BY TEMESGEN G/MARIAM
OUT LINE
ANATOMY
CONTENTS
PATHOLOGIC ENTITIES
CLINICAL PRESENTATION
DIAGNOSTIC EVALUATION
TUMORS
CYSTS
Anatomy
PATHOLOGIC ENTITIES
ASM MM PS
thymoma(19%)
primary cysts(18%)
lymphomas(13%)
GCT(10%)
Posterior 16%
Middle 25%
Dyspnea
Cough
Fever
SVS
Pericardial tamponade
CHF
Chylothorax
Rhythm disturbance
Vocal cord paralysis
Horners syndrome
Phrenic nerve paralysis
Spinal cord compression
Pancoasts syndrome
pulmonary stenosis
Dysphagia
Stridor
Paraneoplastic syndrome
Hypertensionpheochromocytoma
Hypercalcemiaparathyroid tumor
Thyrotoxicosis intrathoracic goiter
Gynecomastia choriocarcinoma
Pel-Ebsten fever Hodgkins disease
Myastenia gravis thymoma
Ostiodystrophy neurogenic tumors
Diarhea neuroblastoma
DIAGNOTIC EVALUATION
History
P/E
General- chronically sick, Cushinoid face, in distress
Ptosis
Sign of SVS
Flaring, audible stridor, retractions, palpable chest masses
through the thoracic inlet
Sign of effusion, collapse
Sign of CHF
HM, Abdomenal mass
Limitation of movement, vertebral mass, vertebral deformity
Smymptx/sign of spinal compression
Skin lesions
Investigations
CBC, U/A, OFT
Imaging techniques
CXR (PA & lateral)
Ultrasound
Barium swallow
Echocardiography
CT scan
MRI
FNA
Core-needle biopsy
Oesophagoscopy
Thoracotomy
Biochemical markers
VMA
Metanephrine- Pheochromocytoma
Alpha fetoprtein
THYMOMA
Most common anterior mediastinal neoplasm in adults
(31%)
2nd most common mediastinal tumor (19%)
Rare in children
C/F
Asymptomatic (40-60%)
Mass effects
10-50%--MG sxs
Lymphoepithelial (50%)
Epithelial (27%)
Lymphocytic (22%)
spindle cell variety, a subtype of epithelial thymoma
Cell types as cortical, medullary, or mixed on the
basis of immunohistochemistry and light
microscopy
May be malignant(3060%) or benign(40-70%)
Benign no capsular invasion
Degenerative changes- hemorrhage, calcification,
necrosis, cystic changes
Pathological staging-(Masaoka)
Based on:
Invasion of capsule & surrounding stractures
Metastasis
Stage 1-encapsulated,no invasion
Stage 2-capsular invasion-mediastinal fat,pleura
Stage 3-gross invasion-pericardium,lung,great vss
Stage 4-a-pleural or pericardial dissemination
b-metastasis (lymphatic / hematogenous)
Rx.
Stage I purely surgery
Debulking + RRx +
Cisplastin based chemotherapy
Prognosis--- 5 Yr survival
Stage I 85-100%
Stage II 60-80%
Stage IV 50%
PET scan
Occasionaly curative
Radiotherapy
Neoadjuvant therapy
Thymolipomas
Benign
Mature teratomas
Dermoid cysts
Malignant
Seminomas
Nonseminomatous germ cell tumors
Immature teratoma
Teratoma with malignant components
Choriocarcinomas
Embryonal cell carcinomas
Endodermal cell (yolk sac) tumors
Mixed germ cell tumors
TERATOMA
Most common type of med.GCTs(60-70%)
2 or 3 embryonic layers, skin, hair (ectodermal),
cartilage and bone (mesodermal), or bronchial,
intestinal, or pancreatic tissue (endodermal).
Peak incidence is in the second and third decades of
life.
No gender predisposition.
