Volume 4 | March 2017
Image | Pancreas/Biliary

Solid-Cystic Pancreatic Tail Desmoid Tumor with Beta-Catenin Positivity

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Harsh D. Patel, MD1, Neil R. Desai, MD2, Aniruddh Som, MD3, Shinil K. Shah, DO2,4, and Nirav C. Thosani, MD3

1Department of Internal Medicine, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX
2Department of Surgery, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX
3Department of Gastroenterology, Hepatology and Nutrition, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX
4Department of Surgery, Division of Minimally Invasive and Elective General Surgery, Memorial Hermann Hospital, Texas Medical Center, Houston, TX
5Ertan Digestive Disease Center, Memorial Hermann Hospital, Texas Medical Center, Houston, TX

ACG Case Rep J 2017;4:e40. http://dx.doi.org/10.14309/crj.2017.40. Published online: March 15, 2017.

Case Report

A 19-year-old Hispanic woman with a history of polycystic ovarian syndrome presented with worsening abdominal pain, nausea, and vomiting. She had stable vitals and lower/suprapubic abdominal pain on exam, as well as a positive urinalysis. She was initially admitted for treatment of a urinary tract infection. An abdominal computed tomography scan, performed due to persistent abdominal pain, revealed a cystic lesion anterior to the pancreatic tail adjoining the splenic flexure and colonic diverticulosis, which was concerning for phlegmon formation (Figure 1).

Figure 1. Abdominal computed tomography scan of a cystic lesion anterior to the pancreatic tail adjoining the splenic flexure and colonic diverticulosis.

Magnetic resonance imaging showed a 4.1-cm pancreatic tail soft tissue lesion with a 3.4-cm unilocular nonenhancing cystic lesion. Carcinoembryonic antigen and CA19-9 antigen levels were normal. The patient underwent endoscopic ultrasound with fine-needle aspiration of the pancreatic lesion, and histopathology showed uniform spindle-cell proliferation (Figure 2). A colonoscopy, performed to exclude colonic involvement, had unremarkable gross findings. Laparoscopic distal pancreatectomy, splenectomy, partial gastrectomy, and partial segmental colectomy were performed. Gross examination revealed an infiltrative poorly circumscribed 4-cm mass with a 2.5-cm unilocular cystic area (Figure 3). Immunohistological evaluation led to a diagnosis of desmoid tumor primarily involving the pancreas, with cells showing nuclear positivity to beta-catenin. The patient recovered well postoperatively and was discharged with follow-up with a genetic counselor.

Figure 2. Endoscopic ultrasound showing a solid-cystic pancreatic mass.

Desmoid tumors make up 0.03% of all neoplasms with an incidence of 2–3 per million population each year, arising largely sporadically, with 5–15% linked to familial adenomatous polyposis (FAP).1,2 With no potential for metastasis or dedifferentiation, desmoid tumors can cause complications through compression or obstruction of surrounding structures. FAP-associated tumors occur primarily intraabdominally, while non-FAP associated tumors occur extraabdominally and on the pelvic/abdominal wall.3 Immunohistochemical testing is required to establish a definitive diagnosis, with nuclear beta-catenin staining supporting the diagnosis with 80% and 67% positivity in sporadic and FAP-associated cases, respectively.4 Pancreatic desmoid tumors are particularly rare and require pathological evaluation for differentiation from other pancreatic lesions. In a literature review, there have been 12 reported cases of such lesions, primarily occurring sporadically and within the pancreatic tail.

The generally indolent nature of these tumors allows treatment to be dictated by location, risk to surrounding structures, and the extent of infiltration. With extraabdominal or abdominal wall tumors, surgical resection with negative margins is the treatment of choice. Surgical intervention becomes more complex with intraabdominal tumors, as does the inherent risk of reoccurrence. While the decision was made for surgical resection in this case given concern for multi-organ infiltration, further exploration on the role of multimodal treatment involving radiation and systemic therapy is needed.

Figure 3. Gross image of an infiltrative, poorly circumscribed 4-cm mass with a unilocular cystic area measuring 2.5 cm.


Disclosures

Author contributions: HD Patel wrote the manuscript and searched the literature. NR Desai wrote the manuscript and obtained the images. A. Som searched the literature. SK Shah reviewed the manuscript. NC Thosani wrote and edited the manuscript and is the article guarantor.

Financial disclosure: None to report.

Informed consent was obtained for this case report.

Correspondence: Harsh D. Patel, University of Texas Health Science Center at Houston, McGovern Medical School, 6431 Fannin St, Suite MSB 1.134, Houston, TX, 77030 (harik81@yahoo.com).

Received November 21, 2016; Accepted Feburary 9, 2017


References

  1. Reitamo JJ, Häyry P, Nykyri E, Saxén E. The desmoid tumor. I. Incidence, sex-, age- and anatomical distribution in the Finnish population. Am J Clin Pathol. 1982;77(6):665–73. PubMed
  2. Nieuwenhuis MH, Casparie M, Mathus-Vliegen LM, Dekkers OM, Hogendoorn PC, Vasen HF. A nation-wide study comparing sporadic and familial adenomatous polyposis-related desmoid-type fibromatoses. Int J Cancer. 2011;129(1):256–61. Article | PubMed
  3. Schlemmer M. Desmoid tumors and deep fibromatoses. Hematol Oncol Clin North Am. 2005;19(3):565–71. Article | PubMed
  4. Carlson JW, Fletcher CD. Immunohistochemistry for beta-catenin in the differential diagnosis of spindle cell lesions: Analysis of a series and review of the literature. Histopathology. 2007;51(4):509–14. Article | PubMed

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© 2017 Patel et al. This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by-nc-nd/4.0.