TUMOR DAERAH LEHERTUMOR DAERAH LEHER

KISTA DUKTUS TIROGLOSUSKISTA DUKTUS TIROGLOSUS

SISA PROSES PERTUMBUHAN SISA PROSES PERTUMBUHAN KEL. TIROID.KEL. TIROID.

SERING DITEMUKAN PADA USIASERING DITEMUKAN PADA USIA

2 – 7 TAHUN2 – 7 TAHUN

LOKASI, MID LINE ,KRANIAL TIROIDLOKASI, MID LINE ,KRANIAL TIROID

HINGGA PANGKAL LIDAHHINGGA PANGKAL LIDAH

KLINISKLINIS

DAPAT BERUPA KISTA, SINUS DAN DAPAT BERUPA KISTA, SINUS DAN FISTULA.FISTULA.

BILA BERHUBUNGAN DENGAN RONGGA BILA BERHUBUNGAN DENGAN RONGGA MULUT SERING INFEKSIMULUT SERING INFEKSI

> 75%, DIDAPATI HUBUNGAN DENGAN > 75%, DIDAPATI HUBUNGAN DENGAN PANGKAL LIDAH, SEHINGGA BILA PANGKAL LIDAH, SEHINGGA BILA DISURUH MENJULURKAN LIDAH MASSA DISURUH MENJULURKAN LIDAH MASSA IKUT BERGERAK KE ATAS. IKUT BERGERAK KE ATAS.

KLINISKLINIS

BISA TERJADI RADANG AKUTBISA TERJADI RADANG AKUT BILA LOKASI DEKAT PANGKAL LIDAH BILA LOKASI DEKAT PANGKAL LIDAH

DAN SANGAT BESAR DAPAT DAN SANGAT BESAR DAPAT MENIMBUKAN, DISPAGIA, DISFONI, MENIMBUKAN, DISPAGIA, DISFONI, SESAK NAFAS, PERDARAHAN DAN SESAK NAFAS, PERDARAHAN DAN SAKIT.SAKIT.

FISTULA DAPAT TERJADI SPONTAN FISTULA DAPAT TERJADI SPONTAN ATAU AKIBAT INSISI ABSES,ATAU AKIBAT INSISI ABSES,

DIFERENSIAL DIAGNOSADIFERENSIAL DIAGNOSA

PEMBESARAN KEL. GETAH BENINGPEMBESARAN KEL. GETAH BENING KISTA DERMOIDKISTA DERMOID PANGKALLIDAH:LINGUALTONSIL, PANGKALLIDAH:LINGUALTONSIL,

FIBROMA, ANGIOMA, SARKOMA / FIBROMA, ANGIOMA, SARKOMA / CARSINOMA LIDAH CARSINOMA LIDAH

PATOLOGIPATOLOGI

DAPAT DIJUMPAI JAR. TIROIDDAPAT DIJUMPAI JAR. TIROID EPITEL, BISA SKUAMOSA DAN EPITEL, BISA SKUAMOSA DAN

KOLUMNAR KOLUMNAR

TERAPITERAPI

INFEKSI AKUT, KOMPRES LOKAL DAN INFEKSI AKUT, KOMPRES LOKAL DAN PEMBERIAN ANTI BIOKTIKA.PEMBERIAN ANTI BIOKTIKA.

ABSES, DRAINASEABSES, DRAINASE MASSA, EKSISI.MASSA, EKSISI. OS HYOID HARUS IKUT DIEKSISI.OS HYOID HARUS IKUT DIEKSISI. EN BLOK SAMPAI PANGKAL LIDAH. EN BLOK SAMPAI PANGKAL LIDAH.

KISTA BRANCHIALKISTA BRANCHIAL

KELAINAN DAPAT BERUPA, SINUS, KELAINAN DAPAT BERUPA, SINUS, KISTA, SISA TULANG RAWAN & FISTEL KISTA, SISA TULANG RAWAN & FISTEL DILEHER.DILEHER.

SISA CELA KANTUNG INSANG .SISA CELA KANTUNG INSANG . DILAPISI SEL EPITEL SKUAMOSA.DILAPISI SEL EPITEL SKUAMOSA.

KLINISKLINIS

SINUS & FISTEL DIJUMPAI ANTERIOR SINUS & FISTEL DIJUMPAI ANTERIOR DARI M STERNOKLAIDOMASTOIDEUS.DARI M STERNOKLAIDOMASTOIDEUS.

DAPAT DETEMUKAN WAKRU LAHIR DAN DAPAT DETEMUKAN WAKRU LAHIR DAN SERING PADA USIA 10 TAHUN.SERING PADA USIA 10 TAHUN.

