Retroperitoneal angioleiomyomatosis
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Retroperitoneal intravenous leiomyomatosis is a rare benign tumor that can spread through veins carrying significant morbidity. The challenge of its management lies within the complexity of completely excising the tumor, which if carried out improperly can result in neurological or vascular complications requiring complex reparative surgeries.

A 35-year-old patient with two children underwent laparoscopic retroperitoneal pelvic tumor excision. The patient presented with chronic left leg pain for many years. At admission, ultrasound examination and MRT image findings were described as a mucinous cystadenoma of the left ovary (9.2?×?5.6 cm with relative irregular outline and filling the pouch of Douglas), with the differential diagnosis of a broad ligament leiomyoma with intrinsic tumor degeneration and necrotic features. By combining the laparoscopic route, in-bag morcellation, and micro-surgical technics, it was possible to ensure complete resection of the retroperitoneal tumor without intraoperative vascular or neural complications. At histological examination, the cytology of peritoneal fluid showed mild subacute inflammation with activated mesothelial cells, suggestive of exudative peritoneal effusion. Sections of the soft tissue neoplasm consisted of cellular sheets and irregular fascicles spindled cells with the formation of glomeruloid architecture and epithelioid features.

The lesion demonstrated numerous anastomosing structures resembling blood vessels covered by endothelium-like cells embedded in a fibrous stroma. No evidence of malignancy or tumor necrosis was reported. The tumor was positive for immunomarkers CD31, CD34, D2-40, CD68, Ki-67 (1–2% positive nuclear staining) as well for smooth muscle markers SMA (Smooth muscle actin) and Desmine. HHV-8, S100, Synaptophysin, and Calretinin were negative. The postoperative course was uneventful.

Source:https://link.springer.com/article/10.1007/s13224-020-01404-7
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