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Special Feature |

Image of the Month—Diagnosis FREE

Arch Surg. 2010;145(1):100. doi:10.1001/archsurg.2009.242-b.
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Ganglioneuroma is a rare, benign tumor that is found most commonly in children and young adults and arises from the sympathetic ganglia. It is composed of mature Schwann cells, ganglion cells, and nerve fibers. The posterior mediastinum is the most frequent site of origin of ganglioneuroma. Less frequently it arises in the retroperitoneum and, quite rarely, in the adrenal glands.1,2

Characteristically, this tumor does not produce excess catecholamines or steroid hormones,3 and it usually presents as a clinically silent lesion incidentally detected in imaging studies for unrelated reasons. Surgery is not mandatory if a certain diagnosis of ganglioneuroma is made.

The rarity and the lack of understanding of ganglioneuroma biology often lead to inappropriate diagnosis and/or treatment.46 Unfortunately, there are no specific diagnostic signs or symptoms allowing preoperative discrimination between active ganglioneuroma and pheochromocytoma. In our case, the gradient between plasma catecholamine (normal) and urinary metanephrine levels (elevated) and, furthermore, the atypical appearance on computed tomographic scan (absence of irregular central areas representing necrosis or hemorrhage that, when present in large adrenal masses, are strongly suggestive for pheochromocytoma) most likely should have discouraged the hypothesis of pheochromocytoma. Nevertheless, the large diameter of the lesion was itself a strong indication for surgery because malignancy had to be excluded.

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Submissions

The Editor welcomes contributions to the Image of the Month. Manuscripts should be submitted via our online manuscript submission and review system (http://manuscripts.archsurg.com). Articles and photographs accepted will bear the contributor's name. Manuscript criteria and information are per the Instructions for Authors for Archives of Surgery (http://archsurg.ama-assn.org/misc/ifora.dtl). No abstract is needed, and the manuscript should be no more than 3 typewritten pages. There should be a brief introduction, 1 multiple-choice question with 4 possible answers, and the main text. No more than 2 photographs should be submitted. There is no charge for reproduction and printing of color illustrations.

Correspondence: Gabriele Materazzi, MD, Dipartimento di Chirurgia, Università de Pisa, Via Roma 67, 56100 Pisa, Italy (gmaterazzi@yahoo.com).

Accepted for Publication: April 1, 2009.

Author Contributions:Study concept and design: Materazzi and Miccoli. Acquisition of data: Berti, Conte, and Faviana. Drafting of the manuscript: Materazzi and Conte. Critical revision of the manuscript for important intellectual content: Berti, Faviana, and Miccoli. Study supervision: Materazzi, Berti, Conte, Faviana, and Miccoli.

Financial Disclosure: None reported.

F  Arredondo MartínezM  Soto DelgadoA  Benavente FernandezB  Basquero GonzalezA  Zurera CosanoR  Linares Armada Adrenal ganglioneuroma: report of a new case. Actas Urol Esp 2003;27 (3) 221- 225
PubMed Link to Article
T  YoshidaJ  SaitoT  Takao Adrenal ganglioneuroma: a case report. Hinyokika Kiyo 2005;51 (2) 93- 96
PubMed
B  GeoergerB  HeroD  HarmsJ  GrebeK  ScheidhauerF  Berthold Metabolic activity and clinical features of primary ganglioneuromas. Cancer 2001;91 (10) 1905- 1913
PubMed Link to Article
N  FukumitsuH  AshidaS  Ogi A case of ganglioneuroma in which 131I-6beta-iodomethyl-19-norcholest-5(10)-en-3beta-ol scintigraphy showed high uptake in the adrenal gland leading to a misdiagnosis. Ann Nucl Med 2006;20 (1) 69- 73
PubMed Link to Article
KJ  SinghA  SuriV  VijjanP  SinghA  Srivastava Retroperitoneal ganglioneuroma presenting as right renal mass. Urology 2006;67 (5) 1085.e7- 1085.e8
CN  StasikTJ  GiordanoPG  Gauger Ganglioneuroma manifesting as an incidental adrenal mass in an adult with Turner's syndrome. Endocr Pract 2005;11 (6) 382- 384
PubMed Link to Article

Figures

Tables

References

F  Arredondo MartínezM  Soto DelgadoA  Benavente FernandezB  Basquero GonzalezA  Zurera CosanoR  Linares Armada Adrenal ganglioneuroma: report of a new case. Actas Urol Esp 2003;27 (3) 221- 225
PubMed Link to Article
T  YoshidaJ  SaitoT  Takao Adrenal ganglioneuroma: a case report. Hinyokika Kiyo 2005;51 (2) 93- 96
PubMed
B  GeoergerB  HeroD  HarmsJ  GrebeK  ScheidhauerF  Berthold Metabolic activity and clinical features of primary ganglioneuromas. Cancer 2001;91 (10) 1905- 1913
PubMed Link to Article
N  FukumitsuH  AshidaS  Ogi A case of ganglioneuroma in which 131I-6beta-iodomethyl-19-norcholest-5(10)-en-3beta-ol scintigraphy showed high uptake in the adrenal gland leading to a misdiagnosis. Ann Nucl Med 2006;20 (1) 69- 73
PubMed Link to Article
KJ  SinghA  SuriV  VijjanP  SinghA  Srivastava Retroperitoneal ganglioneuroma presenting as right renal mass. Urology 2006;67 (5) 1085.e7- 1085.e8
CN  StasikTJ  GiordanoPG  Gauger Ganglioneuroma manifesting as an incidental adrenal mass in an adult with Turner's syndrome. Endocr Pract 2005;11 (6) 382- 384
PubMed Link to Article

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