: sheets and cords of uniform, sheets and cords of uniform, polygonal cells with sparse connective polygonal cells with sparse connective tissue. tissue.

Functional status can not be predicted Functional status can not be predicted from morphology.from morphology.

Adenoma cells Adenoma cells are small to are small to medium-sized medium-sized containing containing

moderate to abundant amounts moderate to abundant amounts of finely of finely granular cytoplasm granular cytoplasm

Round to oval nuclei showing Round to oval nuclei showing finely-stippled chromatin finely-stippled chromatin and and indistinct to small nucleoli.indistinct to small nucleoli.

The cytoplasm can be The cytoplasm can be eosinophilic, basophiliceosinophilic, basophilic, , acidophilic, or chromophobe acidophilic, or chromophobe recapitulating normal pituitary recapitulating normal pituitary cell types. cell types.

This tumor is arranged in This tumor is arranged in pseudo-acinarpseudo-acinar formations with a formations with a central lumen-like space central lumen-like space (arrowhead). (arrowhead).

Tumor Tumor necrosisnecrosis is generally not is generally not present present unless infarction unless infarction develops develops because of rapid growth. because of rapid growth.

You will see clusters and cords of the tumor You will see clusters and cords of the tumor cells, and it may be tricky to distinguish the cells, and it may be tricky to distinguish the tumor from the surrounding normal pituitary.tumor from the surrounding normal pituitary.

Pituitary adenome with extensive interstitial and perivascular Pituitary adenome with extensive interstitial and perivascular fibrosis.fibrosis.

SymptomsSymptoms

HeadacheHeadache

LethargyLethargy

Nasal drainageNasal drainage

Nausea and vomitingNausea and vomiting

Problems with the sense of smellProblems with the sense of smell

Visual changesVisual changes Double visionDouble vision Drooping eyelidsDrooping eyelids Visual field lossVisual field loss

Tumor StagesTumor Stages

Type A: Type A: Tumor bulges into the chiasmatic cisternTumor bulges into the chiasmatic cisternType B: Type B: Tumor reaches the floor of the 3Tumor reaches the floor of the 3rdrd ventricleventricleType C: Type C: Tumor is more voluminous with Tumor is more voluminous with extension into the 3extension into the 3rdrd ventricle up to the foramen ventricle up to the foramen of Monroof MonroType D: Type D: Tumor extends into temporal or frontal Tumor extends into temporal or frontal fossafossa

EvaluationEvaluation

MRIMRI

Visual field assessmentVisual field assessment

Endocrine evaluationEndocrine evaluation Tests of normal gonadal, thyroid, and adrenal Tests of normal gonadal, thyroid, and adrenal

functionfunction Radioimmunoassays – for hormone levelsRadioimmunoassays – for hormone levels

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Prolacinomas Prolacinomas

Present in Present in ::Abnormal milk flow from the breast in a woman who is not pregnant or nursing (galactorrhea)

Breast tenderness

Decreased sexual interest

Headache

Infertility

Stopping of menstruation not related to menopause

Vision changes

Prolacinomas Prolacinomas

Present in Present in : : Decreased sexual interest

Enlargement of breast tissue (gynecomastia)

Headache

Impotence

Infertility

Vision changes

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A- The tumor consists of multiple densely irregular sheets cellular micronodular structures with disruption of normal pituitary sinusoidal vasculostromal network. (see arrow)

Multinucleation and discrete cytoplasmic accumulations of finely granular eosinophilic material (secretory granules) displacing the nucleus at the periphery

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Benign tumor Benign tumor from ( Rathke's from ( Rathke's cleft cleft – source of– source of

adenohypophysis).adenohypophysis).

-Suprasellar expansion.-Suprasellar expansion.

-Often cystic, similar structure as adamantinoma -Often cystic, similar structure as adamantinoma

of the jaws.of the jaws.

CraniopharyngCraniopharyngiiomaoma

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Gross picture:Gross picture:

Large capsulated solid or cystic mass.Large capsulated solid or cystic mass.

Areas of calcification.Areas of calcification.

