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Alveolar soft part sarcoma of the orbit, a case report of a rare tumor
Insights
Alveolar Soft Part Sarcoma (ASPS) is a rare soft tissue tumor. Early diagnosis and long-term follow-up are crucial due to its indolent course and metastasis potential.
Area of Science:
- Oncology
- Pathology
Background:
- Alveolar Soft Part Sarcoma (ASPS) is a rare malignant tumor of uncertain origin, comprising 0.5-1% of all soft tissue tumors.
- ASPS predominantly affects the head and neck, particularly the orbit and tongue, often in infants, with initially nonspecific symptoms.
Observation:
- A 4-year-old girl presented with a six-month history of left eyelid swelling, proptosis, and globe displacement.
- Ophthalmological examination revealed decreased visual acuity and restricted eye motility. Imaging showed an extraconal orbital mass.
Findings:
- Histopathological analysis of the excisional biopsy revealed nests of large polygonal cells within fibrous septa and sinusoidal vascular channels, consistent with ASPS.
- The patient experienced a favorable post-operative outcome with no evidence of metastasis or local recurrence.
Implications:
- ASPS, despite its rare occurrence and often indolent clinical course, possesses a significant propensity for distant metastases.
- This case underscores the necessity for early diagnosis and vigilant long-term monitoring in managing Alveolar Soft Part Sarcoma.
Background:
Alveolar Soft Part Sarcoma is a rare malignant tumor of uncertain histogenesis, representing 0.5-1% of all soft tissue tumors. It occurs predominantly in head and neck regions, especially the orbit and the tongue, in infants, causing no specific symptoms for an extended period of time.
Case:
A four year old girl was brought to consultation because of persistent swelling of her left upper and lower eyelid for the past six months. A 4 mm proptosis was noted on her left eye with significant upwards displacement of the globe. A firm, not pulsating and non tender mass was palpated, and motility examination revealed deficient abduction and infraduction. Visual acuity was 20/40 and 1/200 in the right and left eye, respectively. The computed tomography revealed an extraconal mass on the inferotemporal aspect of the left orbit, with no bony erosion or globe invasion. An excisional biopsy was made, finding a tumor with nests of clusters of large polygonal cells, separated by fibrous septa and a sinusoidal vascular channel. An Alveolar Soft Part Sarcoma was diagnosed. Our patient had a favorable post-operative follow up, and oncologic evaluations have not shown metastasis or local recurrences. Observations and Conclusions: Alveolar Soft Part Sarcoma is a rare malignant tumor of indolent course, but with propensity to distant metastases, making early diagnose and long term follow up necessary.