Jejunal gangliocytic paraganglioma

Paragangliomas (PGs) are neuroendocrine tumours originating from extra-adrenal chromaffin cells.^1,2 They are rare, with an incidence of 1.5 cases per million population.² Intestinal PGs are usually located in the second part of the duodenum, with location in the jejunum being exceptionally rare.^3–5 We present the case of a patient who had a jejunal gangliocytic paraganglioma (JGP) and review the literature.

This was a 66-year-old man with a medical history of high blood pressure, dyslipidaemia, grade II obesity, duodenal ulcer and acute myocardial infarction, treated with stents and dual antiplatelet therapy. He went to Accident and Emergency due to melaena, which he had had for a year but which had worsened upon initiation of dual antiplatelet therapy. On physical examination he was in good general condition, with no relevant findings. Lab tests revealed a haemoglobin level of 7.4 mg/dl. Gastroscopy was performed, revealing gastric and duodenal angioectasias, which were treated with argon gas. Computed tomography (CT) scan of the abdomen and pelvis revealed rounded endoluminal parietal thickening at the mesenteric edge of the first jejunal loop with a soft tissue density of 23 × 18 mm, which could indicate jejunal cancer. Enteroscopy was performed, identifying a jejunal lesion, with signs of recent bleeding. It was biopsied and reported as a low-grade neuroendocrine tumour positive for synaptophysin and chromogranin. The patient underwent surgery; a lesion was palpated in the first intraluminal and mobile jejunal loop, performing an enterotomy and revealing a mamelonated and pedunculated lesion, which was resected. The rest of the gastrointestinal tract was examined, finding no other associated lesions. The histology result was low-grade gangliocytic paraganglioma with submucosal infiltration and without lymphovascular or perineural invasion. Immunohistochemistry was positive for s100, synaptophysin, chromogranin and neuron-specific enolase. The patient's postoperative recovery was favourable (Clavien 0) and he was discharged after three days. At the six-month check-up, he had not had any further gastrointestinal symptoms. The terms used for the PubMed search without language or time limits were “jejunal” AND “gangliocytic” AND “paraganglioma”. We included articles that included patients with JGP. Those that included PGs in another part of the gastrointestinal tract were excluded.

PGs are extra-adrenal neuroendocrine tumours.¹ Their aetiology is unknown.^4–6 Most PGs are sporadic, but they can occur in patients with certain endocrine syndromes.⁴ PGs are characterised by having three cell lines: epithelioid, ganglion and spindle cells.^6–8 Immunohistochemically, they are positive for s100, synaptophysin, chromogranin and neuron-specific enolase.^6,8 PG excision is the basis of any treatment, as these tumours are usually chemoresistant,⁴ with 8% of cases metastasizing.^6,7 The five-year survival of patients with malignant PG is around 60%.^2,7 Of the patients who have surgery, 16% have late recurrences (>10 years), making prolonged follow-up necessary.⁷

Intestinal PG is located almost exclusively in the second part of the duodenum (90% of cases),³ while a jejunal location is extremely rare (1.6% of all intestinal PGs).³ Our search for JGP revealed few previously published cases (Table 1). The most common symptoms of the published JGPs are haemorrhage (3 patients), abdominal pain (1)³ and intestinal obstruction (1).^4,6 Our patient's initial symptom was melaena. The average age is 58.4 years, a little younger than our case.⁵ In the cases described, four occurred in women and only one in a man, like our case (Table 1). The diagnosis was made through a variety of diagnostic tests.

Table 1.

Cases published in the literature.

Author, year	Gender	Age	Debut symptom	Diagnosis	Treatment	IHC
Grouls, 1987	Female	54	Gastrointestinal bleeding	NA	Afferent loop in Billroth II: endoscopic	Neuron-specific enolaseS100CytokeratinVimentinSerotonin
Aung, 1995	Female	54	Melaena	Laparotomy	Enterotomy and lesion excision	ChromograninSynaptophysinNeuron-specific enolaseS100Somatostatin
Kazim, 2015	Male	70	Abdominal painVomiting	CT	Segmental resection	Neuron-specific enolase
Caballero, 2016	Female	44	Intestinal obstruction	MR enterography	Lesion excision	SynaptophysinNeuron-specific enolaseS100
Fontana, 2021	Female	69	Melaena	CT/ERCP	Duodenal-jejunal junction:CholecystectomyBiliary drainageEnterotomy and lesion excisionLymphadenectomy	ChromograninSynaptophysinS100CK8/18Somatostatin
Viñas, 2023	Male	66	Melaena	CTEnteroscopy	EnterotomyLesion excision	ChromograninSynaptophysinNeuron-specific enolaseS100

IHC: immunohistochemistry; NA: not available.

Treatment for JGP is complete excision.⁵ Endoscopic resection seems a reasonable option if the tumour can be completely removed with this approach, but it has only been performed on one occasion.^5,7 If it is not feasible, surgical excision should be performed, either by enterotomy and resection of the lesion with free margins (3 patients) or by performing an intestinal resection (1 patient). Due to the small number of patients, we do not know which of the two techniques is better.^4,5

In conclusion, a very small number of jejunal PGs have been reported in the literature. Complete excision by enterotomy or intestinal resection is the treatment of choice.

Funding

No funding was received for preparing this manuscript.

Conflicts of interest

The authors have no conflicts of interest to declare.