Occipitofrontalis muscle cancer is a very rare form of soft-tissue sarcoma that arises in the epicranius (occipitofrontalis) muscle covering the scalp. Like other muscle-based cancers, it most often presents as a subtype of rhabdomyosarcoma or other soft-tissue sarcoma. Early recognition and a multidisciplinary approach to diagnosis and treatment are essential for improving outcomes. Below is a comprehensive, plain-English, SEO-friendly overview covering all aspects of this condition.
Anatomy of the Occipitofrontalis Muscle
-
Structure & Location
The occipitofrontalis (epicranius) is a paired, broad, thin muscle overlying the skull. It has two bellies—frontal and occipital—connected by the epicranial (galea) aponeurosis. It covers the forehead and posterior skull WikipediaKenhub. -
Origin
-
Frontal belly: intermediate tendon on the epicranial aponeurosis Wikipedia
-
Occipital belly: superior nuchal line of the occipital bone and mastoid region of the temporal bone Wikipediawww.elsevier.com
-
-
Insertion
-
Blood Supply
-
Frontal belly: supraorbital and supratrochlear arteries (branches of ophthalmic artery) and frontal branch of superficial temporal artery WikipediaKenhub
-
Occipital belly: occipital artery and posterior auricular branches (from external carotid system) KenhubRadiopaedia
-
-
Nerve Supply
Innervation is via the facial nerve (CN VII): -
Functions (6 key actions)
Types of Occipitofrontalis Muscle Cancer
Most tumors of the occipitofrontalis muscle are classified under soft-tissue sarcomas, especially rhabdomyosarcomas, because they arise from muscle cells. Major types include:
-
Embryonal Rhabdomyosarcoma (ERMS)
-
Most common subtype in children (around 60% of cases) PMCCancer Research UK
-
Often arises in head and neck, including scalp
-
-
Alveolar Rhabdomyosarcoma (ARMS)
-
Accounts for ~20% of cases; more aggressive with quicker spread PMC
-
More common in adolescents and young adults
-
-
Pleomorphic Rhabdomyosarcoma
-
Rare in children; usually affects adults over 50 Cleveland Clinic
-
-
Spindle Cell and Botryoid Subtypes
-
Variants of ERMS with unique microscopic features and prognosis PMC
-
-
Other Soft-Tissue Sarcoma Variants
-
Leiomyosarcoma, liposarcoma, undifferentiated pleomorphic sarcoma may rarely involve the epicranius Home
-
Causes and Risk Factors
-
Genetic Syndromes
-
Neurofibromatosis type 1 PMC
-
Costello syndrome PMC
-
Noonan syndrome PMC
-
Recklinghausen disease PMC
-
Prior radiation exposure (therapeutic or accidental) Mayo Clinic
-
Parental smoking (father’s smoking linked to RMS) Home
-
Chemical exposures (vinyl chloride, herbicides) Home
-
Immunosuppression (e.g., post‐transplant) Mayo Clinic
-
Advanced age (for pleomorphic subtype) Cleveland Clinic
-
Male sex (slightly higher incidence in boys) PMC
-
Chronic lymphedema Home
-
Ultraviolet (UV) exposure (scalp sun exposure) Home
-
Human papillomavirus (HPV) (possible link in head/neck cancers) Home
-
Human immunodeficiency virus (HIV) Mayo Clinic
-
Epstein-Barr virus (EBV) (in some soft-tissue tumors) Mayo Clinic
-
Inherited DNA repair defects PMC
-
Environmental pollution Home
-
Obesity (pro-inflammatory state) Home
-
Chronic scalp infections (promote abnormal cell growth) Mayo Clinic
Symptoms
Depending on tumor size and location, patients may experience:
-
A firm, painless lump on the scalp Mayo Clinic
-
Rapid growth of the lump Mayo Clinic
-
