Clinical characteristics and clinical observation of granulocyte sarcoma in children / 中华实用儿科临床杂志

ABSTRACT

Objective To discuss the mode of onset,clinical characteristics,treatment and prognosis of children with granulocyte sarcoma (GS),in order to provide guidance for early diagnosis and effective treatment of GS.Methods Six cases of children with GS diagnosed at the Department of Hematology,Children's Hospital Affiliated to Soochow University between June 2009 and June 2014 were analyzed,the data including the mode of onset,clinical manifestation,diagnosis,treatment and outcome.Results There were 2 cases with a painless mass onset (1 case was 2 years old,characterized by right waist mass,about 10 cm × 5 cm;the other case was 6 years old,characterized by axillary lump,about 2 cm × 3 cm),and both of them received surgical removal of the tumor,then the postoperative tumor was examined by pathologic and immunohistochemical method,and at last the primary granulocyte sarcoma was diagnosed.The third case was a 7 years old girl,she was onset characterized by scalp lump,about 2 cm × 3 cm,and was diagnosed by the pathologic and immunohistochemical method,and changes in hematological system appeared a month later and acute myeloid leukemia(AML) was confirmed by bone marrow examination.The onset ages of other 3 cases were in 10 months,1 year and 7 months,13 years and 3 months old respectively,characterized by scalp lump (about 2 cm × 3 cm),spinal canal tumor (about 1.0 cm × 1.5 cm),intracranial tumors (6.0 cm × 4.9 cm),with AML occurring at the same time,which was confirmed by surgical pathology,immunohistochemistry and bone marrow cell morphology,immune classification,chromosome,and fusion gene diagnosis.Four cases were hematopoietic malignancies by pathology,2 cases of then belonging to small round cell tumor.The immune pathology showed 5 cases of myeloperoxidase positive,CD68-positive,3 cases of CD43-positive,CD123-positive.All children CD3,CD20 levels in all children were negative.Four cases underwent surgery combined with chemotherapy,other 2 cases received surgery and then gave up treatment,1 case discontinued follow-up 3 months later,and the other case died of intracranial hemorrhage after 3 months,which induced by thrombocytopenia.The treated 4 cases were followed up 3 to 58 months,and all had disease-free survival.Conclusions Children with GS have low incidence and non-specific diagnostic criteria,its diagnosis depends on immune pathology,and the treatment is mainly in accordance with AML program for high-dose chemotherapy.The systematic chemotherapy helps to prolong overall survival;at the same time,the hematopoietic stem cell transplantation with bone marrow may help to improve the prognosis.