LETTERS TO THE EDITOR

 

Bowel obstruction secondary to yeyunal intussusception due to gangliocytic paraganglioma

Obstrucción intestinal secundaria a invaginación yeyunal como forma de presentación de paraganglioma gangliocítico

 

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Key words: Gangliocytic paraganglioma. Intestinal invagination.

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Dear Editor,

Gangliocytic paraganglioma is an uncommon entity. It accounts for approximately two hundred cases reported in the medical literature (1). The jejunum location is extremely rare: there are only five cases reported in the medical literature (5).

 

Case report

We present the case of a 44-year-old woman suffering from intestinal obstruction. An image approximately 14 cm in length, with target morphology in its section, was detected by magnetic resonance enterography (Fig. 1). It included a polyp in one end, probably submucosal. A surgical resection of the lesion was performed. Twenty five months later, the patient remains without suspicion of relapse.

 

 

Histopathology confirmed a 2.5 cm submucosal polypoid tumor, which ulcerated the mucosa. It was composed of epithelioid-like cell nests with some disperse ganglion-like cells. On immunohistochemical analysis, the S-100 protein showed a high positive expression in most cells (Fig. 2), and enolase and synaptophysin showed positivity in ganglion-like cells (Fig. 3). The proliferation index was less than 10%.

 

 

 

Discussion

The gangliocytic paraganglioma is a rare tumor whose histologic origin remains unknown. Despite being considered as a benign entity (1), cases of lymph node metastasis and even bone and liver metastasis have been described (4). It is formed by epithelioid cell nests, spindle and ganglion-like cells. Immunohistochemistry showed a high positivity for neuronal specific enolase, synaptophysin and S-100 protein (1,2,4). The depth of invasion and the fact that cases of lymph node metastasis have been reported in 8% of the patients tested mean that it should be considered as a true neoplasm.

With regard to tumor location, 90.1% of cases are located in the duodenum and just 1.6% (1) of them in the jejunum. The most common symptom is gastrointestinal bleeding, followed by abdominal pain and anemia (1-4).

Histological diagnosis via forceps biopsies can be complicated, on account of the submucosal origin of the lesion (1,3). The treatment consists in the complete excision of the tumor.

 

Eugenia Caballero-Rodríguez¹, Beatriz Arencibia-Pérez² and Guillermo Hernández-Hernández^1
1Department of General Surgery and Digestive Diseases.
Hospital Universitario Nuestra Señora de Candelaria. Santa Cruz de Tenerife, Spain.
²Department of General Surgery and Digestive Diseases.
Hospital Universitario de Gran Canaria Doctor Negrín. Las Palmas de Gran Canaria, Spain

 

References

1. Okubo Y, Wakayama M, Nemoto T, et al. Literature survey on epidemiology and pathology of gangliocytic paraganglioma. BMC Cancer 2011;11:187. DOI: 10.1186/1471-2407-11-187.         [ Links ]

2. Nuño-Guzmán CM, Arróniz-Jáuregui J, Alvarez-López F, et al. Obstructing gangliocytic paraganglioma in the third portion of the duodenum. Case Rep Gastroenterol 2012;6:489-95. DOI: 10.1159/000341586.         [ Links ]

3. Sánchez-Pobre P, Sáenz-López S, Rodríguez S, et al. Safe endoscopic resection of gangliocytic paraganglioma of the major duodenal papilla. Rev Esp Enferm Dig 2004;96:660-4. DOI: 10.4321/S1130-01082004000900008.         [ Links ]

4. Park HK, Han HS. Duodenal gangliocytic paraganglioma with lymph node metastasis. Arch Pathol Lab Med 2016;140:94-8. DOI: 10.5858/arpa.2014-0456-RS.         [ Links ]

5. Aung W, Gallagher HI, Joyce WP, et al. Gastrointestinal haemorrhage from a jejunal gangliocytic paraganglioma. Clin Pathol 1995;48:84-5. DOI: 10.1136/jcp.48.1.84.         [ Links ]