Localized pain or tenderness Cleveland Clinic
-
Swelling or visible bulge Mayo Clinic
-
Scalp ulceration or skin changes Mayo Clinic
-
Hair loss over the tumor Cleveland Clinic
-
Headaches (if compressing skull) Radiopaedia
-
Numbness or tingling in scalp Radiopaedia
-
Facial asymmetry (if spreading) Radiopaedia
-
Vision changes (if orbital extension) Cleveland Clinic
-
Ear pain or discharge (if para‐auricular spread) Cleveland Clinic
-
Bleeding or oozing from lesion Mayo Clinic
-
Fever (paraneoplastic) Cleveland Clinic
-
Night sweats Cleveland Clinic
-
Unexplained weight loss Cleveland Clinic
-
Fatigue Cleveland Clinic
-
Enlarged lymph nodes in head/neck Mayo Clinic
-
Difficulty swallowing (if deep extension) Radiopaedia
-
Hoarseness (if compressing laryngeal nerves) Radiopaedia
-
General malaise Cleveland Clinic
Diagnostic Tests
-
Clinical examination Mayo Clinic
-
Skin ultrasound Radiopaedia
-
X-ray of skull Radiopaedia
-
CT scan of head and neck Radiopaedia
-
MRI with contrast Radiopaedia
-
PET-CT scan Radiopaedia
-
Core-needle biopsy Mayo Clinic
-
Excisional biopsy Mayo Clinic
-
Histopathology (H&E stain) PMC
-
Immunohistochemistry (myogenin, desmin) PMC
-
Cytogenetics (PAX-FOXO1 fusion detection) PMC
-
FISH analysis PMC
-
Flow cytometry PMC
-
CBC & metabolic panel Cleveland Clinic
-
Liver function tests Cleveland Clinic
-
Kidney function tests Cleveland Clinic
-
Bone scan Radiopaedia
-
Bone marrow biopsy (if metastasis suspected) PMC
-
Echocardiogram (pre-chemo assessment) Cleveland Clinic
-
Chest CT (lung metastasis check) Cleveland Clinic
Non-Pharmacological Treatments
(Note: These include both curative and supportive modalities beyond drug therapy.)
-
External-beam radiotherapy Home
-
Brachytherapy Home
-
Proton therapy Home
-
Stereotactic radiosurgery Home
-
Hyperthermia therapy Home
-
Cryoablation Home
-
Radiofrequency ablation Home
-
Photodynamic therapy Home
-
Thermal ablation (HIFU) Home
-
Surgical wide local excision (also detailed in surgery) Home
-
Reconstructive flap surgery PMC
-
Physical therapy (maintain scalp mobility) Mayo Clinic
-
Occupational therapy (daily living support) Mayo Clinic
-
Speech/swallow therapy Radiopaedia
-
Nutritional counseling Cleveland Clinic
-
Psychological counseling Cleveland Clinic
-
Massage therapy Mayo Clinic
-
Acupuncture (pain relief) Mayo Clinic
-
Mindfulness & meditation Mayo Clinic
-
Yoga & tai chi Mayo Clinic
-
Art/music therapy Cleveland Clinic
-
Support groups Cleveland Clinic
-
Palliative care services Cleveland Clinic
-
Hospice care Cleveland Clinic
-
Lymphedema therapy Mayo Clinic
-
Skin care & wound management Mayo Clinic
-
Pain management techniques (TENS, relaxation) Mayo Clinic
-
Occupational scalp prosthetics Cleveland Clinic
-
Nurse-led care coordination Cleveland Clinic
-
Telemedicine follow‐up Cleveland Clinic
Drugs (Chemotherapy & Targeted Agents)
-
Vincristine Cleveland Clinic
-
Actinomycin D (Dactinomycin) Cleveland Clinic
-
Cyclophosphamide Cleveland Clinic
-
Ifosfamide Cleveland Clinic
-
Doxorubicin Cleveland Clinic
-
Etoposide Cleveland Clinic
-
Cisplatin Cleveland Clinic
-
Carboplatin Cleveland Clinic
-
Irinotecan Cleveland Clinic
-
Temozolomide Cleveland Clinic
-
Topotecan Cleveland Clinic
-
Vinblastine Cleveland Clinic
-
Vinorelbine Cleveland Clinic
-
Bleomycin Cleveland Clinic
-
Pirarubicin Cleveland Clinic
-
Pazopanib (TKI) Home
-
Sorafenib (TKI) Home
