|
| Retrorectal Chordoma: Case Report and Literature Review |
| Martínez Hernández Magro Paulino1*, Martínez Salcedo Néstor1, Koelliker Ayala Enrique2 and Guerrero Medrano Jaime3 |
| 1Colorectal Surgery Department, Hospital Guadalupano de Celaya, Celaya Gto, México |
| 2Pulmonology, Hospital Guadalupano de Celaya, Celaya Gto, México |
| 3Pathology Department, Hospital Guadalupano de Celaya, Celaya Gto, México |
| *Corresponding author: |
Paulino Martínez Hernández Magro
Eje Vial Norponiente
No 200 Consultorio 509
Col. Villas de la Hacienda, CP 38100
Celaya Gto,
México
Tel: +52 (461) 6162123
E-mail: paulinomhm@hotmail.com |
|
| |
| Received March 24, 2012; Accepted April 12, 2012; Published April 18, 2012 |
| |
| Citation: Paulino MHM, Néstor MS, Enrique KA, Jaime GM (2012) Retrorectal
Chordoma: Case Report and Literature Review. J Clinic Case Reports 2:121.doi:10.4172/2165-7920.1000121 |
| |
| Copyright: © 2012 Paulino MHM, et al. This is an open-access article distributed
under the terms of the Creative Commons Attribution License, which permits
unrestricted use, distribution, and reproduction in any medium, provided the
original author and source are credited. |
| |
| Abstract |
| |
| Objective: To present a case of retrorectal chordoma and a literature review of this condition. |
| |
| Introduction: Tumors of the retrorectal area are rather rare. Retrorectal lesions can be cystic or solid, benign or
malignant, and can be classified as congenital, neurogenic, osseous or miscellaneous. Diagnosis is frequently delayed
until the tumors reach considerable size. Wide en bloc resection should be performed once the diagnosis is made. |
| |
| Case report: A 63 year old male patient, who complaint of progressive and recent onset constipation, and light
pain at the coccigeal area. At the digital examination of the rectum we appreciate a firm, smooth presacral mass
swelling the posterior rectal wall, with intact rectal mucosa. The MRI showed a multilobulated retrorectal mass; and the
patient underwent to a wide en bloc resection of the tumor. The histopathological study of the specimen corroborates
the presence of chordoma. |
| |
| Conclusions: Retrorectal tumors are rare; their diagnosis is difficult and late. Treatment is surgical with wide
resection; chordomas have poor sensitivity to radiotherapy and chemotherapy. |
| |
| Keywords |
| |
| Retrorectal tumors; Sacrococcygeal chordoma; Presacral
tumors |
| |
| Introduction |
| |
| The retrorectal space can harbor heterogeneous and rare lesions
that may be silent and are thus difficult to diagnose. Tumors of the
retrorectal area are rather rare; their incidence has been estimated to
be 1 in 40,000 hospital admissions [1,2]. Retrorectal lesions can be
cystic or solid, benign or malignant, and can be classified as congenital,
neurogenic, osseous or miscellaneous [2,3]. |
| |
| The sacrococcygeal chordoma although rare is the most common
malignancy of this area. Approximately one half of these lesions is
located in the sacrococcygeal area, predominates in men rather than
woman and is rarely founded in young patients. |
| |
| Diagnosis is not infrequently delayed until the tumors reach
considerable size. The relative rarity and anatomical position of
retrorectal tumors may lead to difficulty in diagnosis and surgical
treatment 4. Most surgeons encounter few of these tumors during their
professional life; we present a case of a patient with a retrorectal tumor
and constipation as a main symptom. |
| |
| Case Report |
| |
| A 63 year old male patient with history of chronic obstructive
pulmonary disease secondary to chronic cigarette smoking that comes
to evaluation for a recent apparition and progressive constipation and
light pain at the coccigeal area. He notes that his constipation starts two
months ago, with straining at stool and denies bleeding, weight loss,
and family history of cancer. At the physical examination he looks thin,
with barrel chest, cyanosis in the nail beds, and expiratory wheezes, but
with good general appearance. Abdominal region soft and not tender
with no palpable organs or masses, inguinal area with no palpable
nodes, perineal region appears normal, but at the digital examination
of the rectum we appreciate a firm, smooth presacral mass swelling the
posterior rectal wall, but with intact rectal mucosa at the rectoscopy
confirming the extrarectal origin of the tumor. Colonoscopy discards
other intra-colonic lesions, and the carcinoembryonic antigen was
normal. Due to the presence of a presacral mass we perform a MRI
that showed a multilobulated retrorectal mass localized below S5, with
this findings our preoperative impression was limited to a retrorectal tumor, (biopsy should be avoided unless the lesion seems unresectable)
(Figure 1). At pulmonologist evaluation they founded in chest films
hyperinflation with increased lucency of the lungs, and low flattened
