Prognostic factors and outcome in Askin-Rosai tumor: a review of 104 patients.
OBJECTIVE
To evaluate the prognostic factors and treatment outcome of patients with Askin-Rosai tumor of the chest wall treated at a single institution.


METHODS
Treatment comprised multiagent chemotherapy and local therapy, which was either in the form of surgery alone, radical external-beam radiotherapy (EBRT) alone, or a combination of surgery and EBRT. Thirty-two patients (40%) were treated with all three modalities, 21 (27%) received chemotherapy and radical EBRT, and 19 (24%) underwent chemotherapy followed by surgery only.


RESULTS
One hundred four consecutive patients aged 3-60 years were treated at the Tata Memorial Hospital from January 1995 to October 2003. Most (70%) were male (male/female ratio, 2.3:1). Asymptomatic swelling (43%) was the most common presenting symptom, and 25% of patients presented with distant metastasis. After a median follow-up of 28 months, local control, disease-free survival, and overall survival rates were 67%, 36%, and 45%, respectively. Median time to relapse was 25 months, and the median survival was 76 months. Multivariate analysis revealed age ≥18 years, poor response to induction chemotherapy, and presence of pleural effusion as indicators of inferior survival. Fifty-six percent of patients with metastatic disease at presentation died within 1 month of diagnosis, with 6-month and 5-year actuarial survival of 14% and 4%, respectively.


CONCLUSIONS
Primary tumor size, pleural effusion, response to chemotherapy, and optimal radiotherapy were important prognostic factors influencing outcome. The combination of neoadjuvant chemotherapy, surgery, and radiotherapy resulted in optimal outcome.
Laskar
Siddhartha
S
Department of Radiation Oncology, Tata Memorial Hospital, Mumbai, India. laskars2000@yahoo.com
Nair
Chandrika
C
Mallik
Suman
S
Bahl
Gaurav
G
Pai
Suresh
S
Shet
Tanuja
T
Gupta
Tejpal
T
Arora
Brijesh
B
Bakshi
Ashish
A
Pramesh
C S
CS
Mistry
Rajesh
R
Qureshi
Sajid
S
Medhi
Seema
S
Jambhekar
Nirmala
N
Kurkure
Purna
P
Banavali
Shripad
S
Muckaden
Mary Ann
MA
D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003037:Cobalt Radioisotopes / Q000627:therapeutic use; D003131:Combined Modality Therapy / Q000379:methods / Q000401:mortality; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000401:mortality / Q000473:pathology / Q000556:secondary / Q000628:therapy*; D010996:Pleural Effusion / Q000401:mortality; D011379:Prognosis; D011879:Radiotherapy Dosage; D012074:Remission Induction; D015996:Survival Rate; D013899:Thoracic Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D035441:Thoracic Wall; D047368:Tumor Burden; D055815:Young Adult
D003037:Cobalt Radioisotopes