A 17-year-old boy presented with fatigability & weakness in limbs, drooping
of eyelids, nasal regurgitation of fluids & nasal twang
since four months. He also had a history of intermittent dyspnea on exertion.
A provisional diagnosis of myasthenia gravis with
acute onset bulbar paresis was made. On examination, there was truncal, neck
and eye closure muscle weakness. Motor power
was 4/5 proximally and 5/5 distally. Pulmonary function tests showed no improvement
following neostigmine.
IMAGING FINDINGS:
The frontal chest radiograph showed a large antrerir mediastinal soft tissue
mass on the right side adjacent to the heart.
Fig.1
A plain and contrast enhanced
CT Chest showed a large, well defined, lobulated, anterior and superior mediastinal
mass with
cystic components. There was no contrast enhancement. In view of the clinical
presentation this lesion was thought to be thymoma.
Fig.2
Fig.3
At surgery, a multilobulated
cystic mass was removed from the mediastinum.
Fig.4
On histopathology, the
findings were characteristic of mixed cystic thymoma.
The patient had an uncomplicated post operative course.
DISCUSSION:
Thymic cysts account
for 1-2% of all anterior mediastinal tumors. Patients are mostly asymptomatic
unless the cysts are very
large or if hemorrhage occurs.
Etiology: These cysts probably result from developmental anomaly and represent
persistent tubular remnants of the third
pharyngeal pouch, the fetal thymo-pharyngeal duct.
Pathophysiology: As they arise from the thymopharyngeal duct, these tumors
can occur anywhere along the site of embryologic descent of the thymus - from
mandible to the level of the diaphragm. Cysts are unilocular or multilocular
and lined by squamous, transitional, simple cuboidal or columnar epithelium.
Imaging: Chest radiographs demonstrate mediastinal mass. Lesion appears to
be cystic on CT and Ultrasound. MRI shows signal characteristics of water
ie. hypointense on T1W & hyperintense on T2W images.
The differential diagnosis includes localised thymoma, teratoma, dermoid cyst,
Hodgkins disease, Non Hodgkins lymphoma