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Table of Contents
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Year : 2021  |  Volume : 4  |  Issue : 4  |  Page : 752-755

A rare tumor of the large bowel in a young boy


1 Department of Pediatric Oncology, Homi Bhabha Cancer Hospital, Varanasi, Uttar Pradesh, India
2 Department of Pediatric Surgery, Homi Bhabha Cancer Hospital, Varanasi, Uttar Pradesh, India

Date of Submission11-Aug-2021
Date of Decision20-Oct-2021
Date of Acceptance30-Oct-2021
Date of Web Publication29-Dec-2021

Correspondence Address:
Shyam Srinivasan
Department of Pediatric Oncology, Homi Bhabha Cancer Hospital, Varanasi - 221 010, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/crst.crst_186_21

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How to cite this article:
Srinivasan S, Saha S. A rare tumor of the large bowel in a young boy. Cancer Res Stat Treat 2021;4:752-5

How to cite this URL:
Srinivasan S, Saha S. A rare tumor of the large bowel in a young boy. Cancer Res Stat Treat [serial online] 2021 [cited 2022 Jan 21];4:752-5. Available from: https://www.crstonline.com/text.asp?2021/4/4/752/334178




  Case History Top


A 10-year-old boy presented with features of subacute intestinal obstruction with a visible lump in the left upper abdomen for 20 days. On examination, a firm mass was palpable in the left hypochondriac region with no guarding or rigidity. A contrast-enhanced computed tomography scan of the abdomen showed a large, intra-abdominal mass measuring 10.3 cm × 7.6 cm with the epicenter at the transverse mesocolon, with luminal narrowing of the distal transverse colon, splenic flexure, and aneurysmal dilation of the proximal colon [Figure 1].
Figure 1: (a) Axial and (b) Coronal contrast enhanced computer tomography images demonstrating a 8.7 cm × 5.3 cm × 10 cm mass with epicenter (red star) in the transverse mesocolon

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Subsequently, the patient underwent an ultrasonography-guided biopsy. Histopathological examination revealed spindle-shaped cells in a collagenous stroma [Figure 2]a. What is the diagnosis?
Figure 2: (a) Histology showing spindled myofibroblasts within a collagenous stroma. Immunohistochemistry showing nuclear positivity for beta-catenin (b) and focal desmin positivity (c)

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What should be done next? Once you have finalized your answer, turn to the next page to read on.


  Differential Diagnosis and Further Management Top


In this case, considering the young age of the patient, the large abdominal mass, and the relatively short duration of symptoms, the differential diagnoses included soft-tissue sarcoma, non-Hodgkin lymphoma, and extraskeletal Ewing sarcoma.[1] The histology was not compatible with hematolymphoid malignancies, and immunohistochemistry was negative for CD45, FLI1, and MyoD1 but focally positive for smooth muscle actin, desmin, and β-catenin [Figure 2]b and [Figure 2]c. The diagnosis was consistent with desmoid-type fibromatosis.

During the process of evaluation, the patient was admitted to the hospital and conservatively managed with nasogastric tube insertion to decompress the bowel while being supplemented with total parenteral nutrition. In view of the persistent obstructive symptoms, he underwent surgery after counseling the family. Intraoperatively, the mass was seen to arise from the transverse mesocolon, involving the wall of the distal transverse colon, splenic flexure, proximal descending colon, and part of the greater curvature of the stomach [Figure 3]. The abdomen was opened through the left thoracoabdominal approach, and the mass was excised along with the involved part of the large bowel and stomach wall. The gastric wall was closed primarily, and a double-barreled colostomy was performed; 6 months later, a side-to-side colocolic anastomosis was performed following re-laparotomy. Postoperatively, the histopathology report showed the colonic and stomach wall margins to be microscopically involved by the tumor. The patient was started on adjuvant therapy once he was deemed medically fit. Injection methotrexate and vinblastine along with oral tamoxifen were given once a week for 18 weeks, followed by oral tamoxifen and imatinib for 36 weeks. The change in therapy after 18 weeks of injectables was made as the patient was unable to travel to the hospital in view of the COVID-19 pandemic. He tolerated the therapy with no adverse effects and is now on routine surveillance by clinical evaluation and ultrasonography of the abdomen performed once every 3 months. He continues to be disease free 24 months following the initial diagnosis.
Figure 3: (a) Schematic diagram of the mass before excision. (b) Schematic diagram of the operating field, after excision of the mass. (c) Resected specimen

