Childhood Vulvar Botryoid-Type Embryonal Rhabdomyosarcoma

Preferred Label : Childhood Vulvar Botryoid-Type Embryonal Rhabdomyosarcoma;

NCIt synonyms : Childhood Sarcoma Botryoides of the Vulva; Vulvar Childhood Botryoid-Type Embryonal Rhabdomyosarcoma; Childhood Botryoid-Type Embryonal Rhabdomyosarcoma of the Vulva; Childhood Vulvar Embryonal Rhabdomyosarcoma;

NCIt definition : A morphologic variant of embryonal rhabdomyosarcoma occurring in female children. The neoplasm arises from the vulva. It is characterized by the formation of a cambium layer in the affected tissue and polypoid nodules with an abundant myxoid stroma.;

Neoplastic status : Malignant;

Details

Origin ID

C36098;

UMLS CUI

C1332946;

Automatic exact mappings (from CISMeF team)
- vulvar childhood botryoid-type embryonal rhabdomyosarcoma [DO Concept]
DO Cross reference
- vulvar childhood botryoid-type embryonal rhabdomyosarcoma [DO Concept]
Disease excludes normal cell origin
- Epithelial Cell [NCIt concept]
- Neuron, Neuroepithelial Cell, and Supporting Cell of the Nervous System [NCIt concept]
Excludes anatomical site(s)
- Bone [NCIt concept]
Has associated anatomic sites
- Connective and Soft Tissue [NCIt concept]
- Female Reproductive System [NCIt concept]
- Genitourinary System [NCIt concept]
- Musculoskeletal System [NCIt concept]
- Reproductive System [NCIt concept]
- Vulva [NCIt concept]
Has cytogenetic abnormalities
- Loss of Chromosome 11p [NCIt concept]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
UMLS correspondences (same concept)
- vulvar childhood botryoid-type embryonal rhabdomyosarcoma [DO Concept]
disease_excludes_finding
- Benign Cellular Infiltrate [NCIt concept]
- Indolent Clinical Course [NCIt concept]
- Intermediate Filaments Present [NCIt concept]
- Intracytoplasmic Eosinophilic Inclusion [NCIt concept]
- Well-Circumscribed Lesion [NCIt concept]
disease_excludes_normal_tissue_origin
- Epithelial Tissue [NCIt concept]
disease_has_abnormal_cell
- Malignant Cell [NCIt concept]
- Malignant Rhabdomyoblast [NCIt concept]
- Malignant Skeletal Muscle Cell [NCIt concept]
- Neoplastic Cell [NCIt concept]
- Neoplastic Connective and Soft Tissue Cell [NCIt concept]
- Neoplastic Muscle Cell [NCIt concept]
- Neoplastic Skeletal Muscle Cell [NCIt concept]
- Primitive Malignant Skeletal Muscle Cell [NCIt concept]
disease_has_finding
- Cambium Layer [NCIt concept]
- Childhood Lesion [NCIt concept]
- Exophytic Polypoid Lesion [NCIt concept]
- High Grade Lesion [NCIt concept]
- Malignant Cellular Infiltrate [NCIt concept]
- Myxoid Stroma Formation [NCIt concept]
- Rapidly Enlarging Mass [NCIt concept]
disease_has_normal_cell_origin
- Connective and Soft Tissue Cell [NCIt concept]
- Muscle Cell [NCIt concept]
- Skeletal Muscle Cell [NCIt concept]
disease_has_normal_tissue_origin
- Connective and Soft Tissue [NCIt concept]
- Muscle Tissue [NCIt concept]
- Skeletal Muscle Tissue [NCIt concept]
- Soft Tissue [NCIt concept]
disease_has_primary_anatomic_site
- Female Reproductive System [NCIt concept]
- Vulva [NCIt concept]
disease_may_have_cytogenetic_abnormality
- Rearrangement of 12q13 [NCIt concept]
- Rearrangement of 1p11-q11 [NCIt concept]
- del(11p15) [NCIt concept]
disease_may_have_molecular_abnormality
- FGFR4 Gene Mutation [NCIt concept]

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