Dig Dis Sci DOI 10.1007/s10620-014-3172-9


Intestinal Benign Glomus Tumor: Description and Review of the Literature J. P. Campana • J. Goransky • E. G. Mullen E. M. Palavecino

Received: 25 February 2014 / Accepted: 15 April 2014 Ó Springer Science+Business Media New York 2014

Keywords Intestinal glomus tumor  Gastrointestinal tumors  Glomus tumor  Gastrointestinal bleeding

Introduction Glomus tumors (GT) are very rare mesenchymal neoplasm, which represent 1–4.5 % of soft tissue tumors [1]. They origin from the glomus body, a special type of arteriovenous anastomosis without an intermediate capilar net, which acts as a chemoreceptor and regulates skin temperature. Glomus bodies are present throughout the skin but are more concentrated in the digits. This explains why GT are more common on the extremities, especially in the subungual area of the digit [2]. In less than 25 % of the cases, these tumors can be found in extracutaneous locations where glomus bodies do not exist physiologically [3]. Gastrointestinal location is extremely infrequent.

J. P. Campana (&)  J. Goransky  E. M. Palavecino General Surgery Service, Hospital Italiano de Buenos Aires, Juan D. Pero´n 4190, C1181ACH Buenos Aires, Argentina e-mail: [email protected] J. P. Campana Avenida Segurola 3293, 4to piso B, C1417BBY Buenos Aires, Argentina E. G. Mullen Pathology Service, Hospital Italiano de Buenos Aires, Juan D. Pero´n 4190, C1181ACH Buenos Aires, Argentina

Case Presentation A 51-year-old man is admitted to our emergency department with 3 episodes of melena in the last 24 h, associated with symptoms of orthostasis, without acute abdominal pain. In the digital rectal examination, bright red blood was observed. A clear nasogastric aspirate was obtained after gastric lavage. Blood tests showed an initial hematocrit concentration of 30 %, which reduced to 23.5 % in the first 12 h, and the hemoglobin was 80 g/L. He was transfused 2 units of red blood cells (URBC), and an upper and lower gastrointestinal endoscopies were performed in which no signs of bleeding were found. He persisted with melena and hematocrit concentration decline; thus, two more URBC were transfused. A CT enterography and an angiography (Fig. 1) were performed, and a hypervascular tumor of 17 mm was found in the proximal ileum. The patient underwent a laparoscopic exploration. The tumor was located 1 m distal to de Treitz angle. It was successfully resected, and an enteroenteric anastomosis was performed. The patient had no further bleeding and was discharged 48 h later. The resected specimen measured 11 cm with an indurated area of 3.7 cm of maximum diameter that involved 50 % of the circumference (Fig. 2). A definite diagnosis was made by histological section (Fig. 3) and immunohistochemical examination. After 2 years of follow-up, the patient has no signs of recurrence.

Discussion GT are unusual mesenchymal neoplasms that originate from the glomus bodies of the skin [1]. These tumors are commonly found in the acral soft tissue [3], whereas gastrointestinal location is extremely rare. The vast majority of


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Fig. 1 a Sagittal image of a CT enterography. The green circle shows the tumor, located in the proximal ileum. b Angiography that shows the hypervascular pattern of the tumor

Fig. 2 Operative specimen. The green arrow shows an ulcerative lesion of the mucosa

Fig. 3 a Histological section that reveals that the tumor is located in the muscular layer (H&E stain, magnification 940). b The tumor is composed of multiple cellular nodules with round uniform nucleus and well-delimitated cell membrane, surrounded by vascular


the reported cases are those that involve the stomach atrium [4]. Before 1985, the Russians reported four cases, two of which were malignant [5–8]. However, there is no report of a benign intestinal GT in the English medical literature so far. GT occur predominantly in female and appear at a median age of 55 years [9]. The most common symptoms are gastrointestinal bleeding, diffuse inespecific abdominal pain, ulcer-like symptoms [9] and nausea and bilious vomiting secondary to bowel obstruction [4]. In some cases, these tumors can be found incidentally during a nonrelated surgery or endoscopy. The main differential diagnoses of these tumors are GISTs, paragangliomas, carcinoid tumors, hemangiopericytomas and lymphomas. Histological and immunohistochemical examination is paramount to achieve the final diagnosis. GT stain positive for a-smooth muscle actin and

structures (H&E stain, magnification 9400). Mitotic activity is less than 5/50 high-power field (HPF) and less than 5 % of Ki67 stain positive in 10 fields 9400, which confirmed it as a benign tumor

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vimentin and stain negative for KIT, CD34, chromogranin, synaptophysin and leukocyte-specific markers. GT are considered benign when they meet the following criteria: less than 5 mitosis in 50 fields 4009 in the H&E stain, less than 5 % of Ki67 stain positive in 10 fields 4009 and absence of metastasis, the three of which were met by the case presented in this article [10]. In conclusion, gastrointestinal GT are very rare neoplasms, yet more when they comprise the small bowel. The final diagnosis is made by histological and immunohistochemical examination. They should be taken in consideration in patients with gastrointestinal bleeding and no clear endoscopic diagnosis. Conflict of interest


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3. Schiefer TK, Parker WL, Anakwenze OA, Amadio PC, Inwards CY, Spinner RJ. Extradigital glomus tumors: a 20-year experience. Mayo Clin Proc Mayo Clin. 2006;81(10):1337–1344. 4. Abu-Zaid A, Azzam A, Amin T, Mohammed S. Malignant glomus tumor (glomangiosarcoma) of intestinal ileum: a rare case report. Case Rep Pathol. 2013;305321. PubMed PMID: 23691399. Pubmed Central PMCID: 3638502. 5. Dasaev AN, Stepanov VA. Glomus tumor of the small intestine with metastasis to the liver. Klin Med. 1985;63:110–111. PubMed PMID: 2987610. Glomicheskaia opukhol’ tonkoi kishki s metastazom v pechen’. 6. Rykov VA. Malignant glomus tumor of the jejunum with distant metastases. 19771125 DCOM- 19771125(0004-1955 (Print)). rus. 7. Lenskaia MA, Treshchan O. Glomus tumor of the small intestine. Khirurgiia. 1976;127–128. PubMed PMID: 181636. Glomicheskaia opukhol’ tonkoi kishki. 8. Vaza AM, Kaem RI, Chikunova BZ. Glomic tumor of the ileum with perforation and hemorrhage. Klin Med. 1974;52:129–130. PubMed PMID: 4371251. Epub 06/01/1974. Glomicheskaia opukhol’ podvzdoshnoi kishki s perforatsiei i krovotecheniem. rus. 9. Miettinen M, Paal E, Lasota J, Sobin LH. Gastrointestinal glomus tumors: a clinicopathologic, immunohistochemical, and molecular genetic study of 32 cases. Am J Surg Pathol. 2002;26(3):301–311. 10. Folpe AL, Fanburg-Smith JC, Miettinen M, Weiss SW. Atypical and malignant glomus tumors: analysis of 52 cases, with a proposal for the reclassification of glomus tumors. Am J Surg Pathol. 2001;25(1):1–12.


Intestinal benign glomus tumor: description and review of the literature.

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