Lipomatosis coli, a mimicker of familial polyposis

Lipomatosis coli, a mimicker of familial polyposis

Annals of Diagnostic Pathology 17 (2013) 210–213 Contents lists available at SciVerse ScienceDirect Annals of Diagnostic Pathology Case Reports Li...

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Annals of Diagnostic Pathology 17 (2013) 210–213

Contents lists available at SciVerse ScienceDirect

Annals of Diagnostic Pathology

Case Reports

Lipomatosis coli, a mimicker of familial polyposis Neda Zarrin-Khameh MD, MPH a,⁎, Eric M. Haas MD b, Jae Ro MD, PhD c, Michael J. Thrall MD c a b c

Department of Pathology, Baylor College of Medicine, Houston, Texas 77030, USA Minimally Invasive Colon and Rectal Surgery Fellowship, University of Texas Medical School at Houston, Houston, Texas 77054, USA Department of Pathology, The Methodist Hospital, Houston, Texas 77030, USA

a r t i c l e Keywords: Lipomatosis coli

i n f o

a b s t r a c t Multiple intestinal lipomas (lipomatous polyposis) are quite rare, and they can be quite challenging to diagnose because this condition may be clinically confused with familial adenomatous polyposis with a suggestive family history. Herein, we present a case of lipomatous polyposis that was presented with abdominal pain and, in colonoscopy, had more than 100 polyps. The patient was admitted for surgery with diagnosis of familial polyposis. Resected colon specimen had multiple polyps ranging from 0.1 to 1.5 cm. Microscopically, the polyps were composed of mature adipose tissue with normal overlying mucosa. There were also increased fat cells in the submucosa of the colon adjacent to the polyps. Lipomatous polyposis rarely occurs and can be confused with familial polyposis. Polypectomy is a simple and cost-effective procedure to help in diagnosis and prevent a major surgery. © 2013 Elsevier Inc. All rights reserved.

1. Introduction Although lipomas are reported as the second most common benign tumor of the colon, after adenomatous polyps [1], they are relatively rare [2]. In autopsy series, its incidence has been reported from 0.02% to 5.8% [3,4]. Intestinal lipomas are usually single, sessile, submucosal, and asymptomatic. Most are located in the right colon [5,6]. Diffuse lipomatosis of colon is extremely rare, and not many cases have been reported in the literature. Here, we describe a patient with colonic lipomatous polyposis who presented with abdominal pain and bloating. Colonoscopy demonstrated multiple polyps; therefore, clinically, the diagnosis of familial adenomatous polyposis was made. 2. Case A 50-year-old African American woman was admitted with chronic abdominal pain with occasional bloating. Colonoscopy demonstrated more than 100 polyps throughout the entire colon (Fig. 1). Multiple polyps were also seen in esophagus and stomach in the upper gastrointestinal endoscopy. The diagnosis of familial polyposis was made clinically. She underwent a laparoscopic handassisted total proctocoloectomy with ileoanal J pouch and diverting loop ileostomy, a retroperitoneal lymphadenectomy, and repair of small bowel enterorrhaphy.

⁎ Corresponding author. Tel.: +1 713 873 4460. E-mail address: [email protected] (N. Zarrin-Khameh). 1092-9134/$ – see front matter © 2013 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.anndiagpath.2011.08.007

The specimen was a 104 × 7 × 5 cm total proctocolectomy with an unremarkable 5 × 2.5 × 0.8 cm appendix. On opening, more than 100 submucosal sessile and pedunculated polyps ranging from 0.1 to 1.0 cm were present throughout the colon and rectum (Fig. 2). Most of the polyps were less than 0.3 cm. A 1.5 cm in diameter submucosal mass was present that on cross-section appeared to be a submucosal lipoma (Figs. 3 and 4). All of the large polyps (N0.5 cm) and representative sections of the smaller polyps were submitted for the microscopic evaluation. No other lesions were identified. Microscopic examination of the polyps shows diffuse thickening of the submucosa and marked increase of the submucosal fat (Fig. 5). The numerous smaller polyps had collections of mature adiopcytes with or without vascular core and/or lymphoid aggregates (Fig. 6). The adjacent colonic mucosa also had increased submucosal mature adipocytes (Fig. 7). No adenomatous or hyperplastic polyps were identified. No dysplasia or malignancy was seen in any of these polyps.

3. Discussion Gastrointestinal lipoma is the most common nonepithelial (mesenchymal) tumor of the large bowel [1,5] and can occur anywhere from esophagus to rectum. Large bowel is the most common site of involvement. Intestinal lipomas are usually single, asymptomatic, and more common in females [1,7-9]. Although they have been seen in all ages, they are found most frequently in patients in the fifth or sixth decades of life [8,10,11]. They can be sessile or pedunculated [7]. Most reports of multiple lipomas present 2 or 3 and rarely more than 4 lipomas [1,9,11,12]. Diffuse lipomatous polyposis

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Fig. 1. Endoscopic picture from colon shows many polyps. Most of the polyps are small.

