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1131129-高雄地区第504次小儿科联合病例讨论会--高雄市医师公会.辫诲蹿

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Ks doctor
Ks doctor

日期：113年11月29日 主題：淺談兒童固態腫瘤 講師：高醫大附設醫院小兒血液腫瘤科 許琬宜 醫師

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淺談兒童固態腫瘤 高雄醫學大學附設中和紀念醫院 小兒血液腫瘤科 許琬宜 醫師
Solid Tumor in Children (excluding CNS tumor) - General concepts - Epidemiology - Cancer screen in children ? - Differential diagnosis - Lymphadenopathy - Mediastinal mass - Abdominal mass - Unusual presentation - (Oncogenic Emergency ) - Germ cell tumor - Lymphoma - Neuroblastoma
1 1 2 Incidence of Cancer among Children and Adolescents 2 3 3
依照台湾儿童癌症基金会的统计：
依照台湾儿童癌症基金会的统计：
依照台湾儿童癌症基金会的统计：
依照台湾儿童癌症基金会的统计：
1131129-高雄地区第504次小儿科联合病例讨论会--高雄市医师公会.辫诲蹿
1131129-高雄地区第504次小儿科联合病例讨论会--高雄市医师公会.辫诲蹿
Who are at risk ? ? Certain infectious agents ? Children who have survived therapy for another cancer ? Children with the genetic predispositions to cancer (1~10% cases)
Who are at risk ? ? Certain infectious agents ? Epstein-Barr virus (EBV) ? B-cell lymphomas, especially Burkitt lymphoma; peripheral T-cell lymphomas, Hodgkin lymphoma, hemophagocytic lymphohistiocytosis, and nasopharyngeal carcinoma ? Hepatitis B and C infections : hepatocellular carcinoma ? Human papilloma virus : cervical cancer
Who are at risk ? ? Certain infectious agents ? Children who have survived therapy for another cancer
Who are at risk ? ? Certain infectious agents ? Children who have survived therapy for another cancer ? Four to six folds more likely to develop second malignant neoplasms(SMN) than the general population ? More aggressive, harder to treat, worse outcomes
Who are at risk ? ? Certain infectious agents ? Children who have survived therapy for another cancer (Front Oncol. 2021 Oct 15;11:710163.)
Radiation-related cancers ? Longer latency period ( at least 5 to 10 years after treatment) ? Associated with the cumulative dose, field of radiation, and age at treatment ? Solid tumor為主： ? breast, thyroid cancer, brain tumors, sarcomas, and basal cell carcinomas Chemotherapy-related cancers ? Occur within 2 to 5 years following treatment, typically declining after 10 years ? Alkylating agents, topoisomerase inhibitors ? Solid tumor(少數) 與therapy-related AML/MDS(多數)皆有可能
Who are at risk ? ? Certain infectious agents ? Children who have survived therapy for another cancer ? Long-term follow-up
Who are at risk ? ? Certain infectious agents ? Children who have survived therapy for another cancer ? Children with the genetic predispositions to cancer (1~10% cases)
Children with the genetic predispositions to cancer ? How tumor forms ?
? How tumor forms ? Account for the majority of childhood cancer Only the minority…
Children with the genetic predispositions to cancer
Children with the genetic predispositions to cancer Down syndrome ? nearly 20-fold increased risk of leukemia ? <2 y/o : ? AML >ALL, esp. acute megakaryocytic leukemia ? Older age ? ALL > AML, 易 relapse 與 infection Phenotypic female with part or all of a Y chromosome ? Gonadoblastoma ? should have prophylactic surgery to remove their gonads 47XXY ? 50-fold increased risk of extra gonadal germ cell tumors Very-low birth weight (<1500gm) baby ? hepatoblastoma
Children with the genetic predispositions to cancer
Cancer Diagnosis that merit a Genetics Evaluation independent of Family history
Children with the genetic predispositions to cancer
Who are at risk ? ? Certain infectious agents ? Children who have survived therapy for another cancer ? Children with the genetic predispositions to cancer (1~10% cases)
