A Rare Case of Askin’s Tumor in the 6th rib

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Presentation transcript:

A Rare Case of Askin’s Tumor in the 6th rib Authors Dr. S Ganesh Kamath Dr Vasudev B Pai Dr Kapil Minocha( Presenter) Dr Sambhram Shetty Dr. Guruprasad Rai Kasturba Medical College, Manipal Manipal University

Clinical Presentation A 23 years old female presented with pain and swelling over right anterior chest. She was afebrile and did not have any other symptoms. The onset was insidious with progress over 3 months. On examination 7X5 cms oval swelling felt over the anterior chest wall right, anterior axillary line over 6th rib, hard in consistency. Overlying skin was normal. No other significant findings.

Patient positioned supine with slight tilt towards left Coastal margin Mass

Investigations Chest X Ray: Soft tissue opacity at the anterior end of 6th rib non homogenous. The lungs were clear. High resolution computerized tomography Scan, revealed An expansile mixed lytic sclerotic lesion anterior end of 6th rib with periosteal reaction.no infiltration of Right lung. There were two small opacity in the right basal segments less than 1cms.

Chest Xray CT Scan cut section

Operative Specimen

Surgery Patient taken up for Excisional biopsy. The 6th rib was excised with the whole mass with wide margin 4cms to the rib posterior and anteriorly up ti the sternum. There was pleural reaction and lung was not involved Histopathological Report Reported as Ewing’s Tumour/ Primitive Neuroectodermal tumour with full clearance

Discussion Askin’s tumor belong to Ewing sarcoma family of Tumour (ESFT)  is a primitive neuroectodermal tumor, developing from the soft tissues of the chest wall. Treatment includes radical surgical resection, neoadjuvant and adjuvant chemotherapy and radiation. The rarity of the lesion requires complex diagnosis approach and a multidisciplinary team with Multimodal chemtherapy treatment. Significant advances in the neoadjuvant and adjuvant chemotherapeutic regimens, as well as improved facility in diagnosing these tumors through cytogenetic and immunohistochemical analysis, improve long-term disease-free survival.

References Askin FB, Rosai J, Sibley RK, Dehner LP, Mc Alister WH. Malignant small cell tumour of the thoracopulmonary region in childhood: a distinctive clinicopathologic entity of uncertain histogenesis. Cancer. 1979;43:2438–2451. doi: 10.1002/1097-0142(197906)43:6<2438: 11. Tazi I, Zafad S, Madani A, Harif M, Quessar A, Benchekroun S. Askin tumor: a case report with literature review. Cancer Radiothérapie. 2009;13:771–774. doi: 10.1016/j.canrad.2009.05.016. [PubMed] [Cross Ref] : Kimber C, Michalski A, Spitz L, Pierro A. Primitive neuroectodermal tumours: anatomic location, extent of surgery, and outcome. J Pediatr Surg. Jan 1998;33(1):39-41. Batsakis JG, Mackay B, el-Naggar AK. Ewing's sarcoma and peripheral primitive neuroectodermal tumor: an interim report. Ann Otol Rhinol Laryngol. Oct 1996;105(10):838-43. Castro EC, Parwani AV. Ewing sarcoma/primitive neuroectodermal tumor of the kidney: two unusual presentations of a rare tumor. Case Report Med. 2012;2012:190581. [Medline]. [Full Text].