-
Imatinib (for PDGFR-positive sarcoma) Home
-
Bevacizumab (anti-VEGF) Home
-
Temsirolimus (mTOR inhibitor) Home
Surgical Procedures
-
Wide local excision (complete tumor removal with margins) Home
-
Mohs micrographic surgery (layer-by-layer margin control) Home
-
Craniotomy with en bloc resection (for bone‐invasive tumors) Radiopaedia
-
Reconstructive flap surgery (scalp defect repair) PMC
-
Free tissue transfer (microvascular flap) PMC
-
Sentinel lymph node biopsy Mayo Clinic
-
Neck dissection (regional lymph node removal) Radiopaedia
-
Debulking surgery (for symptom relief) Cleveland Clinic
-
Excisional biopsy (diagnostic and therapeutic) Mayo Clinic
-
Craniofacial resection (extensive head and neck involvement) Radiopaedia
Prevention Strategies
-
Minimize therapeutic radiation when possible Mayo Clinic
-
Avoid tobacco smoke exposure Home
-
Wear sun protection to limit UV on scalp Home
-
Screen for genetic syndromes in high-risk families PMC
-
Regular scalp examinations (self and physician) Mayo Clinic
-
Reduce chemical exposures (industrial, agricultural) Home
-
Maintain healthy weight Home
-
Vaccinate against HPV where indicated Home
-
Manage chronic infections of scalp promptly Mayo Clinic
-
Promote occupational safety (protective gear) Home
When to See a Doctor
Promptly consult a healthcare provider if you notice:
-
A new or changing lump on your scalp Mayo Clinic
-
Rapid enlargement of a painless bump Mayo Clinic
-
Skin ulceration or bleeding from a scalp lesion Mayo Clinic
-
Persistent headaches or neurological changes Radiopaedia
-
Unexplained systemic symptoms (fever, weight loss) Cleveland Clinic
Frequently Asked Questions
-
What is occipitofrontalis muscle cancer?
A rare sarcoma arising in the epicranius muscle on the scalp. Penn Medicine -
How common is this cancer?
Extremely rare—most scalp sarcomas are <1% of all soft-tissue sarcomas. Penn Medicine -
Who is at risk?
Children with genetic syndromes; prior radiation exposure; males slightly more. PMCHome -
What are early symptoms?
A painless scalp lump that grows over weeks or months. Mayo Clinic -
How is it diagnosed?
Imaging (MRI/CT), biopsy, histology, and genetic tests. Radiopaedia -
Can it spread?
Yes—through blood to lungs or bones and via lymph nodes. PMC -
What treatments are available?
Surgery, radiotherapy, chemotherapy, and supportive care. Home -
What is the prognosis?
Depends on subtype and stage; 5-year survival up to 70% in localized cases. PMC -
Are there side effects of treatment?
Hair loss, skin changes, fatigue, nausea, and risk of secondary tumors. Cleveland Clinic -
How can I cope with treatment?
Supportive therapies like nutrition, physiotherapy, and counseling help. Cleveland Clinic -
Is genetic testing useful?
Yes—for diagnosing subtypes and guiding family counseling. PMC -
Can it recur?
Recurrence risk exists; regular follow-up imaging is essential. PMC -
What follow-up is needed?
Periodic MRI/CT, chest imaging, blood tests, and physical exams. Cleveland Clinic -
Are there clinical trials?
Yes—many centers offer trials of novel drugs and immunotherapies. Home -
Where can I find support?
Cancer support groups, sarcoma foundations, and specialized centers. Cleveland Clinic
Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.
The article is written by Team Rxharun and reviewed by the Rx Editorial Board Members
Last Updated: April 27, 2025.