II. Epidemiology

  1. Incidence: One in 100,000 aged 10 to 19 years old
    1. Second most common Bone Cancer (Osteosarcoma is most common)
    2. Accounts for one third of U.S. Bone Cancer cases
  2. Race
    1. Rare in black children (contrast with Osteosarcoma)
    2. More common in white and asian patients
  3. Age
    1. Median onset age 15 years old
  4. Gender
    1. Slightly more common in males

III. Risk Factors

  1. Chromosome 11 and 22 translocation (95% of cases)
  2. Chromosome 21 and 22 translocation (5-10% of cases)

IV. 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

V. Pathophysiology

  1. Small round cell undifferentiated tumor
    1. Likely derives from primitive neuroectodermal and neural crest cells or possibly stem cells
    2. Grouped with other blue round cell tumors (based on histology)
  2. Associated with Chromosome translocation t(11,22)
  3. Primary tumor distribution
    1. Pelvis (most common)
    2. Femoral diaphysis
    3. Tibia diaphysis
    4. Humerus diaphysis
    5. Ribs
    6. Scapula
  4. Metastatic locations
    1. Lungs
    2. Other bones

VI. Symptoms

  1. Pain and swelling limits range of motion of joint
  2. Constitutional (Fever, Weight loss)
  3. May present as Pediatric Limp

VII. Imaging

  1. XRay (may be normal)
    1. Bone destruction (75% of cases)
    2. Soft tissue extension (64% of cases)
    3. Reactive bone formation (25%)
    4. Classic onion-skinning appearance (lamellated periosteal reaction) in 23% of cases
    5. Sunburst pattern (radiating calcification) in 20% of cases
  2. MRI of lesion
    1. Defines extent of tumor involvement

VIII. Differential Diagnosis

  1. Osteosarcoma
  2. Osteomyelitis
  3. Rhabdomyosarcoma (if soft tissue lesion)
  4. Primary Lymphoma involving bone
  5. Langerhans Cell Histiocytosis
  6. Metastatic Neuroblastoma

IX. Evaluation

  1. Biopsy by surgeon or CT-guided biopsy
  2. Imaging to identify metastases
    1. Chest CT
    2. Bone scan
    3. Bone Marrow Biopsy

X. Management

  1. Surgical Excision with clear margin excision
  2. Chemotherapy (neoadjuvant before surgery and adjuvant after surgery)
    1. Cyclophosphamide
    2. Doxorubicin
    3. Etoposide
    4. Ifosamide
    5. Vincristine
  3. Radiation Therapy Indications (contrast with Osteosarcoma, in which radiation is not used)
    1. Lung metastases
    2. Palliative Care
    3. Paraspinal tumors with neurologic compromise
    4. Positive margins on resection
    5. Unresectable tumor

XI. Prognosis

  1. Small, distal, localized tumors: 75% cure
  2. See Osteosarcoma for positive and negative prognostic factors
    1. Osteosarcoma share the same prognostic factors
    2. Pelvic tumors are also associated with worse prognosis

XII. 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

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Related Studies

Ontology: Ewings sarcoma (C0553580)

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 (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 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.
Definition (NCI_NCI-GLOSS) A type of cancer that forms in bone or soft tissue.
Definition (CSP) malignant tumor of bones which always arises in medullary tissue, occurring more often in cylindrical bones, with pain, fever, and leukocytosis.
Concepts Neoplastic Process (T191)
MSH D012512
ICD10 M9260/3
SnomedCT 76909002
English Ewing's Tumor, Ewings Tumor, Sarcoma, Ewings, Tumor, Ewing's, Ewing's sarcoma NOS, Ewing's sarcoma stage unspecified, EWING SARCOMA, ES, Ewing sarcoma, Ewings sarcoma, 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, Sarcoma, Ewing's, Ewing Sarcoma, Sarcoma, Ewing, Sarcoma, Ewing [Disease/Finding], Tumor, Ewing, Ewing Tumor, Ewing's Sarcoma, Ewings Sarcoma, Ewing's sarcoma, Ewing's tumor, Ewing's tumour, Ewing's sarcoma (morphologic abnormality), sarcoma, Ewing's, Ewing's family of tumors
Dutch Ewings tumor, Ewing-sarcoom stadium niet-gespecificeerd, Ewing-tumor, Ewing-sarcoom NAO, Ewing-sarcoom
French Sarcome d'Ewing SAI, Sarcome d'Ewing stade non précisé, Tumeur d'Ewing, 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, ユーイングニクシュNOS, ユーイングニクシュ, ユーイングニクシュビョウキフメイ, ユーイングシュヨウ, 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
Finnish Ewingin sarkooma
Czech Ewingův sarkom blíže neurčeného stadia, Ewingův sarkom NOS, Ewingův nádor, sarkom Ewingův, Ewingův sarkom
Polish Mięsak Ewinga, Guz Ewinga
Hungarian Ewing-sarcoma k.m.n., Ewing-tumor, Nem meghatározott stádiumú Ewing-sarcoma, Ewing-sarcoma, Ewing-daganat
Croatian Sarkom, Ewingov, Ewingov sarkom
Norwegian Ewings sarkom

