Coccygectomy for refractory coccydynia, old-fashioned but effective procedure: A retrospective analysis.

PURPOSE: Coccydynia, characterized by persistent pain in the coccygeal region, significantly impacts patients' quality of life. While various treatment modalities exist, including conservative measures and surgical interventions like coccygectomy, optimal management remains unclear. This retrospective cohort study aimed to compare the clinical outcomes, functional improvements, and quality of life in patients with chronic coccydynia undergoing either infiltrative treatment or coccygectomy. METHODS: Data from patients treated at our institution from January 2018 to December 2022 were analyzed. Participants meeting inclusion criteria were divided into two groups: Group A underwent coccygectomy, while Group B received conservative therapy. Clinical assessments, radiographic evaluations, and patient-reported outcomes were collected preoperatively and at follow-up intervals. RESULTS: Of the 223 initially examined patients, 55 met inclusion criteria. Group A (n = 21) underwent coccygectomy, while Group B (n = 34) received conservative therapy. Both groups showed significant pain reduction post-intervention, with sustained improvement in Group A. Functional outcomes favoured Group A, with significant improvements in disability and quality of life measures. Complications were minimal, with only one case of superficial wound infection in Group A. CONCLUSION: Our findings suggest that coccygectomy provides superior and lasting pain relief, functional improvement, and quality of life improvement compared to conservative therapy. While complications were minimal, further research with larger cohorts is warranted to validate these results and explore long-term outcomes. Despite its historical association with complications, advancements in surgical techniques and perioperative care have led to improved outcomes and reduced complication rates. Thus, coccygectomy should be considered in the treatment algorithm for patients with debilitating coccydynia.

[Coccygodynia-An often underestimated clinical picture]. / Kokzygodynie ­ ein häufig unterschätztes Krankheitsbild.

One of the most frequently underestimated symptoms in orthopedic practices is coccygodynia. The clinical picture of coccygodynia was first described in 1859. Nevertheless, discussions about the importance of coccygeal problems remain a controversial topic. All patients have in common that they have gone through a long ordeal with many therapists, examinations and therapeutic approaches without having received a real diagnosis. The main symptom of coccygodynia is pain directly on the lowest segment of the coccyx, which often only occurs when sitting and is intensified by a change in position, usually from a sitting to a standing position. Trauma can only be described as the trigger in 50% of cases. Women are four times more likely to be affected than men. The gold standard for imaging should be viewed from standing and seated dynamic lateral radiographs of the coccyx. After the diagnosis has been made, conservative treatment should first be started with oral nonsteroidal anti-inflammatory drugs (NSAID), relief with a coccyx cushion with a recess and, if necessary, physiotherapy to strengthen or loosen the pelvic floor. Local infiltration with a glucocorticoid and a local anesthetic directly in the painful area are also often promising. If the symptoms persist for more than 6 months, surgical treatment in the sense of removing the coccyx can be discussed with the patient. The literature shows a success rate of 80-90% if the indications are correct.

Minimally Invasive Open Reduction and Maintenance Technique for Anterior Sacrococcygeal Dislocation: A Case Report.

Anterior dislocation of the coccyx is rare, but it can occur due to trauma. Conservative treatment is usually performed. However, dislocation reduction may be required to control severe pain in the acute phase or to prevent chronic complications. If manual reduction fails, open reduction is required. The extent of the incision and the method used to maintain the reduction should be considered during open reduction. A 56-year-old male patient experienced a dislocation of the sacrococcygeal joint after falling backwards. Despite conservative treatment, the patient complained of persistent pain during sitting and when using the bathroom. A manual reduction was attempted but failed. We performed joystick reduction via minimal incision and maintained the reduction using a one-strand trans-osseous suture passing through the skin. The patient was advised to use a soft cushion when sitting or lying down for four weeks after surgery. The supine position was not restricted. The patient's symptoms significantly improved after surgery. At the 6-month follow-up, the sacrococcygeal joint showed good alignment and no surgical complications occurred. During the treatment of sacrococcygeal dislocation, the rapid alleviation of acute pain and minimizing potential complications are key points. If open reduction is needed, the minimally invasive reduction technique with a one-strand trans-osseous suture may offer patient satisfaction and a good surgical outcome.

A novel radiological classification for displaced os coccyx: the Benditz-König classification.

