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DNA Index (14,15) คา DNA index ทวดดวยวธ Flow cytometry มความสมพนธกบจำานวนโครโมโซมและพยากรณโรค ถากอนมะเรงม hyperdiploid DI ( มคาเทากบ 1 หรอกวานน) มกเปนโรคระยะตนตอบสนองตอเคม
บำาบดดกวากอนทม diploid DI ( มคาเทากบ 1) ทมกจะเปนมะเรงระยะหลงมการแพรกระจายและใชเคมบำาบดไดผลนอย(14) วธนทำาไดงายกวาวธนบโครโมโซมและมประโยชนในการวางแผนการรกษาและพยากรณโรคด
ตารางท 17.4 Recommended Criteria at the International Neuroblastoma Staging
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System (INSS) Conference for Diagnosis of Neuroblastoma (40)
A diagnosis of neuroblastoma is established if:(1) Unequivocal pathologic diagnosisa is made from tumor tissue by light microscopy (with or without immunohistology, electron microscopy) , and/or increased urine or serum catecholamines or metabolitesb:OR(2) Bone marrow aspirate or trephine biopsy contains unequivocal tumor cellsa (e.g. syncytia or immunocytologically positive clumps of cells) and increased urine or serum catecholamines or metabolites.b
หมายเหตa If histology is equivocal, karyotypic
abnormalities in tumor cells characteristic of other tumors {e.g. t(11:22)}, excludes a diagnosis of neuroblastoma, whereas genetic features characteristic of neuroblastoma (1 p deletion, N-myc amplification) would support this diagnosis.
b Includes dopamine, HVA, and/or VMA (levels must be >3.0 SD above the mean per milligram creatinine for age to be considered increased, and at least two of these must be measured).
CT and/or MRI scana with 3D measurements; MIBG scan, ifavailableb
Metastatic sitesb
Bone marrow
Bilateral posterior iliac crest marrow aspirates and trephine (core) bone marrow biopsies required to exclude marrow involvement. A single positive site documents marrow involvement. Core biopsies must contain at least 1 cm of marrow (excluding cartilage) to be considered adequate
Bone MIBG scan; 99Tc scan required if MIBG scan negative orunavailable, and plain radiographs of positive lesions are recommended
ตารางท17.7 เปรยบเทยบ Staging Systems สำาหรบ Neuroblastoma แบบตาง ๆ
CCSG System
POG System INSS System
Stage I. Tumor confined to the organ or structure of origin
Stage A. Complete gross resection of the primary tumor, with or without microscopic residual disease. Intracavity lymph-nodes not adhered to the primary tumor must be histologically free of tumor. Nodes adhered to the surface of or within the primary may be positive. If primary tumor in abdomen or pelvis, liver must be histogically
Stage 1. Localized tumor with complete gross excision, with or without microscopic residual disease; representative ipsilateral lymph nodes negative for tumor microscopically (nodes attached to and removed with the primary tumor may be positive)
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free of tumor
ตารางท17.7 เปรยบเทยบ Staging Systems สำาหรบ Neuroblastoma แบบตาง ๆ (ตอ)
CCSG System
POG System INSS System
Stage II. Tumor extending in continuity beyond the organ or structure of origin, but not crossing the midline. Regional lymph nodes on the ipsilateral side may be involved
Stage B. Grossly unresected primary tumor. Nodes and liver, the same as in stage A
Stage 2A. Localized tumor with incomplete gross excision; representative ipsilateral nonadherent lymph nodes negative for tumor microscopicallyStage 2B. Localized tumor with or without complete gross excision, with ipsilateral nonadherent lymph nodes positive for tumor. Enlarged
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contralateral lymph nodes must be negative microscopically
Stage III. Tumor extending in continuity beyond the midline. Regional lymph nodes may be involved bilaterally
Stage C. Complete or incomplete resection of primary. Intercavitary nodes not adhered to primary must be histologically positive for tumor. Liver as in stage A
Stage 3. Unresectable unilateral tumor infiltrating across the midline,a with or without regional lymph node involvement; or localized unilateral tumor with contralateral regional lymph node involvement; or midline tumor with bilateral extension by infiltration (unresectable) or by lymph node involvement
ตารางท17.7 เปรยบเทยบ Staging Systems สำาหรบ Neuroblastoma แบบตาง ๆ (ตอ)
CCSG POG System INSS System
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Neuroblastoma
SystemStage IV. Remote disease involving the skeleton, bone marrow, soft tissue and distant lymph node groups (see stage IV-S)
Stage D. Dissemination of disease beyond intracavitary nodes (i.e. extracavitary nodes, liver, skin bone marrow, bone, etc.)
