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THYMIC TUMORS . GENERAL THORACIC SURGERY CHAPTER 167. Thymic tumor. Almost in the anterior mediastinum. Secondary to neurogenic tumor in mediastinal tumor. Rare in children younger than 16 y/o. . Thymic tumor. Separated into three histologic categories— Thymoma.

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thymic tumors




thymic tumor
Thymic tumor
  • Almost in the anterior mediastinum.
  • Secondary to neurogenic tumor in mediastinal tumor.
  • Rare in children younger than 16 y/o.
thymic tumor4
Thymic tumor
  • Separated into three histologic categories—


Thymic carcinoma.

Neuroendocrine tumor.

  • 95% in anterior mediastinum.
  • Neck.
  • Left hilar region.
  • Within lung parenchynma.
  • Anterior cardiophrenic angle.
  • All thymoma derive from thymic epithelial cell.
  • Predominantly lymphocytic thymoma (more than 66% lymphocyte).
  • Predominantly epithelial thymoma (more than 66% epithelial cell).
  • Mixed lymphoepithelial thymoma.
  • Spindle cell tumor.
  • Most important gross feature — The presence or absence of encapsulation of tumor and the gross invasion into adjacent structure.
  • The invasion present — The thymoma must be considered malignant lesion regardless the microscopic appearance.
  • Extensive spread.
  • Incidence of distal metastasis is 3%.
  • Stage.
another class
Another class
  • Cortical.
  • Medullary.
  • Mixed thymoma.
  • Immunohistochemistry.
clinical presentation
Clinical presentation
  • 50-60 y/o.
  • Sex distribution — Equal.
  • s/s — chest pain, SOB, cough, SVC syndrome, paralysis of hemidiaphragm, hoarseness, weight loss, fatigue, fever, night sweats.
parathymic syndrome
Parathymic syndrome
  • 40% with parathymic syndrome.
  • Myasthenia gravis.
  • Pure red cell aplasia.
  • Immunoglobulin deficiency.
  • Systemic lupus erythematosus—infrequently, 2.5%, poor prognosis.
  • Nonthymic cancer—17-21%.
  • Inappropriate antidiuretic hormone secretion(SIADH)-- rarely, malignant thymoma and spindle cell thymoma.
myasthenia gravis
Myasthenia gravis
  • Most commonly associated disease.
  • 30% of patient with thymoma associate with MG.
  • Only 5-15% patient with MG have thymoma.
  • 10-15 older than patient with MG without tumor, younger than patient with thymoma without MG.
  • Any type of thymoma except spindle type, marked associated squamous elements in thymus.
  • Little affect on local presention, clinical behavior, prognosis.
  • Better prognosis than patient with thymoma without MG.
pure red cell aplasia
Pure red cell aplasia
  • Anemia.
  • Suppression erythrogenesis in bone marrow.
  • Mechanism--Not clear, IgG antibodies inhibit erythropoietin or hemoglobin synthesis, cytotoxic to erythroblast, decrease B cell.
  • 50 % patients with red cell aplasia have thymoma, 5% thymoma with red cell aplasia.
  • Most (70%)are non-invasive spindle cell.
  • 25-33% patient with red cell aplasia benefit from excision of the thymoma.
immunoglobulin deficiency
Immunoglobulin deficiency
  • Spindle cell type.
  • Acquired hypogammaglobulinemia.
  • Suppressor T-cell inhibiting immunoglobulin synthesis.
diagnostic studies
Diagnostic studies
  • Standard posteroanterior and lateral chest radiographies.
  • CXR—Smooth or lobulated mas, right side the silhouette sign present, left side the sign abscent.
  • Calcification — 10%.
  • CT—Delineate the extent of mass, cannot not differentiating benign and malignant, assessing intrathoracic spread of an invasive thymoma.
surgical biopsy
Surgical biopsy
  • Unnecessary for a suspected locally symptomatic thymoma, because the capsule of tumor may be violated by invasive procedure.
  • Only distinguish the tumor from the other malignant tumor, or locally symptomatic, clearly nonresectable, biopsy is to establish the diagnosis before making decision of therapy.
  • Fine needle biopsy by CT or sono-guide.
  • Extend substernal mediastinoscopy.
  • Anterior mediastinotomy.
  • Lateral thoracotomy.
  • VATS.
  • Depend on clinical presentation.
  • Surgical resection — thymoma is encapsulated and free from adjacent structure.
  • Radiation — in atage II, III.
  • chemotherapy — in locally nonresectable, presence distal metastasis, neoadjuvant therapy for initially advanced local diasease or in locally recurrent disease.
surgical excision
Surgical excision
  • All patient with thymoma should undergo as complete resection as possible.