BACKGROUND: Treatment of coccygodynia is still a challenging entity. Clear surgical selection criteria are still lacking. The aim of the investigation was to establish a novel radiological classification for surgical decision-making in coccygodynia cases. MATERIAL AND METHODS: Retrospective analysis of standing and sitting X-rays of coccygodynia patients referred to a single centre from 2018 to 2020. The sacro-coccygeal angle (SCA), the intra-coccygeal angle (ICA) and the difference of the intervertebral disc height (∆IDH) were measured. All coccyges were distributed in subtypes and correlated with the patients' treatment. RESULTS: In total, 138 patients (female/male: 103/35) with a mean age of 45.6 ± 15.4 years were included in the study. In total, 49 patients underwent coccygectomy. Four different subtypes of displaced coccyges were identified: Type I with a non-segmented coccyx, anterior pivot, increased SCA and ICA from standing to sitting, ∆IDH = 1.0 ± 1.5 mm. Type II with a multisegmented coccyx, anterior pivot, increased SCA and ICA standing/sitting, ∆IDH = 1.1 ± 1.6 mm. Type III showed a posterior pivoted coccyx, negative SCA and ICA, ∆IDH = 0.6 ± 1.6 mm. Type IV is characterized by an anterior-posterior dissociation of the tail bone with a positive SCA, and the ICA shifted from a posterior to an anterior orientation. ∆IDH was - 0.6 ± 1.8 mm. CONCLUSION: The presented radiological classification could help to facilitate the surgical decision-making for patients with displaced os coccyx. In addition, lateral and sitting X-rays were easy to perform and did not need unnecessary ionizing radiation like in CT scans and were more cost-effective than MRI investigations. The subtypes III and especially IV were more likely leading to surgery.

Coccygectomy for refractory coccygodynia: a systematic review and meta-analysis.

PURPOSE: We sought to systematically assess and summarize the available literature on outcomes following coccygectomy for refractory coccygodynia. METHODS: PubMed, Scopus, and Cochrane Library databases were systematically searched in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Data concerning patient demographics, validated patient reported outcome measures (PROMs) for pain relief, disability outcomes, complications, and reoperation rates were extracted and analyzed. RESULTS: A total of 21 studies (18 retrospective and 3 prospective) were included in the quantitative analysis. A total of 826 patients (females = 75%) received coccygectomy (720 total and 106 partial) for refractory coccygodynia. Trauma was reported as the most common etiology of coccygodynia (56%; n = 375), followed by idiopathic causes (33%; n = 221). The pooled mean difference (MD) in pain scores from baseline on a 0-10 scale was 5.03 (95% confidence interval [CI]: 4.35 to 6.86) at a 6-12 month follow-up (FU); 5.02 (95% CI: 3.47 to 6.57) at > 12-36 months FU; and 5.41 (95% CI: 4.33 to 6.48) at > 36 months FU. The MCID threshold for pain relief was surpassed at each follow-up. Oswestry Disability Index scores significantly improved postoperatively, with a pooled MD from baseline of - 23.49 (95% CI: - 31.51 to - 15.46), surpassing the MCID threshold. The pooled incidence of complications following coccygectomy was 8% (95% CI: 5% to 12%), the most frequent of which were surgical site infections and wound dehiscence. The pooled incidence of reoperations was 3% (95% CI: 1% to 5%). CONCLUSION: Coccygectomy represents a viable treatment option in patients with refractory coccygodynia.

Anococcygeal Nerve and Sitting Pain: Differential Diagnosis and Treatment Results.

