Askin's tumor is a rare tumor arising from the chest wall. It is a subset of Ewing sarcoma characterized histologically by the presence of small round blue cells. It is a highly malignant tumor with guarded prognosis, which is dependent upon the extension of tumor at the time of diagnosis. A dual paper of Askin's tumors in young boys is being presented here.
Ewing sarcoma comprises of tumors which are characterized by small round blue cells. These include classical Ewing sarcoma of bone, extraskeletal Ewing sarcoma, Askin’s tumor of the thoracic wall, and peripheral primitive neuroectodermal tumor [
3 years old male toddler, presented with 15 days of cough and 1 day of chest pain, breathlessness, and low-grade fever. He had no other associated complaints, and his past history was unremarkable. Chest radiograph PA view showed a large homogenous opacity on right upper lobe, suggestive of a mass lesion (Figure
The tumor regressed significantly with 8 cycles of chemothearpy. Surgery was undertaken after the course of chemotherapy. Operative findings showed tumor which was arising from the 1st rib, stuck to the apex of the right lung. There was also intense desmoplastic reaction surrounding the tumor, and adhesions were present between the tumor and the right subclavian vessel and superior venacava. Thoracotomy with excision of the tumor and resection of the first rib was done, preserving subclavian vein and brachial plexus. There were no postoperative complications. Postoperative histopathological examination of the mass showed small round blue cell tumors with hyperchromatic nuclei and scanty cytoplasm with tiny foci of calcifications. Immunohistochemistry was strongly positive for CK and CD-99 and negative for LCA and CD-34 (Figure
Postoperatively, he was given ifosfamide, carboplatin, and etoposide (ICE) chemotherapy [
Chest X-ray of Case
FNAC from the lesion in Case
Immunohistochemistry from the lesion showing CK and CD 99 positive in Case
9-year-old boy had presented with chest pain and fever of 7 days duration. There was no history of breathlessness, cough, hemoptysis, or weight loss. His past history and family history were unremarkable. Radiograph of the chest PA view showed large homogenous opacity covering almost the entire right hemithorax. CT scan chest delineated a large mass (9.5 cm × 4.5 cm × 7.3 cm) along the right posterolateral chest wall which was causing scalloping of the inner surface of the 8th rib with obliteration of the extrapleural fat in places with free well defined and irregular border medially. FNAC from the lesion showed small round blue cells, with scanty cytoplasm and relatively large nuclei. Biopsy of the lesion showed small round blue cells in sheets with ill-defined rosette-like structures, histomorphologically peripheral neuroectodermal tumor-possibly Askin’s tumor (Figure
He was given chemotherapy with vincristine, adriamycin and cyclophosphamide (VAC), alternating with ifosfamide and etoposide (IE) as per RCT II protocol [
Histopathlogical examination of the biopsy in Case
Chest X ray postsurgery in Case
Askin’s tumor is a subset of Ewing’s sarcoma which arises from the chest wall. It is characterized histologically by features of small round blue cell tumors. It was described by Askin et al. in 1979 [
Askin’s tumor is genetically defined by reciprocal translocation t(11 : 22) (q24 : q12) with EWS-FLI-1 fusion gene [
Askin’s tumor usually presents with common respiratory symptoms. It may include cough, chest pain, fever, breathlessness, and so forth. Local pain may be present which might reduce in intensity at night but not disappear completely. It may present with local paraesthesia and rarely as pathological fracture or metastasis related symptoms [
Radiography of the chest in Askin’s tumor may show classical onion-peel appearance of the tumor or just homogenous opacity [
The treatment includes chemotherapy, radiotherapy and surgery. Surgical therapy has the most important implication; however, because of its position, surgery has to be individualized. Complete surgical resection is associated with a survival advantage [
Askin’s tumor is a rare tumor of childhood which usually presents with common respiratory symptoms. Pediatrician must be far sighted to suspect this rare entity with such common presentations. It would require a high index of suspicion on behalf of the general pediatrician for the early recognition of this tumor so that chances of better outcome and prognosis shall be there. However, considering its aggressive nature, complicated course and recurrence tendency, long term followup is warranted.
Informed consent has been taken from the parents for the publication of the cases, along with the accompanying photographs.
The authors declare that they have no conflict of interests.
B. Shrestha conceived and drafted the paper. B. N. Kapur was the treating physician for both the cases. K. Karmacharya contributed in obtaining the histological photographs. S. Kakkar was involved in the pathological diagnosis of both the cases. R. Ghuliani edited the entire paper and supervised the literature. All the authors have read and agreed about the paper.