Malignant epithelial tumor of salivary…

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Malignant epithelial tumor of salivary glands

ORPHA:276145Disease

What it is

A rare neoplastic disease characterized by the presence of a tumor located in the parotid, sublingual, submandibular and/or minor salivary glands, which presents with a wide spectrum of clinical features depending on the location, size and type of salivary gland involved, ranging from clinically asymptomatic, slow-growing, painless mass(es), that may or may not be fixed to underlying skin or muscles, to rapidly growing mass(es) associated with pain, facial weakness/nerve palsy, otorrhoea, dysphagia, palatal/parapharyngeal fullness, nasal obstruction/bleeding, voice hoarseness/change, dyspnea, trismus, palate bone erosion, telangiectasia, mucosal/skin ulceration and/or cervical adenopathy.

Key facts

Prevalence
1-9 / 1 000 000 (annual incidence, Europe)
Age of onset
Adult
Inheritance
Not applicable
Classified as
Disease

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Gene

PRKD1Disease-causing somatic mutation(s)

ICD-10 codes

C07ICD-10 uses a narrower term
C08.0ICD-10 uses a narrower term
C08.1ICD-10 uses a narrower term
C08.8ICD-10 uses a narrower term

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

MONDO 0017167UMLS C5190832

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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