Interhospital Pediatric Conference Ramathibodi Hospital 16 November 2010

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Interhospital Pediatric Conference Ramathibodi Hospital 16 November 2010

Interhospital Pediatric Conference Ramathibodi Hospital 16 November 2010

ประวตปจจบน ตรวจเพมเตมทโรงพยาบาลจงหวด Flim TL spine: compression fracture T 12 PPD skin test: negative CT

ประวตปจจบน ตรวจเพมเตมทโรงพยาบาลจงหวด Flim TL spine: compression fracture T 12 PPD skin test: negative CT chest: normal CT Whole abdomen: hepatosplenomegaly

ประวตปจจบน MRI spine: multiple ring enhancement at multiple vertebral lesion with pathological fracture T

ประวตปจจบน MRI spine: multiple ring enhancement at multiple vertebral lesion with pathological fracture T 12 Bone scan: increased uptake lesions at upper thoracic spine and all joint Bone marrow aspiration: no abnormal cell Bone marrow biopsy: reactive hyperplasia

ประวตปจจบน MRI spine: multiple ring enhancement at multiple vertebral lesion with pathological fracture T

ประวตปจจบน MRI spine: multiple ring enhancement at multiple vertebral lesion with pathological fracture T 12 Bone scan: increased uptake lesions at upper thoracic spine and all joint Bone marrow aspiration: no abnormal cell Bone marrow biopsy: reactive hyperplasia

Physical Examination A Thai boy, mild pallor, no jaundice T 39 oc PR 112/min

Physical Examination A Thai boy, mild pallor, no jaundice T 39 oc PR 112/min RR 20/min BP 120/70 mm. Hg • Wt 45 kg Ht 150 cm • Heart and lungs: normal • Abdomen: liver 4 cm below RCM mild tender, spleen 2 cm below LCM

Physical Examination • Back: no scoliosis, tender along distal thoracic to lumbar spine •

Physical Examination • Back: no scoliosis, tender along distal thoracic to lumbar spine • Extremities: no deformity, no limitation of movement • Neuro: motor power upper extremities V lower extremities IV DTR 2+ all

Problem lists and Discussion

Problem lists and Discussion

Initial investigations CBC: Hb 9. 6 g%, Hct 30%, MCV 71. 7 fl, MCH

Initial investigations CBC: Hb 9. 6 g%, Hct 30%, MCV 71. 7 fl, MCH 23. 1 pg, MCHC 32. 2 g/d. L WCB 10, 400/mm 3, N 73%, L 16%, M 11% Plt 435, 000/mm 3 UA: normal Anti HIV: negative

Initial investigations (2) Electrolytes: Na 134, K 3. 9, Cl 94, CO 2 26.

Initial investigations (2) Electrolytes: Na 134, K 3. 9, Cl 94, CO 2 26. 5 mmol/L Ca 12. 7, P 3. 8, Mg 1. 6 mg/d. L BUN 20, Cr 0. 9 mg/d. L LDH: 670 U/L CRP: 265 mg/L

Initial investigations (3) Liver function test AST 55, ALT 27 U/L, TB 0. 6,

Initial investigations (3) Liver function test AST 55, ALT 27 U/L, TB 0. 6, DB 0. 3 mg/d. L, Total protein 78 g/L, albumin 31. 5 g/L, Alkaline phosphatase 212 U/L GGT 85 U/L

Investigation (4) Melioid titer: negative Hemo c/s: no growth PPD skin test: no induration

Investigation (4) Melioid titer: negative Hemo c/s: no growth PPD skin test: no induration

Investigations Left sided L 1 transpedicular biopsy: Necrotic material with some inflammation cells, no

Investigations Left sided L 1 transpedicular biopsy: Necrotic material with some inflammation cells, no acid fast bacilli, no fungus

Investigations BMA: normal cellularity, normal maturation of erythroid, myeloid and megakarycyte, no abnormal cell

Investigations BMA: normal cellularity, normal maturation of erythroid, myeloid and megakarycyte, no abnormal cell BM Biopsy: normal cellularity 80%, M: E ratio 3: 1 Active trilinear hematopoiesis Blast 5% of nucleated cells No fibrosis, no granuloma

MRI knee Extensive varying-size Nodular appearing marrow infiltrating lesions Metastatics deposit Malignancy process Infectious

MRI knee Extensive varying-size Nodular appearing marrow infiltrating lesions Metastatics deposit Malignancy process Infectious process

Pathology BCL-2 CD 20 CD 3 CD 10

Pathology BCL-2 CD 20 CD 3 CD 10

Investigations Bone biopsy: left proximal tibia Primary non-Hodgkin lymphoma of bone, diffuse B cell