Most in the ASM, although 3% to 8% PM
Rarely, focus of carcinoma;these malignant
teratomas(or teratocarcinomas) are locally aggressive
characteristic CT findings of a multilocular cystic
tumor, encapsulated with combinations of fluid, soft
tissue, calcium, and/or fat attenuation in the anterior
compartment
Rx-surgical resection for benign
Treatment with cisplatin-based chemotherapy is
instituted for malignant
Poor prognosis for malignant even after treatment
5-year survival rate of 61 %for patients responding to
chemotherapy and < 10% for non-responders has
been reported
SEMINOMAS:
50% of malignant germ cell tumors and 2% to 4% of all
mediastinal masses.
large cells with round nuclei, scant cytoplasm, and
abundant glycogen.
Advanced disease at time of diagnosis
Local compression than metastasis
Rx-Radiation therapy best response (90%)
Surgical resection- small asymptomatic or residual after
chemotherapy
chemotherapy-cisplatin-based
75%-complete response
NONSEMINOMATOUS GCTS
Bulky,irregular tumors
Involvment of adjacent stractures
Metastasis to regional LNs,pleura,& lungs,brain
s/mic Children with these tumors may present with
precocious puberty
chromosomal abnormalities are associated
Ix -CT- areas of low attenuation
-Serum markers(LDH,AFP,hCG)
Rx -chemotherapy
Cisplatin,bleomycin,etoposide
2yr survival=67%,5yr survival=60%
surgical resection of residual mass/salvage chemotherapy
rare radiosensitivity
NEUROGENIC TUMORS
Arise from nerve sheath cells,ganglion cells,or
paraganglionic system; vagus & phrenic nerves
Commonest tumor in the mediastinum
Commonly arises from posterior mediastinum
Age-correlates with incidence , cell type,& malignancy
risk
In children, most neurogenic tumors are malignant
(50%)
Rate of malignancy low in adults (10%)
Could have intraspinal extension=>Cord
compression
Chest pain, dyspnea, hoarse voice
Horners syndrome - unusual
Classification
NERVE SHEATH TUMORS
20% of all mediastinal tumors
>95%-benign(neurilemmoma or neurofibroma)
NEURILEMMOMA(schwanomas)
Arise from intercostal nerves
Benign,firm,well encapsulated
Malig. Tumors lack encapsulation & no Antoni features
Dx:
Hx
VATS
Complete resection
Combined thoracic and neurosurgical approach is
indicated for tumors with intraspinal extension
Recurrence is rare for benign tumors
Local recurrence is common for malignant tumors
and overall prognosis is poor
NEUROFIBROMA
Both nerve sheath & nerve cell components
25% of nerve sheath tumors
70 % -benign
Malignant degeneration(25-30%)
Risk - with age, vRh disease, radiation exposure
Prognosis- growth rate , local invasion along nerve
bundles
Rx-complete resection
5yr survival rate-53%
GANGLION CELL TUMORS
From sympathetic chain or adrenal medulla
GANGLIONEUROMA:
Well differentiated cystic degeneration
Asymptomatic, young adults & childhood
Intraspinal canal extension
Rx-complete resection
GANGLIONEUROBLASTOMA:
mature and immature ganglion cells.
Nodular-metastasizes
Diffuse-rarely metastasizes
Infants & children<3yrs
Rx-resection & chemotherapy.
5yr servival=80%
NEUROBLASTOMA
Highly malignant
Most common intrathoracic malignancy of childhood
Adrenal gland-common site but 14% arise from thorax
spontaneously regress, mature, or proliferate
aggressively
Extension in to spinal canal & ossious invasion
Children <2 yrs(>50%)-
Rx-surgical resection
chemotherapy
better prognosis than neuroblastomas occurring
elsewhere
PARAGANGLIONIC TUMORS
PHEOCHROMOCYTOMA:
one of the most rare tumors
10% are malignant
Highly vascular
Site-costovertebral sulcus
-visceral compartment
Preoperative care includes alpha- and beta-adrenergic
blockade to prevent intraoperative malignant hypertension
and arrhythmias
CHEMODECTOMA:
Rare
30% malignant, rarely secrete catecholamines
Site-aortic arch,vagus,aorticosympathetic
MEDIASTINAL CYSTS
PRIMARY MEDIASTINAL CYST
25% of med.masses are benign cysts
Middle compartment
Bronchoscopy
Ba study
CT, MRI
Rx-
Resection