Document Type : Case Report

Authors

Department of Surgical Oncology, Chittaranjan National Cancer Institute, West Bengal, India

Abstract

Non-islet cell tumor hypoglycemia (NICTH) is an exceedingly rare paraneoplastic condition and often its commonest presenting symptom is hypoglycaemia. Most cases of NICTH are associated with underlying mesenchymal or epithelial neoplasm. However our case is unique as NICTH was associated with well differentiated liposarcoma, which has never been described before. Most of the reported cases of NICTH were diagnosed on the basis of biochemical tests. However NICTH can also be a diagnosis of exclusion as highlighted by our case report. This case also highlights both the diagnostic dilemma and the surgical challenges in the management of such cases. The elderly lady presented with repeated episodes of loss of unconsciousness for which she was hospitalised twice. Her symptoms closely resembled that of a cerebrovascular accident patient. However CT brain did not reveal any brain lesion. However she also had spontaneous episodes of hypoglycaemia which led to further investigations. Ultrasonography abdomen revealed presence of huge retroperitoneal mass on FNAB which was malignant. Subsequently, she was put on dextrose drip and thorough investigations ruled out metastatic disease. She underwent laparotomy and the mass was excised enbloc. Postoperative recovery was smooth and the hypoglycaemia resoled spontaneously. Final histopathologic examination was suggestive of well differentiated liposarcoma. At the 6-month follow-up, she was free from hypoglycemic episodes. This case highlights that NICTH can be a difficult diagnosis given its propensity to mimic several other benign conditions. NICTH can also be caused by liposarcomas. Diagnosis by excluding all other causes of hypoglycaemia is also an option where costly biochemical tests are unavailable. Surgical excision is the main stay of treatment.

Keywords

  1. Escobar GA, Robinson WA, Nydam TL, Heiple DC, Weiss GJ, Buckley L, et al. Severe paraneoplastic hypoglycemia in a patient with a gastrointestinal stromal tumor with an exon 9 mutation: a case report.  BMC Cancer. 2007;7:13.
  2. Service FJ, McMahon MM, O'Brien PC, Ballard DJ. Functioning insulinoma--incidence, recurrence, and long-term survival of patients: a 60-year study. Mayo Clin Proc. 1991;66(7):711-9.
  3. Blackman MR, Rosen SW, Weintraub BD. Ectopic hormones. Adv Intern Med. 1978;23:85-113.
  4. Odell WD, Wolfsen AR. Humoral syndromes associated with cancer. Annu Rev Med. 1978;29:379-406.
  5. Sluiter WJ, Marrink J, Houwen B. Monoclonal gammopathy with an insulin binding IgG(K) M-component, associated with severe hypoglycaemia. Br J Haematol. 1986;62(4):679-87.
  6. Stuart CA, Prince MJ, Peters EJ, Smith FE, Townsend CM 3rd, Poffenbarger PL. Insulin receptor proliferation: a mechanism for tumor-associated hypoglycemia. J Clin Endocrinol Metab. 1986;63(4):879-85.
  7. Kiang DT, Bauer GE, Kennedy BJ. Immunoassayable insulin in carcinoma of the cervix associated with hypoglycemia. Cancer. 1973;31(4):801-5.
  8. Silbert CK, Rossini AA, Ghazvinian S, Widrich WC, Marks LJ, Sawin CT. Tumor hypoglycemia: deficient splanchnic glucose output and deficient glucagon secretion. Diabetes. 1976;25(3):202-6.
  9. Dutta P, Aggarwal A, Gogate Y, Nahar U, Shah VN, Singla M, et al. Non-islet cell tumor-induced hypoglycemia: a report of five cases and brief review of the literature. Endocrinol Diabetes Metab Case Rep. 2013;2013:130046.
  10. Dimitriadis GK, Gopalakrishnan K, Rao R, Grammatopoulos DK, Randeva HS, Weickert MO, et al. Severe paraneoplastic hypoglycemia secondary to a gastrointestinal stromal tumour masquerading as a stroke. Endocrinol Diabetes Metab Case Rep. 2015;2015:150062.