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  Discussion Top


Desmoid-type fibromatosis is a benign but aggressive tumor of mesenchymal origin, with a high predilection for local recurrence. The disease is rare with an approximate incidence of four to six cases per million persons.[2],[3] Intra-abdominal fibromatosis is an uncommon subtype, seen in about 8%–15% of patients with fibromatosis making it even more rare. [Table 1] summarizes the cases of intra-abdominal fibromatosis in children and adolescents reported in the literature.
Table 1: Literature review of cases of children and adolescents with intra-abdominal fibromatosis

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In our review of the literature, we observed that most cases involved either the stomach or the small bowel mesentery; the large bowel was involved only in two patients.[8],[11] In fact, due to the frequent occurrence in the small bowel mesentery, the term “mesenteric fibromatosis” is often used for this entity.[15] Prior abdominal surgery and familial adenomatous polyposis, which are known to be predisposing factors for intra-abdominal fibromatosis, were not seen in our patient.[16]

Considering that intra-abdominal fibromatosis is known for spontaneous regression and in view of the surgery-related morbidities, the European Organisation for Research and Treatment of Cancer guidelines recommend a wait-and-watch strategy before performing a surgery.[17],[18],[19],[20] On the other hand, some of these tumors due to their proximity to vital organs are known to cause aneurysm and bowel obstruction making the initial strategy of observation difficult.[21],[22] In our case, the patient was symptomatic with a large mass causing aneurysmal dilation of the colon and subacute intestinal obstruction, prompting us to opt for a surgical intervention rather than a conservative approach.

Although the need for a wide surgical margin has been the norm while operating on patients with fibromatosis, it has been shown that surgical margins do not consistently correlate with recurrence.[23] Our literature review showed that two out of three patients with a positive margin experienced recurrence of disease. In our patient, in view of the positive surgical margin, we opted for adjuvant medical therapy, and the patient was offered a combination of chemotherapy (methotrexate and vinblastine), antihormonal therapy (tamoxifen), and targeted therapy (imatinib), all of which have shown efficacy in fibromatosis.[20],[24]


  Conclusion Top


Intra-abdominal desmoid-type fibromatosis is a rare entity, even more so in the pediatric population. Hence, a high degree of suspicion is required to make a diagnosis. Asymptomatic patients should be managed conservatively, with surgery being reserved for patients who present with signs of intestinal obstruction. Due to the rarity of these tumors, several aspects including the surgical extent, role of negative tumor margins, and appropriate medical management continue to be controversial.

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 initial s 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.



 
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2.
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Kloeze J, van Veen M. Spontaneous regression of a sporadic intra-abdominal located desmoid-type fibromatosis. J Surg Case Rep 2019;2019:rjz037.  Back to cited text no. 17
    
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Bonvalot S, Eldweny H, Haddad V, Rimareix F, Missenard G, Oberlin O, et al. Extra-abdominal primary fibromatosis: Aggressive management could be avoided in a subgroup of patients. Eur J Surg Oncol 2008;34:462-8.  Back to cited text no. 18
    
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Bonvalot S, Ternès N, Fiore M, Bitsakou G, Colombo C, Honoré C, et al. Spontaneous regression of primary abdominal wall desmoid tumors: More common than previously thought. Ann Surg Oncol 2013;20:4096-102.  Back to cited text no. 19
    
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Koyama R, Maeda Y, Minagawa N, Shinohara T. Rapidly growing desmoid-type fibromatosis of the mesentery of the small intestine after distal gastrectomy for gastric cancer. Case Rep Gastroenterol 2020;14:197-205.  Back to cited text no. 21
    
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Aggarwal G, Shukla S, Maheshwari A, Mathur R. Desmoid tumour: A rare etiology of intestinal obstruction. Pan Afr Med J 2015;22:158.  Back to cited text no. 22
    
23.
Salas S, Dufresne A, Bui B, Blay JY, Terrier P, Ranchere-Vince D, et al. Prognostic factors influencing progression-free survival determined from a series of sporadic desmoid tumors: A wait-and-see policy according to tumor presentation. J Clin Oncol 2011;29:3553-8.  Back to cited text no. 23
    
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