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Tumor of endocrine organ

Dr. Muhartono, M.Kes, Sp.PA


Tumor of the pituitary gland
The vas majority of neoplasms located in
sella turcica are benign pitutary adenoma
derived from adenohypophysial cells
In many cases, they can be succesfully
removed by minimally invasive
transspenoidal surgery
Hoever their biology is complex and they
can cause a variety of endocrin syndrome
and disorders.
Pitutary tumor
Difinition:
Terletak pada sela tursica
mayoritas pitutary adenoma
Sel-sel adenohypofise
Sedikit: pitutary carcinoma

WHO Clasification
Pitutary adenoma
Typical adenoma 8272/0
Atypical adenoma 8272/1
Pitutary carcinoma 8272/3
Epidemiology
Pitutary adenoma
10-15% dari intracranial neoplasia
27% autopsi
Jarang pada anak-anak 2% dari pitutary
adenoma
Clinical features
Pitutary adenoma hormone excess
(PRL, GH, TSH, ACTH, FSH, LH)
Mass effect intracranial mass
Headeche
Loss of normal anterior pitutary hormon
production
Visual field disturbance
Pemeriksaan
imaging
Histopatology
Dibedakan menjadi
Acidofilik
Basofilik
Chromophobe
Mayoritas monomorphic proliferation
Mitosis jarang
Ki67/MIB-1 <3%
P53+

Dengan penemuan imunohistokimia,
hormon profile, gambaran ultrastruktur
maka tumor ini diklasifikasikan lebih detail
GH adenoma;PRL;TSH;
ACTH;gonadotropin; null; plurihormonal;
pitutary carcinoma
Growth patterns
Expansive growth
Tumor dikelilingi jaringan ikat, biasanya tidak
berkapsul
Invasive growth
Melibatkan tulang, saraf, pembuluh darah,
duramater
Pitutary carcinoma
Metastasis craniospinal/sistemik
etiology
Pitutary tumourigenesis
Oncogen
Ras
Cyclin D1
Tumor suppressor gene
Men1
RB
TP53
P16
GADD45
Therapy
Surgical
Medical
radiation
Surgical
Transsphenoidal minimal invasive
Indikasi
Tumor with progressive mass efect, e.g visual loss
Hyperfunction: chusing disease, achromegaly,
hypertyroidisme
Failure of prior treatment such as medical teraphy
Massive acute haemorrhagic necrosis of adenoma
Medical treatment
Dopamin agonist PRL
Somatostatin GH, TSH
GH antagonist
Radiation teraphy
Preop
Incomplet
recurrent
Tumor of the tyroid
Papillary carcinoma
Follicular carcinoma
Poorly diferentiated carcinoma
Undifferentiated carcinoma
Medulary carcinoma

Follicular adenoma
Papilary carcinoma
Difinition
A malignant epithelial tumor showing
evidence of follicular cell differentiation
and caracterized by distinctive nuclear
features.
Epidemiology
Paediatric thyroid malignancy
20-50 tahun
Female: male 4:1
Execellent > 90% khususnya pasien <45
tahun

incidence
Tertinggi diantara karsinoma tyroid

etiology
Radiation exposure
localization
Tyroid
Ectopic thyroid tissue struma ovarii
Clinical features
Massa tyroid
Cervical lymphadenopaty
Tyroid function test
Tidak membantu diagnosis
TSH, T3,T4
Fine needle aspiration biopsy
Sitologi
Kelompokan sel sel atipik yang tersusun
papil-papil dengan inti powdery
chromatin, thick nuclear membran,
pseudoinclusion, nuclear groove,
multinucleated giant cell
Psamoma bodies

Imaging
Ultrasound
Radioactive iodine scans
CT scan
MRI

macroscopy
Massa abu-abu putih
Batas tidak tegas
Infiltrasi ke organ parenchym
Dystrophic calcification
Bone formation
Cystic change
Tyroglossal duct cyst dapat muncul
Nodal metastasis
Fat, sceletal muscle, oesofagus, larynx dan
trachea



Tumor spread
Lymmphatic chanel regional nodal
metastasis
Walaupun jarang venous invasion dapat
terjadi


Stage grouping
< 45
>45
< 45
Stage I
Any T any N M0
Stage II
Any T any N M1

> 45 tahun
Stage I
T1 N0 M0
Stage II
T2 N0 M0
Stage III
T3N0M0;T1-3N1aM0
Stage IV
T4aN0-1aM0; T1-3N1bM0
T1 <2
T2 2-4
T3 >4
T4a : menyebar melebihi kapsul;subcutan
tissue, larynx, trachea, esofagus recurrent
laryngeal nerve
T 4b; prevertebral fascia; a. carotis; v.
mediastinal
N0 belum
N1 regional node metastasis
N1a pretracheal, paratracheal,
prelaryngeal
N1b cervical; superior mediastinal

M0 belum
M1 sudah metastasis jauh
Histopatology
Ground glass appearance
Nuclear groove
Pseudoinclusion

Papillary arcitecture
Metaplasia squamosa
Psammoma bodies




imunnochemistry
Cytokeratin
Tyroglobulin and tyroid transcription factor-
1 (TTF-1)
Variant
Follicular variant
Macrofollicular variant
Oncocytic variant
Clear cell variant
Difuse sclerosing variant
Tall cell variant
Columnnar cell variant
Solid variant
Grading
Well diffferentiated
Tumor necrosis, vascular invasion,
mitosis, inti atipia prognosis yang buruk
Histogenesis
Follicular cell
Somatic genetic
RET/PTC rearrangement 10q11.2
Prognostic and predictive factor
10ysr 90%98%untu pasien muda
Usia
Stadium
Variant tall cell dan columnar buruk.

Follicullar carcinoma
Keganasan tyroid yang menunjukkan
deferensiasi sel-sel folikel.
Tanda:
Invasi ke kapsul
Invasi ke pembuluh darah
Imunohistokimia
Reaktif terhadaf
Tyroglobulin
TTF-1
Derajat invasi
Minimally
widely
Metastasis
Tulang tengkorak
Tulang panjang
Tulang vertebra
Paru-paru
dll
Poorly differentiated
Insular carcinoma
Usia lebih tua dari kelompok well
differentiated
Groosly invasive
Insular pattern
Periteliomatoiu
s pattern
Imunohistokimia
Reaktif: tyroglobulin dan TTF-1
Tidak calcitonin
TTF-1
trabecular

Mikroskopis
Insular pattern
Peritheliomatous pattern
Undifferentiated carcinoma
=anaplatik
Tua
Gejala: hoarseness, dysphagia, dyspneu
Gross: highly necrotic and hemorrhagic
solid tumor

type
Spindel
Giant cell


Kebanyakan dari transformasi dari tipe
well differentiated:
Papillary ca
Follicular ca
Poorly diff ca
Harapan hidup 6 bulan struktur vital
pada leher
Medullary carcinoma
Keganasan berasal dari sel C
= solid carcinoma; hialin carcinoma; C-cell
carcinoma

Solid proliferation of round to polygonal
cells of granular amphophilic cytoplasm
and medium nucleus
Dipisahkan highly vascular stroma,
kolagen dan amyloid



Imunohistokimia
calcitonin.TTF-1, chromogranin +,CEA

CEA+
bentuk
Sporadic 80% 45 tahun
Familial 20% 35 tahun
Dihubungkan dengan MEN IIA/IIB
RET mutation

Penyebaran
Kgb leher dan mediastinum
Paru, liver, skeletal

Pengobatan
Tyroidectomy+ cervical lymphadenectomy

Lokal ruccerent 35%
5ysr 70-80%

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