Hematology and Oncology Book

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Ewing Sarcoma

Aka: Ewing Sarcoma, Peripheral Primitive Neuroectodermal Tumor, PPNET
  1. Epidemiology
    1. Incidence: 2.1 cases/million U.S. children
    2. Rare in black children
  2. Types: Ewing Sarcoma Family of Tumors
    1. Ewing Sarcoma of Bone and Soft Tissue
    2. Peripheral Primitive Neuroectodermal Tumor (PPNET)
    3. Askin Tumors (primary chest wall tumors)
      1. When large, may interfere with breathing
  3. Pathophysiology
    1. Small round cell undifferentiated tumor
    2. Associated with chromosome translocation t(11,22)
  4. Symptoms
    1. Pain and swelling limits range of motion of joint
    2. Constitutional (Fever, Weight loss)
  5. Imaging
    1. XRay
      1. Lytic bone lesion
      2. Classic onion-skinning appearance
    2. MRI of lesion
      1. Defines extent of tumor involvement
  6. Differential Diagnosis
    1. Osteosarcoma
    2. Osteomyelitis
    3. Rhabdomyosarcoma (if soft tissue lesion)
    4. Primary Lymphoma involving bone
    5. Langerhans Cell histiocytosis
    6. Metastatic neuroblastoma
  7. Evaluation
    1. Biopsy by surgeon or CT-guided biopsy
    2. Imaging to identify metastases
      1. Chest CT
      2. Bone scan
      3. Bone Marrow Biopsy
  8. Management
    1. Muti-faceted approach
      1. Surgical resection with or without radiation
      2. Multi-drug Chemotherapy
  9. Prognosis
    1. Small, distal, localized tumors: 75% cure
    2. Factors associated with worse prognosis
      1. Pelvic tumors
      2. Metastatic disease
  10. Follow-up: Careful and long-term observation
    1. Relapse may occur even a decade after diagnosis
    2. Cardiotoxicity due to Chemotherapy (Anthracycline)
    3. Secondary malignancy (e.g. Osteosarcoma)
      1. Especially if radiation used

Ewings sarcoma (C0553580)

