Colonic Gastrointestinal Autonomic Nervous Tumor in a Patient with Crohn’s Disease

Veloso, Fernando Tavarela; Pereira, Pedro; Saraiva, António; Capelinha, Ana Filipa; Lopes, José Manuel

doi:10.1007/s10620-005-2865-5

Cited by 4 publications

(4 citation statements)

References 25 publications

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“…A diet with MCT improves the symptoms of PIL and reduces mortality rate [ 9 ]. Aoyagi et al [ 10 ] reviewed nine patients with intestinal lymphangiectasia; patients who did not respond to a low-fat diet, were treated with TPN or EN with elemental diet and polymeric diet. EN could be as effective as TPN providing a safe alternative therapy for patients with PIL but not in our case - due to chronic intestinal failure EN was not sufficient enough, therefore we had to combine EN + PN.…”

Section: Discussionmentioning

confidence: 99%

Home parenteral nutrition a life-saving therapy in a primary intestinal lymphangiectasia patient affecting the entire GI tract – 3 year follow-up case report

Mańkowska-Wierzbicka

Kloska

Karwowska

et al. 2021

Annals of Medicine &Amp; Surgery

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Introduction and Importance Primary intestinal lymphangiectasia (PIL) is a rare protein-losing gastroenteropathy of unknown etiology, characterized by impaired lymphatic vessels drainage. The pathological changes in PIL result in usually localized or diffuse dilatation of intestinal lacteals, leading to leakage of lymphatic fluid rich of proteins, lymphocytes, and immunoglobulins into the intestinal lumen. PIL may be asymptomatic or mildly symptomatic in moderate forms of the disease. In some patients, though, the outcome may be poor or even life-threatening. This case report demonstrates the severity of protein malnutrition, in some cases, and the extent of GI tract affected, requiring to start PN early and the need for its continuation as home parenteral nutrition (HPN). Case presentation We present a case of 39-year-old male with Factor V Leiden deficiency, who presented initially with symptoms of malnutrition and anasarca. The diagnosis was confirmed by histopathological findings pathognomonic for PIL from biopsies of the stomach, small intestine and colon. Clinical discussion The patient was started on low fat, high protein parenteral nutrition from the beginning of the treatment and required a long-term HPN for 3 years, because trials of tapering off and discontinuation of PN led to worsening of the biochemical results and recurrence of symptoms. Patient gradually improved and stabilized with persistent nutritional support. Conclusions The presented case report shows the magnitude of nutritional support (HPN) needed for severe PIL patients. HPN offers PIL patients with poor outcome and life-threatening complications a chance to improve and lead a normal life.

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Section: Discussionmentioning

confidence: 99%

Home parenteral nutrition a life-saving therapy in a primary intestinal lymphangiectasia patient affecting the entire GI tract – 3 year follow-up case report

Mańkowska-Wierzbicka

Kloska

Karwowska

et al. 2021

Annals of Medicine &Amp; Surgery

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“…Immunohistochemical studies usually display evidence of neural differentiation and negativity for smooth muscle antigens, but the key for identifying a tumor as GANT is the presence of dendritic processes with dense neuroendocrine granules on ultrastructural examination [7]. Furthermore, in contrast to GIST, GANT is rather more common in the small intestine than stomach [8], but has been reported form the other sites such as oesophagus [9], colon [10], retroperitoneum, omentum, mesentery and peritoneum [3,11]. …”

Section: Discussionmentioning

confidence: 99%

Gastrointestinal autonomic nerve tumor of the stomach

Meshikhes

et al. 2014

Am J Case Rep

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Patient: Female, 32Final Diagnosis: Gastrintestinal Autonomic Nerve Tumor (GANT)Symptoms: anemia • anorexia • fatigue • fever • hearburn • nausea • weight lossMedication: —Clinical Procedure: —Specialty: Gastroenterology and HepatologyObjective:Rare diseaseBackground:Gastrointestinal autonomic nerve tumors (GANT) are extremely rare tumors that are related to gastrointestinal autonomic nervous plexuses. They are distinguished from stromal tumors by their unique ultrastructural features. Hence, their diagnosis is usually made on electron microscopy and immunohistochemical analyses. Although they are apparently slow-growing tumors, they run an aggressive clinical course and often associated with poor prognosis which eventually leads to death.Case Report:We report on a case of gastric GANT in a young female who was treated surgically by total gastrectomy. The disease, however ran an aggressive course with the development of distant (nodal, liver, lung, adrenal and musculo-skeletal) metastases two months after the radical resection.Conclusions:We believe this could be the first reported case of adrenal and musculo-skeletal metastases from gastric GANT soon after the radical gastric resection.

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“…24 -26 GANT secreting catecholamines with its associated symptoms of flushing, fluctuating hypertension, and raised urinary 5-hydroxyindoleacetic acid was also reported. 27 GANTs may also arise in the setting of other medical conditions such as von Recklinghausens disease (neurofibromatosis type 1), 23 , 24 Crohn's disease, 6 Meckel's diverticulum, 28 c o l o r e c t a l a d e n o m a o r c a r c i n o m a , 1 0 a d r e n a l ganglioneuroma, 29 , 30 Carney's triad, 31 , 32 and renal cell carcinoma. 33 Multiple familial GANTs in association with small intestinal neuronal dysplasia have also been reported.…”

Section: Clinical Presentationmentioning

confidence: 99%

“…1 , 11 , 52 , 57 , 61 , 65 In other cases, metastatic sites appeared soon after an apparently radical surgical resection. 6 62 Meshikhes et al reported a case of gastric GANT in a young female who developed distant musculoskeletal, lung, and adrenal metastases only 2 months after radical total gastrectomy. 21 A case of metastases to the liver and peritoneum occurring 8 years after a radically resected duodenal stromal tumor with benign features was reported in a young woman.…”

Section: Prognosismentioning

confidence: 99%