Abstract
Primary carcinoid tumor (well-differentiated neuroendocrine tumor) of the bone involving the sacrum is extremely rare. We report the case of a 72-year-old man who presented with a 20-year history of intermittent low back pain and was found to have an intraosseous sacral mass on imaging. A needle biopsy revealed that this lesion was a well-differentiated neuroendocrine tumor. Workup did not show any primary tumor or other metastatic disease. There was no associated tailgut cyst or sacrococcygeal teratoma. The lesion was treated with radiation therapy because a surgical approach was rejected. The patient is free of metastatic disease after 28 years evolution of the lesion, retrospectively seen to be present on a conventional radiography performed in 1980. A review of the literature revealed 20 case reports of neuroendocrine tumors arising from the presacral region (with or without associated tailgut cyst or sacrococcygeal teratoma) and sometimes extending to the sacrum. One additional case was located within the neural canal and involved the sacrum, the presacral region, and the rectal wall. Our case is the only tumor arising primarily from the sacrum. The long evolution of this lesion without any other location makes metastatic disease very improbable and this case appears to be a unique example of primary intraosseous sacral carcinoid tumor.
Similar content being viewed by others
References
Zuetenhorst JM, Taal BG. Metastatic carcinoid tumors: a clinical review. Oncologist 2005; 10: 123–131.
Taal BG, Visser O. Epidemiology of neuroendocrine tumours. Neuroendocrinology 2004; 80: 3–7.
Modlin IM, Lye KD, Kidd M. A 5-decade analysis of 13,715 carcinoid tumors. Cancer 2003; 97: 934–959.
Quaedvlieg PF, Visser O, Lamers CB, et al. Epidemiology and survival in patients with carcinoid disease in The Netherlands. An epidemiological study with 2391 patients. Ann Oncol 2001; 12: 1295–1300.
Gerber S, Ollivier L, Leclère J, et al. Imaging of sacral tumours. Skeletal Radiol 2008; 37(4): 277–289.
Raina V, Milroy R, al-Dawoud A, Dunlop D, Soukop M. Extrapulmonary small cell carcinoma of bone. Postgrad Med J 1992; 68(796): 147–148.
Scarsbrook AF, Ganeshan A, Statham J, Thakker RV, Weaver A, Talbot D. Anatomic and functional imaging of metastatic carcinoid tumors. Radiographics 2007; 27: 455–476.
Zuetenhorst JM, Hoefnagel CA, Boot H, et al. Evaluation of (111) In-pentetreotide, (131) I-MIBG and bone scintigraphy in the detection and clinical management of bone metastases in carcinoid disease. Nucl Med Commun 2002; 23: 735–741.
Meijer WG, van der Veer E, Jager PL, et al. Bone metastases in carcinoid tumors: clinical features, imaging characteristics, and markers of bone metabolism. J Nucl Med 2003; 44: 184–191.
Kirshbom PM, Kherani AR, Onaitis MW, Feldman JM, Tyler DS. Carcinoids of unknown origin: comparative analysis with foregut, midgut, and hindgut carcinoids. Surgery 1998; 124(6): 1063–1070.
Banzo J, Abos MD, Prats E, et al. Carcinoid tumor and bone metastases: diagnosis by somatostatin receptor scintigraphy. Rev Esp Med Nucl 2004; 23(6): 394–402.
Arasi M, Toy H, Tavli L. Primary neuroendocrine carcinoma arising within a mature sacrococcygeal teratoma. Orthopedics 2007; 30(10): 878–879.
Luong TV, Salvagni S, Bordia C. Presacral carcinoid tumour: review of the literature and report of a clinically malignant case. Dig Liver Dis 2005; 37(4): 278–281.
Krasin E, Nirkin A, Issakov J, Rabau M , Meller I. Carcinoid tumor of the coccyx. Spine 2001; 16(19): 2165–2167.
Jacob S, Dewan Y, Joseph S. Presacral carcinoid tumour arising in a tailgut cyst: a case report. Indian J Pathol Microbiol. 2004; 47(1): 32–33.
Theunissen P, Fickers M, Goei R. Primary large cell neuroendocrine carcinoma of the presacral region. J Clin Pathol 2001; 54(11): 880–882.
Matthieu 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.
Mourra N, Caplin S, Parc R, Flejou J-F. Presacral neuroendocrine carcinoma developed in a tailgut cyst: report of a case. Dis Colon Rectum 2003; 46: 411–413.
Prasad AR, Amin MB, Randolph TL, Lee CS, Ma CK. Retrorectal cystic hamartoma: report of 5 cases with malignancy arising in 2. Arch Pathol Lab Med 2000; 124: 725–729.
Oyama K, Embi C, Rader AE. Aspiration cytology and core biopsy of a carcinoid tumor arising in a retrorectal cyst: a case report. Diagn Cytopathol 2000; 22: 376–378.
Gorski T, Khubchandani IT, Stasik JJ, et al. Retrorectal carcinoid tumor. South Med J 1999; 92: 417–420.
Horenstein MG, Erlandson RA, Gonzales-Cueto DM, et al. Presacral carcinoid tumors. Report of three cases and review of the literature. Am J Surg Pathol 1998; 22: 251–255.
Edelstein PS, Wong WD, La Valleur J, Rothenberger DA. Carcinoid tumor: an extremely unusual presacral lesion. Report a case. Dis Colon Rectum 1996; 39: 938–942.
Lin SL, Yang AH, Liu HC. Tailgut cyst with carcinoid: a case report. Zhonghua Yi Xue Za Zhi (Taipei) 1992; 49: 57–60.
Addis BJ, Rao SG, Finnis D, Carvell JE. Pre-sacral carcinoid tumour. Histopathology 1991; 18: 563–565.
Hood DL, Petras RE, Grundfest-Broniatowski S, Jagelman DG. Retrorectal cystic hamartoma: report of five cases with carcinoid tumors arising in two [abstract]. Am J Clin Pathol 1988; 89: 433.
Fiandaca MS, Ross WK, Pearl GS, Bakay RA. Carcinoid tumor in a presacral teratoma associated with an anterior sacral meningocele: case report and review of the literature. Neurosurgery 1988; 22(3): 581.
Aparicio SR, Cowen PN, Croft CB. Argentaffin carcinoma arising in a sacrococcygeal teratoma. J Pathol 1971; 107: 49–53.
Schnee CL, Hurst RW, Curtis MT, Friedman ED. Carcinoid tumor of the sacrum: case report. Neurosurgery 1994; 35: 1163–1167.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Dujardin, F., Beaussart, P., de Muret, A. et al. Primary neuroendocrine tumor of the sacrum: case report and review of the literature. Skeletal Radiol 38, 819–823 (2009). https://doi.org/10.1007/s00256-009-0693-y
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00256-009-0693-y