Definition (NCI) A spectrum of malignant tumors, affecting mostly males under age 20, characterized morphologically by the presence of small round cells. Ewing sarcoma and peripheral primitive neuroectodermal tumor represent the ends of a spectrum, with Ewing sarcoma lacking evidence of neural differentiation and the markers that characterize the peripheral primitive neuroectodermal tumor. Ewing sarcoma and peripheral primitive neuroectodermal tumor may share cytogenetic abnormalities, proto-oncogene expression, cell culture and immunohistochemical abnormalities. These tumors may occur in the soft tissues or the bones. Pain and the presence of a mass are the most common clinical symptoms.
Definition (NCI) A group of cancers that includes Ewing tumor of bone (ETB or Ewing sarcoma of bone), extraosseous Ewing (EOE) tumors, primitive neuroectodermal tumors (PNET or peripheral neuroepithelioma), and Askin tumors (PNET of the chest wall). These tumors all come from the same type of stem cell.
Definition (NCI) Round cell sarcomas with varying degrees of neuroectodermal differentiation. There is a recurrent cytogenetic aberration, most commonly t(11;22)(q24;q12) and t(21;22)(q22;q12).
Definition (MSH) A malignant tumor of the bone which always arises in the medullary tissue, occurring more often in cylindrical bones. The tumor occurs usually before the age of 20, about twice as frequently in males as in females.
Definition (CSP) malignant tumor of bones which always arises in medullary tissue, occurring more often in cylindrical bones, with pain, fever, and leukocytosis.
Definition (NCI) A small round cell tumor that lacks morphologic, immunohistochemical, and electron microscopic evidence of neuroectodermal differentiation. It represents one of the two ends of the spectrum called Ewing's sarcoma/peripheral neuroectodermal tumor. It affects mostly males under age 20, and it can occur in soft tissue or bone. Pain and the presence of a mass are the most common clinical symptoms.
Concepts Neoplastic Process (T191)
MSH D012512
ICD10 M9260/3
SnomedCT 76909002
English Ewing Tumor, Ewing's Tumor, Ewings Tumor, Sarcoma, Ewings, Tumor, Ewing's, EWING TUMOR, SARCOMA, EWING, Ewing Family of Tumors, Ewing's Sarcoma/Peripheral Primitive Neuroectodermal Tumor, Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor, Ewing's Sarcoma, Ewing's sarcoma NOS, Ewing's sarcoma stage unspecified, EWING SARCOMA, ES, Ewing sarcoma, Ewings sarcoma, Sarcoma, Ewing's [Disease/Finding], ewing's sarcoma, ewing's tumors, ewings tumors, ewings sarcoma, ewing sarcoma, ewings's sarcoma, sarcoma ewing, ewing tumor, ewing's tumor, ewings tumor, sarcoma ewing's, sarcoma ewings, Ewing sarcoma family of tumors, EFTs, Sarcoma, Ewing's, Ewing Sarcoma, Sarcoma, Ewing, Ewing's sarcoma, Ewing's tumor, Ewing's tumour, Ewing's sarcoma (morphologic abnormality), sarcoma, Ewing's, Ewing's family of tumors, Tumors of Ewing's Family, Tumors of the Ewing's Family, Ewing's Family of Tumors, Ewing's Family of Tumours
Dutch Ewings tumor, Ewing-sarcoom stadium niet-gespecificeerd, Ewing-tumor, Ewing-sarcoom NAO, Ewing-sarcoom
French Tumeur d'Ewing, Sarcome d'Ewing SAI, Sarcome d'Ewing stade non précisé, Sarcome d'Ewing
German Ewing-Tumor, Ewing-Sarkom, Stadium unspezifisch, Ewing-Sarkom NNB, Ewing-Sarkom, Omoblastom, Sarkom, Ewing-
Italian Sarcoma di Ewing, stadio non specificato, Sarcoma di Ewing NAS, Tumore di Ewing, Sarcoma di Ewing
Portuguese Sarcoma de Ewing estádio NE, Sarcoma de Ewing NE, Sarcoma de Ewing, Tumor de Ewing
Spanish Sarcoma de Ewing, estadio no especificado, Sarcoma de Ewing NEOM, sarcoma de Ewing (anomalía morfológica), sarcoma de Ewing, tumor de Ewing, Sarcoma de Ewing, Tumor de Ewing
Japanese ユーイング肉腫ＮＯＳ, ユーイング肉腫、病期不明, ﾕｰｲﾝｸﾞﾆｸｼｭNOS, ﾕｰｲﾝｸﾞﾆｸｼｭ, ﾕｰｲﾝｸﾞﾆｸｼｭﾋﾞｮｳｷﾌﾒｲ, ﾕｰｲﾝｸﾞｼｭﾖｳ, Ewing腫瘍, Ewing肉腫, ユーイング肉腫, ユーイング腫瘍, 内皮性骨髄腫, 広汎性骨内皮腫, 骨血管内皮腫, 骨髄原性肉腫, 肉腫-Ewing's, 肉腫-Ewing, 肉腫-ユーイング, 肉腫-骨髄原性, 腫瘍-Ewing, 腫瘍-ユーイング
Swedish Sarkom, Ewings
Czech sarkom Ewingův, Ewingův sarkom blíže neurčeného stadia, Ewingův sarkom NOS, Ewingův nádor, Ewingův sarkom
Finnish Ewingin sarkooma
Russian IUINGA OPUKHOL', SARKOMA IUINGA, ENDOTELIOMA KOSTEI DIFFUZNAIA, OMOBLASTOMA, ОМОБЛАСТОМА, САРКОМА ЮИНГА, ЭНДОТЕЛИОМА КОСТЕЙ ДИФФУЗНАЯ, ЮИНГА ОПУХОЛЬ
Polish Mięsak Ewinga, Guz Ewinga
Hungarian Ewing-sarcoma k.m.n., Ewing-tumor, Nem meghatározott stádiumú Ewing-sarcoma, Ewing-sarcoma, Ewing-daganat
Sources
Derived from the NIH UMLS (Unified Medical Language System)


Ewings sarcoma-primitive neuroectodermal tumor (PNET) (C0684337)

