|Year : 2018 | Volume
| Issue : 3 | Page : 104-107
Gastrointestinal stromal tumor presenting as a recurrent mesenteric cyst: A rare and important entity
Chandan Roy Choudhury
Department of General Surgery, Medical College and Hospital, Kolkata, West Bengal, India
|Date of Web Publication||10-Sep-2018|
Dr. Chandan Roy Choudhury
Department of General Surgery, Medical College and Hospital, 88 College Street, Kolkata - 700 073, West Bengal
Source of Support: None, Conflict of Interest: None
Gastrointestinal stromal tumors (GISTs) arise from interstitial cells of Cajal identified in 1893 by Santiago Ramon y Cajal and present with a myriad of symptoms including gastrointestinal bleeding, obstruction, and lump. However, cystic presentation of GIST is a rare presentation and few cases have been reported in literature till date. Our patient presented as a recurrent abdominal cyst with provisional diagnosis of the mesenteric cyst. On surgery, a huge cyst adhered to the mesentery was found adhered to a part of the jejunum which required resection and anastomosis. Postoperative biopsy revealed it to be a case of GIST with cystic degeneration due to hemorrhage and necrosis. The author tries to find out whether preoperatively any features are predictive of this rare diagnosis to enable R0resection for good prognosis.
Keywords: Gastrointestinal stromal tumor, mesenteric cyst, recurrent
|How to cite this article:|
Choudhury CR. Gastrointestinal stromal tumor presenting as a recurrent mesenteric cyst: A rare and important entity. Saudi Surg J 2018;6:104-7
| Introduction|| |
Gastrointestinal stromal tumors (GISTs) were initially thought to be a smooth-muscle tumor arising from the gut wall and were historically classified as leiomyoma or leiomyosarcoma till 1980 when improved histochemical techniques and electron microscopy studies made a diagnosis of GIST possible. These tumors arise from interstitial cells of Cajal identified in 1893 by Santiago Ramon y Cajal and named them interstitial due to its location between nerve endings and smooth-muscle cells in the gut wall.
GISTs are uncommon tumors; accounts for 0.1% to 3% of all gastrointestinal (GI) neoplasm. The most common site of involvement is stomach (60%–70%), small intestine (20%–30%), and rarely at extra-GI sites such as omentum and mesentery called as extra-GI stromal tumor (EGIST). The incidence of GIST is 10–20 cases per million population and mostly seen in patients older than 50 years, accounting for <1% of all GI tumors. Most of the tumors are seen in the stomach and rarely in the mesentery.
Reports of GIST presenting as a mesenteric mass has been reported. Due to the rarity and different modes of presentation of GISTs, the preoperative diagnosis is extremely difficult. A total of 25% of GISTs are found incidentally during imaging or surgery for other disorders.
However, GIST presenting as a cystic lesion in the mesentery is a very rare entity and very few cases have been reported in literature till date. I do hereby report a case of GIST masquerading as recurrent mesenteric cyst in a female patient and it is a very rare entity.
| Case Report|| |
A 56-year-old female patient attended our outpatient department with complaints of a recurrent abdominal swelling [Figure 1] which was operated 1 year back. Previous biopsy reports available showed it to be a case of mesenteric cyst of size 8 cm and histopathology report mentions it to be a mesenteric cyst with occasional spindle cells the exact nature of which was not understood. A contrast-enhanced computed tomography (CECT) scan of the abdomen revealed it to be a case of cystic lesion of the abdomen with irregular thickening and peripheral enhancement [Figure 2]. On exploration, a huge mesenteric cyst [Figure 3] was found which was highly vascular and on careful dissection, it was found to be attached to jejunum close to duodenojejunal junction [Figure 4]. Resection of small part of attached jejunum with the cyst with jejuno jejunal anastomosis performed. The cyst weighing 2.32 Kg was removed and cut section revealed red hemorrhagic fluid [Figure 5]. Histopathology reported it to be an epitheloid variety of GIST with wide areas of hemorrhage and necrosis with mitotic figure >5–50/hpf and immunohistochemistry shows CD117 positivity consistent with GIST of high malignant potential. The patient is now treated with imatinib therapy 400 mg daily and is on follow-up.
|Figure 2: Contrast-enhanced computed tomography showing irregular thickening of wall with peripheral enhancement|
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| Discussion|| |
GI stromal tumors are defined as spindle, epithelioid, or occasionally pleomorphic mesenchymal tumors of GI tract expressing the kit protein detected at immunohistochemistry. GISTs are uncommon tumors; accounts for 0.1% to 3% of all GI neoplasm. They are predominantly found in the stomach (60%–70%) followed by the small intestine (20%–25%), colorectal (5%), and esophagus (<5%). In up to 10% of cases, GIST arises from outside bowel wall (e.g., omentum, mesentery, peritoneum, gallbladder, and liver) so called as EGIST.
The importance of this entity is for various reasons. First, GIST must be considered in the differential diagnosis of any abdominal cyst, especially if it arises from the upper abdomen.
