SCARE

Sarcoma Histology Acronym

Author: Lily Guo, MD

Editor: Alyssa Haag, MD

Editor: Ian Mannarino MD, MBA

Editor: Kelsey LaFayette, DNP, ARNP, FNP-

Illustrator: Jannat Day

Modified: Jan 06, 2025

What is sarcoma?

Sarcoma is a form of cancer that affects bones and soft tissue, including fat, muscle, blood vessels, nerves, ligaments, and tendons. Sarcomas are rarely associated with lymph node involvement, only occurring in <5% of all cases of soft tissue sarcoma. The sarcoma subtypes represented by the acronym SCARE are those most frequently associated with lymph node involvement via metastasis from the primary source. Sarcoma constitutes 1% of all adult malignancies.

An infographic detailing the sarcoma histology acronym, SCARE.

What is SCARE?

SCARE is an acronym that describes the five main types of sarcoma, specifically soft tissue sarcoma, that are most commonly associated with lymph node involvement. Lymph nodes are part of the body’s adaptive immune system and contain white blood cells that help fight infection. Sarcomas that metastasize to the lymph nodes more frequently require more aggressive treatment with chemotherapy compared to other sarcoma subtypes. The overall survival rate of sarcoma depends on tumor type, size, and 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 grow from soft tissues in the lungs or abdomen. It is commonly diagnosed in genetic males younger than 30 years of age. On molecular testing, those with synovial sarcoma will typically have a translocation between chromosome X and 18, which leads to the expression of SS18:SSX fusion proteins and is pathognomonic (i.e., distinctly characteristic) for synovial sarcoma. Diagnosis of synovial carcinoma includes a biopsy of the mass and radiographic tests including computed tomography (CT) scans, magnetic resonance imaging (MRI), and positron emission tomography (PET) scans. Treatment modalities 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 a tender spot associated with a tendon or tendon sheath. It is often found in the legs, specifically the ankles, feet, and thighs. A small percentage of individuals may have ulceration of the overlying skin. Clear cell sarcomas arising from the upper extremities, abdomen, chest wall, and around the scapula have been described but are much rarer. There is no gender 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 causing a fusion of the EWSR1 and ATF1 genes. Diagnosis of clear cell carcinoma includes biopsy and radiographic 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 presents as a raised purple-red papule (i.e., raised lesion <1cm wide) or may be confused with a bruise. As the tumor grows in size, it can become ulcerated and bleed easily. Angiosarcomas are more commonly found in the head and neck region, particularly in the scalp, and are typically diagnosed in genetic males with advanced age. Angiosarcomas can occur sporadically (i.e., spontaneously), however, 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 mutations in the DNA repair genes BRCA1 and BRCA2 are associated with angiosarcoma development. Diagnosis includes biopsy, imaging to detect tumor cell spread including MRI of the brain, a CT scan of the chest, and a CT scan of the abdomen and pelvis. Treatment includes surgical resection, chemotherapy (e.g., doxorubicin and paclitaxel), and combination radiation therapy with chemotherapy.

What does the “R” in SCARE mean?

The “R” in SCARE stands for rhabdomyosarcoma which is derived from muscle and can present anywhere on 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, the masses can be painful and have overlying erythema or inflammation. Rhabdomyosarcoma of the bladder can present with hematuria (i.e., blood in urine) and urinary obstruction resulting in difficulty or pain with urination and decreased urine output. Most cases are diagnosed in children under the age of 6 with a slight predominance for genetic males. 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 includes imaging (e.g., CT or MRI scan) of the tumor, abdomen, and chest; bone scan; bone marrow aspiration; and biopsy of the tumor. Treatment includes 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 extremity (e.g., fingertips) of young adult genetic males, generally between 10 to 45 years of age. Tumors can also be located on the skin of the forearm, knee, or lower leg. Epithelioid sarcomas show loss of expression of integrase interactor-1 (INI-1) which is part of a chromatin remodeling complex ultimately allowing for transcription of DNA. Diagnosis of epithelioid sarcoma includes biopsy and work-up to look for metastatic disease including ultrasound scanning 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 sarcoma that commonly metastasize to lymph nodes. Sarcoma is a rare form of cancer that affects the bones and soft tissue, such as the 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 on the legs; "A" stands for angiosarcoma, appearing with bruise-like lesion on the scalp or head and neck regions of older genetic males; "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.

References

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