ARTICLE IN PRESS
A 21-year-old woman with right lower quadrant abdominal pain Eric J. Finnesgard, BA, Benjamin Zendejas, MD, MSc, and Stephanie F. Heller, MD, Rochester, MN
From the Department of Surgery, Mayo Clinic, Rochester, MN
A 21-YEAR-OLD WOMAN presented to the emergency department with a 5-day history of periumbilical abdominal pain, which gradually migrated toward her right lower quadrant (RLQ). Associated symptoms included nausea, emesis, and constipation. She had a medical history significant for chronic nausea, anorexia, and marijuana abuse. Her obstetric background consisted of 3 gestations and 1 natural spontaneous vaginal delivery. At presentation, she was afebrile and hemodynamically stable. Laboratory studies were remarkable for a serum leukocyte count of 15.8 3 109 cells/L (normal 3.5–10.5 3 109 cells/L) and a neutrophil count of 13.67 3 109 cells/L (normal 1.7–7.0 3 109 cells/L). Physical examination revealed mild RLQ tenderness to deep palpation with no guarding or other signs of peritoneal irritation. Ultrasound of the abdomen failed to visualize the appendix and showed no evidence of RLQ inflammation or fluid collection. Abdominal computed tomography demonstrated a small amount of free fluid in the pelvis and findings suggestive of appendiceal calcifications (Fig 1). With the working diagnosis of an appendiceal mucocele, the patient was taken to the operating room for planned laparoscopic appendectomy. However, laparoscopy revealed a benignappearing appendix and a mass in the terminal ileum, 5 cm proximal to the ileocecal valve (Fig 2). No enlarged or suspicious-appearing regional lymphadenopathy was appreciated. However, because of concerns for this mass representing a carcinoid, a gastrointestinal stromal tumor, or a small bowel adenocarcinoma, an open ileocecectomy with stapled side-to-side, functional
Accepted for publication February 25, 2016. Reprint requests: Eric J. Finnesgard, BA, Department of Surgery, Mayo Clinic, Rochester, MN 55905. E-mail: finnesgard.
[email protected]. Surgery 2016;j:j-j. 0039-6060/$ - see front matter Ó 2016 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.surg.2016.02.031
Fig 1. Coronal image of a contrast-enhanced abdominal computed tomographic scan demonstrating a calcifying focus within the right lower quadrant of the abdomen.
end-to-end, ileocolonic anastomosis was performed. Pathologic evaluation of the ileal mass revealed a calcifying fibrous pseudotumor, measuring 3.5 3 2 3 1.5 cm, confined to a subserosal location. The patient’s postoperative course was uneventful. Her chronic nausea and anorexia persisted. DISCUSSION First reported in 1988 by Rosenthal and AbdulKarim,1 calcifying fibrous pseudotumors (CFPs) are benign lesions of an unknown etiology and pathogenesis. CFPs have a characteristic morphology of dense hyalinized collagenous tissue containing dystrophic calcifications and inflammatory SURGERY 1
ARTICLE IN PRESS 2 Finnesgard, Zendejas, and Heller
Surgery j 2016
This case was particularly challenging to diagnose preoperatively given a presenting symptomology suggestive of acute appendicitis and an anatomic location that made the CFP difficult to differentiate from an appendiceal mucocele on abdominal computed tomography. While CFPs are rare, this case demonstrates that they can occur within the walls of the ileum and should be considered as a differential diagnosis when appendiceal mucocele is suspect or when a calcified mass is seen in the RLQ.
Fig 2. Laparoscopic view of the right lower abdominal quadrant depicting a mass within the terminal ileum. (Color version of this figure is available online.)
infiltrate.2 Although widely considered to be nonmalignant, CFP recurrences have been reported.3 CFPs have most commonly been identified within the neck, limbs, and membranes of the pericardium, peritoneum, and pleura. There have also been reports in the literature of CFPs in the stomach and liver.4,5 To our knowledge, this is the first reported case of a CFP located in the ileum.
REFERENCES 1. Rosenthal NS, Abdul-Karim FW. Childhood fibrous tumor with psammoma bodies. Clinicopathologic features in two cases. Arch Pathol Lab Med 1988;112:798-800. 2. Fetsch JF, Montgomery EA, Meis JM. Calcifying fibrous pseudotumor. Am J Surg Pathol 1993;17:502-8. 3. Maeda A, Kawabata K, Kusuzaki K. Rapid recurrence of calcifying fibrous pseudotumor (a case report). Anticancer Res 2002;22:1795-7. 4. Nobili C, Rosso E, Oussoultzoglou E, Casnedi S, Jaeck D, Bachellier P. Image of the month: hepatic calcifying fibrous pseudotumor. Arch Surg 2011;146:237-8. 5. Zhang H, Jin Z, Ding S. Gastric calcifying fibrous tumor: a case of suspected immunoglobulin G4-related gastric disease. Saudi J Gastroenterol 2015;21:423-6.