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Desmoid tumor of Meckel's diverticulum presenting as intestinal obstruction: A rare case report with literature review


 Department of General Surgery, Jawaharlal Nehru Medical College, Wardha, Maharashtra, India

Date of Submission07-May-2020
Date of Decision16-Jul-2020
Date of Acceptance15-Sep-2020
Date of Web Publication17-Jul-2021

Correspondence Address:
Aditya Vijay Mundada,
Department of General Surgery, Jawaharlal Nehru Medical College, Sawangi, Wardha, Maharashtra
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcrt.JCRT_582_20

 > Abstract 


Meckel's diverticulum (MD) is among the many known congenital malformations of intestine. It is seen in approximately 2% of the population. Desmoid tumors are unusual. They are unique, well-differentiated, and fast-growing musculoaponeurotic fibromatosis tumors, contemplated as Grade 1 fibro sarcoma. They offer exclusive management challenges to surgeons. The most common presentation of desmoid tumors is of painless masses with sluggish growth. Rarely, they may present as intestinal obstruction leading to surgical emergency. We present the only case in history till date along with review of the relevant literature of a 65-year-old gentleman who presented to us with features of intestinal obstruction who successfully underwent elective segmental resection of ileum containing MD with its desmoid tumor. A multimodality approach is needed to tackle such kind of diseases with a team comprising oncosurgeons, oncophysicians, and radiation oncologist to design a standard treatment protocol.

Keywords: Desmoid tumor, intestinal obstruction, Meckel's diverticulum, segmental resection



How to cite this URL:
Mundada AV, Tote D, Zade A. Desmoid tumor of Meckel's diverticulum presenting as intestinal obstruction: A rare case report with literature review. J Can Res Ther [Epub ahead of print] [cited 2021 Jul 29]. Available from: https://www.cancerjournal.net/preprintarticle.asp?id=321712




 > Introduction Top


One of the most common malformations of small bowel is a Meckel's diverticulum (MD). MD is accounted as a real diverticulum of the small bowel, usually present within 2 feet from the ileocecal junction. It is seen due to failure of regression and obliteration of the vitello-intestinal duct. MD is found in 2% of the general population.[1] Only 4% of all the cases with MD will be symptomatic or complicated. Symptomatic MD manifests as intestinal obstruction in 36.5% of cases, intussusceptions in 13.7% of cases, diverticulitis in 12.7% of cases, and gastrointestinal bleeding in 11.8% of cases.[1] The mean length of MD was found to be 2.99 cm, with a range of 1–11 cm in a study comprising 227 patients with MD.[2] It has been reported that there are only 3.2%[1] cases that present with a tumor in the MD mostly benign.[3] Almost all of the malignant tumors of MD are recognized either during operative procedure or disclosed on histopathological examination. The literature concerning malignant MD includes only case reports and case series. The cardinal malignancies of MDs include lymphomas, metastases, adenocarcinomas, gastrointestinal stromal tumors (GISTs), and neuroendocrine tumors with the prevalence increasing in that order.[1],[4],[5]

Considering all the neoplasms, desmoid tumors (DTs) make up only 0.03% of it. Moreover, DT forms no more than 3% of all soft tissue tumors.[6] DT has an incidence of 2–4 cases per million per year.[7] DTs are mostly seen in the range of 30–40 years with a minimal female predisposition. They are not uncommon in persons with familial adenomatous polyposis (FAP), where the risk is 850 times more than in the general population with only a few cases occurring as sporadic.[8] The origin of the desmoids tumors which are also known as aggressive fibromatosis is from the mesenchymal cell lines. DTs are benign in nature and carry no metastatic potential. DTs may locally infiltrate the surrounding organs and may cause organ dysfunction of the adjacent organs by pressure effect. Desmoid tumors are categorized as extra-abdominal, abdominal, or intra-abdominal.[9] Intra-abdominally, the most common sites of genesis are the mesentery, omentum, and retroperitoneum with small intestine considered as a more rare origin. Desmoids are among the most common mesenteric tumors and perhaps exhibit a varied clinical course in contrast to small bowel desmoids. Certainly, desmoids of the small bowel are extremely rare with desmoid of MD not even reported in the literature. These pathologies offer exclusive management challenges to surgeons. There has not been a single case reported till date of desmoid tumor arising from MD.[10]

Here, we present the only case in history till date along with review of the relevant literature of a patient who presented to us with features of intestinal obstruction who successfully underwent elective segmental resection of ileum containing MD with its desmoid tumor.


