ORPHA:213531

Metaplastic carcinoma of the breast

Type: Disease
Group: Disorder
Age of onset: Adult
Status: active

Metaplastic carcinoma of the breast is a rare, aggressive subtype of invasive breast carcinoma characterized by rapid growth, relatively large tumor size and a tendency to metastasize to distant organs, particularly the lungs, with relatively less frequent involvement of the axillary lymph nodes. Histologically, the tumor shows high-grade cellularity and heterologous differentiation, including chondroid, osseous, pleomorphic/sarcomatoid, spindled, and squamous elements. Patients usually present with a fast-growing, large, well-circumscribed, mobile lump in the breast, which can become painful and involve the chest wall and the skin, leading to ulceration.

Names in Orphadata languages

12 of 12 languages

Lang	Name	Synonyms
en	Metaplastic carcinoma of the breast
fr	Carcinome métaplasique du sein
de	Metaplastisches Karzinom der Brust
es	Carcinoma metaplásico de mama
it	Carcinoma metaplastico della mammella
nl	Metaplastisch carcinoom van borst
pt	Carcinoma metaplásico da mama
pl	Metaplastyczny rak piersi
cs	Metaplastický karcinom prsu
uk	Метапластична карцинома молочної залози
zh	乳腺化生性癌
tr	Metaplastik meme karsinomu

Definition in:

Cross-references

qualifier = Orphanet mapping relation

Source	Code	Qualifier	ICD relation	Validation
ICD-10	C50.0	BTNT	attributed	validated
ICD-10	C50.1	BTNT	attributed	validated
ICD-10	C50.2	BTNT	attributed	validated
ICD-10	C50.3	BTNT	attributed	validated
ICD-10	C50.4	BTNT	attributed	validated
ICD-10	C50.5	BTNT	attributed	validated
ICD-10	C50.6	BTNT	attributed	validated
ICD-10	C50.8	BTNT	attributed	validated
ICD-11	2C6Y	NTBT	index	validated
MONDO	0006043	E		validated
UMLS	C1334708	E		validated
GARD	10804	E		validated
MedDRA	10073100	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):

ORPHA:250908Rare neoplastic disease

Parents in the classifications (1):

ORPHA:180257Rare malignant breast tumor
rare neoplastic diseases; rare gynecological and obstetric diseases

No children.

Genes

0 direct

Orphanet records no gene directly on this code.

Prevalence and incidence

24 records

Type	Class	Value	Area	Qualification	Validation	Source
Annual incidence	<1 / 1 000 000	0.06 / 100 000	Europe	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence	<1 / 1 000 000	0.074 / 100 000	Estonia	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence	<1 / 1 000 000	0.065 / 100 000	Finland	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence	<1 / 1 000 000	0.043 / 100 000	Iceland	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence	<1 / 1 000 000	0.096 / 100 000	Ireland	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence	<1 / 1 000 000	0.084 / 100 000	Italy	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence	<1 / 1 000 000	0.06 / 100 000	Latvia	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence	<1 / 1 000 000	0.031 / 100 000	Malta	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence	<1 / 1 000 000	0.045 / 100 000	Poland	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence	<1 / 1 000 000	0.082 / 100 000	Portugal	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence	<1 / 1 000 000	0.074 / 100 000	Slovakia	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence	<1 / 1 000 000	0.07 / 100 000	United Kingdom	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence	1-9 / 1 000 000	0.226 / 100 000	Belgium	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence	1-9 / 1 000 000	0.11 / 100 000	Czech Republic	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence	1-9 / 1 000 000	0.136 / 100 000	Germany	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence	1-9 / 1 000 000	0.108 / 100 000	Lithuania	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence	1-9 / 1 000 000	0.104 / 100 000	Norway	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence	1-9 / 1 000 000	0.163 / 100 000	Slovenia	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence	1-9 / 1 000 000	0.128 / 100 000	Spain	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence	1-9 / 1 000 000	0.135 / 100 000	Switzerland	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence	1-9 / 1 000 000	0.238 / 100 000	Netherlands	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence	<1 / 1 000 000	0.081 / 100 000	Austria	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence	<1 / 1 000 000	0.027 / 100 000	Bulgaria	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence	<1 / 1 000 000	0.059 / 100 000	Croatia	Value and class	Validated	RARECARE surveillance of rare cancers in Europe[OTHER]

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

Sources for this card

Orphanet page: orpha.net/en/disease/detail/213531
This record as JSON: /data/d/213531.json