Solid Tumor in Children (excluding CNS tumor) - General concepts - Epidemiology - Cancer screen in children ? - Differential diagnosis - Lymphadenopathy - Mediastinal mass - Abdominal mass - Unusual presentation - (Oncogenic Emergency )
It is often difficult to diagnose childhood cancer in its early stages…… - Because many of the presenting signs and symptoms are nonspecific and mimic common childhood diseases
兒童癌症九大警訊 (from兒癌基金會) 1. 不明原因的發燒:持續兩週以上不明原因的發燒。 2. 不明原因的疼痛:如關節酸痛、腹痛、頭痛等。 3. 不明原因的腫塊:小孩皮膚表面有不明原因之腫塊且有增大的傾向。 4. 淋巴腺腫大:不明原因之全身性或局部性淋巴結腫大(如:頸部、腋下、鼠蹊部淋巴結腫)且無壓痛感。 5. 肝脾腫大:肝及脾臟緊貼兩側肋骨的下方，若以手按壓可摸出硬塊即是腫大。 6. 臉色蒼白:小孩無故臉色蒼白，即唇及眼結膜較無血色，伴隨倦怠、食慾不振等。 7. 紫斑或出血傾向:皮膚表面時常無故出現瘀斑或紫紅色出血小點，或有黏膜出血(如:流鼻血、牙齦出 血等)。 8. 神經方面的症狀: 如無故的嘔吐、走路不穩、頭痛，顔面神經麻痺等。 9. 眼球有異常反射光: 嬰幼兒的眼球對光線産生不正常反射光(如:貓眼)。
Lymphadenopathy Indication for biopsy: ? Chronic, persistent, progressive adenopathy, in the absence of an infectious etiology ? Any nodes larger than 2.5 cm in diameter, in the absence of signs of infection ? Supraclavicular adenopathy ? with Systemic symptoms What we should do before biopsy ? ? Check viral titer (ex. EBV, CMV), E.S.R., LDH ? Check CXR, arrange abd./neck echo ? Biopsy tissue culture: TB, bacteria, fungi ? obtain tissue chromosome if malignancy is suspected
Thoracic mass ? Virtually all primary intrathoracic tumors arise in the mediastinum ? Rarely tumor in lung/rib cage - pleuropulmonary blastomas, desmoplastic small round cell tumors, rhabdomyosarcomas
Mediastinal mass Germ cell tumor
Mediastinal mass Oncogenic Emergency !! -> Superior vena cava Syndrome
Mediastinal mass What we do before biopsy ? ? Vital sign & airway stabilize ? Check LDH, AFP, beta-hCG,… ? Chest CT, cardiac echo Indication for biopsy: ? All mediastinal mass, excepting for germ cell tumor w/ elevating tumor marker& characteristic image finding (but biopsy is still preferred)
Abdominal mass ? Age plays a major role in the diagnostic considerations ? Neonates ? Most mass are benign, excepting Wilms’ tumor and neuroblastoma ? Infant/Children ? 1st Neuroblastoma → 2nd Wilms’ tumor → 3rd Lymphoma Calcification in X-ray ? Neuroblastoma, Hepatoblastoma, teratoma
Unusual presentation ? Disseminated Intravascular Coagulation ? Kaposiform hemangioendothelioma ? Acute promyelocytic leukemia(APML) ? Possible to cause intracranial bleeding ? Rarely: T-ALL, neuroblastoma ? Acquired von Willebrand disease ? Wilms tumor ? Rarely: lymphoma, Ewing sarcoma ? Blueberry muffin syndrome in newborn ? Congenital leukemia cutis ? Histiocytosis ? Neurublastoma
Unusual presentation ? Disseminated Intravascular Coagulation ? Kaposiform hemangioendothelioma ? Acute promyelocytic leukemia(APML) ? Possible to cause intracranial bleeding ? Rarely: T-ALL, neuroblastoma ? Acquired von Willebrand disease ? Wilms tumor ? Rarely: lymphoma, Ewing sarcoma ? Blueberry muffin syndrome (in newborn) ? Congenital leukemia cutis ? Histiocytosis ? Neurublastoma ? Raccoon eyes (Periorbital ecchymosis) ? Metastatic Neuroblastoma ? Rarely: Kaposi’s sarcoma ? Must rule out: basal skull fractures, child abuse
Unusual presentation ? Disseminated Intravascular Coagulation ? Kaposiform hemangioendothelioma ? Acute promyelocytic leukemia(APML) ? Possible to cause intracranial bleeding ? Rarely: T-ALL, neuroblastoma ? Acquired von Willebrand disease ? Wilms tumor ? Rarely: lymphoma, Ewing sarcoma ? Blueberry muffin syndrome (in newborn) ? Congenital leukemia cutis ? Histiocytosis ? Neurublastoma ? Hypertension ? Renal or adrenal tumor ? Neuroblastoma ? Sexual abnormality / Cushing syndrome ? Gonadal or Adrenal tumor ? Raccoon eyes (Periorbital ecchymosis) ? Metastatic Neuroblastoma ? Rarely: Kaposi’s sarcoma ? Must rule out: basal skull fractures, child abuse
Thank You !

