Acromegaly and a Giant Retroperitoneal Liposarcoma Producing IGF-1

Abstract

<jats:sec><jats:title>Objective:</jats:title><jats:p> Liposarcoma is the most common histotype of retroperitoneal sarcomas, representing up to 45% of all cases. We report a rare combination of acromegaly and liposarcoma in the same individual. </jats:p></jats:sec><jats:sec><jats:title>Methods:</jats:title><jats:p> Laboratory and imaging studies including an oral glucose tolerance test, measurements of growth hormone (GH) and insulin-like growth factor-1 (IGF-1), and a computed tomography scan were performed. </jats:p></jats:sec><jats:sec><jats:title>Results:</jats:title><jats:p> The patient was a 60-year-old male with a history of acromegaly diagnosed on the basis of elevated IGF-1 at 1,373 ng/mL (age-appropriate reference range is 87 to 225 ng/mL) and macroadenoma treated with transsphenoidal surgery. He presented 8 years later with a history of abdominal distension and weight loss. Physical examination was notable for a right-sided abdominal mass that was tense and non-fluctuant. Two years earlier, he had a post oral glucose tolerance test GH level &lt;0.25 ng/mL and IGF-1 level of 256 ng/mL (age-appropriate reference range is 55 to 206 ng/mL). Pituitary magnetic resonance imaging reported a 3.7 × 2.0-mm left-sided parasagittal lesion. Computed tomography scan showed a 25.0 × 22.0 × 32.3-cm heterogeneous giant mass in the right abdomen corresponding to a liposarcoma causing displacement of kidney, liver, and bowel loops. The patient was treated with a complete en bloc resection of the liposarcoma with the right kidney (45 × 33 × 17 cm) and tumor (9,400 g). Immunohistochemical examination revealed positive IGF-1 and GH staining. The patient suffered postoperative gastrointestinal bleeding that resulted in hemorrhagic shock and died on the 29th postoperative day after a cardiorespiratory arrest. </jats:p></jats:sec><jats:sec><jats:title>Conclusion:</jats:title><jats:p> Acromegalic patients are at increased risk of developing various types of neoplasms. This is the first documented coexistence of liposarcoma and history of acromegaly. </jats:p></jats:sec>

A Giant retroperitoneal liposarcoma encasing entirely the right kidney: A case report and a review of nephrectomy indications

“…The only definite indication for nephrectomy is kidney invasion or perirenal fat infiltration. This should at best be suspected on preoperative imaging and must be confirmed intraoperatively [8][9][10] .…”