SCARE · Sarcoma Histology Acronym

Published: Aug 17, 2026
Author: Lily Guo, MD
Editor: Alyssa Haag, MD
Editor: Ian Mannarino, MD, MBA
Editor: Kelsey LaFayette, DNP, ARNP, FNP-C
Illustrator: Jannat Day
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What is sarcoma?

Sarcoma is a type of cancer that affects bones and soft tissue, including fat, muscle, blood vessels, nerves, ligaments, and tendons. It constitutes 1% of all adult malignancies. Sarcomas are rarely associated with lymph node involvement, which only occurs in <5% of soft tissue sarcoma cases.  

The SCARE acronym represents the sarcoma subtypes most frequently associated with lymph node involvement via metastasis from the primary tumor.  

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What is SCARE?

SCARE is an acronym that describes the five main types of soft tissue sarcoma most commonly associated with lymph node involvement. Lymph nodes, as part of the body’s adaptive immune system, contain white blood cells that help fight infections. Sarcomas that metastasize to lymph nodes often require more aggressive treatment with chemotherapy compared to other sarcoma subtypes. The overall survival rate depends on the tumor type, size, and extent of spread.  

What does the “S” in SCARE mean?

The “S” in SCARE stands for synovial sarcoma. Synovial sarcoma typically presents as a slow-growing, painless lump near the joints of the knee, hips, wrist, shoulder, or ankle. In extremely rare cases, synovial sarcoma can also originate from soft tissues in the lungs or abdomen. It is commonly diagnosed in males assigned at birth younger than 30 years of age. On molecular testing of a synovial sarcoma sample, the pathognomonic (i.e., distinctly characteristic) sign is a translocation between chromosome X and 18, which leads to the expression of SS18:SSX fusion proteins. Diagnosis of synovial sarcoma involves a tumor biopsy and imaging, such as computed tomography (CT) scans, magnetic resonance imaging (MRI), and positron emission tomography (PET) scans. Treatment strategies include surgical resection, radiation therapy, chemotherapy (e.g., doxorubicin, ifosfamide), and tyrosine kinase inhibitors (e.g., pazopanib, apatinib).  

What does the “C” in SCARE mean?

The “C” in SCARE stands for clear cell sarcoma. This sarcoma subtype commonly presents as a small swelling or tender spot near a tendon or tendon sheath, with a small percentage of individuals showing ulceration of the overlying skin. It is often found in the legs, specifically the ankles, feet, and thighs. Clear cell sarcomas originating in the upper extremities, abdomen, chest wall, and around the scapula have been described but are much rarer. There is no sex predilection, and the median age of affected individuals is 39 years, with rare cases reported in children. Clear cell sarcoma is caused by a translocation of chromosomes 12 and 22, resulting in a fusion of the EWSR1 and ATF1 genes. Diagnosis of clear cell carcinoma involves tumor biopsy and imaging, including X-ray and MRI. Treatment commonly includes wide surgical resection and radiotherapy, as well as chemotherapy (e.g., doxorubicin and cisplatin) in those with metastatic disease.  

What does the “A” in SCARE mean?

The “A” in SCARE stands for angiosarcoma, which typically presents as a raised purple-red papule (i.e., raised lesion <1cm wide) that can be mistaken for a bruise. As the tumor grows in size, it can become ulcerated and bleed easily. Angiosarcomas more commonly occur in the head and neck region, particularly in the scalp, and are typically diagnosed in older males assigned at birth. While angiosarcomas can occur sporadically (i.e., spontaneously), they have been linked to previous radiation therapy and exposure to chemicals including vinyl chloride, thorium dioxide, radium, and arsenic. Additionally, some case reports suggest that mutations in the DNA repair genes BRCA1 and BRCA2 are associated with angiosarcoma development. Diagnosis involves biopsy and imaging to assess tumor spread, including brain MRI, chest CT scan, and CT scans of the abdomen and pelvis. Treatment includes surgical resection, chemotherapy (e.g., doxorubicin and paclitaxel), and combined radiation therapy and chemotherapy.  

What does the “R” in SCARE mean?