Definition (NCI) A type of cancer that forms in bone or soft tissue.
Definition (MSH) A group of highly cellular primitive round cell neoplasms which occur extracranially in soft tissue and bone and are derived from embryonal neural crest cells. These tumors occur primarily in children and adolescents and share a number of characteristics with EWING SARCOMA.
Definition (NCI) A small round cell tumor with neural differentiation arising from the soft tissues or bone.
Concepts Neoplastic Process (T191)
MSH D018241
ICD10 M9364/3
SnomedCT 253096008, 73676002, 254764001
English Neuroectodermal Tumor, Peripheral, Neuroectodermal Tumors, Peripheral, Peripheral Neuroectodermal Tumors, Tumor, Peripheral Neuroectodermal, Tumors, Peripheral Neuroectodermal, Peripheral neuroectodermal tum, PERIPHERAL PRIMITIVE NEUROECTODERMAL NEOPL, NEUROECTODERMAL NEOPL PERIPHERAL PRIMITIVE, PNE - Periph neuroepithelioma, Extracranial Primitive Neuroectodermal Tumor, Primitive Neuroectodermal Tumor, Extracranial, Neuroectodermal Neoplasm, Peripheral Primitive, Peripheral Primitive Neuroectodermal Neoplasm, (pPNET) Peripheral Primitive Neuroectodermal Tumors, Neuroectodermal Tumor, Peripheral Primitive, Neuroectodermal Tumors, Primitive, Peripheral, Peripheral Primitive Neuroectodermal Tumors, NEUROEPITHELIOMA, PERIPHERAL, PNE, Ewings sarcoma-primitive neuroectodermal tumor (PNET), Neuroectodermal Tumors, Primitive, Peripheral [Disease/Finding], Ewing tumor of bone, peripheral neuroectodermal tumor (PNET), pnet, Ewing sarcoma, Peripheral neuroectodermal tumor, Peripheral neuroectodermal tumour, Peripheral neuroepithelioma, PNE - Peripheral neuroepithelioma, Peripheral neuroectodermal tumor (disorder), Peripheral neuroectodermal tumor (morphologic abnormality), Peripheral neuroepithelioma (disorder), Peripheral primitive neuroectodermal tumor, Peripheral primitive neuroectodermal tumour, PPNET, neuroepithelioma, peripheral, pPNET, peripheral neuroectodermal tumor, peripheral primitive neuroectodermal tumor, primitive neuroectodermal tumor, peripheral, primitive peripheral neuroectodermal tumor, Ewing's sarcoma/primitive neuroectodermal tumor (PNET), Peripheral Neuroectodermal Tumor, Peripheral Neuroectodermal Neoplasm, Peripheral Neuroepithelioma, Peripheral PNET, Peripheral Primitive Neuroectodermal Tumor
Italian Neuroepitelioma periferico, Tumore neuroectodermico primitivo periferico, Tumori neuroectodermici periferici, Tumori neuroectodermici primitivi periferici (pPNET)., Neoplasia neuroectodermica primitiva periferica, Tumori neuroectodermici primitivi periferici
Japanese 末梢性神経上皮腫, ﾏｯｼｮｳｾｲｼﾝｹｲｼﾞｮｳﾋｼｭ, 末梢神経外胚葉性腫瘍, 神経上皮腫, 神経外胚葉腫-末梢, 末梢神経外胚葉腫瘍, 神経外胚葉性腫瘍-末梢, 神経外胚葉腫瘍-末梢, 末梢未分化神経外胚葉性腫瘍, 神経外胚葉性腫瘍-未分化-末梢, 末梢原始神経外胚葉性腫瘍, 神経外胚葉性腫瘍-原始-末梢, 未分化神経外胚葉性腫瘍-末梢, 末梢神経外胚葉腫, 原始神経外胚葉性腫瘍-末梢, 神経外胚葉性腫瘍-末梢原始
Swedish Neuroektodermala tumörer, primitiva, perifera
Czech neuroektodermové nádory primitivní periferní, Periferní neuroepiteliom
Finnish Primitiiviset perifeeriset neuroektodermaaliset kasvaimet
Russian NEIROEKTODERMAL'NYE NOVOOBRAZOVANIIA NEDIFFERENTSIROVANNYE PERIFERICHESKIE, NEIROEPITELIOMA, NEIROEKTODERMAL'NAIA OPUKHOL' PERIFERICHESKAIA, NEIROEKTODERMAL'NOE NOVOOBRAZOVANIE PERIFERICHESKOE, НЕЙРОЭКТОДЕРМАЛЬНАЯ ОПУХОЛЬ ПЕРИФЕРИЧЕСКАЯ, НЕЙРОЭКТОДЕРМАЛЬНОЕ НОВООБРАЗОВАНИЕ ПЕРИФЕРИЧЕСКОЕ, НЕЙРОЭКТОДЕРМАЛЬНЫЕ НОВООБРАЗОВАНИЯ НЕДИФФЕРЕНЦИРОВАННЫЕ ПЕРИФЕРИЧЕСКИЕ, НЕЙРОЭПИТЕЛИОМА
Croatian Not Translated[Neuroectodermal Tumors, Primitive, Peripheral]
Polish Guzy neuroektodermalne pierwotne obwodowe
Hungarian Peripheriás neuroepithelioma
Spanish neuroepitelioma periférico (trastorno), neuroepitelioma periférico, tumor neuroectodérmico periférico (anomalía morfológica), tumor neuroectodérmico periférico (trastorno), tumor neuroectodérmico periférico, Neuroepitelioma periférico, Tumores Neuroectodérmicos Periféricos Primitivos, Tumores Neuroectodérmicos Primitivos Periféricos, Tumor Neuroectodérmico Periférico, Tumor Neuroectodermico Periferico, Tumores Neuroectodermicos Perifericos Primitivos, Tumores Neuroectodermicos Primitivos Perifericos
Portuguese Neuroepitelioma, Tumores Neuroectodérmicos Periféricos Primitivos, Tumores Neuroectodérmicos Primitivos Periféricos, Tumor Neuroectodérmico Periférico
Dutch perifeer neuro-epithelioom, Neuro-ectodermale tumor, perifere, Perifere primitieve neuro-ectodermale tumor, Perifere primitieve neuro-ectodermale tumoren, Tumor, neuro-ectodermale primitieve perifere, Tumoren, neuro-ectodermale primitieve perifere
French Neuro-épithéliome périphérique, Neuroblastome périphérique, Tumeurs neuro-ectodermiques primitives périphériques, Tumeurs neuroectodermiques primitives extra-crâniennes, Tumeurs neuroectodermiques primitives périphériques, Tumeurs neuroectodermiques périphériques primitives
German peripheres Neuroepitheliom, Neuroektodermaler Tumor, peripherer, Neuroektodermale Tumor, primitive periphere, Peripherer primitiver neuroektodermaler Tumor
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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