SERING HANYA PADA SATU SISI, SERING HANYA PADA SATU SISI, BILATERAL DITEMUKAN PADA 10 % BILATERAL DITEMUKAN PADA 10 % KASUS.KASUS.

DIFERENSIAL DIAGNOSADIFERENSIAL DIAGNOSA

LIMFADENITIS GRANULOMATOSA.LIMFADENITIS GRANULOMATOSA. HAEMANGIOMA, HIGROMA KOLI.HAEMANGIOMA, HIGROMA KOLI. KAROTID BODY TUMOR.KAROTID BODY TUMOR. LIMFOMA.LIMFOMA. METASTASE PADA KGB.METASTASE PADA KGB.

TERAPITERAPI

INFEKSI AKUT, KOMPRES DAN BERI INFEKSI AKUT, KOMPRES DAN BERI ANTI BIOTIKA. ANTI BIOTIKA.

ABSES, DRAINASE.ABSES, DRAINASE. KISTA, EKSISI.KISTA, EKSISI.

LIMFANGIOMALIMFANGIOMA

DISEBUT JUGA HIGROMA SISTIKA ATAU DISEBUT JUGA HIGROMA SISTIKA ATAU HIGROMA KOLI.HIGROMA KOLI.

TUMOR JINAK SISTEM LIMFATIK.TUMOR JINAK SISTEM LIMFATIK. DIJUMPAI SEJAK LAHIR, TERUTAMA DIJUMPAI SEJAK LAHIR, TERUTAMA

DATANG PADA USIA 1 – 2 TAHUN.DATANG PADA USIA 1 – 2 TAHUN. DAPAT DETEMUKAN PADA SELURUH DAPAT DETEMUKAN PADA SELURUH

TUBUH, SERING DI LEHER DAN AKSILA.TUBUH, SERING DI LEHER DAN AKSILA.

KLINISKLINIS

KISTIK, LUNAK DAN TRANSILUMINASI & KISTIK, LUNAK DAN TRANSILUMINASI & BERLOBUS LOBUS.BERLOBUS LOBUS.

PADA BAYI BARU LAHIR DAPAT PADA BAYI BARU LAHIR DAPAT DITEMUKAN PADA SELURUH LEHER, DITEMUKAN PADA SELURUH LEHER, LIDAH, LARING DAN MENGGANGGU LIDAH, LARING DAN MENGGANGGU JALAN NAFAS. JALAN NAFAS.

HISTOPATOLOGIHISTOPATOLOGI

SIMPLE.SIMPLE. KAVERNOSAKAVERNOSA KISTIKKISTIK

TERAPITERAPI

PEMBEDAHAN, BERUPA EKSISI, PADA PEMBEDAHAN, BERUPA EKSISI, PADA USIA YANG TEPAT.USIA YANG TEPAT.

PARATHYROIDPARATHYROID

DevelopmentDevelopment

■■Superior glands develop from fourth pharyngeal pouch.Superior glands develop from fourth pharyngeal pouch.

■■Adult position fairly constant with respect to the superior lobes Adult position fairly constant with respect to the superior lobes of thyroidof thyroid

■■Inferior glands develop from third pharyngeal pouch, in Inferior glands develop from third pharyngeal pouch, in conjunction with thymusconjunction with thymus

■■Inferior glands have more variable position (intrathymic or Inferior glands have more variable position (intrathymic or even superior to superior glands)even superior to superior glands)

AnatomyAnatomy

■■Weight <50 mg/glandWeight <50 mg/gland

■■3x3x3mm3x3x3mm

■■Yellow-brown tissue similar to surrounding fatty tissueYellow-brown tissue similar to surrounding fatty tissue

■■Histology : Normal gland contains mainly chief cells, with occasional Histology : Normal gland contains mainly chief cells, with occasional oxyphilsoxyphils

■■Vasculature : Inferior thyroid arteries ; superior, middle, and inferior Vasculature : Inferior thyroid arteries ; superior, middle, and inferior thyroid veinsthyroid veins

Physiology / Calcium HomeostasisPhysiology / Calcium Homeostasis

Primary functionPrimary function : Endocrine regulation of calcium and phosphate : Endocrine regulation of calcium and phosphate metabolismmetabolism

■■Regulators of calcium and phosphate metabolism : Regulators of calcium and phosphate metabolism : Parathyroid hormone (PTH), vitamin D, calcitoninParathyroid hormone (PTH), vitamin D, calcitonin

■■Organ systemsOrgan systems involved : Gastrointestinal (GI) tract, bone, involved : Gastrointestinal (GI) tract, bone, kidney kidney

PTHPTH

■■Synthesized in precursor from by the parathyroids .Synthesized in precursor from by the parathyroids .

