raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:97285

Thyroid lymphoma

Type
Disease
Group
Disorder
Age of onset
No data available
Status
active

A rare primary organ-specific lymphoma characterized by primary origin in the thyroid gland, sometimes involving cervical lymph nodes, and infrequently more distant sites. Diffuse large B-cell lymphoma is most common, followed by MALT lymphoma, and follicular lymphoma. More rare types include T-cell lymphomas, Burkitt lymphoma, or classic Hodgkin lymphoma. The condition is usually associated with Hashimoto thyroiditis. Patients typically present with a mass in the thyroid, with or without cervical lymphadenopathy. Hoarseness and dyspnea may occur, while constitutional symptoms are rare. Prognosis is favorable for patients with localized tumors.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enThyroid lymphoma
frLymphome thyroïdien
deSchilddrüsenlymphom
esLinfoma tiroideo
itLinfoma tiroideo
nlSchildklierlymfoom
ptLinfoma da tiroide
plChłoniak tarczycy
csLymfom štítné žlázy
ukЛімфома щитовидної залози
zh甲状腺淋巴瘤
trTiroid lenfoması

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10C85.7NTBTattributedvalidated
ICD-112B33.5NTBTindexvalidated
MONDO0019962Evalidated
UMLSC1336753Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (2):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

1 record
TypeClassValueAreaQualificationValidationSource
Annual incidenceUnknown—WorldwideClass onlyNot yet validated23714679[PMID]

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

6

HPO phenotypes

14 annotations · validated 2016-06-08
HPOTerm (as annotated by Orphanet)Diagnostic
Obligate (100%)
HP:0000853Goiter
HP:0002665Lymphoma
Very frequent (99-80%)
HP:0000475Broad neck
Frequent (79-30%)
HP:0002015Dysphagia
HP:0002094Dyspnea
HP:0000872Hashimoto thyroiditis
HP:0001609Hoarse voice
HP:0000821Hypothyroidism
HP:0002716Lymphadenopathy
HP:0010307Stridor
HP:0002781Upper airway obstruction
Occasional (29-5%)
HP:0000836Hyperthyroidism
HP:0012531Pain
Very rare (<4-1%)
HP:0002098Respiratory distress

Run the differential on the 3 obligate / very frequent terms

Annotation source: 23714679[PMID]_24077086[PMID]_20497878[PMID]_12417514[PMID]

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Sources for this card