 > Case Report Top


In February 2020, a 65-year-old gentleman presented with features of constipation, pain in abdomen, gradually increasing abdominal distension, and vomiting for 5 days to the casualty of a rural hospital in Central India. The patient denied any history of significant weight loss, fever, and bladder complaints. The patient gave no history of smoking, alcoholism, and tobacco chewing. His medical history and personal history were not significant. He denied any family history of any malignancy, Crohn's disease, or ulcerative colitis. Physical examination revealed distended abdomen with absence of guarding and rigidity. Bowel sounds were exaggerated and loud. A palpable lump was present in the hypo gastric region. It was firm in consistency, nontender, mobile in horizontal direction. Ultrasonography of the abdomen revealed solid cystic mass in the infraumbilical region with minimal vascularity on Doppler. Magnetic resonance imaging (MRI) with contrast to the abdomen and pelvis was suggestive of a heterogeneously enhancing solid mass lesion in pelvis superior to the bladder measuring approximately 7 cm × 11 cm × 13.5 cm. The lesion was seen extending superiorly up to the aortic bifurcation and inferiorly it was indenting over the superior surface of the bladder with maintained fat plane with displacement of the bowel loops. The mass appeared heterogeneously hyperintense on T2WI and had low intensity on T1WI. The mass showed post contrast enhancement. There was another well-defined soft tissue signal intensity at the anterior part of right lumbar region measuring 5 cm × 3.8 cm, which was separate from the bowel loops [Figure 1]. The small bowel loops appeared mildly dilated with diameter of small bowel loop up to 2.8 cm, suggesting subacute small intestinal obstruction with fecal loaded large bowel.
Figure 1: Magnetic resonance imaging showing intra-abdominal mass arising from the bowel

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The patient underwent exploratory laprotomy and mass resection after taking written informed consent. A standard midline laprotomy incision was taken from 4 cm above the umbilicus to pubic symphysis. There was notable distension of the small bowel with fecal loaded colon. There was a highly vascular mass, which was seen to be arising from a diverticulum present on ileum's anti mesenteric border approximately 2 feet from the ileocecal valve, most likely to be MD [Figure 2]. The tumor was free from the urinary bladder, colon, and major vessels without any presence of adhesions or infiltrations. Rest of the abdominal cavity did not exhibit any signs of distant spread. There was another similar mass, which was seen arising from the greater omentum [Figure 3]. A segmental resection with primary anastomosis was performed along with excision of mass from the omentum with adequate margins. The final histopathology report was suggestive of desmoid tumor arising from the MD with clear margins. Post-operatively, the patient developed hospital acquired pneumonia with septicemia leading to multiorgan dysfunction syndrome. The patient died due to respiratory failure 5 days after the procedure.
Figure 2: Intra-operative photo showing mass arising from a diverticulum present on the ileum's antimesenteric border approximately 2 feet from the ileocecal valve

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Figure 3: Intra-operative photo showing a mass arising from the omentum

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 > Discussion Top


MD is among the many known congenital malformations of the intestine. MD is found in approximately 2% of the population.[11],[12] MD is present on the distal ileum's anti-mesententeric border approximately 2 feet ahead of the ileocecal junction. It results from incomplete obliteration of the omphalomesentric duct. Men and women are equally affected. The mucosa present in MD is heterotrophic in nature, the most common being gastric mucosa found in 50% of cases. Colonic and pancreatic mucosa (5%) constitutes the rest. Most of the MDs are found coincidentally during the post-mortem studies, barium studies or operative procedures. The most common clinical manifestations of the MDs are gastrointestinal hemorrhage (from the ulcer bed in the ileum close to MD due to continuous secretion of acid by gastric mucosa), intestinal obstruction, and inflammation of diverticulum. There is a 0.5-3.2% chance of finding a tumor in the MD.[13],[14],[15] Majority of the tumors are benign tumors such as lipomas, leiomyomas, and angiomas. GIST, adenocarcinomas, carcinoid tumors, and sarcomas are some of the malignant tumors found within the MD.[3] There has not been a single case in the literature where a desmoid tumor is seen arising from the MD. Our case is the first and the only study, which is being reported as desmoid tumor arising MD presenting as intestinal obstruction.

