Carcinoma of unknown primary origin presenting as adult intussusception

May 25, 2007
Nicole Schimke, DO

Surgical Rounds, September 2006, Volume 0, Issue 0

Nicole Schimke, Resident in General Surgery, Department of Surgery, Mercy Medical Center? Des Moines, Des Moines, IA; Dennis Whitmer, Attending Surgeon, Department of Surgery, Mercy Medical Center?Des Moines, Des Moines, IA

Nicole Schimke, DO

Resident in General

Surgery

Department of Surgery

Mercy Medical Center-

Des Moines

Des Moines, IA

Dennis Whitmer, DO

Attending Surgeon

Department of Surgery

Mercy Medical Center-

Des Moines

Des Moines, IA

Only 5% of intussusceptions occur in adults.1-12 Symptoms of adult intussusception mimic those of bowel obstruction and may be acute or chronic, as opposed to the acute onset typically observed in children.2,8,11,13-17 Reports identify a discrete lesion in up to 95% of cases,8,10-13,15-18 with malignancy accounting for up to 48% of small bowel lesions11; thus, surgical exploration is mandatory.7,8,10-12,18?We report a case of adult intussusception that was found to be caused by a carcinoma of unknown primary origin.

Case report

A 62-year-old white woman with a 50-pack-year history of smoking was admitted to the hospital for evaluation of intermittent gastrointestinal bleeding and periumbilical pain that worsened over 24 hours. Her medical history was significant for T1 N0 M0 adenocarcinoma of the left upper lobe of the lung, which was diagnosed 7 years earlier. She had undergone primary definitive radiation therapy and had complete resolution of the adenocarcinoma. Her medical history also included hyperlipidemia, chronic obstructive pulmonary disease, hypertension, congestive heart failure, and a myocardial infarction (MI). The patient's daily medications included digoxin, enalapril, furosemide, atorvastatin, lorazepam, pantoprazole, diltiazem, nebulizer treatments, and a multivitamin. She was intolerant to beta-blocking agents and allergic to penicillin and metoprolol. Her surgical history included repair of an abdominal stab wound when she was an adolescent, appendectomy, cholecystectomy, umbilical hernia repair, and coronary artery stent placement after her MI. She had no family history of colon cancer or colonic polyps. ?

The patient's initial symptoms began 4 months earlier and manifested as right-sided flank pain. Evaluation at that time included a computed tomography (CT) scan, which revealed a 7-cm, right adrenal mass that appeared to be metastatic. Needle biopsy confirmed a hemorrhagic lesion but showed no evidence of a neoplasm. The patient's pain resolved but recurred 3 months later as left-sided flank pain. CT scans at that time showed the right adrenal mass, which did not change in size, and a new 5.6-cm left adrenal mass with hemorrhage. A positron emission tomography (PET) scan was subsequently obtained and showed significant uptake in the bilateral adrenal glands, the right pelvis anterior to the sacrum, and the left lower quadrant, raising concern of malignancy. There was no uptake within the lungs.

Shortly after the PET scan, the patient began having episodes of profound anemia and required several blood transfusions. She noticed ongoing dark-colored stools, which were found to be heme-positive. The patient underwent gastrointestinal evaluation at this time since she was also experiencing nausea, dry heaves, diminished appetite, and fatigue, and reported losing 10 pounds over the previous 5 months. Gastroscopy was unremarkable, and colonoscopy revealed only a rectal hyperplastic polyp, which did not explain the bloody stools. She was scheduled for capsule endoscopy to further evaluate the small bowel. Upon presenting for the study, she reported worsening abdominal pain and was subsequently admitted to the hospital.

During this hospitalization, the patient was found to have normocytic, normochromic anemia with a hematocrit of 25%. Coagulation parameters were within normal limits. Mild elevations were seen in alkaline phosphatase and liver enzymes, and urinalysis showed a trace of blood. Physical examination revealed a soft, nondistended abdomen with tenderness across the lower abdomen on palpation. No peritoneal signs, masses, or hernias were noted. A contrast-enhanced CT scan of the abdomen and pelvis showed bilateral adrenal gland masses (Figure 1). There were multiple dilated small bowel loops exhibiting high-grade obstruction, and the target appearance of intussusception was observed within the right hemipelvis (Figure 2). A soft tissue nodule measuring 1.5-cm was also associated with the intussuception (Figure 3). No free air was visible.