Investigations Bone biopsy: left proximal tibia Primary non-Hodgkin lymphoma of bone, diffuse B cell lymphoma Immunohistochemistry CD 20 CD 79 a CD 3 CD 10 CD 68 CD 99 BCL-2 Td. T Desmin Sarcomeric actin positive in large lymphoid cells negative in large lymphoid cells positive in histiocytic cells negative negative

Chest x-ray

Chest x-ray

Film spine Destruction and collapse T 12, L 4 -5 vertebrae

Film spine Destruction and collapse T 12, L 4 -5 vertebrae

Bone survey multiple osteolytic lesions

Bone survey multiple osteolytic lesions

CT chest and abdomen mediastinum and intraabdominal lympadenopathy mild hepatosplenomegaly Infiltrative involvement both kidney,

CT chest and abdomen mediastinum and intraabdominal lympadenopathy mild hepatosplenomegaly Infiltrative involvement both kidney, no hydronephrosis Multiple osteolytic lesions including clavicles, scapulars, head of humerus, whole spines, pelvic bone and femur

Diagnosis Primary non-Hodgkin lymphoma of bone, diffuse B cell lymphoma

Diagnosis Primary non-Hodgkin lymphoma of bone, diffuse B cell lymphoma

Treatment Start chemotherapy Burkitt protocol Supportive care

Treatment Start chemotherapy Burkitt protocol Supportive care

After Treatment

After Treatment

Primary Bone Lymphoma Primary bone lymphomas (PBLs) are rare, less than 1% of all

Primary Bone Lymphoma Primary bone lymphomas (PBLs) are rare, less than 1% of all malignant lymphomas PBLs is defined as a lymphoma that is confined to bone or BM without evidence of systemic involvement 2002 WHO classification of tumors of soft tissue and bone, the criteria for a diagnosis of PBL a single skeletal tumor without regional LN involvement multiple bone lesions without visceral or LN involvement Arch Pathol Lab Med Vol 133, November 2009

Primary Bone Lymphoma Most are diffuse large B-cell lymphomas The middle-aged to elderly population,

Primary Bone Lymphoma Most are diffuse large B-cell lymphomas The middle-aged to elderly population, median age of 48 yrs Common presentation: bone pain and less-frequent a palpable mass and bone fracture Very rarely, paraplegia from compression Rarely, hypercalcemia may be present Arch Pathol Lab Med Vol 133, November 2009

Anatomic Location Beal et al reported a series of PBDLBCL that included 82 patients

Anatomic Location Beal et al reported a series of PBDLBCL that included 82 patients Involvement was femur (27%), pelvis (15%), tibia/fibula (13%), polyostotic (13%), humerus (12%), spine (9%), other (5%), mandible (2%), radius/ulna (1%), scapula (1%), and skull (1%) Rarely, small bones of the hands and feet are involved in PBDLBCL Arch Pathol Lab Med Vol 133, November 2009

Radiographic Findings The metaphysis is the most common site of occurrence in long bones

Radiographic Findings The metaphysis is the most common site of occurrence in long bones The lesion shows varying areas of sclerosis and osteolysis, producing a ‘‘moth-eaten’’ appearance Arch Pathol Lab Med Vol 133, November 2009

Case Reports 1. A 13 -year-old girl presented with a 6 month history of

Case Reports 1. A 13 -year-old girl presented with a 6 month history of pain in the lower thoracic region Examination and investigations: mass at right thoracic and pelvic region, osteolytic lesion Biopsy: lymphoblastic lymphoma, CD 20, CD 79 positive 2. A 6 -year-old boy, progressive pain left knee and rapidly, enlarging mass. Exam and Ix: mass at left knee 5*5 cms, osteolytic lesion at metaphyseal of distal femur Biopsy: burkitt lymphoma, CD 19, CD 20 positive Eur J Pediatr 2001, 160: 239 -242

Case Reports) con’t( Treatment with B-cell protocol NHL consecutive blocks of polychemotherapy Vincristine, cytarabine,

Case Reports) con’t( Treatment with B-cell protocol NHL consecutive blocks of polychemotherapy Vincristine, cytarabine, dexamethasone, doxorubicine, etoposide, cyclophosphamide and high dose methotrexate with leucovorin rescue Complete remission both cases Follow up of 24, 18 months respectively, alive without disease Eur J Pediatr 2001, 160: 239 -242

Case Reports) con’t( PLB, defined localized disease and treat with local radiation of primary

Case Reports) con’t( PLB, defined localized disease and treat with local radiation of primary site Treatment of adult PLB of RT had 50% overall long term survival Lymphoma in children as systemic disease, local RT not sufficient Pediatric oncology group treatment with multiagent chemotherapy without RT 95% of a 5 year event-year free rate Eur J Pediatr 2001, 160: 239 -242

Thank you

Thank you