|Year : 2016 | Volume
| Issue : 4 | Page : 1318-1320
The diagnosis of mesenteric fibromatosis: A 90-month five patients case report
Peixin Li, Zhongtao Zhang, Shengqi Qin, Jianshe Li
Department of General Surgery, Medical and Health Center, Beijing Friendship Hospital, Capital Medical University, Beijing, China
|Date of Web Publication||7-Feb-2017|
Department of General Surgery, Medical and Health Center, Beijing Friendship Hospital, Capital Medical University, 95 Yong'an Road, Xicheng, Beijing, 100050
Source of Support: None, Conflict of Interest: None
Mesenteric fibromatosis (MF) is a rare tumor (2–4 cases per 1 million people annually) with few presented features. In this case study, we reported five MF patients, one of whom suffered a recurrence. Patients received renogram, colonoscopy, cystoscopy, and gastrointestinal examinations. Histology and immunohistochemistry evaluations were performed after the surgical resection. Hormone levels were measured before and after the surgery. It was found that clinical imaging readily confirmed an abnormal mass but was unable to distinguish MF from other tumors. Histology and immunohistochemistry were definitive diagnoses because the tissue was vimentin ± β–catenin ± CD117−/CD34−. Furthermore, the patient who suffered a recurrence showed an elevated estrogen level. A 6-month postoperative administration of letrozole drove the estrogen down to normal level. Our study showed that vimentin, β–catenin, CD117, and CD34 were the markers for MF whereas medical imaging, and estrogen level could be used for the complimentary purpose.
Keywords: β–catenin, estrogen, mesenteric fibromatosis, vimentin
|How to cite this article:|
Li P, Zhang Z, Qin S, Li J. The diagnosis of mesenteric fibromatosis: A 90-month five patients case report. J Can Res Ther 2016;12:1318-20
| > Introduction|| |
Fibromatosis is a rare type of benign tumor that is locally invasive but not metastatic. It occurs primarily in the abdominal wall and in extremely rare cases, can originate from the mesentery. Mesenteric fibromatosis (MF) is particularly challenging for diagnosis because of its lack of specific presenting features. Previous case reports showed that pyrexia, ileus, and abdominal mass were most common but not definite features., In our case study, our reported five patients with MF, one of whom suffered a recurrence 1 year after the surgical resection.
| > Case Report|| |
Five MF patients were identified at Beijing Friendship Hospital. Informed consents were obtained from all patients. Their symptoms and surgical history can be found in [Table 1].
Blood concentrations of carcinoembryonic antigen, alpha-fetoprotein, CA125, and CA199 in all patients were measured before and after the surgical resection. A preoperative increased level of CA199 was observed but it fell to normal levels after the surgery. All the other protein concentrations remained in the physiological ranges throughout the surgery. It was noteworthy that the estradiol concentration of patient 5a reached 199.20 mol/L in a follow-up examination 1 year after the primary surgical resection. The estradiol concentration remained abnormally high in an examination 2 weeks after the secondary resection. However, the concentration fell down to normal range after a 6-month administration of letrozole.
Ultrasound examinations of patients 1–5a showed tumor masses without clear borders or blood flow [Figure 1]a. The masses in patients one and two were cystic-solid whereas those in patients 3, 4, and 5a solid. According to computed tomography (CT) scans, tumor masses in patients 1–4 showed clear borders [Figure 1]b. In addition, tumor masses in patient one, and four were solid whereas those in patient two and three cystic-solid. Furthermore, patient one showed an enlargement of sigmoid colon whereas the intestine and bladder of patient four depressed and dislodged. Cystic gas was observed in patient two. Plain scanning yielded a CT value between 20 and 41 Hu whereas an enhanced scanning between 34 and 91 Hu [Figure 1]c. According to magnetic resonance imaging, patient four showed a weak T1W1 but strong T2W1 [Figure 1]d. Gastrointestinal examinations of patient 1–4 showed a space-occupying lesion to varying degrees in pelvic cavity (patient one) or abdomen (patient 2–4). Sigmoid colon and colon were depressed and dislodged in patient one whereas intestinum tenue and sigmoid colon depressed and dislodged in patient four. According to colonoscopy and cystoscopy, patient 5a's bladder and the opening of appendix got depressed. Renogram confirmed a partial blockage of ureters in patient four.
|Figure 1: Diagnostic imaging results. (a) B-mode ultrasound; (b) computed tomography (plain scanning); (c) computed tomography (enhanced signal); (d) magnetic resonance imaging|
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An extended surgical resection was performed in all patients in that the tumor mass had invaded the intestinal tissues. To ensure the tumor mass was completely removed, a section of intestine (>10 cm) from both side of the tumor mass was also removed. Histology confirmed that the tissue at the edge of resected intestine was free of tumor masses. A follow-up examination 1 year after the surgery showed that patient 5a suffered a recurrence of MF at the surgical site. The tumor mass was removed in a secondary surgical resection. The surgical details and follow-up record can be found in [Table 2].
| > Discussion|| |
MF is a rare subtype of fibromatosis (2–4 cases per 1 million people annually), which usually occurs in the small intestine but occasionally in colon. It was reported that familial adenomatous polyposis (FAP) might significantly increase the likelihood of fibromatosis. The correlation between fibromatosis and FAP is further pronounced in patients with Gardner's syndrome. Epidemiological study showed that MF could occur in patients aged between 1 and 60 with males accounting for 42%. The histological signatures of fibromatosis include spindle cells with a homogeneous morphology, collagen deposition and invading borders. In contrast, gastrointestinal stromal tumor (GIST) features spindle cells or epithelioid cells with a heterogeneous morphology and myxoid or hyalinized stroma.
Clinically, MF lacks unique presenting features. Abdominal mass, pain and/or nephrological disorders are the most frequent complaints by MF patients. According to medical imaging results, MF can be categorized into four types based on their morphology, i.e., heterogeneous solid mass, homogeneous solid mass, cystic mass, and pervasive mass. Cystic features are usually present in masses of a large volume. If gas is observed in the mass, it suggests that the tumor has invaded into the lumen of the intestine.
At present histology and immunohistochemistry of tumor masses harvested during the surgery are the most reliable diagnostic means. Cells in the tumor mass showed a spindle-like morphology with significant collagen fibers [Figure 2]. Lymphocytes could also be observed in large tumor masses. MF tissue was vimentin ± β–catenin ± CD117−/CD34−, which can be used as the biomarkers for diagnostic purposes., Moreover, β–catenin has been used to distinguish MF from GIST or sclerosing mesenteritis. Interestingly, clinical data showed that females were more prone than males to MF because of estrogen as a growth factor. Previous studies suggested that an increased intake of estrogen might lead to the occurrence of MF. On the other hand, menopause often predates the peak of MF growth. In addition, MF tumor masses were found to feature a higher concentration of estradiol receptors. All these clinical data suggested a correlation between estrogen and MF, although the underlying mechanism remains elusive.
|Figure 2: Histology of mesenteric fibromatosis tissues retrieved during the surgery|
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Surgical resection remains the gold standard treatment for MF. Given that MF frequently invades adjacent tissues, an extended resection is recommended. However, it must be noted that postoperative chemotherapy remains controversial. Moreover, tamoxifen, sulindac, and cyclo-oxygenase-2 inhibitor can also be used to treat MF.
In summary, we reported a five-patient case study on MF with a comprehensive diagnostic and treatment results. Our findings suggested that estrogen level, vimentin, and β–catenin may be useful biomarkers for MF. In addition, an extended resection is recommended to minimize the chance of recurrence.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]
[Table 1], [Table 2]