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| Discussion |
A 13-yr-girl was admitted with complaints of fever and breathlessness
since 4-5 days. She had developed acute flaccid paralysis in the last three
days. Three months back, the patient had been admitted for fever and productive
cough.
On examination, child was febrile; air entry was decreased in the left lung.
The child was papraparetic with a power of 2/5 in both lower limbs.. Routine
investigations were normal.
RADIOLOGICAL Examination:
The chest radiograph revealed an approximately 12x8 cm, lobulated mass in
the left posterior mediastinum without calcification; scalloping of the
posterior segment of upper ribs on the left side was seen .
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Fig. 1
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Fig. 2
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Fig. 3
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MRI chest better delineated the epidural extension of lesion with compression
of the spinal cord.
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Fig. 4
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Fig. 5
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Fig. 6
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CT guided biopsy of the tumor revealed an invasive small cell malignant
tumor, and was reported as a primitive neuroectodermal tumor ( ASKIN Tumor
) of the chest wall.
DISCUSSION:
Askin tumor is rare Ewing's
family pediatric tumor seen involving the thoracopulmonary region. The tumor
mostly affects the chest wall and rib. It is a neuroectodermal tumor having
malignant behaviour. It was first proposed by Askin et al in 1979.
The tumor is
commonly seen in the age group of 4 months to 20 years, the mean age being
14.5 yrs. Clinically, it presents with pain and shortness of breath if there
is pleural effusion. The term Askin tumor refers to the cells seen on histopathology
i.e. small cell tumors.
It needs to be differentiated from other tumors on the basis of age, sex,
clinical presentation and histology coupled with other necessary special
stains and immunochemistry. Histologically it is more related to a neuroepithelima.
On imaging, there is
rib destruction with a large soft tissue mass which may show calcification.
The affected rib shows increased uptake on radionuclide scanning.
Diagnosis is made by biopsy.
Treatment options are
chemotherapy and resection of the mass and affected ribs or radiotherapy.
The primary malignant
small round cell tumor of chest wall and lung excluding the mediastinum
in childhood and early adolescent are infrequent. However Ewing's sarcoma,
embryonal rhabdomyosarcoma, neuroblastoma , malignant histiocytoma and malignant
lymphoma account for 1.8% of chest wall pediatric neoplasms. Therefore,
these should be considered in the differential diagnosis of Askin tumors.
Biopsy is confirmatory.