Premium
Leptomeningeal sarcoma 16 years after cranial radiation in a child with acute lymphoblastic leukemia that spread to the central nervous system (CNS)
Author(s) -
Llena Josefina,
Weidenheim Karen,
Radel Eva,
Goodrich James,
Small Trudy
Publication year - 2008
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.22.1_supplement.706.23
Subject(s) - medicine , radiation therapy , sarcoma , frontal bone , rhabdomyosarcoma , primitive neuroectodermal tumor , bone marrow , skull , surgery , pathology
With constant ongoing improvement in radiotherapy, tumors occuring after cranial radiation in children with acute lymphoblastic leukemia are increasingly rare. The latest review article is by Rimm I J et al in Cancer 59:1506–1508, 1987. Their Table 1 listed 22 cases; 20 gliomas, 1 melanocytoma and 1 meningeal fibrosarcoma. Average radiation dose was 2400 cGy, age range was 2 to 19 years and latency period range was 3 to 11 years. The meningeal fibrosarcoma was described by Tiberin P et al in J. Neurosurg 1984; 61:772–776. It was a frontal lobe tumor in an 8 year old girl irradiated at age 2. Our patient was 3 1/2 years old at diagnosis. He was treated with a regimen including 2400 cGy cranial radiation. 10 months after completing treatment, his tumor relapsed in the bone marrow and CNS. He received intensive chemotherapy including intrathecal and intraventricular chemotherapy for 9 months. About 3 years later, while in complete remission, he received an unmodified bone marrow transplant from his HLA‐identical sister after total body irradiation of 1375 cGy. At age 20, he presented with headache and seizures. Neuroradiology showed a 1 cm enhancing mass in the left frontal opercular region. A circumscribed leptomeningeal gray‐tan firm tumor located under the central sulcus was completely resected. The tumor was an undifferentiated high grade sarcoma with epithelioid and myxoid features.