Thymic carcinoma in children: a report from the Polish Pediatric Rare Tumors Study.

Pediatr Blood Cancer 2010 Jul;54(7):916-20

Department of Pediatrics, Hematology, Oncology and Endocrinology, Medical University, Gdansk, Poland.

Background: Invasive thymomas and thymic carcinomas are rare tumors jointly accounting between 0.2% and 1.5% of malignancies in adults. They are usually at an advanced stage when diagnosed and have both high recurrence and poor survival rates. In this report, the aim is to explore our experience in the treatment of thymic carcinomas in Polish children.

Procedure: The clinical data of nine children with thymic carcinomas, treated between 1992 and 2008 in the Polish oncological and surgical centers was retrospectively analyzed.

Results: In five cases, presenting symptoms resulted from the compression of the respiratory ways by the mediastinal tumor. In two children paraneoplastic autoimmune syndromes were associated with thymic carcinoma. In accordance with the Masaoka classification, two patients had stage II, five had stage III, and two had stage IV of the disease. Diagnostic biopsy of mediastinal tumor was performed on eight patients and one underwent complete primary resection and subsequently received radiotherapy; he has passed 11 years since the conclusion of therapy. Six patients received multi-drug chemotherapy with or without steroids. Delayed surgery was performed in four children (R0-2, R1-1, and R2-1). After complete resection, one child received chemotherapy. In three patients, chemotherapy and radiotherapy was administered. Seven patients died, including six due to progression of the disease with the other as a result of complications following chemotherapy; only two patients classed at stage II remain alive.

Conclusions: Most thymic tumors in pediatric patients are inoperable at diagnosis, which results in poor prognosis. Improved chemotherapy approaches are needed.

Download full-text PDF

Source
http://doi.wiley.com/10.1002/pbc.22482
Publisher Site
http://dx.doi.org/10.1002/pbc.22482DOI Listing
July 2010
34 Reads

Publication Analysis

Top Keywords

thymic carcinomas
12
rare tumors
8
thymic carcinoma
8
mediastinal tumor
8
patients
7
thymic
6
stage
5
chemotherapy
5
primary resection
4
complete primary
4
patients underwent
4
biopsy mediastinal
4
tumor performed
4
performed patients
4
resection subsequently
4
underwent complete
4
received radiotherapy
4
therapy patients
4
patients received
4
received multi-drug
4

Similar Publications