BACKGROUND: The plastic surgeon is often asked to reconstruct the sacral area related to pilonidal cysts or a tumor, or after other surgery, such as coccygectomy. When sitting pain is not due to the pudendal or posterior femoral cutaneous nerve injury, the anococcygeal nerve (ACN) must be considered. Clinically, its anatomy is not well known. Rather than consider coccygectomy when the traditional nonoperative treatment of coccydynia fails, resection of the ACN might be considered. METHODS: A review of traditional anatomy textbooks was used to establish classical thoughts about the ACN. A retrospective cohort of patients with sitting pain related to the coccyx was examined, and those operated on, by resecting the ACN, were examined for clinicopathologic correlations. RESULTS: When the ACN is described in anatomy textbooks, it is with varying distributions of innervated skin territory and nerve root composition. Most include an origin from sacral 5 and coccygeal 1 ventral roots. Most agree that the ACN forms on the ventral side of the sacrum/coccyx, alongside the coccygeus muscle, to emerge laterally and travel dorsally to innervate skin over the coccyx and lower sacrum. A review of 13 patients with sitting pain due to the ACN, from 2015 to 2019, demonstrated a mean age of 54.6 years. Eleven were female. The etiologies of ACN injury were falls (9), exercise (3), and complication from surgery (1). Six of the 9 patients who had surgery were able to be followed up with a mean length of 36.3 months (range, 11-63 months). Overall, 3 had an excellent result, 2 had a good result, and 1 was not improved. The one with a failed result showed improvement with coccygectomy. CONCLUSIONS: The ACN must be included in the differential diagnosis of sitting pain. It is most often injured by a fall. The ACN can be evaluated with a diagnostic nerve block, can be identified at surgery, and can be resected, and its proximal end can be implanted into the coccygeus muscle. This surgery may prove an alternative to coccygectomy.

[Coccygodynia: Case Reports and Literature]. / Coccygodynie: vlastní kazuistiky a prehled literatury.

Coccygodynia, or tailbone pain, is the most common in women after trauma (complicated childbirth, fall). This pain can be treated conservatively (by using analgesics, local injections, physiotherapy) or by surgical coccygectomy. In the presented article, a set of five female patients is evaluated, in whom, after the failing conservative therapy, coccygectomy was indicated for persistent coccygodynia. In all female patients, improvement of their clinical condition and alleviation of pain were reported. Coccygectomy has its place in the management of coccygodynia and in correctly chosen patients significant pain reduction can be expected. Key words: coccygodynia, coccyx, coccygectomy, trauma.

Conservative treatment for chronic coccydynia: a 36-month prospective observational study of 115 patients.

PURPOSE: To describe long-term outcomes of conservative treatment for chronic coccydynia. METHODS: We conducted a 36-month prospective observational study. Adults with chronic coccydynia (> 2 months) were included. The first-line treatment was coccygeal corticosteroid injection. The second-line treatment was either manual therapy or coccygectomy. The primary endpoint was the mean variation from baseline of coccydynia intensity at 6 and 36 months, using a numeric rating scale (0, no pain; 10, maximal pain). Evolution was considered unfavorable when coccydynia intensity was > 3 of 10 points at 36 months or coccygectomy had been performed. We carried out bivariate and multivariate analyses to identify variables associated with an unfavorable evolution. RESULTS: We included 115 participants. Mean (SD) age was 43.5 (12.3) years, duration of coccydynia 18.4 (21.6) months and coccydynia intensity 6.5 (2.0) of 10 points. Mean variations for coccydynia intensity were - 1.5 (3.0) at 6 months and - 2.8 (3.2) at 36 months. At 36 months, 59/115 (51%) participants had an unfavorable evolution. In bivariate analysis, posterior coccyx dislocations were numerically more frequent in participants with an unfavorable evolution compared to others (29/59 (48%) versus 17/56 (30%), p = 0.057). In multivariate analysis, longer duration of coccydynia was associated with an unfavorable evolution (OR = 1.04, 95% CI from 1.01 to 1.07, p = 0.023). CONCLUSION: In adults with chronic coccydynia receiving conservative treatment, symptoms decrease overtime, but significantly persist at 36 months in more than half of them. For patients with posterior coccyx dislocation, coccygectomy may be considered rapidly.

Coccygodynia Due to a Sacrococcygeal Anomaly: A Rare Cause of Constipation.

ABSTRACT: We report a case of an uncommon sacrococcygeal anomaly in a healthy girl initially presenting to the emergency department with coccygodynia and a past history of longstanding constipation. The clinical evolution was satisfactory once the bony anomaly was removed (coccygectomy). This unusual case exemplifies the importance of the medical history and physical examination to make an accurate diagnosis. An inadequate intervention may result in persistent pain, worsening longstanding constipation, and psychosocial and medical consequences.

Pedunculated Perianal Mass in a Neonate: A Rare Case Report.

Congenital perianal pedunculated masses are a rare occurrence in neonates. They are benign in nature and can be excised completely for definitive diagnosis. Here is a report of a rare case of pedunculated perianal mass occurring in a neonate managed successfully.

