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Image of the Month—Diagnosis FREE

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Section Editor: E. Bredenberg Carl, MD

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Arch Surg. 2008;143(10):1018. doi:10.1001/archsurg.143.10.1018.
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ANSWER: RETRORECTAL CYSTIC HAMARTOMA (TAILGUT CYST)

Exploratory laparotomy and pelvic exploration with resection of the mass was performed. We approached the resection through a midline incision. Intraoperatively, we found a large, multicystic, fluid-filled mass that involved the superior rectal artery and significantly compressed the adjacent structures. The tumor was resected in continuity with the rectum and anus; a coccygectomy was also performed. No evidence was found of metastatic disease or lymphadenopathy.

Histologic examination of the specimen revealed a moderately differentiated adenocarcinoma with colorectal-type morphologic features arising within a tailgut cyst. The colorectal phenotype was confirmed immunohistochemically by positive stains for CDX2 and CK20 and a negative stain for CK7. Nine local lymph nodes were negative for malignant neoplasm. The surgical margins were free of malignant neoplasm. The patient elected to undergo adjuvant locoregional chemoradiotherapy, as recommended by our institutional tumor board.

A tailgut cyst, or retrorectal cystic hamartoma (RCH), is a rare congenital lesion that arises from vestiges of the most caudal portion of the primitive hindgut and typically presents as a retrorectal mass.1,2This embryologic remnant usually regresses during the 7th to 8th week of development. These tumors occur most commonly in middle-aged women (3:1) and often have a nonspecific clinical presentation, with pain reported in 51% of patients.3The tumors often contain many types of epithelial tissue but always contain some elements of intestinal epithelium. An RCH rarely undergoes malignant transformation of the epithelial components of the tumor, but it nonetheless necessitates meticulous gross and microscopic evaluation of the surgical specimen to rule out malignant neoplasm. In 2000, Prasad et al4noted 12 cases in the literature of a malignant neoplasm within an RCH. We identified 14 more through June 2006 and, including our case, 26 cases of malignant transformation within an RCH have been reported during the past 75 years; 17 were adenocarcinomas2,49and 9 were carcinoid tumors.9An elevated preoperative carcinoembryonic antigen level may indicate malignant transformation and may be useful in tracking the recurrence of adenocarcinomas that arise within an RCH after surgical extirpation.8

Complete surgical resection of an RCH is necessary to prevent recurrence and the development of draining sinuses and to evaluate for malignant transformation. A combined anterior and posterior pelvic approach with complete resection of the tumor and adjacent involved structures is recommended for cysts with malignant transformation. The incidental finding of a retrorectal mass during laparotomy also necessitates resection and has been advocated by several authors.5,6Local chemoradiotherapy is frequently recommended for an adenocarcinoma that arises within an RCH because of the frequently large size of these tumors, the difficulty in evaluating surgical margins, and the compression of adjacent structures. Follow-up with computed tomography or magnetic resonance imaging after treatment is recommended.

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The Editor welcomes contributions to the Image of the Month. Manuscripts should be submitted via our online manuscript submission and review system (http://manuscripts.archsurg.com). Articles and photographs accepted will bear the contributor's name. Manuscript criteria and information are per the Instructions for Authors for Archives of Surgery (http://archsurg.ama-assn.org/misc/ifora.dtl). No abstract is needed, and the manuscript should be no more than 3 typewritten pages. There should be a brief introduction, 1 multiple-choice question with 4 possible answers, and the main text. No more than 2 photographs should be submitted. There is no charge for reproduction and printing of color illustrations.

Correspondence:Kevin G. Billingsley, MD, Department of Surgery, Oregon Health and Science University, 3181 SW Sam Jackson Park Rd, Mail Code L223A, Portland, OR 97239.

Accepted for Publication:December 21, 2006.

Author Contributions:Study concept and design: Mayo, Dobos, and Billingsley. Acquisition of data: Mayo and Huang. Analysis and interpretation of data: Mayo and Huang. Drafting of the manuscript: Mayo and Huang. Critical revision of the manuscript for important intellectual content: Mayo, Dobos, and Billingsley. Study supervision: Dobos and Billingsley. Histologic photographs, diagnosis, and interpretation: Huang.

