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Arch Surg. 2012;147(1):94. doi:10.1001/archsurg.2011.702b.
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ANSWER: LOW-GRADE NEUROENDOCRINE CARCINOMA ARISING FROM A TAILGUT CYST

The final pathologic diagnosis was low-grade neuroendocrine carcinoma arising from a tailgut cyst. At the initial 3-month follow-up, the patient was doing well, with no signs of recurrence on physical examination and transrectal ultrasonography. His follow-up will consist of physical examination and transrectal ultrasonography every 3 months for the first year, every 6 months for the second, and yearly after that for a total of 5 years. Computed tomographic scan of the abdomen and pelvis will be obtained every year for 5 years. Regular colonoscopy protocol will be followed as per average-risk patients.

Tailgut cysts, or cystic hamartomas, are rare developmental cysts derived from tailgut remnants, an embryological precursor of the gastrointestinal system, thus explaining the common finding of glandular columnar epithelium within.1 The differential diagnoses include other congenital developmental (epidermoid cysts, dermoid cysts, and teratomas) and miscellaneous (chordomas, anterior meningocele, rectal duplications, and adrenal rest tumors) cystic lesions, inflammatory lesions (granulomas and abscesses), neurogenic neoformations (neurofibromas, neurolemmomas, ependymomas, ganglioneuromas, and neurofibrosarcomas), osseous tumors (osteomas, osteogenic sarcomas, sacral bone cysts, and Ewing tumor), and other neoplastic lesions (metastases, lymphangiomas, desmoid tumors, leiomyomas, and fibrosarcomas).2 Congenital lesions account for 55% to 70% of retrorectal lesions, and 60% of them are developmental cysts.3 Tailgut cysts can occur at any age but are more frequent in middle-aged women and most of the time are asymptomatic and incidentally diagnosed by routine physical examination or imaging studies obtained for nonspecific and often unrelated symptoms.4 Malignant transformation is unusual but the development of adenocarcinomas, carcinoids, squamous cell carcinomas, and sarcomas has been described.5 Digital rectal examination can provide essential information on the location, consistency, and mobility of the lesion, while endoscopic ultrasonography, computed tomographic scan, and magnetic resonance imaging are valuable tools in defining texture, extent, and relationships with surrounding structures of the lesion, the latter being the diagnostic imaging tool of choice.1 The role of transrectal or presacral needle biopsy is controversial because of the low likelihood of obtaining a significant sample for an adequate diagnosis, along with the potential risk of tumor seeding.6 A primary carcinoid tumor arising in a retrorectal tailgut cyst is a rare finding, with only 12 cases previously reported in the literature.5,7 Neuroendocrine carcinomas arising in tailgut cysts have a real malignant potential, as the occurrence of distant metastases has been reported in the literature.8 Besides the oncological concerns, the need for surgical excision of tailgut cysts resides in the potential for infection and fistulization.9 Surgical resection is the keystone in the management of all retrorectal tumors, aiming to excise the mass intact and surrounded by macroscopically intact tissue. For low lesions (below the fourth sacral element), a posterior approach is preferred to the transabdominal route to better assess local invasion without violating the rectal wall, unless it is involved.10 As confirmed in this case, a parasacrococcygeal approach with resection of the coccyx provides optimal exposure, thus facilitating radical en bloc resection of the tumor, sparing the uninvolved surrounding structures, with minimal surgical trauma and morbidity and fast recovery.

ARTICLE INFORMATION

SECTION EDITOR: CARL E. BREDENBERG, MD

Correspondence: Alessandro Fichera, MD, 5841 S Maryland Ave, MC 5095, Chicago, IL 60637 (afichera@surgery.bsd.uchicago.edu).

Accepted for Publication: April 27, 2011.

Author Contributions:Study concept and design: Zoccali, Hart, and Fichera. Acquisition of data: Zoccali. Drafting of the manuscript: Zoccali and Fichera. Critical revision of the manuscript for important intellectual content: Hart and Fichera. Study supervision: Hart and Fichera.

Financial Disclosure: None reported.