The “R” in SCARE stands for rhabdomyosarcoma. Rhabdomyosarcoma originates from muscle and can occur anywhere in the body, including the head and neck, extremities, and bladder. When on the head and neck, the mass is usually painless and slow growing. When on the extremities, masses can be painful and present overlying erythema or inflammation. Rhabdomyosarcoma of the bladder can manifest with hematuria (i.e., blood in urine) and urinary obstruction, resulting in difficult or painful urination and decreased urine output. Most cases are diagnosed in children under 6 years of age, with a slight predominance for males assigned at birth. Rhabdomyosarcoma typically occurs sporadically; however, the disease has been associated with familial syndromes (e.g., neurofibromatosis, Noonan syndrome, Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome). Diagnosis involves imaging (e.g., CT or MRI scan) of the tumor, abdomen, and chest; bone scan; bone marrow aspiration; and tumor biopsy. Treatment options include surgery, chemotherapy (e.g., vincristine, actinomycin D, cyclophosphamide), and radiotherapy.  

What does the “E” in SCARE mean?

The “E” in SCARE stands for epithelioid sarcoma, which typically presents as a painless, slow-growing mass in the distal extremities (e.g., fingertips) of young adult males assigned at birth, generally between 10 to 45 years of age. Tumors can also be originate from the skin of the forearm, knee, or lower leg. Epithelioid sarcoma is characterized by loss of expression of integrase interactor-1 (INI-1), part of a chromatin remodeling complex ultimately allowing DNA transcription. Diagnosis of epithelioid sarcoma involves tumor biopsy and evaluation for metastatic disease, including ultrasound of regional lymph nodes and sentinel lymph node biopsy. Treatment includes surgical resection of the mass, which can be curative, and radiation therapy to mitigate local recurrence.  

What are the most important facts to know about the SCARE acronym?

The SCARE acronym describes five subtypes of soft tissue sarcoma that commonly metastasize to lymph nodes. Sarcoma is a rare form of cancer that affects bones and soft tissue, such as fat, muscle, blood vessels, nerves, ligaments, and tendons. The S in SCARE stands for synovial sarcoma, characterized by slow-growing lumps near joints; C stands for clear cell sarcoma, presenting as tender spots near tendons on the legs; A stands for angiosarcoma, appearing with bruise-like lesion on the scalp or head and neck regions of older males assigned at birth; R stands for rhabdomyosarcoma, commonly found in children and affecting various body areas such as the head, neck, and bladder; and E stands for epithelioid sarcoma, generally found on the distal extremities of young adults.  

Definition

SCARE is the acronym describing the five subtypes of soft tissue sarcoma most commonly associated with lymph node metastasis.  

S 

Synovial sarcoma 

C

Clear cell sarcoma 

A 

Angiosarcoma 

R 

Rhabdomyosarcoma 

E 

Epithelioid sarcoma 

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References


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Burkhard-Meier, A., Jurinovic, V., Berclaz, L.M. et al. Differentiation of benign and metastatic lymph nodes in soft tissue sarcoma. Clin Exp Metastasis. 2024;41: 131–141. doi: https://doi.org/10.1007/s10585-024-10273-7  


Kyriazoglou A, Gkaralea LE, Kotsantis I, Anastasiou M, Pantazopoulos A, Prevezanou M, Chatzidakis I, Kavourakis G, Economopoulou P, Nixon IF, Psyrri A. Tyrosine kinase inhibitors in sarcoma treatment. Oncol Lett. 2022 Jun;23(6):183. doi: 10.3892/ol.2022.13303. Epub 2022 Apr 21. PMID: 35527786; PMCID: PMC9073578.  


Serban B, Cretu B, Cursaru A, Nitipir C, Orlov-Slavu C, Cirstoiu C. Local recurrence management of extremity soft tissue sarcoma. EFORT Open Rev. 2023 Aug 1;8(8):606-614. doi: 10.1530/EOR-23-0095. PMID: 37526250; PMCID: PMC10441250. 


Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ. Angiosarcoma. Lancet Oncol. 2010;11(10):983-991. doi:10.1016/S1470-2045(10)70023-1