Primary Cutaneous NUT Adnexal Carcinoma

Preferred Label : Primary Cutaneous NUT Adnexal Carcinoma;

NCIt synonyms : NUT Sweat Gland Carcinoma; NUT Adnexal Carcinoma; Primary Cutaneous NUT Sweat Gland Carcinoma;

NCIt definition : A very rare carcinoma that arises from the sweat glands. It is characterized by mutations and rearrangement of the NUT gene. It presents as a papular or nodular skin lesion and has been described in the hand, shoulder, trunk, and lower limb. Histologically, there is a proliferation of malignant epithelial cells in the dermis and/or subcutaneous tissue. The tumor cells display round nuclei with prominent single nucleoli. Features include the presence of small round ducts and foci of abrupt keratinization.;

Neoplastic status : Malignant;

Concept status : Provisional_Concept;

NCI Metathesaurus CUI : CL1927618;

Details

Origin ID

C205357;

Disease excludes abnormal cell
- Malignant Squamous Cell [NCIt concept]
- Malignant Stromal Cell [NCIt concept]
- Neoplastic Smooth Muscle Cell [NCIt concept]
- Neoplastic Squamous Cell [NCIt concept]
Has associated anatomic sites
- Integumentary System [NCIt concept]
- Skin [NCIt concept]
Has cytogenetic abnormalities
- t(15;19)(q13;p13.1) [NCIt concept]
disease_excludes_finding
- Benign Cellular Infiltrate [NCIt concept]
- Mixed Neoplastic Glandular and Squamous Cell Population [NCIt concept]
disease_has_abnormal_cell
- Adenocarcinoma Cell [NCIt concept]
- Malignant Cell [NCIt concept]
- Malignant Epithelial Cell [NCIt concept]
- Neoplastic Cell [NCIt concept]
- Neoplastic Epithelial Cell [NCIt concept]
- Neoplastic Glandular Cell [NCIt concept]
disease_has_finding
- Adnexal Differentiation [NCIt concept]
- Aggressive Clinical Course [NCIt concept]
- Carcinomatous Component Present [NCIt concept]
- Cutaneous Involvement [NCIt concept]
- Epithelial Component Present [NCIt concept]
- Malignant Cellular Infiltrate [NCIt concept]
- Poorly Differentiated Lesion [NCIt concept]
disease_has_molecular_abnormality
- BRD4-NUT Fusion Protein Expression [NCIt concept]
disease_has_normal_cell_origin
- Epithelial Cell [NCIt concept]
disease_has_normal_tissue_origin
- Epithelial Tissue [NCIt concept]
disease_has_primary_anatomic_site
- Exocrine System [NCIt concept]
- Midline Anatomic Structure [NCIt concept]
- Skin [NCIt concept]
- Sweat Gland [NCIt concept]
disease_mapped_to_gene
- BRD4/NUTM1 Fusion Gene [NCIt concept]

Keyword's position in hierarchy(ies) :

You can consult :

recommendations
documents concerning education
documents for patients