diaphragms, SaO2 84% and at spirometry forced expiratory volume in
one second (FEV1) of 38%, and recommend pulmonary physiotherapy
prior to a surgical procedure. We decide both with the patient planning
a surgical treatment and perform a posterior sagittal approach, with
wide en bloc tumor and coccyx resection (Figures 2-4) under spinal
anesthesia (due to his respiratory condition), thrombosis prophylaxis and complete bowel preoperative preparation, finding a soft,
multilobulated tumor approximately of 10 x 8 x 6 cm, with mucinous
content which was resected in its totality, (resection was complete following the limits of the retrorectal space: the rectum anteriorly, the
pre-sacral fascia posteriorly, and the endopelvic fascia laterally, the
superior border of the space is the posterior peritoneal reflection of the
rectum and the inferior border is Waldeyer fascia) and place a closed
drainage at the resection site which was removed on the third day, with
good outcome and resolution of his symptoms (constipation) and at a
15 month follow up without recurrence. The histopatological study of
the piece confirms chordoma (conventional type) (Figure 5). |
| |
|
Figure 1: MRI with retrorectal mutilobulated tumor, below S5. |
|
| |
|
Figure 2: Posterior sagittal approach. A multilobulated tumor can be appreciated. |
|
| |
|
Figure 3: En bloc resection of the tumor and coccyx. |
|
| |
|
Figure 4: Surgical specimen. |
|
| |
|
Figure 5: Histopatologyc study demonstrates the presence of physaliferous
cells that confirms chordoma. |
|
| |
| Discussion |
| |
| Retrorectal space is located anterior to the sacrum, and posterior
to the rectum, its superior boundary is the peritoneal reflection, and
inferior boundary the rectosacral fascia. Lesions in this area can arise
from a variety of tissues, (this is an area of embryological fusion
between hindgut and proctodeum, neural elements and bone) so a good
knowledge of the embryology and anatomy of this space is essential to
provide appropriate treatment [5]. |
| |
| Retrorectal lesions can be classified as either congenital or
neoplastic. Congenital lesions represent 40% and neoplastic lesions
60%. Developmental cysts are the most common retrorectal cystic
lesions in adults, occurring mostly in middle-aged women [6]. The
Mayo clinic reported that 43% of all retrorectal tumors were malignant
[7]. |
| |
| The first description of a sacrococcygeal chordoma was in 1900
by Henning. The sacrococcygeal chordoma is a tumor arising from
remnants of the notochord [8-10], characterized by a slow and
progressive growth that usually spans a period of years. Its invasion is
by direct extension. Usually thought like a local disease but has been
demonstrated distant metastases in > 40% of cases. The usual sites of
distribution of chordoma are 50% sacrococcygeal, 35% sphenooccipital
and vertebral in 15% [8]. The male: female ratio is 2:1 [8,11], and over
one half of the lesions occur in persons aged 50-70 years [12]. The
principal symptom is pain in 82% of the cases [10,13], symptoms are
produced as the tumor proliferates, often reaching considerable size
before diagnosis is made [10,14], the second most common symptom
is constipation. If a malignant tumor invades sacral roots (S2-S4), fecal
or urinary incontinence may ensue [13]. |
| |
| Chordomas are lobulated, pseudoencapsulated, gelatinous masses,
with variable consistency, the lesions are malignant and tend to be
locally aggressive, they often spread along the nerve roots in the sacral plexus, and usually do not infiltrate adjacent organs but recurrent
tumors have this tendency [5]. Microscopically the large vacuolated
cells (physaliferous cells) are characteristic of this tumor [8]. |
| |
| Nearly all of the patients have a palpable retrorectal mass on digital
examination, so rectal examination is therefore the most important,
most effective, and least expensive means to identify the tumor.
Endoscopic and barium enemas can show extrinsic compression. Plain
pelvic radiographs could show bone destruction in sacrum or coccyx.
Endoluminal ultrasound can help to distinguish solid from cystic
lesions. CT or IRM should be performed to confirm the presence of
a retrorectal mass and to evaluate not only the tumor size but also its
spatial relationships with pelvic organs and sacrum to decide the best
approach for surgery. |
| |
| It is recommended that all the retrorrectal lesions be extirpated,
although they be benign and without symptoms [2]. Chordomas have
poor sensitivity to radiotherapy and chemotherapy; they mainly are
treated by surgery [8,15,16]. Complete removal of the tumor at the time
of initial surgery is important for good prognosis. Based on CT or MRI
findings, a surgical plan can be made for tumor excision, small and low
lying (below level of S3) can be removed by a posterior approach [17].