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

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.
Definition (NCI_NCI-GLOSS) A type of cancer that forms in bone or soft tissue.
Concepts Neoplastic Process (T191)
MSH D018241
ICD10 M9364/3
SnomedCT 703707001, 253096008, 73676002
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, 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, Ewings sarcoma-primitive neuroectodermal tumor (PNET), Neuroectodermal Tumors, Primitive, Peripheral [Disease/Finding], Ewing tumor of bone, peripheral neuroectodermal tumor (PNET), pnet, peripheral neuroectodermal tumor, malignant neoplasm primary peripheral neuroectodermal tumor, peripheral neuroectodermal tumor (diagnosis), Ewing sarcoma / peripheral neuroectodermal tumor (morphologic abnormality), Ewing sarcoma / peripheral neuroectodermal tumor, Ewing sarcoma / PNET, Ewing sarcoma / peripheral neuroectodermal tumour, Peripheral neuroectodermal tumor, Peripheral neuroectodermal tumour, Peripheral neuroectodermal tumor (disorder), Peripheral neuroectodermal tumor (morphologic abnormality), Peripheral primitive neuroectodermal tumor, Peripheral primitive neuroectodermal tumour, PPNET, neuroepithelioma, peripheral, pPNET, 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
Swedish Neuroektodermala tumörer, primitiva, perifera
Czech neuroektodermové nádory primitivní periferní
Finnish Primitiiviset perifeeriset neuroektodermaaliset kasvaimet
Japanese 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
Italian Tumore neuroectodermico primitivo periferico, Tumori neuroectodermici periferici, Tumori neuroectodermici primitivi periferici (pPNET)., Neoplasia neuroectodermica primitiva periferica, Tumori neuroectodermici primitivi periferici
Croatian Not Translated[Neuroectodermal Tumors, Primitive, Peripheral]
Polish Guzy neuroektodermalne prymitywne obwodowe
Norwegian Perifere primitive nevroektodermale svulster, Primitive perifere nevroektodermale tumorer
Spanish tumor neuroectodérmico periférico (anomalía morfológica), tumor neuroectodérmico periférico (trastorno), tumor neuroectodérmico periférico, Tumores Neuroectodérmicos Periféricos Primitivos, Tumores Neuroectodérmicos Primitivos Periféricos, Tumor Neuroectodérmico Periférico
French Neuroblastome périphérique, Tumeurs neuro-ectodermiques primitives périphériques, Tumeurs neuroectodermiques primitives extra-crâniennes, Tumeurs neuroectodermiques primitives périphériques, Tumeurs neuroectodermiques périphériques primitives
German Neuroektodermaler Tumor, peripherer, Neuroektodermale Tumor, primitive periphere, Peripherer primitiver neuroektodermaler Tumor
Dutch Neuro-ectodermale tumor, perifere, Perifere primitieve neuro-ectodermale tumor, Perifere primitieve neuro-ectodermale tumoren, Tumor, neuro-ectodermale primitieve perifere, Tumoren, neuro-ectodermale primitieve perifere
Portuguese Tumores Neuroectodérmicos Periféricos Primitivos, Tumores Neuroectodérmicos Primitivos Periféricos, Tumor Neuroectodérmico Periférico