  • Pulmonary lesion should be excised at the same time.
  • Tumor encapsulated — total thymectomy.
  • Simple enucleation is avoid except the unusual condition(excision through lateral thoracotomy with unknown preoperative diagnosis).
surgical excision30
Surgical excision
  • Preferred median sternotomy.
  • Posterolateral thoracotomy — for large tumor in hemithorax or tumor from anterior cardiophrenic angle.
  • Bilateral anterior fourth intercostals incisionwith transverse section of sternum (clamshell) — for large midline tumors.
  • The use of video-assisted thoracoscopic removal of thymoma is unacceptable even for stage I tumor.
surgical excision31
Surgical excision
  • Extend procedure the entire thymus and adjacent fat should be removed if possible.
  • Tumor fixation to nonvital adjacent structure should be resect(pleura, lung, pericardium, ).
surgical excision32
Surgical excision
  • One phrenic nerve involve could be resected if patient could tolerate loss of hemidiaphragm function.
  • If both phrenic are involved, only debulking is performed.
surgical excision33
Surgical excision
  • The wall of SVC involve — if no SVC syndrome, lateral wall resection of SVC and replace graft.
  • When the aorta, major pulmonary vessels, recurrent nerve trachea, are involve, only debulking.
  • Operative mortality 3.1%-7.7%.
radiation therapy
Radiation therapy
  • For invasive thymoma.
  • In stage I is uncertain.
  • For resected stage II or completely or incompletely resected stage III disease.
  • 4500-5000 cGy for suspected microscopic residual disease.
  • 6000 cGy for known residual disease.
  • Brachytherapy with I-125 seed placed in gross residual disease at time of operation.
  • For stage III and IV.
  • Cisplatin, doxorubucin, vincristine, cyclophosphamide, neoadjuvant.
treatment of recurrent local disease or distant metastases
Treatment of recurrent local disease or distant metastases
  • Recurrent I — 0-5%, II — 10%, III — 30%, IVa — 33%.
  • Second resection if possible.
  • 5-year survival is 65%.
  • For stage III recurrent — irradiation or chemotherapy.
  • Depend on — stage, tumor size, histology, extent of resection.
  • Better in patient with thymoma associated with MG.
  • Poor in patient with red cell aplasia, hypogammaglobulinemia, SLE.
thymic carcinoma
Thymic carcinoma
  • Low and high grade.
  • Malignant cytologic and architectural feature.
  • Staging not standardized.
squamous cell carcinoma
Squamous cell carcinoma
  • Most common.
  • Men predominant.
  • 60 y/o.
  • Partially encapsulated.
  • s/s — weight loss, chest pain, cough, hemoptysis.
  • Treatment—Surgical resection, sensitive to radiation, combination chemotherapy.
  • Prognosis excellent in well-differentiated squamous cell carcnoma.
lymphoepitheliomalike carcinoma
Lymphoepitheliomalike carcinoma
  • Epstein-Barr virus.
  • Treatment—irradiation therapy, chemotherapy.
tumor of neuroendocrine cell origin
Tumor of neuroendocrine cell origin
  • Thymic carcinoid tumor.
  • Small cell carcinoma.
thymic carcinoid tumor
Thymic carcinoid tumor
  • Large.
  • One-half lesion infiltrative into adjacent structures.
  • Associated Cushing’s syndrome.
  • 3/4 are men.
  • Mean age 42 y/o.
  • s/s — asymptomatic, chest pain, cough dyspnea, SVC syndrome, fatigue fever, night sweat.
thymic carcinoid tumor47
Thymic carcinoid tumor
  • 1/3 have feature of Cushing sundrome — ectopic ACTH production.
  • 15-18% with multiple endocrine neoplasia(MEN)syndrome.
  • Most MEN I.
  • Few MEN II.
  • Thymic carcinoid associated with MEN syndrome is more malignant in behavior.
  • 1/3 with bone metastases.
multiple endocrine neoplasia men
Multiple endocrine neoplasia(MEN)
  • MEN I(Werner syndrome)– Single or multiple parathyroid adenoma, islet cell tumor of pancrease, adrenal neoplasm, thyroid adenoma, multiple lipoma.
  • MEN II(Sipple syndrome)—Thyroid medullary carcinoma, pheochronocytoma, parathyroid neoplasia.
thymic carcinoid tumor49
Thymic carcinoid tumor
  • Treatment—complete surgical resction or debulking tumor, radiation therapy.
  • 73% local recurrence or metastases.
  • Overall cure rate is low — 13%.
  • Mean survival of metastases disease is 3 years.
small oat cell carcinoma
Small (Oat)cell carcinoma
  • Aggressive and metastases extensively,
  • Associated with MEN I.
  • Treatment—radiation therapy and chemotherapy.