If the upper pole of the tumor extends above the S3 level, and anteriorposterior
approach is preferred [9]. In some cases of benign retrorectal
tumors, laparoscopic approach has been reported [18]. |
| |
| The value of biopsy before curative resection is controversial. There
are some reports which indicate that biopsy may cause seeding of
tumor through otherwise unaffected tissue planes [19]. |
| |
| Metastasis is rare, and when it occurs is usually to lymph nodes,
lungs and liver. The incidences of pulmonary and bone metastases
could be high in patients with long term follow-up [8]. Recurrence
after curative resection is frequent and may lead to a slow but relentless
progression until death due to invasion of local pelvic structures [13].
In cases of recurrence, reexcision is a reasonable therapeutic option. |
| |
| Conclusions |
| |
| Retrorectal tumors are rare; their diagnosis is difficult and late.
Once the diagnosis is made, surgical therapy is mandatory even if
the patient is asymptomatic, wide en bloc resection should improve
survival, and decrease recurrence rates. Chordomas have poor
sensitivity to radiotherapy and chemotherapy. |
| |
|
| References |
| |
- Whittaker LD, Pemberton JD (1938) Tumors ventral to the sacrum. Ann Surg 107: 96-106.
- López Cano M, Vilallonga R, Espin Basany E, Sánchez García JL, Lozoya Trujillo R, et al. (2006) Lesiones retrorrectales en adultos. Experiencia en 5 casos. Cir Esp 80: 334-336.
- Jarboui S, Jarraya H, Ben Mihoub M, Abdesselem MM, Zaouche A, et al. (2008) Retrorectal cystic hamartoma associated with malignant disease. Can J Surg 51: E115-E116.
- Localio SA, Eng K, Ranson JHC (1980) Abdominosacral approach for retrorectal tumors. Ann Surg 191: 555-559.
- Christensen MA, Blatchford GJ (1998) Presacral tumors in adults, chapter 24. In Fundamentals of anorectal surgery, 2nd edn, WB Saunders company Ltd, 400-413.
- Dahan H, Arrivé L, Wendum D, Ducou le Pointe H (2001) Retrorectal developmental cysts in adults: Clinical and Radiologic-Histopatologic review, differential diagnosis and treatment. Radio Graphics 21: 575-584.
- Jao SW, Beart RW Jr, Spencer RJ, Reiman HM, Ilstrup DM (1984) Retrorectal tumors: Mayo Clinic experience, 1960-1979. Dis Colon Rectum 28: 644-652.
- Yonemoto T, Tatezaki S, Takenouchi T, Ishii T, Satoh T, et al. (1999) The surgical management of the sacrococcygeal chordoma. Cancer 85: 878-883.
- Dozois RR (1990) Retrorectal tumors: Spectrum of the disease, diagnosis and surgical management. Perspect Colon Rectal Surg 3: 241-255.
- Alfonzo Núñez R, Díaz Blancofombona I, Sierra Mileo JC (2007) Cordoma sacrococcígeo. A propósito de un caso. Rev Mex de Coloproct 13: 53-58.
- Smith J, Ludwig RL, Marcove RC (1987) Sacrococcygeal chordoma: A clinicoradiological study of 60 patients. Skeletal Radiol 16: 37-44.
- Gray SW, Singhabhandhu B, Smith RA, Skandalakis JE (1975) Sacroccocygeal chordoma: report of a case and review of the literature. Surgery 78: 573-582.
- Böhm B, Milsom JW, Fazio WV, Lavery IC, Church JM, et al. (1993) Our approach to the management of congenital presacral tumors in adults. Int J Colorectal Dis 8: 134-138.
- Healey JH, Lane JM (1989) Chordoma: a criteria review of diagnosis and treatment. Orthop Clin North Am 20: 417-426.
- Chandawarkar RY (1996) Sacrococcygeal chordoma. Review of 50 consecutive patients. World J Surg 20: 717–719.
- Bethke KP, Neifeld JP, Lawrence W Jr (1991) Diagnosis and management of sacrococcygeal chordoma. J Surg Oncol 48: 232–238.
- Sabuncuoglu H, Ozdogan S, Dogan H, Ataoglu O, Timurkaynak E (2010) Total resection of inferiorly located sacral chordoma with posterior only approach: case report and review of the literature. Turk Neurosurg 4: 527-532.
- Gunkova P, Martinek L, Dostalik J, Gunka I, Vavra P, et al. (2008) Laparoscopic approach to retrorectal cyst. World J Gastroenterol 14: 6581-6583.
- Jao SW, Beart RW, Spencer RJ, Reima HM, Ilstrup DM (1985) Presacral tumors. Mayo Clinic experience, 1960-1979. Dis Colon Rectum 28: 644-652.
|
| |
| |
|
|
|
This article |
DOWNLOAD |
|
CONTRIBUTE |
|
SHARE |
|
EXPLORE |
|
 |
 |
| |
|
| |
| |
| |
|
Untitled Document
|
|
|
|
|
