Editorial-Orientierung von MedAPI — kein Bestandteil der Kodieranweisung.
C07 denotes a primary malignant neoplasm of the parotid gland, the largest major salivary gland. Parotid malignancies are uncommon and histologically diverse. A malignant-appearing parotid-region mass may also represent lymphoma or metastatic disease involving intraparotid lymph nodes—particularly cutaneous squamous cell carcinoma or melanoma—rather than a primary salivary carcinoma.
Common Histologies
- Mucoepidermoid carcinoma
- Acinic cell carcinoma
- Adenoid cystic carcinoma
- Salivary duct carcinoma
- Carcinoma ex pleomorphic adenoma
- Secretory carcinoma
- Epithelial-myoepithelial carcinoma
- Adenocarcinoma or carcinoma not otherwise specified
Tumor grade and histologic subtype strongly influence recurrence risk, metastatic pattern, and treatment.
Common Symptoms
- Painless (R52), enlarging preauricular (R22.0), infra-auricular (R22.0), or upper-neck mass (R22.1)
- Pain (R52), fixation (Z47.2), skin involvement (R23.9), or rapid growth
- Facial weakness (R29.810), paralysis (G51.9), or suggesting facial nerve involvement (G51.9)
- Trismus (M26.52), numbness (R20.0), otalgia (H92.09), or symptoms of skull-base/perineural spread
- Cervical lymphadenopathy (R59.0)
Diagnosis
Evaluation typically includes head and neck examination with facial nerve assessment, cervical nodal examination, and review for prior or current skin cancers. Ultrasound, contrast-enhanced CT, and MRI help define local extent; MRI is particularly useful when perineural or skull-base involvement is suspected. Fine-needle aspiration or image-guided core biopsy may establish histology, although definitive classification sometimes requires resection. Chest or whole-body imaging may be used for advanced or high-grade disease.
Staging
Primary parotid carcinomas are staged using the TNM system for major salivary glands. Relevant features include tumor size, extraparenchymal extension, facial nerve involvement, invasion of adjacent structures, regional nodal disease, extranodal extension, and distant metastasis. Common distant sites include lung, bone, and liver, with late metastases possible in adenoid cystic carcinoma.
Molecular Markers
Selected findings may support diagnosis or guide therapy:
- CRTC1/3–MAML2 fusions in many mucoepidermoid carcinomas
- MYB or MYBL1 rearrangements in adenoid cystic carcinoma
- ETV6–NTRK3 fusion in secretory carcinoma
- Androgen receptor expression and ERBB2/HER2 amplification or overexpression in subsets of salivary duct carcinoma
- PLAG1 or HMGA2 alterations in some carcinoma ex pleomorphic adenoma cases
Broad molecular profiling may be considered in unresectable, recurrent, or metastatic disease.
Common Treatments
Surgery is the principal treatment for resectable disease, usually with an appropriate parotidectomy and preservation of the facial nerve when oncologically feasible. Therapeutic or elective neck dissection may be indicated according to nodal status, grade, histology, and tumor extent. Postoperative radiation is commonly considered for high-grade tumors, positive or close margins, perineural invasion, advanced local disease, or nodal involvement. Unresectable, recurrent, or metastatic disease may be managed with radiation, systemic therapy, or biomarker-directed treatment such as NTRK-, HER2-, or androgen receptor–targeted therapy in selected tumors.
Scope Note
C07 identifies the anatomic site of a primary malignant neoplasm of the parotid gland. It does not encode histologic subtype, grade, TNM stage, laterality, molecular profile, facial nerve involvement, or treatment status. Metastatic involvement of intraparotid lymph nodes or the parotid by a primary cancer elsewhere should be represented as secondary disease rather than assumed to be a primary C07 malignancy. Lymphoma and tumors arising in other major or minor salivary glands are outside this family.