J Korean Endocr Soc.  2007 Dec;22(6):440-445. 10.3803/jkes.2007.22.6.440.

A Case of Epithelioid Hemangioendothelioma of the Pelvic Retroperitoneum with Hypoglycemia

• Kim JR
• Jeun YK
• Park KT
• Kang YH
• Son SM
• Kim IJ
• Kim YK
• Choi KU
• Lee KJ

• KMID: 1958909
• DOI: http://doi.org/10.3803/jkes.2007.22.6.440

Abstract

Hypoglycemia caused by a non-islet cell tumor (NICT) is a rare condition. The mechanism of NICT-induced hypoglycemia is still unclear, but insulin-like growth factor-II (IGF-II) has been thought to play a major role in its development. NICT is usually of mesenchymal or epithelial cell origin, but reports on NICT of an endothelial cell origin, which causes hypoglycemia, have yet to surface. Here, we report on a case of a 63-year-old female patient who was diagnosed with epithelioid hemangioendothelioma-induced hypoglycemia. Epithelioid hemangioendothelioma is a borderline malignant vascular tumor that is of endothelial cell origin and usually occurs in soft tissue, skin, lung, and liver. It was observed that serum insulin, C-peptide, and IGF-I were reduced, but the IGF-II level was elevated in hypoglycemia. The PET-CT showed no abnormal glucose metabolism in the tumor. Dextrose fluid was administered to the patient to control hypoglycemia until the operation. For treatment and diagnosis, surgical resection of the tumor and total hysterectomy were performed. The specimen was noted to have epithelioid hemangioendothelioma. Hypoglycemia-related symptoms disappeared after surgical resection was performed.

Keyword

epithelioid hemangioendothelioma; hypoglycemia; insulin-like growth factor-II

MeSH Terms

C-Peptide
Diagnosis
Endothelial Cells
Epithelial Cells
Female
Glucose
Hemangioendothelioma, Epithelioid*
Humans
Hypoglycemia*
Hysterectomy
Insulin
Insulin-Like Growth Factor I
Insulin-Like Growth Factor II
Liver
Lung
Metabolism
Middle Aged
Skin
C-Peptide
Glucose
Insulin
Insulin-Like Growth Factor I
Insulin-Like Growth Factor II

Figure

• 

  Fig. 1 MRI. Huge tumor was well enhancing mass and was composed of necrotic and hemorrhagic portion in it. It showed engorged vessels around tumor.

• 

  Fig. 2 Whole body PET-CT. There was no abnormal glucose metabolism. FDG uptake in tumor cell was slightly increased (SUV 1.7), but it was meaningless.

• 

  Fig. 3 Gross histology of the tumor. The tumor mass was composed of pinkish brown fragments and flesh soft tissue, measuring 630 gm in its weight. The largest one measured 6×6×5 cm in dimensions. The cut surface was pinkish and showed multiple small hemorrhagic foci.

• 

  Fig. 4 Microscopic findings of the tumor. A. The tumor mass was composed of solid nests of rounded to slightly spindled endothelial cells (H&E stain, ×200). B. The tumor cells formed small intracellular lumens, which was seen as "vacuoles" (H&E stain, ×400). C, D. The tumor cells expressed CD34 and FVIII related antigen (×400).

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