[Undetected coccyx fracture in a woman with fibromyalgia]. / Unentdeckte Os-coccygis-Fraktur bei Patientin mit Fibromyalgiesyndrom.

This article presents the case of a 46-year-old woman with fibromyalgia with an undetected fracture of the coccyx. The heterogeneity of the symptoms of patients suffering from fibromyalgia including chronic widespread pain, vegetative and functional disorders, mental and physical exhaustion as well as sleep disorders can cause accidentally undetected comorbidities, especially if these are rare and predominantly present with pain as the main symptom. In the present case the reason for symptoms was detected only after 14 months of ineffective therapies and diagnostic procedures. The coccygeal pain was eliminated through a coccygectomy as ultima ratio. It should be nevertheless emphasized that patients with fibromyalgia suffer from a central pain-processing disorder. Indications for operative treatment must be very carefully considered. Surgery should only be considered in consultation with the patient and after failed conservative therapy.

Resection of a Perirectal Leiomyosarcoma via a Posterior Transcoccygeal Approach.

INTRODUCTION: The management of perirectal tumors often requires rectal wall resection, and sometimes a complete proctectomy is required. Access to posterior perirectal masses via a posterior, transcoccygeal approach (Kraske procedure) avoids dissection of the intraperitoneal rectum. PATIENT: The patient was a 63-year-old male who presented to his primary care physician with debilitating perirectal pain of several months' duration. He did not respond to therapy with pain medications and topical agents, and underwent a lateral internal sphincterotomy for what was thought to be an anal fissure, without relief prior to referral. Diagnostic workup showed a low signal intensity mass on magnetic resonance imaging (MRI), and biopsy revealed high-grade leiomyosarcoma with myxoid features. Staging workup included a contrast-enhanced computed tomography chest, abdomen and pelvis, flexible sigmoidoscopy and endoscopic ultrasound. A lytic lesion in his left ilium on MRI was found to be avid on fluorodeoxyglucose-positron emission tomography scan and was therefore consistent with oligometastatic disease. He received six cycles of adriamycin, cyclophosphamide and dacarbazine, with good response. The metastatic lesion was treated with 24 Gy of radiotherapy, while the primary tumor was treated with 50 Gy of radiotherapy. The patient underwent the Kraske approach with radical resection of the perirectal mass. The rectal wall was closed with interrupted silk sutures, and layered closure of incision over a drain was performed. An R0 resection was achieved. A laparoscopic diverting loop ileostomy to protect the rectal repair was performed. CONCLUSION: The Kraske approach allows for adequate resection, while avoiding the morbidity of the transabdominal approach, and allowing the patient to maintain a continent rectum.

The Estimate of the Impact of Coccyx Resection in Surgical Field Exposure During Abdominal Perineal Resection Using Preoperative High-Resolution Magnetic Resonance.

OBJECTIVE: To estimate the improvement in surgical exposure by removal of the coccyx, during abdomino-perineal resection (APR), in rectal cancer patients. METHODS: Retrospective study of 29 consecutive patients with rectal cancer was carried out. Using MR T2 sagittal series, the solid angle was estimated using the angle determined by the anterior resection margin and the tip of coccyx (no coccyx resection) or the tip of last sacral vertebra (coccyx resection). The solid angle provides an estimate of the tridimensional surface area provided by an original angle resulting in the best estimate of the surgeon's view/exposure to the critical dissecting point of choice (anterior rectal wall). The difference ("Gain") in surgical field exposure by removal of the coccyx was compared by the solid angle variation between the two estimates (with and without the coccyx). RESULTS: Routine removal of the coccyx determines an average 42% (95% CI 27-57%) gain in surgical field exposure area facing the anterior rectal wall at the level of the prostate/vagina by the surgeon. Fifteen (51%) patients had ≥30% (median) estimated gain in surgical field exposure by coccygectomy. There was no association between BMI, age or gender and estimated gain in surgical field exposure area. CONCLUSIONS: Routine removal of the coccyx during APR may result in an average increase in 42% in surgical field exposure during APR's perineal dissection. Precise estimation of surgical field exposure gain by removal of the coccyx may be predicted by MR sagittal series for each individual patient.

A Review of Current Treatment Options for Coccygodynia.