Microscopic picture:Microscopic picture:

Masses of squamoid and columnar epithelium Masses of squamoid and columnar epithelium

lining cystic spaces filled with oily fluid in a loose lining cystic spaces filled with oily fluid in a loose

fibrous stroma.fibrous stroma.

CraniopharyngCraniopharyngiioma.oma.

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CraniopharyngCraniopharyngiioma.oma.

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CraniopharyngCraniopharyngiioma.oma.

Multiple endocrine neoplasia (MEN) IMultiple endocrine neoplasia (MEN) I

Multiple endocrine neoplasia (MEN) type I is an inherited Multiple endocrine neoplasia (MEN) type I is an inherited disorder in which one or more of the endocrine glands are disorder in which one or more of the endocrine glands are overactive or form a tumor. Endocrine glands most overactive or form a tumor. Endocrine glands most commonly involved include:commonly involved include:PancreasPancreas

ParathyroidParathyroid

PituitaryPituitary

CauseCause

MEN I MEN I is caused by a defect in a gene that carries the code is caused by a defect in a gene that carries the code for a protein called menin. for a protein called menin.

The disorder may occur at any age, and it affects men and The disorder may occur at any age, and it affects men and women equally. A family history of this disorder raises your women equally. A family history of this disorder raises your risk.risk.

SymptomsSymptoms Abdominal pain, Bloated feeling after meals, Black, tarry stoolsAbdominal pain, Bloated feeling after meals, Black, tarry stools AnxietyAnxiety Burning, aching, or hunger discomfort in the upper abdomen or lower Burning, aching, or hunger discomfort in the upper abdomen or lower

chest that is relieved by antacids, milk, or foodchest that is relieved by antacids, milk, or food Decreased sexual interest, Decreased sexual interest, Fatigue, WeaknessFatigue, Weakness Lack of menstrual periods, infertility, or failure to produce breast milk (in Lack of menstrual periods, infertility, or failure to produce breast milk (in

women)women) Loss of appetite, Loss of body or facial hair (in men)Loss of appetite, Loss of body or facial hair (in men) Loss of coordinationLoss of coordination Mental changes or confusionMental changes or confusion Headache, Muscle painHeadache, Muscle pain Nausea and vomiting, Unintentional weight lossNausea and vomiting, Unintentional weight loss Sensitivity to the coldSensitivity to the cold Vision problemsVision problems Coma (if low blood sugar is untreated), High blood calcium levelComa (if low blood sugar is untreated), High blood calcium level Kidney stones, Low blood pressure, Low blood sugarKidney stones, Low blood pressure, Low blood sugar

Blood TestsBlood Tests

Calcitonin levelCalcitonin level

Blood alkaline phosphataseBlood alkaline phosphatase

Blood calciumBlood calcium

Blood phosphorusBlood phosphorus

Urine catecholaminesUrine catecholamines

Urine metanephrineUrine metanephrine

TreatmentTreatment

The method of treatment depends on the type and size of The method of treatment depends on the type and size of the tumorthe tumor

Observation and medication Observation and medication

SurgerySurgery Transphenoidal endoscopic tumor removalTransphenoidal endoscopic tumor removal Transcranial tumor removal Transcranial tumor removal

Radiation TreatmentRadiation Treatment Stereotactic radiosurgery Stereotactic radiosurgery External-beam radiation External-beam radiation

Hormone TherapyHormone Therapy

Question?????Question?????

72 year old male inpatient recently admitted for 72 year old male inpatient recently admitted for fatigue and hyponatremia who complains of fatigue and hyponatremia who complains of progressively worsening vision in both eyes over progressively worsening vision in both eyes over the past few years, also he had hypertension, the past few years, also he had hypertension, hypercholesterolemia, alcohol abuse, chronic hypercholesterolemia, alcohol abuse, chronic pancreatitis. Regarding to his cause:pancreatitis. Regarding to his cause:

What is your more likely diagnosis. Why? What is your more likely diagnosis. Why?

Histopathology of these disease.Histopathology of these disease.

Mention the important blood test which used to Mention the important blood test which used to confirm the disease. confirm the disease.