A 33-year-old man with no relevant pathological history presented with pain and swelling on the right scapular region and reduced range of motion of the right shoulder. The patient noticed those symptoms a few weeks before.
A radiographic study of the right scapula was conducted showing bone expansion and destruction. These changes are suggestive of the presence of an aggressive lesion.
A chest CT study was performed, which showed the presence of a bulky mass involving the right scapula, measuring about 9 x 14 cm of greater antero-posterior and longitudinal axes. It causes destruction and bone expansion of the body and spine of the scapula, the distal portion of the acromion is spared. Multiple metastatic nodules are also identified in both lungs.
MRI was performed, the solid lesion has smooth lobulated borders, slight high signal comparing with the muscle on T1WI and T2WI. It has an internal nodule appearance with smaller masses separated by thin bands with low intensity on T1WI and T2WI. Some large-caliber vascular structures are identified within the mass.
An ultrasound-guided biopsy was performed and revealed the mass to be an alveolar soft part sarcoma.
Alveolar soft part sarcoma is a rare form of sarcoma, with distinct histology, molecular characteristics and clinical evolution [1]. It accounts for less than 1% of soft tissue tumors, and is characterized by a pseudoalveolar or organoid arrangement of polygonal tumor cells separated by fibrovascular septa and delicate vascular channels of capillary size [1]. It predominantly affects people of a young age group, namely children, adolescents and young adults, with a slight female predominance. This type of sarcoma usually occurs in deep tissues of the buttock and and thigh in adults [1]. In children commonly occurs in the head and neck, predominantly in the orbit and tongue. It usually has an indolent evolution, being noticed when it reaches large dimensions, being metastasized at presentation in up to 65% of the adults and 30% of the children [1,2]. The most common metastatic organs are lung, bone, central nervous system and liver. Unlike other subtypes, alveolar soft part sarcoma often metastasizes to the brain. Surgery is the therapy of choice for localized disease and may be associated with adjuvant radiotherapy. Chemotherapy is usually ineffective. In disseminated disease the prognosis is worse. Features associated with better prognosis include younger age at diagnosis, small tumor size and not disseminated disease. In imaging studies these tumors are usually well defined, lobulated, showing large vessels surrounding and inside the mass, emerging from the poles [2]. It usually shows strong enhancement after contrast administration on CT and MRI, manly peripheral rim enhancement. It may have areas of necrosis. On MRI it is characterized by having iso or high signal comparing to muscle on T1WI It may show a nodular pattern with several smaller nodules separated by thin bands of low signal intensity on T1WI and T2WI. On T2WI it can present a target morphology, with low signal hypointense at periphery and higher signal in the center. The most typical imaging features are the presence of peri and intra-tumoral vessels [2].
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Alveolar soft-part sarcoma
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The patient’s right shoulder X-ray shows soft tissue swelling in the scapular region, with locally increased density. The bony contours remain generally intact, and there is no obvious sign of fracture. Plain and contrast-enhanced CT scans reveal a relatively large mass in the deep soft tissue of the right scapular region, with a well-defined boundary and increased vascular distribution in the area. After contrast enhancement, the lesion shows marked enhancement, and some areas exhibit low-density necrosis/cystic changes. Abundant vascular shadows can be seen within and surrounding the lesion. MRI indicates that, on T1-weighted images, the mass appears similar to or slightly higher than the muscle signal, while on T2-weighted images, the signal is increased. In some regions, a “target sign” may be observed (lower signal at the periphery and higher signal in the center). After enhancement, prominent peripheral enhancement and internal septations are visible. The tumor extends into the surrounding soft tissue and is adjacent to the scapula, though no clear evidence of bone destruction has been found.
Considering the patient’s young age, subtly presenting clinical symptoms, imaging findings of a large soft tissue mass with abundant vascular features, and pathological indicators, the most likely diagnosis is an extra-pulmonary primary soft tissue sarcoma with suspected lung metastases—“a soft tissue sarcoma subtype markedly prone to pulmonary spread,” indicated by suspicious hilar or lung field lesions.
Based on the histological characteristics and clinical course, the final confirmed diagnosis is an alveolar soft part sarcoma (ASPS) with possible lung metastases (“pulmonary metastatic” sarcoma subtype).
Overview of Treatment Strategy:
Rehabilitation and Exercise Prescription (FITT-VP Principle):
If severe pain, significant joint swelling, or deterioration in function occurs during rehabilitation, seek medical evaluation promptly to avoid further tissue damage.
Special Considerations: If there is distant metastasis (especially to the lungs, bones, or central nervous system) or a decline in physical fitness due to chemotherapy or radiotherapy, exercise intensity and modality should be personalized under professional supervision to prevent overexertion.
Disclaimer: This report is for reference only based on the available information and does not substitute for an in-person consultation or professional medical advice. If you have concerns or if symptoms worsen, please consult a physician for further examination and evaluation.
Alveolar soft-part sarcoma