Stage 4. Any primary tumor with dissemination to distant lymph nodes, bone. bone marrow, liver, skin and/or other organs (except as defined for stage 4S)
Stage IV-S. As defined in stage I or II, except for the presence of remote disease confined to the liver, skin, or marrow (without bone metastases)
Stage DS. Infants <1 yr of age with stage IV-S disease (see CCSG).
Stage 4S. Localized primary tumor (as defined for stage 1, 2A or 2B), with dissemination limited to skin, liver, and./or bone marrowb (limited to infants <1 yr of age)
Multifocal primary tumors (e.g., bilateral adrenal primary tumors) should be staged according to
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the greatest extent of disease, as defined above, and followed by a subscript letter M (e.g., 3m).a The midline is defined as the vertebral column. Tumors originating on one side and crossing the midline must infiltrate to or beyond the opposite side of the vertebral column. A grossly resectable tumor arising in the midline from pelvic ganglia or the organ of Zuckerkandl would be considered stage I. A midline tumor that extended beyond one side of the vertebral column and was unresectable would be considered stage 2A. Ipsilateral lymph node involvement would make it stage 2B, whereas bilateral lymph node involvement would make it stage 3. A midline primary tumor with bilateral infiltration that was not resectable would be considered Stage 3. A tumor of any size with malignant ascites or peritoneal implants would be stage 3 (but a thoracic tumor with malignant pleural
ตารางท17.7 เปรยบเทยบ Staging Systems สำาหรบ Neuroblastoma แบบตาง ๆ (ตอ)
effusion unilaterally would be stage 2B).b Marrow involvment in stage 4S should be minimal. i.e., <10% of total nucleated cells identified as malignant on bone marrow biopsy or on marrow aspirate. More extensive marrow involvement would be considered to be stage 4. The MIBG scan should be negative in the marrow. CCSG = Children’s Cancer Study Group; POG = Pediatric Oncology Group; INSS = International Neuroblastoma Staging System Conference (second conference).
Low-dose, sequential CPM/ADR (course repeated every 3-4 weeks)1. Cyclophosphamide (CPM) 150 mg/M2/day, PO or IV, days 1-72. Adriamycin (Doxorubicin) 35 mg/M2
IV, day 8Cisplatin/VM-26
1. Cisplatin 90 mg/M2, IV over 6 hours, day 12. VM-26 (Teniposide) 100 mg/M2, IV over 1 hour, days 3
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MADDOC(Dana-FarberCancer Inst.)
Repeat courses every 3 weeks, total duration 9-12 months1. Nitrogen mustard 4 mg/M2/dose, day 12. Adriamycin 40 mg/M2/dose, day 13. DTIC 750 mg/M2/dose, day 1 and only during every other course (i.e. every 6 weeks)4. Cisplatin (Diamine dichlor cisplatin) 45 mg/M2/dose, day 15. Vincristine (Oncovin) 2 mg/M2/dose, day 1, (maximum single dose 2 mg)6. Cyclophosphamide 750 mg/M2/dose/day1 Total cumulative adrimycin dose is 300 mg/M2. When necessary, reduce myelosuppressive treatment by 25% to maintain absolute neutrophil count (ANC) above 200/cu.mm..
17.10 ตารางท17.10 Modified International Neuroblastoma Response Criteria (INRC)
Definitions of Response to Treatment (40)
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Response
Primary tumor
Metastatic Sites
Complete response
No tumor No tumor; catecholamines normal
Very good partial response
Decreased by 90-99%
No tumor; catecholamines normal; residual 99Tc bone changes allowed
Partial response
Decreased by>50%
All measureable sites decreased by >50%. Bones and bone marrow: number of positive bone sites decreased by >50%; no more than one positive bone marrow site allowedb
Mixed response
No new lesion; >50% reduction of any measurable lesion (primary or metastases) with <50% reduction in any other; <25% increase in any existing lesion
No response
No new lesion; <50% reduction but <25% increase in any existing lesion
Progressive disease
Any new lesion; increase of any measurable lesion by >25%; previous negative marrow positive for tumor
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