■■Calcium levels requlate secretion of cleaved PTH (with negative Calcium levels requlate secretion of cleaved PTH (with negative feed-back mechanism)feed-back mechanism)

■■Bone :Bone :

■■Stimulates osteoclastsStimulates osteoclasts

■■Inhibits osteoblastsInhibits osteoblasts

■■Stimulates bone resorption, releasing calcium and phosphateStimulates bone resorption, releasing calcium and phosphate

■■Kidney :Kidney :

■■Increases reabsorption of calciumIncreases reabsorption of calcium

■■Increases phosphate excretionIncreases phosphate excretion

■■GI tract :GI tract :

■■Stimulates hydroxylation of 25-OH DStimulates hydroxylation of 25-OH D

■■1,25 OH D, then increases absorption of calcium and phosphate1,25 OH D, then increases absorption of calcium and phosphate

■■Promotes mineralizationPromotes mineralization

■■Enhances PTH’s effect on boneEnhances PTH’s effect on bone

CalcitoninCalcitonin

■■Secreted by thyroid C-cellsSecreted by thyroid C-cells

■■Inhibits bone resorptionInhibits bone resorption

■■Increases urinary excretion of calcium and phosphateIncreases urinary excretion of calcium and phosphate

HyperparathyroidismHyperparathyroidism

PRIMARYPRIMARYDue to overproduction of PTH, causing increased absorption of Due to overproduction of PTH, causing increased absorption of

calcium from intestines, increased vitamin Dcalcium from intestines, increased vitamin D33 production, production, and decreased renal calcium excretion, thereby raising the serum and decreased renal calcium excretion, thereby raising the serum

level.level.■■Incidence : 1/800 in the United StatesIncidence : 1/800 in the United States■■Signs and symptoms :Signs and symptoms :

■■””Stones” : Kidney stonesStones” : Kidney stones■■””Bones” : Bone pain, pathologic fracturesBones” : Bone pain, pathologic fractures■■””Groans” : Nausea, vomiting, constipation, pancreatitis, Groans” : Nausea, vomiting, constipation, pancreatitis,

peptic ulcer diseasepeptic ulcer disease■■””Moans” : Lethargy, confusion, depression, paranoiaMoans” : Lethargy, confusion, depression, paranoia

■■Etiology :Etiology :■■Solitary adenoma 85-90%Solitary adenoma 85-90%■■Four-gland hyperplasia 10%Four-gland hyperplasia 10%■■Cancer < 1%Cancer < 1%

■■Preop localization : US, FNA of suspicious US findings, Preop localization : US, FNA of suspicious US findings, Sestamibi scanSestamibi scan■■Diagnosis : Elevation of plasma PTH, with inappropriately high Diagnosis : Elevation of plasma PTH, with inappropriately high serum calciumserum calcium■■Treatment :Treatment :

■■Solitary adenoma : Solitary parathyroidectomySolitary adenoma : Solitary parathyroidectomy■■Multiple gland hyperplasia : Remove three glands, or all Multiple gland hyperplasia : Remove three glands, or all

four with reimplantation of one gland in forearmfour with reimplantation of one gland in forearm■■Outcome :Outcome :

■■First operation has 98% success rateFirst operation has 98% success rate■■Reoperationhas 90% success rate if remaining gland is Reoperationhas 90% success rate if remaining gland is localized preoplocalized preop

SECONDARYSECONDARY

Due to chronic renal failure or intestinal malabsorption that causes Due to chronic renal failure or intestinal malabsorption that causes hypocalcemia with appropriate increase in PTH.hypocalcemia with appropriate increase in PTH.■■Signs and symptoms :Signs and symptoms :

■■Bone pain from renal osteodystrophy and pruritusBone pain from renal osteodystrophy and pruritus■■Patients are often asymptomaticPatients are often asymptomatic

■■Diagnosis : Made by labs in asymptomatic patient Diagnosis : Made by labs in asymptomatic patient ■■Treatment :Treatment :

■■Nonsurgical : In renal failure patients, restrics phosphorus intake, treat with Nonsurgical : In renal failure patients, restrics phosphorus intake, treat with phosphorus-binding agentsphosphorus-binding agents

and calcium/vitamin D supplementation. Adjust dialysate to and calcium/vitamin D supplementation. Adjust dialysate to maximize calcium and minimize aluminiummaximize calcium and minimize aluminium

■■Surgical : Indicated for intractable bone pain or pruritus, or Surgical : Indicated for intractable bone pain or pruritus, or pathologicpathologic

■■3½-gland parathyroidectomy3½-gland parathyroidectomy

TERTIARYTERTIARY

Due to autonomously functioning parathyroid glands, resistand to Due to autonomously functioning parathyroid glands, resistand to negative feedback, for example, persistentnegative feedback, for example, persistent

hypercalcemia following renal transplantationhypercalcemia following renal transplantation