Financial Disclosure:None reported.

Additional Contributions:Chris Corless, MD, PhD (Department of Pathology at Oregon Health and Science University), provided expertise in the pathologic analysis of the specimen.

Caropreso  PRWengert  PA  JrMilford  HE Tailgut cyst—a rare retrorectal tumor: report of a case and review. Dis Colon Rectum 1975;18 (7) 597- 600
PubMed
Moreira  ALScholes  JVBoppana  SMelamed  J p53 Mutation in adenocarcinoma arising in retrorectal cyst hamartoma (tailgut cyst). Arch Pathol Lab Med 2001;125 (10) 1361- 1364
PubMed
Hjermstad  BMHelwig  EB Tailgut cysts:report of 53 cases. Am J Clin Pathol 1988;89 (2) 139- 147
PubMed
Prasad  ARAmin  MBRandolph  TLLee  CSMa  CK Retrorectal cystic hamartoma: report of 5 cases with malignancy arising in 2. Arch Pathol Lab Med 2000;124 (5) 725- 729
PubMed
Maruyama  AMurabayashi  KHayashi  M  et al.  Adenocarcinoma arising in a tailgut cyst: report of a case. Surg Today 1998;28 (12) 1319- 1322
PubMed
Graadt van Roggen  JFWelvaart  Kde Roos  AOfferhaus  GJHogendoorn  PC Adenocarcinoma arising within a tailgut cyst. J Clin Pathol 1999;52 (4) 310- 312
PubMed
Umar  TMikel  JJPoller  DN Carcinoma arising in a tailgut cyst diagnosed on fine needle aspiration (FNA) cytology. Cytopathology 2000;11 (2) 129- 132
PubMed
Schwarz  RELyda  MLew  MPaz  IB A carcinoembryonic antigen-secreting adenocarcinoma arising within a retrorectal tailgut cyst: clinicopathological considerations. Am J Gastroenterol 2000;95 (5) 1344- 1347
PubMed
Mourra  NCaplin  SParc  RFlejou  JF Presacral neuroendocrine carcinoma developed in a tailgut cyst: report of a case. Dis Colon Rectum 2003;46 (3) 411- 413
PubMed

Figures

Tables

References

Caropreso  PRWengert  PA  JrMilford  HE Tailgut cyst—a rare retrorectal tumor: report of a case and review. Dis Colon Rectum 1975;18 (7) 597- 600
PubMed
Moreira  ALScholes  JVBoppana  SMelamed  J p53 Mutation in adenocarcinoma arising in retrorectal cyst hamartoma (tailgut cyst). Arch Pathol Lab Med 2001;125 (10) 1361- 1364
PubMed
Hjermstad  BMHelwig  EB Tailgut cysts:report of 53 cases. Am J Clin Pathol 1988;89 (2) 139- 147
PubMed
Prasad  ARAmin  MBRandolph  TLLee  CSMa  CK Retrorectal cystic hamartoma: report of 5 cases with malignancy arising in 2. Arch Pathol Lab Med 2000;124 (5) 725- 729
PubMed
Maruyama  AMurabayashi  KHayashi  M  et al.  Adenocarcinoma arising in a tailgut cyst: report of a case. Surg Today 1998;28 (12) 1319- 1322
PubMed
Graadt van Roggen  JFWelvaart  Kde Roos  AOfferhaus  GJHogendoorn  PC Adenocarcinoma arising within a tailgut cyst. J Clin Pathol 1999;52 (4) 310- 312
PubMed
Umar  TMikel  JJPoller  DN Carcinoma arising in a tailgut cyst diagnosed on fine needle aspiration (FNA) cytology. Cytopathology 2000;11 (2) 129- 132
PubMed
Schwarz  RELyda  MLew  MPaz  IB A carcinoembryonic antigen-secreting adenocarcinoma arising within a retrorectal tailgut cyst: clinicopathological considerations. Am J Gastroenterol 2000;95 (5) 1344- 1347
PubMed
Mourra  NCaplin  SParc  RFlejou  JF Presacral neuroendocrine carcinoma developed in a tailgut cyst: report of a case. Dis Colon Rectum 2003;46 (3) 411- 413
PubMed

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