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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 9  |  Issue : 1  |  Page : 14-16

Granular cell tumor of the colon presenting as profound weight loss


Department of Surgery, Beaumont Hospital, Troy; Department of Surgery, Henry Ford Macomb Hospital, Clinton Township, Michigan, USA

Date of Submission24-Jul-2019
Date of Decision27-Aug-2019
Date of Acceptance30-Sep-2019
Date of Web Publication28-Mar-2020

Correspondence Address:
Dr. Sarah E Diaz
4047 Woodmere Dr. Waterford, MI 48329
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/WJCS.WJCS_14_19

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  Abstract 


Granular cell tumors (GCTs) are typically benign tumors of neuronal origin that stain positive for S-100. They traditionally occur in the skin and subcutaneous tissue with gastrointestinal tumor sites, such as the colon, being rare. On colonoscopy, they appear as a well-circumscribed, yellow, submucosal lesion. Biopsy prior to endoscopic or surgical removal can be inconclusive due to the submucosal nature of the lesion. Although GCTs are rarely malignant, they can metastasize with a high mortality rate. Endoscopic resection has been described as an appropriate treatment for tumors up to 5 cm in diameter. We present the case of a 19-year-old male who experienced a 60-pound weight loss, which was initially attributed to depression. On clinical examination, a GCT was found in his ascending colon.

Keywords: Colonoscopy, endoscopic submucosal dissection, endoscopic submucosal excavation, granular cell tumor, gastrointestinal tract


How to cite this article:
Diaz SE, Hain JM, Hans SD. Granular cell tumor of the colon presenting as profound weight loss. World J Colorectal Surg 2020;9:14-6

How to cite this URL:
Diaz SE, Hain JM, Hans SD. Granular cell tumor of the colon presenting as profound weight loss. World J Colorectal Surg [serial online] 2020 [cited 2021 Sep 17];9:14-6. Available from: https://www.wjcs.us.com/text.asp?2020/9/1/14/281587




  Introduction Top


Abrikossofffirst described granular cell tumors (GCTs) in 1926 when reviewing a series of tongue tumors thought to be of skeletal muscle origin.[1],[2] GCTs are mesenchymal tumors consisting of cells with eosinophilic granules that appear like Schwann cells and typically stain positive for S-100 proteins.[3] They are most commonly found in the oral cavity, followed by the skin, subcutaneous tissue, breast, and respiratory tract, with only 8% being found in the gastrointestinal tract (GIT).[4] For GCTs found in the GIT, the most common location is the esophagus, followed by the ascending colon.[4],[5] In total, 1%–2% of GCTs are malignant and have a high propensity for metastasis, with a 40%–60% mortality rate.[3],[6] Here we present the case of a 19-year-old male who presented with weight loss attributed to depression and was found to have a GCT in the ascending colon.


  Case Report Top


A 19-year-old-male with past medical history significant for thalassemia presented to his primary care physician with the chief complaint of depression and unintentional weight loss of 60 pounds over 3 months. His laboratory investigations were within normal limits at presentation, except mild chronic anemia secondary to his thalassemia. Although he was treated for his depression, he was referred to a GI specialist 6 months later for persistent weight loss with associated nausea/nonbloody emesis.

The patient underwent an esophagogastroduodenoscopy (EGD) which showed a deformity of the gastric antrum secondary to questionable extrinsic compression with mild extrinsic deformity of the duodenal bulb associated with narrowing of the lumen. Computed tomography (CT) of the abdomen/pelvis was obtained after EGD, which showed a lobulated soft tissue-like mass in the proximal ascending colon with no stomach or duodenal pathology [Figure 1] and [Figure 2].
Figure 1: CT abdomen/pelvis showing soft tissue mass in ascending colon in the axial view

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Figure 2: CT abdomen/pelvis showing soft tissue mass in ascending colon in the coronal view

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A colonoscopy was performed revealing a 5-cm submucosal, nonobstructing mass in the proximal ascending colon involving half of the lumen circumference [Figure 3]. The mass was firm with no evidence of bleeding. Biopsy showed only mucosa with mild hyperplastic changes.
Figure 3: Picture from colonoscopy showing submucosal mass in ascending colon

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The patient underwent a laparoscopic, hand-assisted right hemicolectomy. The pathology exhibited a predominantly nested architecture of large polygonal cells with abundant eosinophilic, granular cytoplasm, and bland small nuclei [Figure 4]. S-100 protein was diffusely and strongly positive, confirming the diagnosis of GCT [Figure 5].
Figure 4: Granular cell tumor showing its predominantly nested architecture of large polygonal cells with abundant eosinophilic, granular cytoplasm, and bland small nuclei

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Figure 5: S-100 immunohistochemical stain confirming the diagnosis of granular cell tumor

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  Discussion Top


GCTs are rare soft tissue tumors. An institutional review of 410,000 surgical specimens over 32 years revealed the overall incidence of GCTs to be 0.03%.[3] Epidemiological data regarding GCTs are conflicting, likely because their incidence is low and most information are available as case reports. Some studies report an equal sex distribution, while others state that the tumor is more common in females.[6],[7] Most reported cases involve patients in their fourth, fifth, and sixth decades of life. Our patient therefore represents a deviation from previously reported data, given his age of 19 years.