■■Usually a short-lived phenomenonUsually a short-lived phenomenon

■■If persistent, surgery is indicated (3½-gland If persistent, surgery is indicated (3½-gland parathyroidectomy)parathyroidectomy)

HypoparathyroidismHypoparathyroidism

■■UncommonUncommon

■■Etiology :Etiology :

■■Surgical-induced : Usually following total thyroidectomy ; Surgical-induced : Usually following total thyroidectomy ; usually transient and treated if symptoms developusually transient and treated if symptoms develop

■■Congenital absence of all four glandsCongenital absence of all four glands

■■DiGeorge syndrome : Absence of parathyroids and DiGeorge syndrome : Absence of parathyroids and thymic agenesisthymic agenesis

■■Functional : Chronic hypomagnesemiaFunctional : Chronic hypomagnesemia

■■Signs and symptoms :Signs and symptoms :

■■Numbness and tingling of circumoral area, fingers, toesNumbness and tingling of circumoral area, fingers, toes

■■Anxiety, confusionAnxiety, confusion

■■May progress to tetany, hyperventilation, seizures, heart blockMay progress to tetany, hyperventilation, seizures, heart block

■■Treatment : Supplementation with calcium and vitamin DTreatment : Supplementation with calcium and vitamin D

■■Pseudohypoparathyroidism : Familial target tissue resistance to Pseudohypoparathyroidism : Familial target tissue resistance to PTH. Patients remain hypocalcemia and hyperphos-PTH. Patients remain hypocalcemia and hyperphos-

phatemic despite bone resorption from elevated PTH. phatemic despite bone resorption from elevated PTH. Treatment consists of calcium and vitamin D Supplementation.Treatment consists of calcium and vitamin D Supplementation.

Parathyroid CancerParathyroid Cancer

Signs and symptoms :Signs and symptoms :

■■Forty to 50% present with firm, fixed massForty to 50% present with firm, fixed mass■■Extremely high calcium, PTH, and alkaline phosphataseExtremely high calcium, PTH, and alkaline phosphatase■■Also associated bone disease, renal insufficiency, and renal stonesAlso associated bone disease, renal insufficiency, and renal stonesPathology :Pathology :■■Pale, white, adherent mass, with a thick fibrous capsule and septaPale, white, adherent mass, with a thick fibrous capsule and septa■■Enlarged hyperchromatic nuclei and varied nuclear sizeEnlarged hyperchromatic nuclei and varied nuclear sizeTreatment : Treatment :

En block surgical resection of mass and surrounding structures, ipsilateralthyroid lobectomy, En block surgical resection of mass and surrounding structures, ipsilateralthyroid lobectomy, regional lymph nodesregional lymph nodes■■Postop external radiation therapy (XRT) and chemotherapy are not usually beneficialPostop external radiation therapy (XRT) and chemotherapy are not usually beneficial■■Five-year survival : 70%Five-year survival : 70%

MULTIPLE ENDOCRINE NEOPLASIA MULTIPLE ENDOCRINE NEOPLASIA (MEN)(MEN)

GeneralGeneral

Autosomal dominant disorders Autosomal dominant disorders resulting in multiple (metachronous resulting in multiple (metachronous or syn-chronous) tumors in different or syn-chronous) tumors in different endocrine organs.endocrine organs.

MEN IMEN I

■■Chromosomal defect : 11q12-13 (deletion)Chromosomal defect : 11q12-13 (deletion)■■Pituitary adenomasPituitary adenomas

■ ■Parathyroid hyperplasiaParathyroid hyperplasia■■Pancreatic islet cell tumorsPancreatic islet cell tumors

■■Involvement :Involvement :■■Two glands : 50%Two glands : 50%■■Three glands : 20%Three glands : 20%

■■Age at presentation :Age at presentation :■■Without known affected family members : 20s to Without known affected family members : 20s to

30s30s■■With known affected members : Prior to age 20 With known affected members : Prior to age 20

(through screening)(through screening)

MEN IIAMEN IIA

■■RET oncogene mutation on chromosom 10q11.2; missense mutations on chromosome 1RET oncogene mutation on chromosom 10q11.2; missense mutations on chromosome 1■■Parathyroid hyperplasiaParathyroid hyperplasia■■Medullary thyroid carcinomaMedullary thyroid carcinoma■■PheochromocytomaPheochromocytoma

MEN IIBMEN IIB

■■RET oncogene mutation on chromosome 10q11.2RET oncogene mutation on chromosome 10q11.2■■Medullary thyroid carcinomaMedullary thyroid carcinoma■■PheochromocytomaPheochromocytoma■■Mucosal neuromaMucosal neuroma■■Mafanoid habitusMafanoid habitus

TERIMAKASIHTERIMAKASIH