PURPOSE OF REVIEW: Coccygodynia is pain within the coccyx area. The diagnosis is made clinically with symptoms of pain in the coccyx region and worsening pain in sitting position. The initial treatment is conservative therapy. For patients who do not respond to conservative therapies, there are further interventions available. This includes local injection of local anesthetics and steroids, neurolysis of sacral nerve roots, caudal epidural block, pulse radiofrequency (PRF), intra-rectal massage and manipulation, ganglion impar block, levator ani massage and stretching, coccyx manipulation, and coccygectomy. The purpose of this review is to evaluate the efficacies of these interventions in the treatment of coccygodynia. RECENT FINDINGS: Literature search was performed with the keywords including coccygodynia, treatment, and coccygectomy, on PubMed and Google Scholar between August 2012 and August 2017. Thirteen studies with patients age 18 and over who underwent treatments for coccygodynia were selected for analysis. These treatments include conservative therapies (physical therapy and capsaicin patch), interventional techniques (local injections with steroids and local anesthetic, pulsed radiofrequency ablation of ganglion impar, extracorporeal shock wave therapy), and surgical techniques (complete and partial coccygectomies). The results from these studies demonstrated that most patients had significant pain relief with these techniques. Our literature review demonstrated various interventions including coccygectomy can be effective in the treatment of coccygodynia refractory to conservative therapies. There is a growing body of clinical evidence to support that coccygectomy is an effective treatment for patients with debilitating pain who had failed interventional therapies. Further randomized control studies should be conducted to examine duration of pain relief after coccygectomy and associated surgical complications.

Use of intraoperative lateral pelvic X-ray to localize and ensure coccyx removal during sacrococcygeal teratoma resection.

En bloc removal of the coccyx during sacrococcygeal teratoma resection is necessary to decrease the risk of recurrence. However, variable anatomy often makes the border between the coccyx and sacrum difficult to identify. We describe the use of intraoperative lateral pelvic X-ray to localize this border and ensure complete coccygectomy.

Coccygodynia review: coccygectomy case series.

BACKGROUND: Coccygodynia is a pain of the coccyx that is typically exaggerated by pressure. Management includes anti-inflammatory medications, physiotherapy, and coccyx manipulation. Coccygectomy is the surgical approach for treating coccygodynia when the conservative management fails. Generally, coccygectomy yields good results. Its most common complication is wound infection. OBJECTIVE: To determine the effectiveness of coccygectomy in patients with coccygodynia. METHODS: A retrospective review of 70 patients (52 females and 18 males) with coccygodynia at King Khalid University Hospital in Riyadh was carried out, and the outcomes were studied. Twenty patients did not respond to conservative management; therefore, bimanual coccyx manipulation was done. Eleven were identified with instability and did not respond to coccygeal manipulation. Coccygectomy was performed on 8 patients while 3 declined. RESULTS: All patients who underwent coccygectomy showed improvement of their symptoms. One case of superficial wound infection and delayed wound healing was encountered. CONCLUSION: Coccygectomy provides effective pain relief to patients not responding to conservative therapies.

Diagnosis and treatment of a rectal-cutaneous fistula: a rare complication of coccygectomy.

BACKGROUND: Coccygectomy may be indicated for the treatment of debilitating coccygodynia unresponsive to non-operative treatment. Perineal contamination and postoperative wound infection following coccygectomy remains a major concern. We present a rare post-coccygectomy complication of rectal-cutaneous fistula. To our knowledge no such case has been previously described. CASE PRESENTATION: A 24-year-old woman presented with recurrent wound infections 1 year after coccygectomy at another institution, which persisted despite two surgical debridements and antibiotic treatment. Wound cultures showed non-specific poly-microbial bacterial growth. MRI scan of the spine and pelvis revealed a sinus track and soft tissue edema with no evidence of abscess or osteomyelitis. Methylene blue injection to the sinus tract confirmed the presence of a rectal-cutaneous fistula. The patient underwent further debridement, fistulectomy and synchronous defunctioning colostomy and resection of the involved colon segment. The wound healed by secondary intention with complete resolution of the infection. Re-anastomosis and closure of the colostomy was performed 6 months later. At 2-year follow-up, the patient had no signs of infection and her initial coccygeal symptoms had improved. CONCLUSION: Postoperative infection following coccygectomy remains a major concern. A discharging sinus at the surgical site may suggest the presence of a rectal-cutaneous fistula, which requires a combined approach of spinal and colorectal surgeons. Methylene blue injection to the sinus tract may facilitate the diagnosis of a rectal-cutaneous fistula.

