TITLE:
Askin Tumor in Egyptian Patients; 5 Years Experience at the National Cancer Institute, Cairo University
AUTHORS:
Ahmed El Sayed Fathalla, Bahaa El Din Ahmed
KEYWORDS:
Chest Wall, Askin Tumor, Management & Outcome
JOURNAL NAME:
Journal of Cancer Therapy,
Vol.7 No.3,
March
18,
2016
ABSTRACT: Background: Askin tumor is a primitive
neuroectodermal tumor of the Ewing sarcoma family arising from soft tissues of
the chest wall. It is the commonest chest wall malignancy in children and
adolescents. Its diagnosis is complex and management requires a
multidisciplinary work including chemotherapy for systemic disease and
radiation therapy to assist local control which is achieved through surgery
with or without reconstruction. Objective: To analyze report and understand the
clinicopathological features, results and outcome of this tumor with assessment
of early and late postoperative complications following resection and chest
wall reconstruction. Materials and Methods: This is a retrospective analysis of
30 cases with chest wall ES/PNET presented to the National Cancer Institute;
Cairo University between January 2011 and December 2015. All patients’ records
were revised for age, sex, clinical presentation, imaging, pathology, operative
notes, different treatment modalities given, early and late postoperative
complications following surgical resection or reconstruction, and outcome
including overall survival (OS) and disease free survival (DFS). Results: Our
cohort included 30 patients with chest wall ES. The median follow up period (n =
30) was 33.7 months (ranging from 3.7 to 69.3 months). The median OS was 54.2
months with cumulative OS at 60 months which was 45.6%. The median DFS was 27.9
months with cumulative DFS at 60 months which was 40.6%. All cases were below
18 years with a male predominance (n = 19). The commonest affected sites were
ribs (n = 22, 73.3%), the scapula (n = 5, 16.6%), the clavicle (n = 2, 6.66%)
and the sternum (n = 1, 3.33%). 23 patients (76.6%) were presented with
localized disease, and 7 patients (23.4%) were metastatic from the start. All
patients received neoadjuvant chemotherapy (4 cycles of VAC/IE) followed by
local control: either surgery (26 cases, 86.6%) or radical radiotherapy (3 cases,
10%). A single case of a rib ES with initial bilateral lung deposited where no
local control was done. After chest wall resection, closure of the defect was
done by 1 ry closure with no reconstruction or double layer prolene mesh and
bone cement that was covered by pedicled flap (latissimus dorsi, serratus
anterior or pectoralis major muscle flap). Postoperative radiation therapy was
given to 9 patients: 4 (13.3%) had postoperative poor chemotherapy effect (