Clinical Imaging xxx (2015) xxx–xxx

Contents lists available at ScienceDirect

Clinical Imaging journal homepage: http://www.clinicalimaging.org

Glomus tumour of the colon: dynamic contrast-enhanced CT findings and review of the literature☆ Tien Jin Tan a,⁎, Malcolm M. Hayes b, Jordan P. Radigan c, Peter L. Munk a a b c

Department of Radiology, Vancouver General Hospital, Vancouver, BC, Canada Department of Pathology, British Columbia Cancer Agency, Vancouver, BC, Canada Department of Pathology, St. Joseph’s General Hospital, Comox, BC, Canada

a r t i c l e

i n f o

Article history: Received 26 December 2014 Received in revised form 15 February 2015 Accepted 26 February 2015 Available online xxxx Keywords: Glomus tumour Gastrointestinal Colon Subepithelial Computed tomography

a b s t r a c t We describe the peculiar enhancement pattern of a subepithelial colonic glomus tumour on dynamic contrastenhanced computed tomography. Discontinuous, peripheral nodular enhancement of the colonic lesion followed by subsequent centripetal filling-in of contrast was reminiscent of a hepatic haemangioma, which has not been described with a glomus or any other subepithelial tumour of the colon. When encountered, this tumour enhancement pattern raises the possibility of a colonic glomus tumour prior to histological confirmation. © 2015 Elsevier Inc. All rights reserved.

1. Introduction Glomus tumours are uncommon mesenchymal lesions with pericytic differentiation and histological features resembling those of perivascular glomus bodies, which in turn are specialised arteriovenous communications that regulate skin temperature [1]. Glomus tumours are classically found in a subungual location in the distal extremities. They have occasionally been reported in other locations, including the gastrointestinal tract, where they occur almost exclusively in the stomach [1]. Glomus tumours of the colon are exceedingly rare, with only a handful of cases reported in the published literature [1–5]. 2. Case report A 74-year-old man presented to the emergency department with several episodes of vomiting and acute abdominal pain. A noncontrast-enhanced computed tomography (CT) scan of the patient’s abdomen and pelvis was performed to exclude a surgical cause for the patient’s symptoms. This revealed bilateral renal cortical cysts but no other significant finding to warrant emergent surgical intervention. A follow-up contrast-enhanced CT scan of the kidneys was however suggested to document interval stability of the dominant cortical cyst in the right kidney that showed focal calcification and thin internal septations on CT but otherwise no aggressive feature. The patient was ☆ The authors declare that they have no sources of support and no conflict of interest. ⁎ Corresponding author. Department of Radiology, Vancouver General Hospital, 899 West 12th Avenue, Vancouver, BC, Canada V5Z1M9. Tel.: +1-604-875-4533; fax: +1-604-875-5195. E-mail address: [email protected] (T.J. Tan).

managed as a case of presumed acute gastritis and was subsequently discharged from the emergency department following resolution of his symptoms. The follow-up dynamic contrast-enhanced CT scan of the patient’s kidneys 1 year later revealed stable, benign appearing bilateral renal cortical cysts. There was however incidental finding of an exophytic, subepithelial solid lesion arising from the splenic flexure measuring 2.5 cm in maximum axial diameter. No calcification or ulceration of the lesion was detected. Discontinuous, peripheral nodular enhancement of the lesion was noted in the post-contrast-enhanced nephrographic phase images, with subsequent centripetal filling-in of contrast in the pyelographic phase, reminiscent of a hepatic haemangioma (Fig. 1). No significant enlarged retroperitoneal or mesenteric lymph node was detected. The exophytic colonic mass was also present in the patient’s prior imaging following retrospective review of the CT scan acquired 1 year earlier, and comparison between the two scans showed that the lesion had more than doubled in size in the interim, measuring approximately 1.0 cm in maximum axial diameter in the previous study. Given the colonic lesion’s imaging features, a gastrointestinal stromal tumour (GIST) was felt to be the most likely diagnosis, although a primary colonic malignancy was also considered in view of the patient’s age. A decision was made for the patient to undergo a laparoscopic left hemicolectomy to remove the colonic tumour. The resected specimen consisted of a 21-cm-length colon with an unremarkable mucosal surface and a 2.2-cm circumscribed haemorrhagic intramural lesion coming to within 1 mm of the serosal surface (Fig. 2). The peritoneal surface was uninvolved. The tumour had a polylobated architecture with a focally irregular border. It was composed of irregular

http://dx.doi.org/10.1016/j.clinimag.2015.02.015 0899-7071/© 2015 Elsevier Inc. All rights reserved.