Preoperative reduction of the intussusception was not attempted. The patient was advised to undergo an exploratory laparotomy, and cardiac clearance was obtained. With the patient under general anesthesia, a mass was palpated in the right lower quadrant. A midline incision was made, and, upon entering the peritoneum, a large amount of ascitic fluid was encountered. Once adequate retraction was obtained, the intra-abdominal contents were examined. No abnormalities were palpable in the liver or colon. The adrenal glands were enlarged, which was consistent with the CT findings, but no attempts at resection were made. At this point, the terminal ileum was identified and the small bowel was run proximally. Intussusception was observed in the distal small bowel and was reduced to reveal serosal dimpling with an intraluminal mass. The small bowel was resected using side-to-side stapled anastomosis, and the specimen was sent to pathology. Gross examination revealed that the specimen measured 8.4 cm in length, with margins measuring 3.7 cm and 2.9 cm in diameter. The lumen contained a 3.2 x 2.7 x 0.8-cm, hemorrhagic, polypoid mass, which was located 2.8 cm from the dilated margin. Frozen-section evaluation indicated the lesion was probably metastatic, most likely adenocarcinoma or melanoma. Two additional masses were encountered within the small bowel, which were also resected and sent for permanent-section pathology. Hand-sewn anastomoses were performed at these sites. Inspection at the ligament of Treitz revealed a large fixed mass within the proximal jejunum in immediate proximity to the superior mesenteric artery, aorta, and inferior vena cava. The mass could not be resected because of its difficult location; thus, an enterotomy was made to remove the bulk of this hemorrhagic lesion and the base was carefully fulgurated. Three units of blood were given intraoperatively. The abdomen was then closed, and the patient was transferred to the surgical intensive care unit (ICU).

She was extubated that evening and transferred out of the ICU on postoperative day 1, at which time parenteral nutrition was initiated. Her postoperative recovery was complicated by a pelvic abscess, which was successfully drained.

Final pathology evaluations of all specimens returned a diagnosis of Grade 3 of 4 metastatic undifferentiated carcinoma, solid type, of large round cell tumor origin. High-grade cellular atypia and frequent mitoses were noted. The associated lymph nodes were negative for tumor, and all resected specimens had clear margins. Metastatic origin of the tumor is supported by Figure 4. Tumor cells are visible throughout the submucosa while normal architecture of small bowel mucosa is maintained. Figure 5 supports a diagnosis of carcinoma because the tumor exhibits cytoplasmic staining for epithelial membrane antigen (EMA), cytokeratin and CAM 5.2. Positive cytokeratin staining helps to exclude the possibility of lymphoma or melanoma. The staining patterns of additional immunohistochemical stains helped exclude malignant melanoma and lymphoma and adrenal gland origin. Upon subsequent review, the histology was not consistent with the patient's previous lung adenocarcinoma; thus, the primary source of the carcinoma was unknown.

In the months following surgery, medical oncology played a large role in managing the patient's care. She continued to have gastrointestinal blood loss, requiring weekly blood transfusions. She was again evaluated with upper and lower endoscopy but no specific source of bleeding was identified. The patient was not a candidate for radiation therapy and repeatedly refused to undergo palliative chemotherapy. She received home hospice care until approximately 3.5 months postoperatively, when she finally decided to pursue a trial of palliative chemotherapy. ACT scan at that time showed slight enlargement of the adrenal masses, interim development of pulmonary metastases, and a mass within the proximal jejunum. Attenuated doses of carboplatin and paclitaxel were administered, but she did not tolerate this well and was readmitted to the hospital with a hematocrit of 15.3%. Once medically stable with a hematocrit of 33.8% and no active bleeding, she was discharged to home hospice care. ?

