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Year : 2013  |  Volume : 40  |  Issue : 3  |  Page : 186-188

A retrorectal tumor: Presacral chordoma

1 Department of Surgery, Kasturba Medical College, Manipal, Karnataka, India
2 Department of Pathology, Kasturba Medical College, Manipal, Karnataka, India

Date of Web Publication19-Oct-2013

Correspondence Address:
Suresh Birur Parmeshwarappa
Department of Surgery, Kasturba Medical College, Manipal - 576 104, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0974-5009.120065

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The retrorectal space contains multiple embryologic remnants derived from various tissues and tumors that develop in this space are both grossly and histologically heterogeneous. Most lesions are benign, but malignant neoplasms are not uncommon. Malignancy is more common in the pediatric population than in adults and solid lesions are more likely to be malignant than are cystic lesions. Here, 50-year-old male presented bleeding with mass per rectum and lower abdominal pain. The computed tomography scan showed retrorectal mass with destruction of the sacrum and excision of the presacral mass with removal of involved sacrum from third sacral vertebra to tip of coccyx. Finally, histopathology was chordoma of sacrum. Patient had received adjuvant external beam radiotherapy.

Keywords: Chordoma, presacral tumor, retrorectal tumor

How to cite this article:
Parmeshwarappa SB, Rao A, Sharma Y, Rao L. A retrorectal tumor: Presacral chordoma. J Sci Soc 2013;40:186-8

How to cite this URL:
Parmeshwarappa SB, Rao A, Sharma Y, Rao L. A retrorectal tumor: Presacral chordoma. J Sci Soc [serial online] 2013 [cited 2020 Sep 21];40:186-8. Available from: http://www.jscisociety.com/text.asp?2013/40/3/186/120065

  Introduction Top

Chordomas arise from the notochord and are the most common malignant tumor of the retrorectal space. Giant cell tumor is the most common differential diagnosis of bony tumor arising from sacrococcygeal region next to chordoma. Other lesions that can give rise to similar pictures are chondrosarcoma, ependymoma, plasmacytoma and a solitary metastatic deposit. [1] Chordomas frequently present with pain and are thought to be more common in men. Chordomas can occur anywhere in the spine, but the most common single site is the sacrococcygeal region (30-50%). These tumors are slow-growing, invasive cancers that show characteristic bony destruction. Radical resection and adjuvant radiotherapy offers the best hope for a cure. [2],[3]

  Case Report Top

A 50-year-old male patient presented with pain in the lower abdominal and bleeding per rectum. Examination of the abdomen and pelvis revealed no palpable mass and organomegaly. Per rectal examination showed a mass in the posterior rectal wall, but there is no breach in the rectal mucosa. Bladder sensation and perianal sensation was normal. No motor or sensory abnormality of lower limb. Biochemical investigation was within the normal limits. The computed tomography (CT) scan of the abdomen and pelvis well defined mass showing few specks of calcification noted in the sacral region, extending from third sacral vertebra along with destruction of vertebra [Figure 1]. Then our provisional diagnosis was retrorectal tumor. Then patient was planned for exploratory laparotomy and excision of involved sacral vertebra. The planned incision was from lateral pubic tubercle to midway between 12 th rib and left iliac crest [Figure 2] and position of the patient was right lateral semi prone position. The posterior rectal mobilization was done. External midline incision over sacrum, involved sacrum from third sacral vertebra to tip of coccyx, along with the retrorectal tumor removed without damaging the sacral nerve plexuses. During this procedure, there was spillage of tumor mass. The titanium clips were put along the resected site in view of post-operative radiotherapy. During the post-operative period, patient had urinary retention. In view of bladder dysfunction urology opinion was taken. They advised continuous intermittent catheterization for one month. However, latter patient recovered from bladder dysfunction. Final histopathology was reported chordoma of sacrum [Figure 3]. Patient advised for adjuvant external beam radiotherapy. Patient was taken a total dose of 60 Gy in 30 fractions over 6 weeks. After regular follow-up of the patient nearly 2 years, no recurrence was observed by radiological investigation.
Figure 1: Computed tomography image showing well defined minimally enhancing soft-tissue lesion (black arrow) with specks of calcification in the sacral region? Chordoma. White arrow showing the rectum (B-bladder, T-tumor and S-sacrum)

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Figure 2: Incision on left side extending from pubic symphsis to midway between 12th rib and iliac crest

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Figure 3: (a and b) Sections of a lobulated tumor with large polygonal cells with eosinophilic to clear cytoplasm, prominent nucleoli, some with bizarre nucleus along with multivacuolated "physaliferous cells." Few of the cells have intracytoplasmic hemosiderin pigment (H and E, ×20)

