Abstract
Ectopic production of biologically active glycoprotein hormones other than hCG has been reported in exceptional cases. A 61-yr-old man came to our Unit complaining of weakness, fatigue and reduced libido with erectile dysfunction. There was also a history of polycythemia, known for about 10 yr and never further investigated. The physical examination showed acne and redness of facial skin and upper chest; no other significant abnormalities were detected. Serum levels of LH were very high, whereas α-subunit and hCG were only slightly increased. Testosterone and 17β-estradiol levels were increased too. Abdominal computed tomography (CT) scan revealed a large hypervascularized mass within the pancreatic tail, which was surgically removed by distal splenopancreatectomy. Diffuse immunoreactivity for LH was detected in more than 70% of the tumor cells. The α-subunit was also positive, while chorionic gonadotropin had only a focal reactivity. Reverse transcriptasepolymerase chain reaction (RT-PCR) and Southern Blot analysis confirmed the synthesis of LH by the tumor. Four weeks after surgery, serum levels of LH, α-subunit, testosterone, hCG and 17β-estradiol were all undetectable. The redness of facial skin and upper chest had disappeared, but libido was still reduced. At a further control, 3 months after surgery, serum levels of LH, FSH, hCG, α-subunit and 17β-estradiol were all within the normal range, as well as hemoglobin concentration and the red blood cells count. Testosterone was slightly below normal, but the patient reported an increase of libido. This is an unusual case of ectopic secretion of LH from an endocrine tumor of the pancreas.
References
Mignon M, Jensen RT eds. Endocrine tumors of the pancreas. Recent advances in research and management. Basel: S. Karger. 1995, 1–482.
Oberg K. State of the art and future prospects in the management of neuronedocrine tumors. Q J Nucl Med 2000, 44: 3–12.
Fraker DL, Jensen RT. Pancreatic endocrine tumors. In: De Vita VT, Hellman S, Rosemberg SA eds. Cancer: principles and practice in Oncology. 5th ed. Philadelphia: Lippencott-Raven. 1997, 1678–84.
Hirshberg B, Conn PM, Uwaifo GI, Blauer KI, Clark BD, Nieman LK. Ectopic luteinizing hormone secretion and anovulation. N Engl J Med 2003, 384: 312–7.
Solcia E, Kloppel G, Sobin LH eds. WHO. Histological typing of endocrine tumors. 2nd ed. Berlin: Springer. 2000, 56–60.
Papotti M, Croce S, Bellò M, et al. Expression of somatostatin receptor types 2, 3 and 5 in biopsies and surgical specimens of human lung tumours. Correlation with preoperative octreotide scintigraphy. Virchows Arch 2001, 439: 787–97.
Leschek EW, Chan WY, Diamond DA, et al. Nodular Leydig cell hyperplasia in a boy with familial male-limited precocious puberty. J Pediatr 2001, 138: 949–51.
Alexanian R. Erythropoietin excretion in man following androgens. Blood 1966, 28: 1007–9.
Dickerman RD, Pertusi R, Zachariah NY, Shaller F. Androgeninduced erythrocytosis. Am J Hematol 1998, 59: 263–4.
Dickerman RD, Pertusi R, Miller J, Zachariah NY. Androgeninduced erythrocytosis: is it erythropoietin? Am J Hematol 1999, 61: 154–5.
Viallard JF, Marit G, Marcié P, Leng B, Reiffers J, Pellegrin JL. Polycythaemia as a complication of transdermal testosterone therapy. Br J Haematol 2000, 110: 237–8.
Katznelson L, Alexander JM, Bikkal HA, Jameson JL, Hsu DW, Klibanski A. Imbalanced follicle-stimulanting hormone β-subunit hormone biosynthesis in human pituitary adenomas. J Clin Endocrinol Metab 1992, 74: 1343–51.
Helzberg JH, McPhee MS, Zarling EJ, Lukert BP. Hepatocellular carcinoma: an unusual course with hyperthyroidism and inappropriate thyroid-stimulating hormone production. Gastroenterology 1985, 88: 181–4.
Gadner H, Weber B, Rjehm H. Adrenocortical carcinoma with ectopic LH production. Z Kinderheilkd 1974, 118: 63–70.
Romer TE, Sachnowska K, Savage MO, et al. Luteinizing hormone secreting adrenal tumour as a cause of precocious puberty. Clin Endocrinol (Oxf) 1998, 48: 367–72.
Dirnhofer S, Freund M, Rogatsch H, Krabichler S, Berger P. Selective expression of trophoblastic hormones by lung carcinoma: neuroendocrine tumors exclusively produce human chorionic gonadotropin alpha-subunit (hCGalpha). Hum Pathol 2000, 31: 966–72.
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Brignardello, E., Manti, R., Papotti, M. et al. Ectopic secretion of LH by an endocrine pancreatic tumor. J Endocrinol Invest 27, 361–365 (2004). https://doi.org/10.1007/BF03351063
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DOI: https://doi.org/10.1007/BF03351063