Pilomatrix carcinoma
Synonyms: Calcified epithelial carcinoma of Malherbe · Calcifying epitheliocarcinoma · Malignant pilomatricoma · Trichomatrical carcinoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Elderly
- Status
- active
A rare skin tumor characterized by an asymptomatic, solitary, often ulcerated nodule most commonly located in the face, involving the deep dermis, subcutaneous tissue, and skeletal muscle and fascia. Histopathologically, the lesion is composed of aggregates of atypical basaloid cells with numerous mitoses. Typical features include shadow cells, keratin cysts, and trichohyalin and keratohyalin granules. The tumor is locally aggressive and shows a tendency to recur after incomplete excision. Regional lymph node or visceral metastasis has been reported.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Pilomatrix carcinoma | Calcified epithelial carcinoma of Malherbe · Calcifying epitheliocarcinoma · Malignant pilomatricoma · Trichomatrical carcinoma |
| fr | Carcinome pilomatriciel | Carcinome trichomatriciel · Epithéliocarcinome de Malherbe · Epithéliome calcifié de Malherbe · Pilomatricocarcinome · Pilomatricome malin |
| de | Pilomatrixkarzinom | Epithelkarzinom, kalzifizierendes · Kalzifiziertes Epithelkarzinom Malherbe · Pilomatrixom, malignes · Trichomatrisches Karzinom |
| es | Carcinoma pilomatricial | Carcinoma epitelial calcificado de Malherbe · Carcinoma tricomatricial · Epiteliocarcinoma calcificado · Pilomatricoma maligno · Pilomatrixoma maligno |
| it | Carcinoma pilomatricale | |
| nl | Pilomatrixcarcinoom | Calcificerend epitheelcarcinoom · Calcificerend epithelioom van Malherbe · Maligne pilomatricoom · Trichomatrisch carcinoom · Verkalkt epitheliaal carcinoom van Malherbe |
| pt | Carcinoma pilomatrix | |
| pl | Rak macierzy włosa | Nabłoniak wapniejący złośliwy · Nabłoniakorak wapniejący · Rak nabłonkowy wapniejący Malherbego |
| cs | Pilomatrix karcinom | Malignant pilomatricoma · Trichomatrical carcinoma · Calcifying epitheliocarcinoma · Calcified epithelial carcinoma of Malherbe · Maligní pilomatrikom · Kalcifikující epiteliokarcinom · Malherbův kalcifikovaný epiteliální karcinom · Trichomatrikální karcinom |
| uk | Піломатріксна карцинома | Кальцинована епітеліальна карцинома Мальхербе · Кальцинуюча епітеліокарцинома · Злоякісна піломатрікома · Трихоматична карцинома |
| zh | 阴唇癌 | Malherbe钙化上皮癌 |
| tr | Pilomatriks karsinomu | Habis pilomatricoma · Trikomatrik karsinom · Kalsifiye epiteliyokarsinom · Malherbe'nin kalsifiye epitel karsinomu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C44.3 | BTNT | attributed | validated |
| ICD-10 | C44.4 | BTNT | attributed | validated |
| ICD-11 | 2C3Y | NTBT | attributed | validated |
| MONDO | 0003414 | E | validated | |
| UMLS | C0585475 | E | validated | |
| MedDRA | 10075614 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:79386Rare skin tumor or hamartoma
rare skin diseases; rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/499182
- This record as JSON: /data/d/499182.json