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  Discussion Top

Retrorectal tumors are rare disease with heterogeneous entities occurring in a potential space between the rectum and sacrum, which is defined by fascia propia of the rectum anteriorly, by presacral fascia overlying the sacrum posteriorly and by the ureters and iliac vessels laterally. The dome of the space is the peritoneal reflection and the floor is the pelvic floor muscles. [4] Chordoma is a rare malignant bone tumor that originates from notochordal remnants and occurs exclusively in the axial skeleton, with a predilection for the sacrum, base of the skull and occasionally the mobile spine. [5] Retrorectal tumors can be completely asymptomatic throughout their courses. The presented symptoms are usually related to compression or invasion to the surrounding pelvis viscera and nerve. Urologic symptoms, change of bowel habits and nerve root signs are common. A careful rectal examination, CT and Magnetic resonance imaging (MRI) provide excellent direct anatomical description regarding the lesion. Both CT and MRI represented the sensitivity of 100% and the derived anatomical information, such as the infiltrating extension to surrounding tissues and the tumor origin, were compatible to all of our surgical findings. Local invasiveness and destructiveness are characteristic features of the disease. On MRI, these neoplasms may have similar signal characteristics to chordoma. Calcification on CT, origin at the sacrococcygeal junction and the presence of internal septations are features that distinguish chordoma from other sacral neoplasm. All tumors in this space should be excised completely. [6] Anterior, posterior and combined approaches were alternative techniques depending upon the size, location, and spatial relationship of the tumors. [7] For small (<1 cm) and low-lying tumors, within the level of sacral promontory, posterior approach is favored. [7] Anterior approach is preferred for larger, high-lying, or high-grade malignant tumors because it provides better visualization of pelvic structures, bleeding control and easier mobilization of rectum. Neurogenic bladder and fecal incontinence occurred in patients undergoing such extensive procedures. [8] Removal should aim to be extensive and complete if not recurrence is certain. The ideal location for sacral dissection, whenever possible, is the S2-S3 junction (preserving the S2 roots) in order to avoid incontinence. Following tumor resection, bowel and bladder dysfunction are the most frequent complications. The radiotherapy, it may prolong the disease-free interval and decrease the symptoms of recurrent lesions. [5] Until date, the role of radiation on managing the tumors was still under debate. Glasgow et al reported in another review article, [9] "aggressive postoperative radiation therapy has been reported for sacrococcygeal chordomas with favorable results. Indications for radiation therapy may include surgically inaccessible lesions, contaminated surgical margins, or incomplete surgical excision of the tumor. Radiation therapy can also be used after removal of the primary tumor or when there is a local recurrence. The interval of follow-up, including repeat MRI or CT scans, depends upon the completeness of the resection.

  Conclusion Top

Sacrococcygeal chordoma, a rare slow growing malignant tumor, presents a difficult diagnostic and therapeutic problem, with a high rate of local recurrence. CT and MRI scans provide sufficient information for diagnosis and pre-operative therapeutic plans. Earlier diagnosis of chordomas is important and requires a high index of suspicion. The surgical margin is the most important predictor of survival and aggressive surgery may help to achieve an adequate margin. Hopefully, one can expect more cures with earlier recognition and aggressive surgical treatment. Adjuvant radiotherapy is still recommended whenever there is inadequate surgical margin or disease is not amenable to surgical excision.

  References Top

1.Soo MY, Wong L. Sacrococcygeal chordoma. J Hong Kong Coll Radiol 2002;5:117-25.  Back to cited text no. 1
2.Cody HS 3 rd , Marcove RC, Quan SH. Malignant retrorectal tumors: 28 years' experience at memorial sloan-kettering cancer center. Dis Colon Rectum 1981;24:501-6.  Back to cited text no. 2
3.Miyahara M, Saito T, Nakashima K, Hiratsuka K, Sato K, Kaketani K, et al. Sacral chordoma developing two years after low anterior resection for rectal cancer. Surg Today 1993;23:144-8.  Back to cited text no. 3
4.Hao-Cheng C, Po-Fang W, Jy-Ming C, Rei-Ping T, Jinn-Shiun C, Chung-Rong C, et al. Outcomes of surgical intervention for retrorectal tumor: Experience of sixteen patients in Chang Gung Memorial Hospital. J Soc Colon Rectal Surg 2010;21:87-93.  Back to cited text no. 4
5.Unni KK. Chondroma. In: Unni KK, editor. Dahlin's Bone Tumors. General Aspects and Data on 11,087 Cases. 5 th ed. Philadelphia: Lippincott-Raven; 1996. p. 291-305.  Back to cited text no. 5
6.Samson IR, Springfield DS, Suit HD, Mankin HJ. Operative treatment of sacrococcygeal chordoma. A review of twenty-one cases. J Bone Joint Surg Am 1993;75:1476-84.  Back to cited text no. 6
7.Lev-Chelouche D, Gutman M, Goldman G, Even-Sapir E, Meller I, Issakov J, et al. Presacral tumors: A practical classification and treatment of a unique and heterogeneous group of diseases. Surgery 2003;133:473-8.  Back to cited text no. 7
8.Wang JY, Hsu CH, Changchien CR, Chen JS, Hsu KC, You YT, et al. Presacral tumor: A review of forty-five cases. Am Surg 1995;61:310-5.  Back to cited text no. 8
9.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. 9


  [Figure 1], [Figure 2], [Figure 3]


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