A rare cause of recalcitrant coccydynia: benign dermoid cyst masquerading as coccygeal pain.

PURPOSE: Coccydynia is a common entity in orthopedic practice, and various etiologies have been described for it. However, benign dermoid cyst causing coccydynia has not yet been reported. METHODS: A 20-year-old male presented with typical symptoms of coccydynia recalcitrant to conservative treatment for 2 years. Since pain interfered with his daily activities, magnetic resonance imaging was performed which showed a circumscribed precoccygeal cystic lesion. RESULTS: The patient underwent coccygectomy along with cyst excision. Histological examination revealed features of benign dermoid cyst. After surgery, the patient had excellent relief of his symptoms. CONCLUSION: The case report identifies that the treating surgeon should be aware of benign dermoid cyst as one of the treatable but rare causes of intractable coccydynia, and MRI should be performed in patients with persistent coccygeal pain.

Coccygectomy for coccygeal spicule: a study of 33 cases.

PURPOSE: To report the results of coccygectomy for coccygeal spicule. METHODS: We report the results of a retrospective series of 33 patients who underwent coccygectomy for coccygeal spicule. There were 31 women and 2 men. The mean age was 42 ± 12 years (range 23-62). There was a pit in the skin overlying the spicule in 14 cases and the spicule was bulky in 8 cases. In three cases, weight loss had preceded the occurrence of the coccygodynia. The coccyx was rigid or had very reduced mobility (less than 5°) in 25 cases and normal mobility (between 5° and 20° of flexion) in 8 cases. All the patients had initially been managed conservatively with injections targeted on the spicule. As they did not obtain sufficient relief, they were offered surgery. Ten patients were followed up for more than 72 months, 10 patients for 48-66 months, and 13 for 30-42 months. The outcome analysis involved functional criteria only. RESULTS: Twenty-six patients (79%) had a very satisfactory outcome and 7 (21%) an unsatisfactory outcome. When asked 'Would you have the surgery again?', only one patient answered in the negative. CONCLUSIONS: Surgical treatment for coccygeal spicules that are causing coccygodynia and are resistant to conservative treatment gives satisfactory outcomes, similar to those obtained from surgery for instability of the coccyx.

Surgical outcomes in adults with benign and malignant sacrococcygeal teratoma: a single-institution experience of 26 cases.

BACKGROUND: Adult sacrococcygeal teratomas are rare, and limited data exist on their management and outcomes following surgery. OBJECTIVE: The aim of this study was to review the outcomes in adult patients undergoing surgery for sacrococcygeal teratomas. DESIGN: A retrospective review of our prospectively maintained surgical pathology and tumor registries was conducted. SETTING: This study was conducted at the Mayo Clinic in Rochester. Information was collected on patients treated between 1980 and 2013. PATIENTS: A total of 26 patients with sacrococcygeal teratoma were identified (19 female), with a median age of 37.5 years. Malignancy was seen in 5 patients. MAIN OUTCOME MEASURES: Data on demographics, clinical presentation, tumor pathology, adjuvant therapy, surgical approach, surgical margins, use of preoperative biopsy, radiological investigations, morbidity, mortality, and local recurrence was collected. Complications were assessed by using the Clavien-Dindo system of classification. RESULTS: Patients most commonly presented with pelvic pain (n = 16) and/or a palpable mass (n = 15). On radiology, 8 lesions were purely cystic, 14 were mixed, and 4 were solid; teratoma was suspected as a diagnosis in 8 patients. Preoperative biopsy (13 patients) had 100% concordance with final pathology. Median tumor size was 6 cm, and the surgical approach was posterior only (n = 15), anterior only (n = 5), and combined anterior-posterior (n = 6). Of 5 patients with malignancy, 3 died of recurrent disease. LIMITATIONS: Limitations of this study include the small number of patients, the long study period, and the heterogeneous nature of these tumors. CONCLUSION: Presacral teratomas require multidisciplinary management and have a risk of malignant transformation. They are more common in females, and the majority are intrapelvic in location in adults. We recommend clinical evaluation, radiological investigation, and image-guided biopsy in all suspicious presacral lesions. A treatment algorithm has been designed to improve the management of these rare tumors.