Alveolar Soft Part Sarcoma. by Keila Torres, MD, PhD and Raphael Pollock, MD, PhD Also available in Chinese, French, Italian, Japanese and Spanish. Cellular origin of synovial sarcoma is unknown, but it is not the synovial cell or any cell involved in the synovium the name synovial sarcoma is a misnomer. like.
Alveolar Soft Part Sarcoma (ASPS): Diagnosis, Treatment and Researchby Keila Torres, MD, Ph. D and Raphael Pollock, MD, Ph. DAlso available in Chinese, French, Italian, Japanese and Spanish. Abstract. Alveolar soft part sarcoma (ASPS) is a rare, poor prognosis neoplasm of unknown histogenesis with a distinctive histology, specific molecular characteristics, and unique clinical behaviors.
· Cancer is not common in young adults, and treating these cancers can be challenging. Learn more about cancer in young adults here. Why sarcoma patients should consider seeking treatment or a second opinion at a sarcoma center. Soft tissue sarcoma is a cancer that starts in your body’s fat, muscles, nerves, tendons, or blood or lymph vessels. Learn more about types and symptoms of soft.
ASPS generally develop in younger patients. Unlike other soft tissue sarcomas, ASPS also metastasizes to the brain. While surgery can improve outcomes even in the setting of metastatic disease, traditional chemotherapeutic agents and ⁄or radiotherapy have failed to demonstrate significant survival advantages.
This article provides an overview of the clinical manifestations, diagnosis, radiographic features, and treatment of ASPS. Background. Alveolar soft part sarcoma (ASPS) is a distinct histologic soft tissue sarcoma subtype.
ASPS is an uncommon tumor, typically occurring in adolescent and younger adult patients. It accounts for 0. Despite a relatively indolent tumor growth pattern, up to 7. The development of therapeutically resistant metastasis contributes to increased mortality.
Credit for the original description of ASPS traditionally goes to Christopherson, then a fellow in surgical pathology at Memorial Sloan Kettering Cancer Center. With the publication of a study of 1. Christopherson et al. This tumor was first defined histologically by the presence of cells arranged in nests ("alveoli") separated by delicate partitions of connective tissue containing sinusoidal vascular channels lined by flattened endothelium. Prior to Christopherson’s publication, the entity ASPS had been described by a variety of other names, including "malignant myoblastoma", "granular cell myoblastoma" and "malignant granular cell myoblastoma."2- 6 While Christopherson et al. ASPS, they did quote an unpublished letter from Dr.
Pierre Masson, who noted the intracytoplasmic crystals. Therefore, credit for the intracytoplasmic crystals belongs to Dr. Masson, who later in 1. Unknown to Christopherson and colleagues, ASPS had been described by Smetana and Scott one year earlier, as malignant tumors of non- chromaffin paraganglia. They chose this term because the tumors resembled non- physiologically active paraganglia, postulating that primitive paraganglia- like structures may perhaps normally occur in the somatic soft tissues (this hypothesis was later discredited). Smetana and Scott also independently observed the intracytoplasmic crystals of ASPS, describing them as rod- shaped, coarse, basophilic bodies of unknown nature. Clinical Manifestations.
ASPS usually presents as a soft, painless, slow- growing mass that rarely causes functional impairment. In adults, the lower extremities are the most common location for this lesion, although it has been described in a variety of locations including the female genital tract, mediastinum, breast, urinary bladder, gastrointestinal tract, and bone. In children, ASPS most often occurs in the head and neck region. These tumors are extremely vascular, and occasionally present as a pulsatile mass with an associated bruit. Figure 1. A: CT scan of the head of a 2. Because of the relative lack of symptoms, in many patients the tumor is easily overlooked and metastasis to the lung or other sites may be the first disease manifestation.
The most common metastatic sites are lung, bone, central nervous system, and liver. Metastasis has been reported as long as 1. Unlike other soft tissue sarcomas, ASPS also metastasizes to the brain, and are described as a common feature of metastatic ASPS (Figure 1). A review of 7. 0 ASPS patients treated at the University of Texas MD Anderson Cancer Center revealed that brain metastases were almost always detected in conjunction with metastatic disease at other sites. Diagnosis of Alveolar Soft Part Sarcoma. Radiologic Findings Accurate diagnosis and treatment of this unusual tumor requires a high index of clinical suspicion coupled with clinicopathologic correlation via appropriate radiographic studies. If the clinical or radiographic interpretation is equivocal, early biopsy is essential to differentiate alveolar soft part sarcoma from arteriovenous malformation.
ASPS tumors appear to be hypervascular on angiography and computed tomographic scan (CT scan), with a dense tumor stain and tortuous, dilated draining veins. Diagnosis of ASPS requires clinicians from different specialties, such as radiologists (a physician who has specialized training in obtaining and interpreting medical images), pathologists (a physician who interprets and diagnoses the changes caused by disease in tissues and body fluids), surgeon oncologists (a physician that deals with the surgical treatment of cancer), and medical oncologists (a physician who uses chemotherapy to treat cancer).
Figure 2. CT scan of a 5. Figure 2 is a CT scan of a 5.
ASPS arising from the anterior lower chest wall.
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