J Korean Endocr Soc.  2008 Dec;23(6):450-455. 10.3803/jkes.2008.23.6.450.

A Case of Retroperitoneal Paraganglioma Manifested as Intractable Constipation with Paralytic Ileus and Aggravated Hyperglycemia

• Hwang SJ
• Kwon MK
• Chon S

• KMID: 1966000
• DOI: http://doi.org/10.3803/jkes.2008.23.6.450

Abstract

Paragangliomas are extra-adrenal pheochromocytomas that arise from specialized neural crest cells. They are distributed anywhere from the upper neck to the pelvic floor, and they are classified on the basis of their anatomic origin. Functioning paragangliomas can cause the same clinical manifestations as pheochromocytoma, such as hypertension, diabetes mellitus, hyperadrenergic spells and so on. We experienced a retroperitoneal paraganglioma that was found in 66 year-old male who suffered from intractable constipation, and his constipation was caused by paralytic ileus and uncontrolled hyperglycemia. After he was diagnosed, removal of the paraganglioma was done and his clinical symptoms and sustained hyperglycemia were successfully resolved.

Keyword

hyperglycemia; paraganglioma; paralytic ileus

MeSH Terms

Constipation
Diabetes Mellitus
Humans
Hyperglycemia
Hypertension
Intestinal Pseudo-Obstruction
Male
Neck
Neural Crest
Paraganglioma
Pelvic Floor
Pheochromocytoma

Figure

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  Fig. 1 Simple abdominal X-ray finding of paralytic ileus. Simple abdomen X-ray photographs shows markedly gas-distended colon with air-fluid level of stepladder pattern. It means severe paralytic ileus.

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  Fig. 2 CT scan finding of paraganglioma. A. Horizontal section. B. Coronal section. Two abdominal CT scan photographs show inhomogenously enhanced hypervascular mass (4.7 × 4.2 × 5.2 cm) with clear boundaries and lobulating contour located in left paraaortic area, L3 level. It contains septa-like structures and less enhanced portions with peripheral rim enhancement.

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  Fig. 3 I123 MIBG scan finding of paraganglioma. I123 MIBG scan photograph shows a lesion of increased uptake of radio-active materials located in left para-aortic area, suggestive of extraadrenal pheochromocytoma.

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  Fig. 4 Gross and microscopic findings of paraganglioma. A. It is a well defined solid mass, measuring 7.8×5×4.5 cm in dimension. Cut surface shows mahogany-colored soft and friable tumor mass associated with hemorrhage and necrosis. The mass is surrounded by thin membranous capsule. B. Some polygonal tumor cell nests make clusters resembling "Zellballen" appearance (H-E stain, ×40). C. Tumor cell has abundant granular eosinophilic cytoplasm with indistinct cell borders (H-E stain, ×100).

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  Fig. 5 Simple abdomen x-ray (post-operation follow up). Post-operation follow up simple abdomen photographs shows decreased colonic distension and bowel gas compared with previous x-ray photographs. It means improvement of the paralytic ileus.

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