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Malignant glomus tumor of the intestinal ileum with multiorgan metastases: A case report and review of literature.

ABSTRACT: BACKGROUND:Glomus tumors (GTs) are rare mesenchymal neoplastic lesions derived from cells of the glomus body. GTs rarely occurs in the visceral organs, where there may be few or no glomus bodies, and the majority of GTs are benign, rarely demonstrating aggressive or malignant behavior and histological features. CASE SUMMARY:We report a patient with malignant GTs of the intestinal ileum with multiorgan metastases who was admitted due to moderate anemia. Capsule endoscopy revealed a bleeding mass in the intestinal ileum, and the patient underwent segmental ileal resection through laparoscopic surgery. The histopathological and immunohistochemical diagnoses were consistent with malignant GT. Long-term follow-up showed that the GT had metastasized to multiple organs such as the colon, brain, and possibly the lung. CONCLUSION:This case was characterized by the highest degree of malignancy and by multiorgan metastases, and it was the first case of intestinal GT uncovered by capsule endoscopy.

SUBMITTER: Chen JH

PROVIDER: S-EPMC7039828 | biostudies-literature | 2020 Feb

REPOSITORIES: biostudies-literature

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Malignant glomus tumor of the intestinal ileum with multiorgan metastases: A case report and review of literature.

Chen Jian-Hong JH Lin Lin L Liu Kui-Liang KL Su Hui H Wang Ling-Ling LL Ding Peng-Peng PP Zhou Quan Q Liu Hong H Wu Jing J

World journal of gastroenterology 20200201 7

<h4>Background</h4>Glomus tumors (GTs) are rare mesenchymal neoplastic lesions derived from cells of the glomus body. GTs rarely occurs in the visceral organs, where there may be few or no glomus bodies, and the majority of GTs are benign, rarely demonstrating aggressive or malignant behavior and histological features.<h4>Case summary</h4>We report a patient with malignant GTs of the intestinal ileum with multiorgan metastases who was admitted due to moderate anemia. Capsule endoscopy revealed a ...[more]

PMID: 32116424

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Project description:BackgroundIntestinal Ganglioneuromatosis (IG) is a rare disorder of the enteric nervous system. In pediatric age it is often associated with genetic syndromes such as Neurofibromatosis 1 (NF1), multiple endocrine neoplasia type 2B (MEN2B) and Cowden syndrome (PTEN mutation), and ganglioneuromas (GNs) may be sometimes the first sign of the disease. Isolated GNs are rare and sporadic. Clinical symptom vary and depend on the size and on the location of the GNs. This disorder affects intestinal motility and it, consequently, causes changes in bowel habits, abdominal pain, occlusive symptoms and rarely lower gastrointestinal bleeding secondary to ulceration of the intestinal mucosa. On the other hand, patients can remain asymptomatic for many years.Case presentationWe describe a 9-year-old boy referred to our emergency department for right lower quadrant abdominal pain. No familial history for gastrointestinal disorders. No history of fever or weight loss. At physical examination, he had diffused abdominal pain. Abdominal ultrasonography showed a hypoechoic formation measuring 41.8 mm by 35 mm in the right lower quadrant of the abdomen. Routine blood tests were normal, but fecal occult blood test was positive. Abdominal TC confirmed the hypodense formation, of about 5 cm in transverse diameter, in the right hypochondrium that apparently invaginated in the caecum-last ileal loop. Colonoscopy showed in the cecum an invaginated polypoid lesion of the terminal ileal loop. Laparoscopic resection of the polypoid lesion was performed. Histological diagnosis of the large neoplasm observed in the terminal ileum was diffuse ganglioneuromatosis. NF1, RET and PTEN gene tests resulted negative for specific mutations. At the 1 year follow-up, the patient presented good general condition and blood tests, fecal occult blood test, esophagogastroduodenoscopy, colonoscopy and MR-enterography were negative.ConclusionsOnly few cases are reported in literature of IG in pediatric age. Although rare, the present case suggests that this disorder must be taken in consideration in every patient with GI symptoms such as abdominal pain, constipation, lower intestinal bleeding, in order to avoid a delayed diagnosis.

Dataset Information

Malignant glomus tumor of the intestinal ileum with multiorgan metastases: A case report and review of literature.

Publications

Malignant glomus tumor of the intestinal ileum with multiorgan metastases: A case report and review of literature.

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