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1131129-高雄地区第504次小儿科联合病例讨论会--高雄市医师公会.辫诲蹿

  • 2. Solid Tumor in Children (excluding CNS tumor) - General concepts - Epidemiology - Cancer screen in children ? - Differential diagnosis - Lymphadenopathy - Mediastinal mass - Abdominal mass - Unusual presentation - (Oncogenic Emergency ) - Germ cell tumor - Lymphoma - Neuroblastoma
  • 3. 1 1 2 Incidence of Cancer among Children and Adolescents 2 3 3
  • 10. Who are at risk ? ? Certain infectious agents ? Children who have survived therapy for another cancer ? Children with the genetic predispositions to cancer (1~10% cases)
  • 11. Who are at risk ? ? Certain infectious agents ? Epstein-Barr virus (EBV) ? B-cell lymphomas, especially Burkitt lymphoma; peripheral T-cell lymphomas, Hodgkin lymphoma, hemophagocytic lymphohistiocytosis, and nasopharyngeal carcinoma ? Hepatitis B and C infections : hepatocellular carcinoma ? Human papilloma virus : cervical cancer
  • 12. Who are at risk ? ? Certain infectious agents ? Children who have survived therapy for another cancer
  • 13. Who are at risk ? ? Certain infectious agents ? Children who have survived therapy for another cancer ? Four to six folds more likely to develop second malignant neoplasms(SMN) than the general population ? More aggressive, harder to treat, worse outcomes
  • 14. Who are at risk ? ? Certain infectious agents ? Children who have survived therapy for another cancer (Front Oncol. 2021 Oct 15;11:710163.)
  • 15. Radiation-related cancers ? Longer latency period ( at least 5 to 10 years after treatment) ? Associated with the cumulative dose, field of radiation, and age at treatment ? Solid tumor為主： ? breast, thyroid cancer, brain tumors, sarcomas, and basal cell carcinomas Chemotherapy-related cancers ? Occur within 2 to 5 years following treatment, typically declining after 10 years ? Alkylating agents, topoisomerase inhibitors ? Solid tumor(少數) 與therapy-related AML/MDS(多數)皆有可能
  • 16. Who are at risk ? ? Certain infectious agents ? Children who have survived therapy for another cancer ? Long-term follow-up
  • 17. Who are at risk ? ? Certain infectious agents ? Children who have survived therapy for another cancer ? Children with the genetic predispositions to cancer (1~10% cases)
  • 18. Children with the genetic predispositions to cancer ? How tumor forms ?
  • 19. ? How tumor forms ? Account for the majority of childhood cancer Only the minority…
  • 20. Children with the genetic predispositions to cancer
  • 21. Children with the genetic predispositions to cancer Down syndrome ? nearly 20-fold increased risk of leukemia ? <2 y/o : ? AML >ALL, esp. acute megakaryocytic leukemia ? Older age ? ALL > AML, 易 relapse 與 infection Phenotypic female with part or all of a Y chromosome ? Gonadoblastoma ? should have prophylactic surgery to remove their gonads 47XXY ? 50-fold increased risk of extra gonadal germ cell tumors Very-low birth weight (<1500gm) baby ? hepatoblastoma
  • 22. Children with the genetic predispositions to cancer
  • 23. Cancer Diagnosis that merit a Genetics Evaluation independent of Family history
  • 24. Children with the genetic predispositions to cancer
  • 25. Who are at risk ? ? Certain infectious agents ? Children who have survived therapy for another cancer ? Children with the genetic predispositions to cancer (1~10% cases)
  • 26. Solid Tumor in Children (excluding CNS tumor) - General concepts - Epidemiology - Cancer screen in children ? - Differential diagnosis - Lymphadenopathy - Mediastinal mass - Abdominal mass - Unusual presentation - (Oncogenic Emergency )
