CASE REPORT
The dilemma in management of desmoid tumour: A case report
 
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1
Department of Urology, Faculty of Medicine and Health Sciences, Universiti Putra Malaysia, Selangor, Malaysia
2
Department of Surgery, School of Medical Sciences, Hospital Universiti Sains Malaysia, Kubang Kerian, Kelantan, Malaysia
3
Department of Surgery, Faculty of Medicine and Health Sciences, Universiti Malaysia Sabah, Kota Kinabalu, Sabah, Malaysia
4
Colorectal Unit, Department of Surgery, Universiti Kebangsaan Malaysia, Cheras, Kuala Lumpur, Malaysia
CORRESPONDING AUTHOR
Firdaus Hayati   

Department of Surgery, Faculty of Medicine and Health Sciences, Universiti Malaysia Sabah, 88450, Kota Kinabalu, Sabah, Malaysia
Submission date: 2022-03-20
Final revision date: 2022-05-26
Acceptance date: 2022-05-30
Online publication date: 2022-09-20
 
 
KEYWORDS
TOPICS
ABSTRACT
Introduction:
Desmoid tumours are rare benign tumours. The tumours may develop sporadically or they may be linked to familial adenomatous polyposis (FAP) in Gardner’s syndrome.

Aim:
This article highlights a young lady with an intra-abdominal desmoid tumour that manifested as an acute abdomen and we discuss our management strategy.

Case study:
A 24-year-old lady with a known FAP who had undergone a panproctocolectomy with an ileal pouch-anal anastomosis and total thyroidectomy, later was complicated with acute abdomen for symptomatic desmoid tumour. Computed tomography of the abdomen showed a large intraperitoneal heterogenous mass with small bowel displacement and was confirmed by magnetic resonance imaging. An exploratory laparotomy and en-bloc resection of the tumour with an end ileostomy were performed.

Results and discussion:
Intra-abdominal desmoid tumours usually present as a painless slow-growing mass, however, in severe form, it can cause bowel ischemia, intestinal obstruction, or deterioration of function in the ileoanal anastomosis, among post total colectomy. Surgery is indicated upfront in specific clinical scenarios namely complications (occlusion, perforation, or bleeding) or major cosmetic issues.

Conclusions:
Desmoid tumour, despite being benign, is challenging to manage due to its compressing nature. As a key point, the diagnosis of a desmoid tumour should be suspected and followed up closely in patients with a previous history of FAP in combination with extracolonic manifestation.

ACKNOWLEDGEMENTS
We would like to thank everybody who has involved directly and indirectly in managing this case.
FUNDING
None declared.
CONFLICT OF INTEREST
The authors have declared no conflict of interest.
 
REFERENCES (13)
1.
Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F. Desmoid-type fibromatoses. WHO Classification of Tumours of Soft Tissue and Bone (IARC WHO Classification of Tumours). 4th ed. Lyon, France: IARC; 2013.
 
2.
Fallen T, Wilson M, Morlan B, Lindor NM. Desmoid tumors – a characterization of patients seen at Mayo Clinic 1976–1999. Fam Cancer. 2006;5(2):191–194. https://doi.org/10.1007/s10689....
 
3.
Sanchez-Mete L, Ferraresi V, Caterino M, et al. Desmoid tumors characteristics, clinical management, active surveillance, and description of our FAP case series. J Clin Med. 2020;9(12):4012. https://doi.org/10.3390%2Fjcm9....
 
4.
Sahid NA, Hayati F, Sani I, Zakaria Z. Extracolonic manifestation of familial adenomatous polyposis in an asymptomatic patient; literature review on current recommendation. GMJ. 2020;31:114–116. http://dx.doi.org/10.12996/gmj....
 
5.
Dinarvand P, Davaro EP, Doan JV, et al. Familial adenomatous polyposis syndrome: An update and review of extraintestinal manifestations. Arch Pathol Lab Med. 2019;143(11):1382–1398. https://doi.org/10.5858/arpa.2....
 
6.
Feng X, Milas M, O'Malley M, et al. Characteristics of benign and malignant thyroid disease in familial adenomatous polyposis patients and recommendations for disease surveillance. Thyroid. 2015;25(3):325–332. https://doi.org/10.1089/thy.20....
 
7.
Chan KH, Sellappan H, Hayati F, Azman A. Uterine rupture mimicking Wunderlich’s syndrome in pregnancy: An unfortunate case. Pol Ann Med. 2022;29(1):74–77. https://doi.org/10.29089/2021.....
 
8.
Garcia-Ortega DY, Martín-Tellez KS, Cuellar-Hubbe M, et al. Desmoid-type fibromatosis. Cancers (Basel). 2020;12(7):https://doi.org/10.3390%2Fcanc....
 
9.
Kasper B, Baumgarten C, Garcia J, Bonvalot S, Haas R, Haller F; Desmoid Working Group. An update on the management of sporadic desmoid-type fibromatosis: a European Consensus Initiative between Sarcoma PAtients EuroNet (SPAEN) and European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG). Ann Oncol. 2017;28(10):2399–2408. https://doi.org/10.1093/annonc....
 
10.
Desmoid Tumor Working Group. The management of desmoid tumours: A joint global consensus-based guideline approach for adult and paediatric patients. Eur J Cancer. 2020;127:96–107. https://doi.org/10.1016/j.ejca....
 
11.
Kaegi-Braun N, Mueller M, Schuetz P, Mueller B, Kutz A. Evaluation of nutritional support and in-hospital mortality in patients with malnutrition. JAMA Netw Open. 2021;4(1):2033433. https://doi.org/10.1001/jamane....
 
12.
Gomes F, Baumgartner A, Bounoure L, et al. Association of nutritional support with clinical outcomes among medical inpatients who are malnourished or at nutritional risk: An updated systematic review and meta-analysis. JAMA Netw Open. 2019;2(11):e1915138. https://doi.org/10.1001/jamane....
 
13.
Howard JH, Pollock RE. Intra-abdominal and abdominal wall desmoid fibromatosis. Oncol Ther. 2016;4(1):57–72. https://doi.org/10.1007/s40487....