Introduction:
Sarcomas are a rare entity with heterogeneous clinical behavior. We herein present the case of a giant retroperitoneal dedifferentiated liposarcoma and aim to remind the clinical, histological, and therapeutic features of this rare tumor.
Presentation of case:
An 80-year-old patient was complaining of significant abdominoscrotal swelling. On physical examination, a 25-cm swelling extending from the right iliac fossa to scrotums, was observed. CT scan revealed a retroperitoneal fatty mass with necrotic areas. Ultrasonography-guided biopsy was carried. Pathological report demonstrated a dedifferentiated sarcoma. An en bloc resection of the tumor was performed through a right iliac incision extended to the scrotum. After one year of follow up, the patient had a 30-cm local recurrence. After complete resection, the patient died two days after the procedure due to neurological distress.
Discussion:
'Giant' liposarcomas over 20kg are extremely rare. CT-Scan and MRI are very useful for defining their size and limits. The resection of a retroperitoneal sarcoma of remarkable size is a challenge for the surgeon owing to the anatomical site that makes it hard to obtain safe margin and to the adherences with the contiguous organs. Dedifferentiated histologic subtypes and negative surgical margins are associated with poor prognosis. This explains the high rate of local recurrence after surgical excision.
Conclusion:
In rare cases, retroperitoneal dedifferentiated liposarcomas can extend through the inguinal canal to the scrotum. Surgical resection obtaining negative margins, remains the curative treatment that reduces the risk of recurrence. Careful follow-up to detect early recurrence is essential for optimal care.