A Young Woman With Refractory GI Symptoms
Article Outline
- Answer to the Clinical Challenges and Images in GI Question: Image 3 Adrenal Ganglioneuroma
- References
- Copyright
Question: A 20-year-old woman presented with a 1- year history of persistent epigastric pain and periodic vomiting. Her past medical and family histories were unremarkable. The abdominal computerized tomography (CT) scan revealed a large, low-attenuation, retroperitoneal tumor, measuring 9 × 7 cm with crescent-shaped calcifications. The mass (white circle in Figure A[c]) was infiltrating the upper pole of the right kidney (Figure A[d, arrow]), and was in proximity with the right adrenal gland, the abdominal aorta and IVC (Figure A[c]), abating the right renal vein and duodenum (Figure 3 [a, d]) and encasing the right renal artery (Figure A[a, b; arrows). Screening to rule out a hormonally active adenoma or pheochromocytoma included serum electrolytes, plasma aldosterone/rennin activity ratio, plasma metanephrine levels and overnight low dexamethasone suppression test (1 mg). The tumor was proved to be hormonally inactive and serum tumor markers were within reference range. A CT-guided biopsy of the mass showed numerous ganglion cells with single nuclei surrounded by bundles of spindle-shaped Schwann cells, which were positive for S-100 protein. Because of the small sample size, the rest of the immunohistochemical markers were not evaluated. The patient underwent laparotomy and the tumor was excised en block along with the right adrenal gland and the upper pole of the ipsilateral kidney. The encased right renal artery was dissected free (Figure B). Frozen section was negative for malignancy. Microscopic examination revealed that the neoplasm developed from the adrenal gland and was extending diffusely to involve the extra-adrenal fat and kidney capsule. Mature ganglion cells of various sizes isolated or in clustering and abundant neuroid bundles surrounded by Schwann cells were recognized. In many locations, the peri-neuroid stroma was myxoid and in some instances perivascular lymphocellular clustering was observed. Neuroblasts were not found (Figure C). Immunohistochemistry showed the presence of abundant neuroid bundles along the neuroid bonds. Ganglion and Schwann cells were strongly immunoreactive for NSE and S100, respectively. Ki67 index was <1%.
What is the diagnosis? Is this a case of a benign or a malignant tumor?
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Answer to the Clinical Challenges and Images in GI Question: Image 3 Adrenal Ganglioneuroma
Ganglioneuromas (GN) are rare, benign, well-differentiated tumors composed of Schwann cells, ganglion cells, fibrous tissue, and nerve fibers. GN can be hormonally active and secrete catecholamines in as many as 37% of cases. They occur more frequently in children and young adults. GNs count for 0.4% of neuroblastic tumors; 21% of GNs are adrenal tumors and approximately 7% are mature. Half of the patients present with GI symptoms.1 Two subtypes of GN are distinguished: mature and maturing.2 There have been few reports of GN in metastatic sites, either spontaneous or induced: GNs can appear de novo or evolve from a ganglioneuroblastoma or neuroblastoma (especially when treated with chemotherapy). Most investigators claim that GN results from the maturation of neuroblastoma cells to ganglion cells.3 Occurrence of metastasized GNs can be explained by spread of matured ganglio-neuroblastoma or neuroblastoma. In this case, despite the extra-adrenal fat and the kidney capsule infiltration, the tumor is considered benign because (1) it had no immature elements (such as neuroblasts, a feature of ganglio-neuroblastoma or neuroblastoma), atypia, mitotic figures, intermediate cells, or necrosis predicting thus a favorable prognosis according to the International Neuroblastoma Pathology Classification, in which GN is accompanied by the term Schwannian stroma-dominant in parentheses2 and (2) a previous history of ganglioneuroblastoma or neuroblastoma was absent. The management of GN is mainly surgical. GN may progress slowly and recur late; therefore, long-term follow up is necessary for early detection of locoregional failure.2 In such cases, reexcision of the tumor should be considered.
References
Conflicts of interest The authors disclose no conflicts.
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PII: S0016-5085(10)00012-0
doi:10.1053/j.gastro.2009.11.058
© 2010 AGA Institute. Published by Elsevier Inc. All rights reserved.