Discussion

This case brings up several clinical points when assessing a patient for bowel obstruction. There are many causes of bowel obstruction in adults, with intussusception accounting for approximately 1%.1,2,8,13,18,19?Our patient experienced intermittent obstructive symptoms over a 4-month period that ultimately resulted in high-grade obstruction. Because of her history of lung cancer and the interim development of bilateral hemorrhagic adrenal masses, a malignant etiology for this obstruction was suspected. The subsequent finding on CT scan of intussusception with high-grade small bowel obstruction further obviated surgical intervention.

When adult intussusception is diagnosed, a pathologic lesion is identified in 86% to 95% of cases.8,10-16,18 The lead point for the intussusception can include benign causes such as adhesions, Meckel's diverticulum, and lipoma8,10,11,18,19; however, the incidence of malignancy can be high, especially in patients with a history of cancer. Begos and colleagues evaluated enteric lesions causing intussusception in their patients and found malignancies in only 14%.10 In comparison, Barussaud and associates found 37% of such lesions to be malignant,18 Nagorney and colleagues reported 46% to be malignant,8 and Azar and Berger reported 48% to be malignant.11 Metastases were responsible for a significant number of such masses.

A 1999 study by Eisen and associates evaluated 27 intussusception cases, of which 7 patients had a previous malignancy.13 Intussusception of the small bowel occurred in 81% of these patients, and all cases were metastatic. The most common primary source was melanoma, followed by lymphoma, lung carcinoma, and sarcoma. Azar and Berger found a similar pattern with regard to the primary source.11?Of the 58 patients they evaluated, 44 had enteric lesions causing intussusception. Forty-eight percent of these were malignant, 96% of which were metastases. The most common primary cancer in their study was melanoma. Other primary sources included lymphoma, sarcoma, and squamous cell carcinoma.

The unknown source of gastrointestinal bleeding in our patient appeared intraoperatively to be secondary to the friable, hemorrhagic tumor we found in the proximal jejunum. Studies show that many adults with intussusception have guaiac-positive stool or hematochezia.4,7-9,11,18 This finding corresponds with the 29% incidence reported by Azar and Berger11; however, it is impossible to determine whether the guaiac-positive stools in our patient were exclusively due to the jejunal mass or if the intussusception also contributed.

We did not attempt to reduce the intussusception before surgery because we knew from the CT scan that it had an enteric location, and, due to the patient's history of lung cancer, we suspected malignancy. Opinions regarding the reduction of intussusception vary in the literature. Data in the 1981 article by Nagorney and associates revealed only 33% of enteric intussusceptions are malignant and they are more commonly metastases, rather than primary small bowel malignancies. The authors suggested that reduction of an intussusception already metastatic to the small bowel would do little to affect the disease course in terms of tumor spread.8 Reduction is preferable if the lesion is metastatic because it allows bowel length to be preserved; however, if a patient's history supports the potential for a primary malignancy, reduction is contraindicated.8?

In 1997, Azar and Berger published a review article that recommends avoiding reduction for all intussusceptions, except those which occur postoperatively and cause bowel obstruction.11 The latter presentation often has its etiology at the site of a suture line or an adhesion and may be reduced intraoperatively, whereas the potential for malignancy with other presentations makes primary resection the most appropriate option.11?

Similarly, Weilbaecher and associates and Barussaud and colleagues recommend resection without reduction due to the potential for tumor seeding12,18; however, Barussaud and colleagues do provide an exception for cases where the disease process may require further bowel resections. Peutz-Jeghers disease was ?cited as a situation where reduction of the intussusception followed by limited resection would be appropriate.18

Conclusion

Adult intussusception is an unusual finding with several distinct features that differentiate it from childhood intussusception. The presenting symptoms, potential comorbidities of the patient, etiology of the intussusception, and proper management are quite different from what is generally experienced in pediatric cases. Whether it is caused by a primary or metastatic malignancy or a benign lesion, the surgeon will need to decide whether reduction is warranted or if primary resection is the best option for the patient. As a general rule, surgeons should have a high index of suspicion for malignancy in cases of adult intussusception and surgical exploration is required.

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