  • 27. It is often difficult to diagnose childhood cancer in its early stages…… - Because many of the presenting signs and symptoms are nonspecific and mimic common childhood diseases
  • 28. 兒童癌症九大警訊 (from兒癌基金會) 1. 不明原因的發燒:持續兩週以上不明原因的發燒。 2. 不明原因的疼痛:如關節酸痛、腹痛、頭痛等。 3. 不明原因的腫塊:小孩皮膚表面有不明原因之腫塊且有增大的傾向。 4. 淋巴腺腫大:不明原因之全身性或局部性淋巴結腫大(如:頸部、腋下、鼠蹊部淋巴結腫)且無壓痛感。 5. 肝脾腫大:肝及脾臟緊貼兩側肋骨的下方，若以手按壓可摸出硬塊即是腫大。 6. 臉色蒼白:小孩無故臉色蒼白，即唇及眼結膜較無血色，伴隨倦怠、食慾不振等。 7. 紫斑或出血傾向:皮膚表面時常無故出現瘀斑或紫紅色出血小點，或有黏膜出血(如:流鼻血、牙齦出 血等)。 8. 神經方面的症狀: 如無故的嘔吐、走路不穩、頭痛，顔面神經麻痺等。 9. 眼球有異常反射光: 嬰幼兒的眼球對光線産生不正常反射光(如:貓眼)。
  • 29. Lymphadenopathy Indication for biopsy: ? Chronic, persistent, progressive adenopathy, in the absence of an infectious etiology ? Any nodes larger than 2.5 cm in diameter, in the absence of signs of infection ? Supraclavicular adenopathy ? with Systemic symptoms What we should do before biopsy ? ? Check viral titer (ex. EBV, CMV), E.S.R., LDH ? Check CXR, arrange abd./neck echo ? Biopsy tissue culture: TB, bacteria, fungi ? obtain tissue chromosome if malignancy is suspected
  • 30. Thoracic mass ? Virtually all primary intrathoracic tumors arise in the mediastinum ? Rarely tumor in lung/rib cage - pleuropulmonary blastomas, desmoplastic small round cell tumors, rhabdomyosarcomas
  • 32. Mediastinal mass Oncogenic Emergency !! -> Superior vena cava Syndrome
  • 33. Mediastinal mass What we do before biopsy ? ? Vital sign & airway stabilize ? Check LDH, AFP, beta-hCG,… ? Chest CT, cardiac echo Indication for biopsy: ? All mediastinal mass, excepting for germ cell tumor w/ elevating tumor marker& characteristic image finding (but biopsy is still preferred)
  • 34. Abdominal mass ? Age plays a major role in the diagnostic considerations ? Neonates ? Most mass are benign, excepting Wilms’ tumor and neuroblastoma ? Infant/Children ? 1st Neuroblastoma → 2nd Wilms’ tumor → 3rd Lymphoma Calcification in X-ray ? Neuroblastoma, Hepatoblastoma, teratoma
  • 35. Unusual presentation ? Disseminated Intravascular Coagulation ? Kaposiform hemangioendothelioma ? Acute promyelocytic leukemia(APML) ? Possible to cause intracranial bleeding ? Rarely: T-ALL, neuroblastoma ? Acquired von Willebrand disease ? Wilms tumor ? Rarely: lymphoma, Ewing sarcoma ? Blueberry muffin syndrome in newborn ? Congenital leukemia cutis ? Histiocytosis ? Neurublastoma
  • 36. Unusual presentation ? Disseminated Intravascular Coagulation ? Kaposiform hemangioendothelioma ? Acute promyelocytic leukemia(APML) ? Possible to cause intracranial bleeding ? Rarely: T-ALL, neuroblastoma ? Acquired von Willebrand disease ? Wilms tumor ? Rarely: lymphoma, Ewing sarcoma ? Blueberry muffin syndrome (in newborn) ? Congenital leukemia cutis ? Histiocytosis ? Neurublastoma ? Raccoon eyes (Periorbital ecchymosis) ? Metastatic Neuroblastoma ? Rarely: Kaposi’s sarcoma ? Must rule out: basal skull fractures, child abuse
  • 37. Unusual presentation ? Disseminated Intravascular Coagulation ? Kaposiform hemangioendothelioma ? Acute promyelocytic leukemia(APML) ? Possible to cause intracranial bleeding ? Rarely: T-ALL, neuroblastoma ? Acquired von Willebrand disease ? Wilms tumor ? Rarely: lymphoma, Ewing sarcoma ? Blueberry muffin syndrome (in newborn) ? Congenital leukemia cutis ? Histiocytosis ? Neurublastoma ? Hypertension ? Renal or adrenal tumor ? Neuroblastoma ? Sexual abnormality / Cushing syndrome ? Gonadal or Adrenal tumor ? Raccoon eyes (Periorbital ecchymosis) ? Metastatic Neuroblastoma ? Rarely: Kaposi’s sarcoma ? Must rule out: basal skull fractures, child abuse