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LETTER TO EDITOR
Year : 2012  |  Volume : 60  |  Issue : 5  |  Page : 544-545

Primary presacral carcinoid tumor with gluteal muscle metastasis


Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China

Date of Web Publication3-Nov-2012

Correspondence Address:
Siqing Huang
Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China

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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.103219

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How to cite this article:
Zhong W, You C, Chen H, Huang S. Primary presacral carcinoid tumor with gluteal muscle metastasis. Neurol India 2012;60:544-5

How to cite this URL:
Zhong W, You C, Chen H, Huang S. Primary presacral carcinoid tumor with gluteal muscle metastasis. Neurol India [serial online] 2012 [cited 2019 Aug 21];60:544-5. Available from: http://www.neurologyindia.com/text.asp?2012/60/5/544/103219


Sir,

A 48-year-old female was admitted with intermittent sacrococcygeal pain of 16-year duration. Physical examination revealed bowel dysfunction and a firm mass in retrorectal space. She had undergone ovariectomy for cystadenoma and hysterectomy for leiomyoma 2 years ago, and a lumbosacral computed tomography (CT) scan done at that time showed a well-circumscribed solid mass in the presacral space closely adhered to the sacrococcyx. Magnetic resonance imaging (MRI) done upon admission showed a larger and aggressive mass, hypointense on T1-weighted and hyperintense on T2-weighted sequences with significantly heterogeneous enhancement [Figure 1]. By a posterior approach, the mass and involved coccyx were totally removed. Pathological examination revealed carcinoid tumor with sacrococcyx invasion [Figure 2], with a proliferation index (Ki-67) of 1%. Postoperative repeat imaging did not reveal any other metastasis. Postoperative chemotherapy included etoposide, cisplatin, adriamycin, and cyclophosphamide. She also received local radiotherapy (50 Gy). At 3-year follow-up, she still has intermuscular metastasis near the sciatic notch.
Figure 1: (a) Lumbosacral MRI revealed a well-circumscribed presacral lesion, which was hypointense on T1-weighted image (b) and hyperintense signal on T2-weighted image (c) and heterogeneous enhanced

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Figure 2: Pathological examination revealed that the lesion had involved the sacrococcyx with intravascular metastasis (arrow)

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Presacral space is a potential space containing multiple embryological remnants. Several types of tumors can arise from this space, and of them chordomas are the most common malignant tumors. [1],[2] Caracinoid tumors are rare, and only about 22 cases have been reported. [3],[4],[5],[6],[7],[8] They may be associated with tailgut cysts and teratomas, suggesting that they are congenital or developmental tumors and may origin from residual neuroendocrine cells within hindgut remnants. [3] A female predominance suggests possible hormonal involvement in the pathogenesis. In our patient, coexistence noncarcinoid tumor at the other locations suggests that there may be potential mutual promoting effect between them. These tumors reported more in female, and the reported average age at presentation was 47 years (range, 19-72 years), while a male predominance has been reported with sacrococcygeal chordomas. [1] The symptoms are often insidious and sometimes the lesion may be an incidental finding. The common manifestations are lower back and rectal pain, bowel and bladder dysfunctions, as a result of local mass effects. [4],[5] Other potential complications include infection manifesting as nonresolving and recurrent perianal or anorectal fistula, bleeding and malignant transformation.

MRI features of presacral carcinoid tumors have rarely been reported, and they are hypointense on T1- weighted and hyperintense on T2-weighted images with significant enhancement as in our patient, similar to that of chordomas. However, the latter is more aggressive and would invade the spine and adjacent soft tissue. [1] Differential diagnoses include many other lesions. Teratoma tends to adhere to the coccyx and is heterogeneous due to the composition of all three germ layers, [9] dermoid cyst is an unilocular cyst with typically high signal on T1-weighted images because of fat contents. [10] An anterior sacral meningocele is a herniation of the dural and arachniod through a sacral defect with the sac in continuity with the subdural space. [1] Enterogenous cyst is in continuity or contiguity with the rectum whereas anal gland cyst is typically in close approximation to the anal sphincter. [10] Although pathological examination is the final diagnostic method, preoperative biopsy is controversial because of the risk of bleeding, serious infection, and tumor seeding through the puncture channel. [1],[5] Moreover, it may be unnecessary if total surgical resection is possible.

