Hematology and Oncology Book

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Ewing SarcomaAka: Peripheral Primitive Neuroectodermal Tumor, PPNET

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  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 (MSH)A malignant tumor of the bone which always arises in the medullary tissue, occurring more often in cylindrical bones. There are conspicuous foci of necrosis in association with irregular masses of small, regular, rounded or ovoid cells with very scanty cytoplasm. The tumor occurs usually before the age of 20, about twice as frequently in males as in females. (From Dorland, 27th ed; Stedman, 25th ed)
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.
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. -- 2004
ConceptsNeoplastic Process (T191)
MSHD012512
EnglishES, Ewing Family of Tumors, Ewing Sarcoma, Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor, Ewing Tumor, Ewing's family of tumors, Ewing's Family of Tumours, Ewing's sarcoma, Ewing's Sarcoma/Peripheral Primitive Neuroectodermal Tumor, Ewing's tumor, Ewing's tumour, Ewings sarcoma, Ewings Tumor, Tumors of Ewing's Family, Tumors of the Ewing's Family
Spanishsarcoma de Ewing, tumor de Ewing
Parent ConceptsBone neoplasms (C0005967), sarcoma (C1261473), osteosarcoma (C0029463), Neoplasms, Embryonal (C0027654), Ewings sarcoma (C0553580), Malignant Childhood Neoplasm (C0278704), childhood solid tumor (C0279068), Malignant Bone Neoplasm (C0279530), Malignant neoplasm of muscle (C0684743), Sarcoma - category (C1299262), Primary malignant neoplasm (C1306459)
SourcesCOSTAR, CSP, DXP, MEDLINEPLUS, MSH, MTH, NCI, NDFRT, OMIM, PDQ, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)


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's Sarcoma (SARCOMA, EWING'S). They may arise from the chest wall, skin, orbit, kidney, and other structures and tend to be locally invasive or metastasize, although relatively benign forms may occur. Characteristic histologic features include a tendency to form Homer-Wright rosettes and to stain positively with neuron-specific enolase and vimentin. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, p2113; J Clin Oncol 1998 Mar;16(3):1150-7)
Definition (NCI)A small round cell tumor with neural differentiation arising from the soft tissues or bone.
ConceptsNeoplastic Process (T191)
MSHD018241
EnglishExtracranial Primitive Neuroectodermal Tumor, NEUROECTODERMAL NEOPL PERIPHERAL PRIMITIVE, Peripheral Neuroectodermal Neoplasm, Peripheral neuroectodermal tumor, Peripheral Neuroectodermal Tumors, Peripheral neuroectodermal tumour, Peripheral neuroepithelioma, Peripheral PNET, PERIPHERAL PRIMITIVE NEUROECTODERMAL NEOPL, Peripheral Primitive Neuroectodermal Neoplasm, peripheral primitive neuroectodermal tumor, Peripheral Primitive Neuroectodermal Tumors, Peripheral primitive neuroectodermal tumour, PNE, PNE - Peripheral neuroepithelioma, PPNET, primitive peripheral neuroectodermal tumor
Spanishneuroepitelioma periferico, tumor neuroectodermico periferico
Parent ConceptsNeuroectodermal Tumor, Primitive (C0206663), Neuroectodermal Tumors (C0206093), Ewings sarcoma (C0553580), Peripheral Nerve Neoplasms, Malignant (C0751428), Malignant Childhood Neoplasm (C0278704), childhood solid tumor (C0279068), Malignant Bone Neoplasm (C0279530), Malignant neoplasm of muscle (C0684743), Skin tumor of neural origin (C0346055), Primary malignant neoplasm (C1306459), Primary malignant neoplasm of skin (C1314758)
SourcesMSH, MTH, NCI, NDFRT, OMIM, PDQ, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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