Desmoid tumors are unusual. They are unique, well-differentiated, and fast-growing musculo-aponeurotic fibromatosis tumors, contemplated as grade 1 fibrosarcoma.[16] Desmoid tumors show a peculiar growth pattern, which is slow and incessant with infiltration of contiguous structures. These tumors are locally invasive but carry no metastatic potential.[17] Studies have shown considerable interconnection with Gardner syndrome, FAP, mutations in beta-catenin genes, and mutations in the adenomatous polyposis coli (APC) gene. There is a speculation that loss of function mutations in the APC gene causes reproduction of beta-catenin, which, in turn, establishes suitable environments for musculoaponeurotic growth. Abdominal trauma and high estrogen states are the other known risk factors.[18],[19],[20] Desmoids may potentially arise from the abdominal wall, the mesentery, or the retroperitoneum. Extra-abdominal sites of desmoids include the shoulder, the thigh, the gluteal region, or the trunk.[21] Intra-abdominal desmoid tumors are rarer still and are often associated with FAP (of which our patient had no family history). Intra-abdominal desmoid tumors which are sporadic in origin constitute only 5%, rest being associated with FAP.[22] Desmoids can affect people of any age groups but are predominantly seen in the age group of 30-40 years. Women have a slightly higher predilection than men.[21],[23] Grossly, desmoid tumors are typically circumscribed lesions with irregular or infiltrating borders in some cases. The cut surface shows white and coarsely trabeculated interior, indistinguishable from scar tissue. Desmoids commonly attain a size of more than 5 cm when seen on imaging or laprotomy with few of them attaining a size of more than a 15 cm in rare cases. Tumor multiplicity is found in 10-15% of cases.[24] On microscope, DTs are made up of fibroblastic cells having stellate or spindle shape being lodged in stroma made up of collagen. The stellate cells are positive for vimentin, smooth muscle actin, and nuclear beta-catenin staining.[25],[26] The radiographic appearance of DTs is unpredictable and relies on the content of fibrosis and collagen, its fibroblastic proliferation, and its vascularity. DTs have varying echogenicity, appearing as hyperechoic, isoechoic, or hypoechoic masses with well-defined and smooth margins.[23] Computed tomography appearance of desmoids tumor is of a well-circumscribed homogeneous isodense or hyperdense mass with a few of them having heterogeneous masses with infiltrative margins. Desmoid tumors may show postcontrast enhancement.[21] MRI shows desmoids as masses of low attenuation on T1WI with variable attenuation on T2WI corresponding to the muscle.[23] Few studies have also shown desmoids appearing from the diaphragm, organ of pancreas and gastroesophageal junction.[18],[19],[20]

The most common presentation of DTs is of painless masses with sluggish growth.[27] The mesentery of small bowel and in some cases ileocolic mesentery, gastrocolic ligaments, and omentum gives rise to mesenteric desmoids.[28] Little do we know about the desmoids arising from the small bowel or with a fistulous connection to the intestine.[10] Singh and colleagues published a case report of a 44-year-old male who underwent a segmental resection for a huge desmoid located in the ileum.[29] Intra-abdominal desmoids do not cause any symptoms until and unless they attain a huge size and cause pressure effects on the neighboring viscera. This mass effect can compress intestines, major vessels, and ureters causing bowel obstruction and bowel ischemia, leading to perforation peritonitis and hydronephrosis respectively.[30] Chang et al. published a case report of a 50-year-old male with gross peritoneal contamination and shock eventually found to have a huge mass appearing from the ileum's anti-mesenteric border.[31] While intestinal obstruction has been reported as a latent consequence of an intra-abdominal desmoid, there have been only 8 cases reported in the literature,[30] our being the 9th case [Table 1].
Table 1: Desmoid tumors presenting as intestinal obstruction

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Surgeons should be able to discriminate small bowel desmoids from the mesenteric desmoids, of which the incidence of latter being more as an intra-abdominal pathology. The prognosis of the patients with small bowel desmoids[10] and precisely of desmoids arising from the MD is obscure due to its singularity. A multimodality approach is needed to tackle such kind of diseases with a team comprising of oncosurgeons, oncophysicians, and radiation oncologist.[10] The accessibility to an ideal treatment protocol for intra-abdominal desmoids is unavailable. The present guidelines for soft tissue sarcomas framed by the National Comprehensive Cancer Network (NCCN) are not specific for small bowel desmoids.[35] Ideally in a non-emergency setting, biopsy of the mass should be taken to confirm the diagnosis and rule out more common intra-abdominal pathologies. The resectable cases should be distinguished from the nonresectable ones as complete surgical resection (R0 resection) remains the gold standard treatment for intra-abdominal desmoids tumors.[10]

The treatment options for unresectable or residual disease include radiation and systemic therapy. Radiation therapy is generally reserved for huge, advanced unresectable tumors and R1 resections, but is not an ideal option for small bowel desmoids due to its toxic effects on adjacent viscera.[10] The systemic therapy made for intra-abdominal DTs include nonsteroidal anti-inflammatory drugs, anti-hormonal therapy, and chemotherapy.[36] Their use is limited because of its unreliable efficacy. Recently, novel drugs such as imatinib, sunitinib, bevacizumab, or sorafenib for IAF are being advocated. Mace et al. in their study suggested a modern therapeutic approach with imatinib mesylate at a dose of 400 mg twice daily for desmoids tumors.[37]

Follow up of the patient should be done every three to six months for first two to three years post-operatively with a suitable imaging modality like CT or MRI. If the patient remains disease free during this term, then the follow up should be every 12 monthly thereafter.[10]


 > Conclusion Top


Desmoid tumor arising from the MD is one of its kinds as not a single case has been reported till date. It presenting as intestinal obstruction is a very rare possibility and should be considered among the differential diagnosis of pelvic masses. The standard management of small bowel desmoids including that arising from the MD based on literature review is segmental resection of intestine containing the mass with primary anastomosis along with adjuvant chemo radiotherapy.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 > References Top

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    Figures

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    Tables

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