It should be kept in mind that presacral lesions may contain malignant elements or possess the potential of malignant degeneration, aggressive surgical resection should be carried out as early as possible, gross total resection with negative margins was the best choice, and sometimes a multidisciplinary cooperation is recommended. The lesion may involve the sacrococcyx, the coccyx and S4-5 vertebral bones could be removed, but the S1, S2, and at least one side of S3 nerve roots should be protected to avoid fecal and urinary continence. [2] Preoperative embolization of the feeding artery or temporary occlusion of abdominal aorta was necessary to control intraoperative bleeding. [6] Although chemotherapy and local radiotherapy have been used in some patients, presacral carcinoid tumors seem to be nonresponsive as in our patient.

The prognosis of presacral carcinoid tumors mainly depends on the pathology and the size of the lesion, the presence of recurrence, and metastasis. [5] Ki-67 index may not fully influence its malignant behavior, and metastasis had been reported as in our patient. The presence of metastasis suggests that carcinoid tumors are malignant tumors, they may metastasize to lung, breast, liver, and ovary, [3],[7],[8] and our patient is probably the first report with local intermuscular space metastasis. The 5-year survival rate will be around 38.5% in the rectal carcinoid tumors with metastasis. [4]

In conclusion, the study of our patient illustrates that presacral carcinoid tumors should be included in the differential diagnosis of presacral mass and close follow-up is of importance for early diagnosis of recurrence and metastasis.

 
  References Top

1.Hobson KG, GhaemmaghamiV, Roe JP, Goodnight JE, Khatri VP. Tumors of the retrorectal space. Dis Colon Rectum 2005;48:1964-74.  Back to cited text no. 1
    
2.Glasgow SC, Birnbaum EH, Lowney JK, Fleshman JW, Kodner IJ, Mutch DG, et al. Retrorectal tumors: A diagnostic and therapeutic challenge. Dis Colon Rectum 2005;48:1581-7.  Back to cited text no. 2
    
3.Oyama K, Embi C, Rader AE. Aspiration cytology and core biopsy of a carcinoid tumor arising in a retrorectal cyst: A casereport. Diagn Cytopathol 2000;22:376-8.  Back to cited text no. 3
    
4.Dujardin F, Beaussart P, de Muret A, Rosset P, Waynberger E, Mulleman D. Primary neuroendocrine tumor of the sacrum: Case report and review of the literature. Skeletal Radiol 2009;38:819-23.  Back to cited text no. 4
    
5.Mourra N, Caplin S, Parc R, Flejou JF. Presacral neuroendocrine carcinoma developed in a tailgut cyst: Report of a case. Dis Colon Rectum 2003;46:411-3.  Back to cited text no. 5
    
6.Schnee CL, Hurst RW, Curtis MT, Friedman ED. Carcinoid tumor of the sacrum: Case report. Neurosurgery 1994;35:1163-7.  Back to cited text no. 6
    
7.Fiandaca MS, Ross WK, Pearl GS, Bakay RA. Carcinoid tumor in a presacralteratoma associated with an anterior sacral meningocele: Case report and review of the literature. Neurosurgery 1988;22:581-8.  Back to cited text no. 7
    
8.Horenstein MG, Erlandson RA, Gonzales-Cueto DM, Rosai J. Presacral carcinoid tumors: Report of three cases and review of the literature. Am J Surg Pathol 1998;22:251-5.  Back to cited text no. 8
    
9.Hassan I, Wietfeldt ED. Presacral tumors: Diagnosis and management. Clin Colon Rectal Surg 2009;22:84-93.  Back to cited text no. 9
    
10.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;21:575-84.  Back to cited text no. 10
    


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