Please cite this article as: Tan TJ, et al, Glomus tumour of the colon: dynamic contrast-enhanced CT findings and review of the literature, Clin Imaging (2015), http://dx.doi.org/10.1016/j.clinimag.2015.02.015

2

T.J. Tan et al. / Clinical Imaging xxx (2015) xxx–xxx

Fig. 1. A 74-year-old man with glomus tumour of the colon. Top: Non-contrast axial CT scan image demonstrates a soft tissue mass arising from the splenic flexure (white arrow). No calcification or ulceration of the lesion was detected. Middle: Axial CT scan image in the nephrographic phase demonstrates discontinuous peripheral nodular enhancement of the lesion arising from the splenic flexure (white arrow). Bottom: Axial CT scan image in the pyelographic phase demonstrates subsequent centripetal filling-in of contrast by the lesion (white arrows).

sheets of polygonal cells that showed moderate nuclear atypia and a variable amount of eosinophilic cytoplasm (Fig. 2). Some cells had vacuolated cytoplasm imparting a clear cell appearance. Mitoses numbered up to 19 per 50 high-power fields. No atypical mitotic figures were identified. The tumour contained many ectatic vascular channels lined by bland flattened endothelial cells. It was separated from the peritoneal surface by a thin pseudocapsule of compressed collagen. The bowel resection margins were negative for neoplasm. Based on the haematoxylin and eosin morphology, the differential diagnoses considered included a gastrointestinal glomus tumour, an epithelioid GIST, a clear cell sarcoma of gastrointestinal type, and a gastrointestinal neuroectodermal tumour. Immunostains showed that the tumour was strongly positive for vimentin, smooth muscle actin (Fig. 2), muscle-specific actin, and synaptophysin. The tumour demonstrated pericellular staining for collagen IV. It was focally positive for caldesmon but negative for S-100 protein, CD34, DOG1, CD117, chromogranin A, desmin, CK20, RCC, PAX8, CD31, CK7, SOX10,

Fig. 2. A 74-year-old man with glomus tumour of the colon. Top: Microscopic section of the resected tumour (T) (haematoxylin and eosin; original magnification ×10) demonstrates a circumscribed intramural lesion with areas of haemorrhage (*) and an intact overlying mucosa (M). Numerous ectatic vascular channels are also visualised within the tumour. Bottom left: Microscopic image of the resected specimen (haematoxylin and eosin; original magnification ×400) shows irregular sheets of polygonal cells with moderate nuclear atypia and a variable amount of eosinophilic cytoplasm. Bottom right: Immunoassaying revealed that the tumour cells were strongly positive for muscle-specific actin (immunostain; original magnification ×200).

HMB45, CD10, and myogenin. The Ki-67 proliferation rate was 15–20%. This immunoprofile was consistent with a gastrointestinal glomus tumour and would exclude the other entities considered in the differential diagnosis. The patient made a good post-operative recovery and was clinically well on follow-up 6 months following surgery. 3. Discussion The CT imaging findings of gastric glomus tumours have been well described in the available literature. These lesions are frequently located in the gastric antrum in a subepithelial location and are usually small in size, measuring less than 3 cm in diameter. Dynamic contrast-enhanced CT demonstrates a well-defined hypervascular lesion, with peripheral nodular or globular enhancement of the tumour being observed in the arterial phase, and subsequent central filling-in of contrast in the portal venous and/or equilibrium phases [6–8]. It has been suggested that this particular pattern of enhancement similar to that of a hepatic haemangioma may be used to differentiate gastroduodenal glomus tumours from other subepithelial lesions such as GISTs, schwannomas, heterotopic pancreatic rests, and leiomyomas [8]. Persistent and

Please cite this article as: Tan TJ, et al, Glomus tumour of the colon: dynamic contrast-enhanced CT findings and review of the literature, Clin Imaging (2015), http://dx.doi.org/10.1016/j.clinimag.2015.02.015

T.J. Tan et al. / Clinical Imaging xxx (2015) xxx–xxx

homogenous enhancement has also been described with gastroduodenal glomus tumours, although this pattern of enhancement was also observed with the other subepithelial lesions included in the study by Hur et al. [8]. To the authors’ knowledge, there has been no previous description of the dynamic contrast-enhanced CT imaging features of a colonic glomus tumour. A detailed search of the available literature revealed a brief description of a glomus tumour of the transverse mesocolon, which manifested as a hypodense mass in the region of the pancreatic tail on CT [9]. The CT scan in our study was performed primarily for the evaluation of renal cysts, and as such, the dynamic imaging phases were not entirely comparable to those described in prior dynamic contrast-enhanced CT imaging studies of gastric glomus tumours. Nonetheless, the imaging findings in our case suggest that when encountered, the peculiar finding of hepatic haemangioma-like enhancement of a subepithelial colonic lesion on dynamic contrastenhanced CT raises the possibility of a colonic glomus tumour prior to histological confirmation. A search of the available literature also reveals that this particular enhancement pattern seen with dynamic contrastenhanced CT has never been described with any other colonic subepithelial lesion. Inclusion of this entity as a differential diagnosis in the management of our case would have obviated the absolute necessity to proceed with immediate hemicolectomy, instead providing an opportunity for close surveillance using dynamic contrast CT imaging. However, the authors recognise that this is the first case report describing the haemangioma-like enhancement pattern of a colonic glomus tumour and, hence, no definitive conclusion can be made with respect to the sensitivity or specificity of this imaging finding at this juncture nor does this entirely exclude an atypical presentation of a more common diagnosis including a GIST or colonic carcinoma. Most gastrointestinal glomus tumours are clinically benign, although nuclear atypia and vascular invasion that are histological features usually associated with malignancy are common [1,5,10]. Folpe et al. studied 52 cases of atypical and malignant glomus tumours of all sites and proposed that malignant behaviour was associated with a combination of a deep location of the tumour and tumour size of over 2 cm, a mitotic count of N5 mitoses per high-power field and moderate-to-high nuclear grade, or the presence of atypical mitotic figures [11]. There is however a marked difference in the frequency of malignant behaviour between the deep glomus tumours of the peripheral soft tissues and those of the stomach. Five out of nine of such deep glomus tumours of the peripheral soft tissues in the study by Folpe et al. metastasized, whilst other studies have shown

