Abdominal Pain

Desmoid tumors are rare, benign, slow-growing, fibroblastic neoplasms that originate from musculoaponeurotic and fascial structures throughout the body. These tumors do not have the ability to metastasize, but they are locally aggressive, with a high rate of recurrence even after surgical resection. Desmoids can cause severe clinical sequelae, including mortality due to infiltration of nearby organs. In this case report, we discuss a patient who presented with right lower quadrant abdominal pain and extraluminal colonic obstruction secondary to a desmoid tumor.

Case Report

A 46-year-old woman with a past medical history of rheumatoid arthritis, who was on weekly methotrexate therapy, presented to her primary care physician com­plaining chiefly of right lower quadrant abdominal pain of 4 weeks’ duration. She described the pain as a progres­sive, dull ache lasting over the preceding 6 months, with intermittent episodes of sharp, severe pain. The pain occasionally radiated to the back and left lower quadrant, but it generally remained in the right lower quadrant. The patient experienced some associated nausea but denied having any associated vomiting, constipation, diarrhea, hematochezia, or weight loss. She had tried taking over- the-counter analgesics and, eventually, simethicone and hyoscyamine (Levsin, Schwarz Pharma), as prescribed by her primary care physician. At a follow-up visit 6 months later, the patient reported no relief with the above therapies and was experiencing progressive symp­toms of dull pain and pressure in the right lower quad­rant. Her past medical history was significant for a ton- sillectomy as a child and for rheumatoid arthritis, which had been diagnosed in 2005 and for which she was receiv­ing weekly methotrexate injections (20 mg/wk). Besides methotrexate, she was also taking folic acid. Her family history was significant for gallstones and kidney stones, but she did not have any history of autoimmune diseases, inflammatory bowel disease, or malignancy. She did not use tobacco, alcohol, or recreational drugs. pharmacy generic viagra

Figure 1. Computed tomography scan

Figure 1. Computed tomography scan revealing a right lower quadrant mass adjacent to the ascending colon (arrow).

On examination, the patient was afebrile with stable vital signs. She was alert and in no acute distress. Her conjunctivae were anicteric, her mucus membranes were moist, and her oropharynx was clear. She had a normal thyroid examination and no cervical or supraclavicular lymphadenopathy. Her lungs were clear to auscultation, and her heart showed regular rhythm with no murmurs, rubs, or gallops. Abdominal examination revealed a nor­mal-appearing abdomen, which was nontender, nondis- tended without any peritoneal signs of rebound or guard­ing, and without appreciable hepatosplenomegaly. Bowel sounds were present in all 4 quadrants. The remainder of her examination was unremarkable.

Given the progressive nature of her symptoms, a computed tomography (CT) scan of the abdomen and pelvis was ordered with oral and intravenous contrast. The CT scan revealed a 6.4-cm X 3.1-cm soft-tissue density adjacent to the ascending colon (Figure 1). Circumfer­ential wall thickening of the adjacent ascending colon was seen, as was a left adnexal mass measuring 4.0 cm X 3.1 cm, which was consistent with an ovarian cyst. Otherwise, the remainder of the examination was unre­markable. The radiologist proposed that the right lower quadrant mass represented an ascending colon neoplasm with adjacent lymph node enlargement or a primary extracolonic mass. Colonoscopy was thus recommended and revealed a 5-mm polyp in the transverse colon and animpassable segment in the mid- to distal ascending colon, corresponding to the location of the abnormality on the patient’s CT scan (Figure 2). The endoscopist noted a twist in the bowel at this segment, which raised concerns for a possible chronic cecal volvulus. A barium enema study was ordered to further evaluate these findings.
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Figure 2. Impassable segment

Figure 2. Impassable segment of the ascending colon seen during colonoscopy.

Despite multiple maneuvers by the barium techni­cian, contrast did not reach the cecum. The barium enema study confirmed that this obstruction was second­ary to a very irregular and narrowed colon in the region of the hepatic flexure and ascending colon, with associated dilation of the proximal bowel (Figure 3). Based upon the study, it was not possible to determine whether this finding was caused by an intraluminal growth or extrin­sic compression. Given these findings, the patient was referred for surgical evaluation for definitive diagnosis and possible resection.

The patient underwent incisional biopsy of the mass and subsequent right hemicolectomy with ileocecectomy, appendectomy, and associated removal of pericolonic fat and mesentery, which included the mass in question. The gross specimen included a 6.2-cm X 6-cm X 3.7-cm well-defined, tan white, firm, trabecular, and slightly whorled mass that was completely free of surgical mar­gins (Figure 4). The mass grossly extended to involve the serosal and muscularis layer of the right colon but did not grossly involve the mucosa. On histologic evaluation, the mass consisted of fibroblastic proliferation with generally low cellularity, bland cytology, and no appreciable mitotic activity. The mass was also densely collagenized. Thetumor cells stained positive for desmin and beta-catenin but negative for CD34, CD117, S-100 protein, and keratin AE1/3, which was consistent with a diagnosis of desmoid tumor (fibromatosis).
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Figure 3. Barium enema study

Figure 3. Barium enema study revealing obstruction of the ascending colon (arrow) caused by either an intraluminal or extracolonic mass.

The patient tolerated the procedure without compli­cations. At a follow-up visit 3 months later, she reportedcomplete resolution of her right lower quadrant pain. Plans for further follow-up included serial clinical and radiographic evaluation (CT scans) every 6 months.

Figure 4. Desmoid tumor with fibroblastic proliferation

Figure 4. Desmoid tumor with fibroblastic proliferation, low cellularity, and dense collagenization (hematoxylin and eosin stain).