GIST can present as a predominantly cystic lesion and needs to be considered in the differential diagnosis of cystic lesions of the duodenum also. Local resection is an attractive option in select cases and avoids a Pancreatico-duodenectomy. A cystic EGIST in omentum falls in the differential diagnoses of omental cystic lesions which include omental cyst, mesenteric teratoma, cystic mesothelioma, cystic spindle cell tumors, pseudomyxoma peritonei, pancreatic pseudocyst, and complicated ascites. Therefore, proper diagnosis of cystic GIST is of great importance in respect to different behavior, management, and complications in comparison to its mimickers and GI counterparts. Naitoh et al., in a case report, described an exophytic GIST with remarkable cystic changes and speculated that as the tumor was large and exophytic, it may have caused congestion, edema, and hemorrhage, giving rise to a multiseptate appearance.
They concluded that GIST should be considered when cystic tumors of unknown origin are found in the abdomen.
The vast majority of GISTs are solid tumors and cystic change is uncommon. There is no significant difference between cystic and solid GISTs in terms of the treatment and prognosis.
For peritoneal cystic lesions, the diagnosis of GIST is not initially considered. Mesenteric GIST, is a rare entity, can be clinically mistaken for mesenteric cyst and can present as acute abdomen due to tumor necrosis. In cases with short duration of abdominal mass arising from mesentery, one has to keep in mind the possibility of mesenteric GIST. Large mass (>5 cm), incomplete resection margins, mitosis >5 HPF, and central necrosis are all poor indices for survival and result in high recurrences. In our patient, we also wrongly diagnosed the cystic mesenteric GIST to be mesenteric cyst.
| Conclusion|| |
After a thorough literature search, the author has concluded certain important take-home lessons:
- Cystic presentations of GIST is a rare entity, but surgeons must keep in the differential Diagnosis of cysts of abdomen, especially mid- and upper abdomen
- This variety of presentation usually represents the aggressive variety of GIST and cystic change occurs due to hemorrhage and necrosis
- Rapid development or quick recurrence (as in our case) of a cyst with a short history might be the clue to diagnosis
- Irregular wall thickening with peripheral contrast enhancement on CECT might be a hint to diagnosis
- Achieving an R0 resection is necessary for long-term survival, so surgeons must be aware of its entity although preoperative diagnosis of such situations may not be easy.
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
Conflicts of interest
There are no conflicts of interest.
| References|| |
Sinha R, Verma R, Kong A. Mesenteric gastrointestinal stromal tumor in a patient with neurofibromatosis. AJR Am J Roentgenol 2004;183:1844-6.
Suryawanshi KH, Patil TB, Damle RP, Dravid NV, Surana A. Gastrointestinal stromal tumour of small intestine presenting as a mesenteric mass. J Clin Diagn Res 2014;8:FD14-6.
Al-Thani H, El-Menyar A, Rasul KI, Al-Sulaiti M, El-Mabrok J, Hajaji K, et al.
Clinical presentation, management and outcomes of gastrointestinal stromal tumors. Int J Surg 2014;12:1127-33.
Burkill GJ, Badran M, Al-Muderis O, Meirion Thomas J, Judson IR, Fisher C, et al.
Malignant gastrointestinal stromal tumor: Distribution, imaging features, and pattern of metastatic spread. Radiology 2003;226:527-32.
Miettinen M, Lasota J. Gastrointestinal stromal tumors – Definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis. Virchows Arch 2001;438:1-2.
Agaimy A, Wünsch PH. Gastrointestinal stromal tumours: A regular origin in the muscularis propria, but an extremely diverse gross presentation. A review of 200 cases to critically re-evaluate the concept of so-called extra-gastrointestinal stromal tumours. Langenbecks Arch Surg 2006;391:322-9.
DeMatteo RP, Lewis JJ, Leung D, Mudan SS, Woodruff JM, Brennan MF, et al.
Two hundred gastrointestinal stromal tumors: Recurrence patterns and prognostic factors for survival. Ann Surg 2000;231:51-8.
Kumar A, Jakhmola CK, Chauhan SS, Singh A. Atypical presentation of gastrointestinal stromal tumor masquerading as a large duodenal cyst: A case report. Int J Surg Case Rep 2015;9:123-6.
Sun KK, Xu S, Chen J, Liu G, Shen X, Wu X, et al.
Atypical presentation of a gastric stromal tumor masquerading as a giant intraabdominal cyst: A case report. Oncol Lett 2016;12:3018-20.
Monabati A, Safavi M, Solhjoo F. Extragastrointestinal stromal tumor presenting as omental cyst. J Gastrointest Surg 2016;20:1275-7.
Naitoh I, Okayama Y, Hirai M, Kitajima Y, Hayashi K, Okamoto T, et al.
Exophytic pedunculated gastrointestinal stromal tumor with remarkable cystic change. J Gastroenterol 2003;38:1181-4.
Joensuu H, Vehtari A, Riihimäki J, Nishida T, Steigen SE, Brabec P, et al.
Risk of recurrence of gastrointestinal stromal tumour after surgery: An analysis of pooled population-based cohorts. Lancet Oncol 2012;13:265-74.
Ramani AS, Huske G, Noronha FP. A rare case of mesenteric gastrointestinal stromal tumor presenting as acute abdomen. Niger J Surg 2017;23:71-2.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]