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Malignant islet cell tumor associated with hypercalcemia

C Mao1, P Carter, P Schaefer

  • 1Department of Surgery, Toledo Hospital, Ohio.

Surgery
|January 1, 1995
PubMed
Summary
This summary is machine-generated.

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Pancreatic islet cell tumors can cause hypercalcemia by producing parathyroid hormone-related protein (PTHrP). These rare, malignant tumors are often vascular and associated with elevated PTHrP levels, not parathyroid hormone (PTH).

Area of Science:

  • Endocrinology
  • Oncology
  • Pathology

Background:

  • Investigated three cases of pancreatic islet cell tumors with hypercalcemia.
  • Reviewed 16 similar cases from English-language literature over 25 years.
  • Aimed to determine the cause of hypercalcemia and clinical features of these tumors.

Observation:

  • Examined tumor tissue using immunohistochemistry for parathyroid hormone (PTH) and PTH-related protein (PTHrP).
  • Measured serum PTH and serum PTHrP levels in affected patients.
  • One patient presented with a critically high serum calcium level of 26.4 mg/dl.

Findings:

  • Two of three tumors stained positive for PTHrP, with one patient showing elevated serum PTHrP.
  • Serum PTH levels were consistently normal or low in all patients.

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  • The majority of tumors (7/8) tested positive for PTHrP, and affected patients (4/4) had elevated PTHrP serum titers.
  • All studied tumors were malignant and highly vascular.
  • In the combined group of 19 patients, hypercalcemia was linked to normal or low serum PTH levels.
  • 17 out of 18 tumors were malignant, typically large but compatible with prolonged survival.

    • Implications:

    • Pancreatic islet cell tumors associated with hypercalcemia likely produce PTHrP as the causative agent.
    • These tumors are predominantly malignant, vascular, and characterized by elevated PTHrP.
    • Further research is needed to definitively classify these as a distinct type of functioning islet cell tumor.