3

that metastatic disease associated with gastric glomus tumours is rare [1,11–14]. It has thus been suggested that these two groups should not be equated in terms of prognostication despite being histologically and immunohistochemically comparable [1]. Although a case of multifocal gastric and colonic glomus tumours with liver metastases has been described [5], the other reported cases of colonic glomus tumours exhibited a benign behaviour [2–4]. Whilst the true metastatic potential of colonic glomus tumours remains uncertain given that so few of these lesions have been described, this is considered to be probably low. Acknowledgement This case was referred to the British Columbia Cancer Agency by Dr. J. Radigan and was also seen in consultation with Dr. A. Pollett, Dr. R. Riddell, and Dr. R. Kirsen. References [1] Miettinen M, Paal E, Lasota J, Sobin LH. Gastrointestinal glomus tumours: a clinicopathologic, immunohistochemical, and molecular genetic study of 32 cases. Am J Surg Pathol 2002;26:301–11. [2] Barua R. Glomus tumour of the colon: first reported case. Dis Colon Rectum 1988;31: 138–40. [3] Tuluc M, Horn A, Inniss S, Thomas R, Zhang PJ, Khurana JS. Case report: glomus tumour of the colon. Ann Clin Lab Sci 2005;35:97–9. [4] Oliphant R, Gardiner S, Reid R, McPeake J, Porteus C. Glomus tumour of the ascending colon. J Clin Pathol 2007;60(7):846. [5] Urbańczyk K, Stachura J, Papla B, Karcz D, Matłok M. Gastric solid glomus tumor and multiple glomangiomyomas of the large bowel with intravascular spread, multifocal perivascular proliferations and liver involvement. Pol J Pathol 2007;58(3):207–14. [6] Kim JK, Won JH, Cho YK, Kim MW, Joo HJ, Suh JH. Glomus tumour of the stomach: CT findings. Abdom Imaging 2001;26(3):303–5. [7] Chou HP, Tiu CM, Chen JD, Chou YH. Glomus tumour in the stomach. Abdom Imaging 2010;35:290–392. [8] Hur BY, Kim SH, Choi JY, Rha SE, Lee MW, Kim SY, et al. Gastroduodenal glomus tumors: differentiation from other subepithelial lesions based on dynamic contrastenhanced CT findings. AJR 2011;197:1351–9. [9] Harper L, Lavrand F, Le Bail B, Brun M, Ferron S, Oses P, et al. Glomus tumor of the mesocolon. J Pediatr Surg 2005;40:E37–8. [10] Haque S, Modlin IM, West AB. Multiple glomus tumors of the stomach with intravascular spread. Am J Surg Pathol 1992;16(3):291–9. [11] Folpe AL, Fanburg-Smith JC, Miettinen M, Weiss SW. Atypical and malignant glomus tumours: analysis of 52 cases, with a proposal for the reclassification of glomus tumours. Am J Surg Pathol 2001;25:1–12. [12] Appelman HD, Helwig EB. Glomus tumours of the stomach. Cancer 1969;23:203–13. [13] Lee HW, Lee JJ, Yang DH, Lee BH. A clinicopathologic study of glomus tumour of the stomach. J Clin Gastroenterol 2006;40(8):717–20. [14] Kang G, Park HJ, Kim JY, Choi D, Min BH, Lee JH, et al. Glomus tumour of the stomach: a clinicopathologic analysis of 10 cases and review of the literature. Gut Liver 2012;6(1):52–7.

Please cite this article as: Tan TJ, et al, Glomus tumour of the colon: dynamic contrast-enhanced CT findings and review of the literature, Clin Imaging (2015), http://dx.doi.org/10.1016/j.clinimag.2015.02.015

Glomus tumour of the colon: dynamic contrast-enhanced CT findings and review of the literature.

We describe the peculiar enhancement pattern of a subepithelial colonic glomus tumour on dynamic contrast-enhanced computed tomography. Discontinuous,...
1MB Sizes 0 Downloads 5 Views