of colon is extremely rare, and to our best knowledge, few cases have been reported in the recent English literature. Although lipomas are usually asymptomatic, the most common symptom is abdominal pain or discomfort [5,8,10]. In adults, they are the most common benign tumor in both small bowel and large bowel intussusceptions, accounting for 38% and 40% of the cases, respectively [13]. They can mimic a malignant tumor by chronic blood loss [10], obstruction, change in bowel habit, or obstruction [5]. Size and location are the most important factors in exhibiting clinical symptoms [5,9,10,14]. Most lipomas are submucosal, like our case, but can rarely be subserosal. Submucosal lipoma is composed of mature adipocytes beneath the muscularis mucosa and can be surrounded by a fibrous capsule derived from the submucosa. The base of them may be quite vascular. The surface epithelium may become ulcerated especially in large tumors and cause significant blood loss [15]. Lipomas are associated with 2 rare syndromes, familial multiple lipomatosis and Weber-Christian disease. Familial multiple lipomatosis, an autosomal dominant disease, is a rare condition that may become complicated by the development of visceral lipomas [16]. It is more common in males and is characterized by multiple and usually symmetric subcutaneous lipomas of various sizes [1]. WeberChristian disease is a rare febrile relapsing nonsuppurative panniculitis. Its etiology and pathogenesis are not well known. Association of lipomatous polyposis and epiploic lipomatosis or massive hypertrophy of the appendices epiploicae of the colon has been reported [17,18]. Lipomas appear as smooth, ovoid, and well-demarcated lesions on barium enema. Some consider the “squeeze sign” as a pathognomonic

sign of lipoma in which the shape of the mass on barium enema changes with external compression or peristalsis [19]. Large colonic lipoma can be well circumscribed on computed tomographic scans because of their fatty nature [20-22] and can be identified mainly on T1-weighted and fat-suppressing images [20]. Endoscopically, submucosal lipoma is smooth, yellowish circular polyp [23,24]. The top of the lesion may be red with gradual change to yellow at base or may have red dots speckled on the yellow surface [25,26]. On probing with a closed biopsy forceps, lipoma appears to be soft and pliable that can be easily indented. It quickly springs back to its original shape upon withdrawal of forceps; therefore, it is called “pillow” or “cushion” sign [24,25,27,28]. The overlying mucosa can be pulled away from submucosa to produce a tent-like appearance, the “tenting sign” [24,25,28]. Endoscopic ultrasonography and chromoendoscopy have been used to assess lipoma [28]. Endoscopic diagnosis of lipoma, however, can be challenging, especially if the lesion is ulcerated, necrotic, or firm [29,30]. Small, less than 2 cm, asymptomatic lipomas can be followed clinically because their growth rate is slow and the risk of complications such as bleeding and obstruction is low [31,32]. Although pedunculated lipomas, as large as 6 cm, can be safely removed endoscopically [33], endoscopic resection of lipomas larger than 2.5 cm has a significant risk of perforation [24,34]. Therefore, treatment of symptomatic, large, or multiple lipomas are surgical excision [9]. Surgery should be done to relieve obstruction or bleeding or secondarily to rule out the possibility of malignancy. Care must be taken to conserve as much of the intestine as is possible. A limited resection of the segment of intestine, which contains the largest concentration of lipomas, may be considered [12]. Subserosal lipomas

Fig. 2. Multiple sessile and pedunculated polyps in the colon. Many of the polyps are small and appear as small bumps (arrow, larger polyps; arrowheads, minute polyps).

Fig. 3. A 1.5 cm in diameter lipoma, the top is yellow, which is usually a clue for lipomatous polyp (arrow, lipoma; arrowheads, minute polyps).

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Fig. 4. Lipoma in cross section has fat in the submucosa. Fig. 6. Lipomatous polyp with vascular core and lymphoid aggregates (original magnification ×20).

Fig. 5. Polyp with marked increase of the submucosal fat. Scattered vessels are also seen (original magnification ×20).

may be removed with simple excision of tumor without opening the bowel lumen [11]. In conclusion, lipomatous polyposis of colon is extremely rare. Although it is usually asymptomatic, it can clinically simulate premalignant and malignant conditions; therefore, it can cause problem in diagnosis and treatment. Surgeons have occasionally resorted to extensive resection because of the difficulty of excluding malignancy on gross appraisal [6], especially in cases like ours with innumerable polyps seen throughout colon and rectum. Polypectomy is helpful in revealing the nature of these polyps and prevents extensive surgery. The presence of the increased fat in the submucosa is a curious finding in our case that has been reported in rare cases. References [1] Taylor BA, Wolff BG. Colonic lipomas. Report of two unusual cases and review of the Mayo Clinic experience, 1976-1985. Dis Colon Rectum 1987;30:888–93. [2] Siegal A, Witz M. Gastrointestinal lipoma and malignancies. J Surg Oncol 1991;47: 170–4. [3] Weinberg T, Feldman Sr M. Lipomas of the gastrointestinal tract. Am J Clin Pathol 1955;25:272–81. [4] Kirshbaum JD. Submucous lipomas of the intestinal tract, as a cause of intestinal obstruction. Am Surg 1935;101:734–9. [5] Rogy MA, Mirza D, Berlakovich G, et al. Submucous large-bowel lipomas— presentation and management. An 18-year study. Eur J Surg 1991;157:51–5.

Fig. 7. The adjacent colonic mucosa of the lipomatous polyp has increased mature adipocytes in submucosa (original magnification ×40).

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