REFERENCES

Hobson  KG, Ghaemmaghami  V, Roe  JP, Goodnight  JE, Khatri  VP.  Tumors of the retrorectal space. Dis Colon Rectum. 2005;48(10):1964-1974.
PubMed   |  Link to Article
Uhlig  BE, Johnson  RL.  Presacral tumors and cysts in adults. Dis Colon Rectum. 1975;18(7):581-589.
PubMed   |  Link to Article
Jao  SW, Beart  RW  Jr, Spencer  RJ, Reiman  HM, Ilstrup  DM.  Retrorectal tumors: Mayo Clinic experience, 1960-1979. Dis Colon Rectum. 1985;28(9):644-652.
PubMed   |  Link to Article
Dahan  H, Arrivé  L, Wendum  D, Docou le Pointe  H, Djouhri  H, Tubiana  JM.  Retrorectal developmental cysts in adults: clinical and radiologic-histopathologic review, differential diagnosis, and treatment. Radiographics. 2001;21(3):575-584.
PubMed   |  Link to Article
Mathieu  A, Chamlou  R, Le Moine  F, Maris  C, Van de Stadt  J, Salmon  I.  Tailgut cyst associated with a carcinoid tumor: case report and review of the literature. Histol Histopathol. 2005;20(4):1065-1069.
PubMed
Cody  HS  III, Marcove  RC, Quan  SH.  Malignant retrorectal tumors: 28 years' experience at Memorial Sloan-Kettering Cancer Center. Dis Colon Rectum. 1981;24(7):501-506.
PubMed   |  Link to Article
Liang  JJ, Alrawi  S, Fuller  GN, Tan  D.  Carcinoid tumors arising in tailgut cysts may be associated with estrogen receptor status: case report and review of the literature. Int J Clin Exp Pathol. 2008;1(6):539-543.
PubMed
Song  DE, Park  JK, Hur  B, Ro  JY.  Carcinoid tumor arising in a tailgut cyst of the anorectal junction with distant metastasis: a case report and review of the literature. Arch Pathol Lab Med. 2004;128(5):578-580.
PubMed
Hjermstad  BM, Helwig  EB.  Tailgut cysts: report of 53 cases. Am J Clin Pathol. 1988;89(2):139-147.
PubMed   |  Link to Article
Abel  ME, Nelson  R, Prasad  ML, Pearl  RK, Orsay  CP, Abcarian  H.  Parasacrococcygeal approach for the resection of retrorectal developmental cysts. Dis Colon Rectum. 1985;28(11):855-858.
PubMed   |  Link to Article

Figures

Tables

References

Hobson  KG, Ghaemmaghami  V, Roe  JP, Goodnight  JE, Khatri  VP.  Tumors of the retrorectal space. Dis Colon Rectum. 2005;48(10):1964-1974.
PubMed   |  Link to Article
Uhlig  BE, Johnson  RL.  Presacral tumors and cysts in adults. Dis Colon Rectum. 1975;18(7):581-589.
PubMed   |  Link to Article
Jao  SW, Beart  RW  Jr, Spencer  RJ, Reiman  HM, Ilstrup  DM.  Retrorectal tumors: Mayo Clinic experience, 1960-1979. Dis Colon Rectum. 1985;28(9):644-652.
PubMed   |  Link to Article
Dahan  H, Arrivé  L, Wendum  D, Docou le Pointe  H, Djouhri  H, Tubiana  JM.  Retrorectal developmental cysts in adults: clinical and radiologic-histopathologic review, differential diagnosis, and treatment. Radiographics. 2001;21(3):575-584.
PubMed   |  Link to Article
Mathieu  A, Chamlou  R, Le Moine  F, Maris  C, Van de Stadt  J, Salmon  I.  Tailgut cyst associated with a carcinoid tumor: case report and review of the literature. Histol Histopathol. 2005;20(4):1065-1069.
PubMed
Cody  HS  III, Marcove  RC, Quan  SH.  Malignant retrorectal tumors: 28 years' experience at Memorial Sloan-Kettering Cancer Center. Dis Colon Rectum. 1981;24(7):501-506.
PubMed   |  Link to Article
Liang  JJ, Alrawi  S, Fuller  GN, Tan  D.  Carcinoid tumors arising in tailgut cysts may be associated with estrogen receptor status: case report and review of the literature. Int J Clin Exp Pathol. 2008;1(6):539-543.
PubMed
Song  DE, Park  JK, Hur  B, Ro  JY.  Carcinoid tumor arising in a tailgut cyst of the anorectal junction with distant metastasis: a case report and review of the literature. Arch Pathol Lab Med. 2004;128(5):578-580.
PubMed
Hjermstad  BM, Helwig  EB.  Tailgut cysts: report of 53 cases. Am J Clin Pathol. 1988;89(2):139-147.
PubMed   |  Link to Article
Abel  ME, Nelson  R, Prasad  ML, Pearl  RK, Orsay  CP, Abcarian  H.  Parasacrococcygeal approach for the resection of retrorectal developmental cysts. Dis Colon Rectum. 1985;28(11):855-858.
PubMed   |  Link to Article

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