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ZARIA THORACIC CLUB MEETING AHMADU BELLO UNIVERSITY TEACHING HOSPITAL,ZARIA,NIGERIA TERATOMA. BY DR SANNI,R.O REGISTRAR. Introduction Epidemiology Classification Aetiopathogenesis Clinical presentation Investigation treatment conclusion. Introduction.
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ZARIA THORACIC CLUB MEETINGAHMADU BELLO UNIVERSITY TEACHING HOSPITAL,ZARIA,NIGERIA TERATOMA BY DR SANNI,R.O REGISTRAR
Introduction • Epidemiology • Classification • Aetiopathogenesis • Clinical presentation • Investigation • treatment • conclusion
Introduction • Terato (Greek : monster, oma : swelling) • Teratomas - embronic neaplasm from totipotent stem cells. • Component derived from all 3 germ layer. • Tissues foreign to the location found. • Relatively common solid Neoplasm in children. • Gonadal and extragonadal location. • Tumor marker
Epidermiology • SCT most common teratoma. • Germ cell tumor-11% childhood tumor,3% malignant. • 25% mediastinal tumor malignant. • Teratoma Commoner in females. -F:M 4:1 SCT. Affect all age group • Increased frequency in the last decade. • No significant geographic predilection.
Aetiology • Gene related • Familial tendency • Polyunsaturated fat (ovarian) • Pyloric stenosis • Turner syndrome • Klinefelter syndrome
Aetiopathogenesis Pathology-solid -cystic -mixed -Benign -Malignant
Pathogenesis • Several theories about its origin. • Abnormal differentiation of fetal germ cell that arise from fetal yolk sac • Normal migration of cell- gonadal tumor • Abnormal to extra gonadal tumor. • Typically found in the midline or gonads.
Frequency of common sites. • Sacroccocygeus 40% • Ovary 25% • Testes 12% • Brain 5% • Others(neck, mediastinum) 18%
Correlation between Degree of immaturity and foci of microscopic malignant cell. • Charoenkwan et al-over expression of P53 in immature teratoma.
Mutter –genetic imprint factor. • Osterhuis et al-suggest tumor group based on chromosomal abnormality’. • MAGE gene family of tumor rejection antigen involved. Hara et al
Mediastinal Teratoma CLINICAL FEATURES • Chest pain ,retrosternal pain. • Cough • Dyspnoea • Fever • Weight loss • Fatigue • Venous congestion • Compressive syptoms • Hoarseness, dysphagia, Horners syndrome
Clinical presentation • Depend on location of tumor. • Prenatal diagnosis-SCT ,mass, pressure effect. • Ovarian mass-abdominal pain ,mass distension. • Testicular tumor –scrotal mass, pain.
Physical examination • Chest • Cardiovascular • Abdominal • Rectal examination • Identify associated anomalies
Investigations • Diagnostic Imaging studies- • CXR • CT scan • Ultra sonography – abdomino-pelvic, testicular.
Tissue Biopsy • Mediastinoscopy. • Video assisted thoracoscopy, biopsy. • Broncoscopy.
Serum- AFP -Beta-Hcg Genetic screening.
FBC, diff. • U E Cr. • LFT • Pulmonary function test. • Others as necessitated by presentation.
Treatment • Goals • Removal, where possible • Relief of symptoms • Prolongation of life • Improve quality of life. • Depends on site, extent of Dx. • Surgical excision. • Chemotherapy
Recurrence • Risk of recurrence related to degree of maturity. • <10% in completely resected mature Teratoma. • 33% immature Teratoma. • Depends on site and completeness of resection. • Decreased by chemotherapy-9.5%(German ). • SCT more likely to re occure.
Follow up Monitor-Examination -AFP -Beta hCG -CXR
Prognosis Depend on the organ of origin and extent of the dx.
Conclusion The incidence of teratoma is gradually increasing ,therefore High index of suspicion is needed for early diagnosis and prompt intervention.