Para-neoplastic secretion of retroperitoneal Hemangiopericytoma

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: MON 306-326-Neoplasia of Endocrine Tissues: Case Reports
Clinical
Monday, June 17, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board MON-308
Fernando Lizcano*1, Alex Valenzuela2 and Guillermo Guzman2
1Universidad de La Sabana, Chia Cundinamarca, Colombia, 2Fundacion Cardio-Infantil, Bogota, Colombia
Background: Hemangiopericytoma (HPC) is a rare tumor of perivascular tissue. Some HPC may be accompanied by Para-neoplastic endocrine manifestations without even knowing what are the factors that determine these alterations. It is likely that these events are the result of epigenetic alterations that underlie the tumor.

Clinical Case: A 43-year-old man showed symptoms of hypoglycemia and difficulty in urination. He was working for a period of 5 years with an electric transformer or converter. For a not evident reason the patient was on treatment with betamethasone for two years.  Patient had facial skin lesions, acromegaly like features and bilateral gynecomastia.  Fasting blood glucose ranging in 30-45 mg / dL. Albumin: 2.9 g/ L (3.5-5.0), TSH: 1.43 mU / L; FT4: 1.2 ug / L; Prolactin 8.41 mg / dL; Cortisol/24h 588.80 mcg/24h  (55-286). 4600.00 ml volume; ACTH: 29.9 (0-46). F Testosterone: 5.98 pg / mL (7.2-23); LH: 2.2 mIU / mL (1.7-8.6); FSH: 2.2 mIU / mL (1.5-12.4); Estradiol: 49.73 (7.6-42.6); DHES: 2.3.

A reduction of IGFBP3: 1880 ng/ml (3300-6600); IGF-1: < 25 ng/mL (101-267); hGH: 0,1; Proinsulin: 5.6 pmol / L (<18.9); Insulin 0.20 mU/ l (2.60-24.90); C-peptide: 0.07 ng/mL (1.1-4.4) was observed with an increase in IGF-2: 756 ng / ml (55-240). An abdominal RNM showed a great 20x12x14cm pelvic mass which demonstrates diameter -occupying most of the pelvic cavity, bladder retro and left lateral displacement straight antero lateral rectus. A biopsy of the tumor showed the typical morpho­logical features of HPC-like vessels, collagenous stroma and patternless architecture of spindled-to-ovoid tumor cells. Immunostaining for CD34 was positive in the tumor cells and in endothelial cells. Because the tumor was not considered for surgery, a chemotherapy treatment was initiated with partial reduction of symptoms of hypoglycemia and estrogen levels.

Conclusion: Epigenetic alterations may influence the origin and biochemical behaviors of HPC. A suppression of growth hormone axis because of the increase of IGF2 and elevation of aromatase activity are characteristic of some HPC

(1) Bokshan SF et al., J Gastrointest Surg. 2012; 16: 2170  (2) Lawson EA et al., J Cllin Endocrinol Metab 2009; 94: 2226 (3) Robinson DR et al., Nat Genet 2013; 45:180.

Nothing to Disclose: FL, AV, GG

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