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Published on: May 31, 2016
Hypercalcemia Associated with Extramammary Paget's Disease.
Tetsuko Sato1, Yoshihisa Wada2, Nobuhiko Kamitani3
1Department of Dermatology, Kawasaki Medical School, Kurashiki, Japan.
Humoral hypercalcemia of malignancy, often caused by parathyroid hormone-related protein (PTHrP), can occur in advanced cancers. Early measurement of serum calcium and PTHrP is crucial for patients with extramammary Paget's disease presenting with altered consciousness.
Area of Science:
- Oncology
- Endocrinology
- Pathology
Background:
- Hypercalcemia of malignancy affects up to one-third of advanced cancer patients.
- Humoral hypercalcemia of malignancy, driven by parathyroid hormone-related protein (PTHrP), is the most common cause.
- Extramammary Paget's disease is a rare cutaneous malignancy that can metastasize.
Observation:
- A 70-year-old male with metastatic extramammary Paget's disease presented with altered consciousness and a genital tumor.
- Elevated serum calcium (15.3 mg/dL) and intact PTHrP (66.1 pg/L) levels were confirmed.
- PTHrP immunohistochemistry was positive in tumor cells, confirming humoral hypercalcemia of malignancy.
Findings:
- Standard treatments for hypercalcemia provided only temporary relief.
- The patient experienced rapid enlargement of liver metastases and general condition deterioration.
- The patient died on day 55, highlighting the aggressive nature of the metastatic disease.
Implications:
- Early detection of hypercalcemia and PTHrP-producing tumors is critical in extramammary Paget's disease patients with altered consciousness.
- This case underscores the challenges in managing hypercalcemia of malignancy in advanced extramammary Paget's disease.
- Further research into novel therapeutic strategies for managing hypercalcemia in rare metastatic cancers is warranted.
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