Most gastrointestinal GCTs are asymptomatic and are found on endoscopic screening.[4] In total, 20% of gastrointestinal GCTs are found in the colon. In case of symptomatic GCTs, the most common symptoms are abdominal discomfort, hematochezia, diarrhea, and constipation.[1] The lesions are typically found in the submucosa and can appear yellow on colonoscopy. Colonic GCTs can be based in the subserosa and muscularis as well.[3]

The diagnosis of these tumors prior to surgical or endoscopic resection can be difficult. No characteristic findings of colonic GCTs appear on computerized tomography (CT) imaging. Endoscopic ultrasound (EUS) has emerged as a useful diagnostic test and classically shows a hypoechoic, homogenous submucosal lesion.[1] Given the submucosal nature of the tumor, endoscopic biopsy is unreliable.[6] In our case, the biopsy specimens obtained during colonoscopy revealed only mucosa with hyperplastic changes, prompting the need for surgical resection to establish a tissue diagnosis.

The surgical pathology in our case demonstrated classic findings of GCTs with plump cells containing small, bland nuclei and abundant granular cytoplasm, necessitating further immunophenotyping to confirm the diagnosis. While the vast majority of GCTs stain positive for S-100 proteins, some reported tumors that lack this property and are called non-neuronal GCTs.[1]

Importantly, albeit rare, GCTs have a malignant potential and the Fanburg-Smith histopathological criteria are used to confirm their malignancy. The criteria include the following histological features: mitotic activity > two mitoses/10 high power fields, increased nuclear to cytoplasmic ratio, pleomorphism, vesicular nuclei with large nucleoli, spindling, and necrosis.[3],[4] If three (or more) criteria are met, there is a high likelihood of malignancy.[4] Risk factors for malignancy have been identified as advanced patient age, tumor size of >4–5 cm, rapid recent growth, and infiltrative growth pattern.[1],[6] Given the malignant potential of GCTs, these tumors should be removed upon diagnosis; however, some sources discuss endoscopic surveillance as an option in a nonsurgical candidate.[1]

Endoscopic treatment of gastrointestinal GCTs has been described in the literature for tumors up to 5 cm. In a study by Chen et al., lesions confirmed to be ≤2 cm in maximum diameter and limited to the mucosa or submucosa on EUS were treated with endoscopic submucosal dissection, while lesions confirmed to be >2 cm in maximum diameter were selected for endoscopic submucosal excavation.[6] All patients completed follow-ups ranging from 11 months to 5 years with no recurrence or metastasis on colonoscopy or EUS.[6] Indications for formal surgical resection are tumor size of ≥5 cm, an infiltrative growth pattern invading the muscular layer, lymphovascular invasion, or aggressive disease.[6]


  Conclusion Top


GCTs of the colon are rare but typically benign. Because of their potential for malignancy, they should be resected either endoscopically or surgically depending on the size and location. Our case represents a unique presentation of GCT in an uncharacteristically young patient.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Barakat M, Kar AA, Pourshahid S, Ainechi S, Lee HJ, Othman M, et al. Gastrointestinal and biliary granular cell tumor: Diagnosis and management. Ann Gastroenterol Ann Gastroenterol 2018;31:439-47.  Back to cited text no. 1
    
2.
Abrikossoff AI. Über myome ausgehend von der quergestreiften willkürlichen muskulatur. Virchows Arch Path Anat. 1926;260:215–233.   Back to cited text no. 2
    
3.
Shrestha B, Khalid M, Gayam V, Mukhtar O, Thapa S, Mandal AK, et al. Metachronous granular cell tumor of the descending colon. Gastroenterology Res 2018;11:317-20.  Back to cited text no. 3
    
4.
Ramai D, Lai J, Changela K, Anand S. Colonic granular cell tumor: An endoscopic and histopathologic review with case illustration. Cureus 2018;10:e2015.  Back to cited text no. 4
    
5.
Leal T, Carvalho S, Costa JM. A granular cell tumor: An unusual colon polyp. Rev Esp Enferm Dig 2019;111:329.  Back to cited text no. 5
    
6.
Chen Y, Chen Y, Chen X, Chen L, Liang W. Colonic granular cell tumor: Report of 11 cases and management with review of the literature. Oncol Lett 2018;16:1419-24.  Back to cited text no. 6
    
7.
Garces-Descovich A, Callery MP, Anderson KR, Poylin VY, Mortele KJ. Synchronous granular cell tumors of the pancreas and cecum. Clin Imaging 2018;52:95-9.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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