Variability in Surveillance Strategies Following Resection of Sacrococcygeal Teratoma.

INTRODUCTION: Surveillance following sacrococcygeal teratoma (SCT) resection varies. The purpose of this study was to describe the clinical characteristics and outcomes of patients undergoing SCT resection and examine current institutional practices to detect recurrence. METHODS: A single-institution retrospective review of children who underwent resection of an SCT from January 1, 2010 to December 31, 2020 was performed. Data were summarized and surveillance strategies compared between histopathologic subtypes using nonparametric methods. RESULTS: Thirty six patients (75.0% female) underwent SCT removal at a median age of 8 d. Histopathology revealed 27 mature teratomas (75.0%), eight immature teratomas (22.2%), and one malignant germ cell tumor (2.8%). Median postoperative follow-up was 3.17 y (interquartile range [IQR]: 2.31-4.38 y). Patients had a median of 2.32 clinic visits per year (IQR: 2.00-2.70), alpha-fetoprotein levels were obtained at a median of 2.01 times per year (IQR: 0-1.66), and surveillance imaging was performed at a median of 2.31 times per year (IQR: 0-2.84). Patients with immature teratomas had alpha-fetoprotein laboratories obtained more frequently than patients with mature teratomas (3.10 times/year versus 0.93 times/year, P = 0.001). There was no significant difference in the number of imaging studies obtained between groups. Two patients (5.6%) developed recurrence, which were identified on magnetic resonance imaging at 191 and 104 d postresection, respectively. CONCLUSIONS: Postoperative surveillance practices varied widely. Recurrence was noted in a single malignant case in the first year following resection. Multi-institutional studies are needed to determine the optimal surveillance strategy to detect recurrence of SCT.

Presacral mature cystic teratoma associated with Currarino syndrome in an adolescent with androgen insensitivity: illustrative case.

BACKGROUND: Currarino syndrome is a rare disorder that classically presents with the triad of presacral mass, anorectal malformation, and spinal dysraphism. The presacral mass is typically benign, although malignant transformation is possible. Surgical treatment of the mass and exploration and repair of associated dysraphism are indicated for diagnosis and symptom relief. There are no previous reports of Currarino syndrome in an androgen-insensitive patient. OBSERVATIONS: A 17-year-old female patient presented with lack of menarche. Physical examination and laboratory investigation identified complete androgen insensitivity. Imaging analysis revealed a presacral mass lesion, and the patient was taken to surgery for resection of the mass and spinal cord untethering. Intraoperative ultrasound revealed a fibrous stalk connecting the thecal sac to the presacral mass, which was disconnected without the need for intrathecal exploration. The presacral mass was then resected, and pathological analysis revealed a mature cystic teratoma. Postoperatively, the patient recovered without neurological or gastrointestinal sequelae. LESSONS: Diagnosis of incomplete Currarino syndrome may be difficult but can be identified via work-up of other disorders, such as androgen insensitivity. Intraoperative ultrasound is useful for surgical decision making and may obviate the need for intrathecal exploration during repair of dysraphism in the setting of Currarino syndrome.

Internal fat prolapse from ischiorectal fossa masquerading as currarino syndrome.

Pelvic magnetic resonance imaging (MRI) is the latest addition to already existing tools for evaluation of anorectal malformation (ARM). It provides detailed information about pelvic floor musculature as well as position of rectal pouch. However, lack of knowledge about normal pelvic floor anatomy can lead to misinterpretation of pelvic MRI which can create confusion and change in approach for surgery. A 14-month-old male child with diagnosis of ARM was evaluated with pelvic MRI. There was finding of abnormal protrusion of fat through the ischiorectal fossa which was misinterpreted as Currarino syndrome which created confusion immediately before posterior sagittal anorectoplasty. Pelvic MRI is highly informative while evaluating a case of ARM. However, a detailed knowledge of pelvic floor anatomy is mandatory to avoid wrong interpretation and misdiagnosis.

Currarino syndrome as an incidental radiologic finding in a patient with acute flank pain: A case report.

Currarino syndrome is an autosomal dominant hereditary disease defined as a triad of anorectal abnormality, sacral dysgenesis, and a presacral mass, primarily an anterior sacral meningocele. It is often seen in children and considered rare in adults. It is mostly found as an incidental finding. We present a 21-year-old man who presented with acute flank pain. He had a history of Hirschsprung's disease and therefore had undergone surgery in his infancy. He also had a history of prolonged constipation and had an episode of admission due to suspected obstruction. On physical examination, he had a severe costovertebral angle tenderness. urine exam revealed microscopic hematuria. Laboratory tests were otherwise unremarkable. Computed tomography scan revealed renal stones as well as a horseshoe kidney. Incidental findings included a large simple cystic structure in the presacral area suggestive of an anterior meningocele and sacral dysgenesis associated with scimitar sacral appearance. These findings suggested a diagnosis of Currarino syndrome. Urinary complications of this disease are reported in few articles. An important takeaway note for physicians is to have a high level of suspicion when encountering patients with gastrointestinal, neurologic, or urologic signs and symptoms and consider a thorough history taking and physical examination alongside proper imaging evaluation.

Risk of malignancy and outcomes of surgically resected presacral tailgut cysts: A current review of the Mayo Clinic experience.

AIM: The aim of this study was to describe the surgical management, outcomes and risk of malignancy of presacral tailgut cysts. METHOD: A retrospective analysis of all patients who underwent resection of tailgut cyst at Mayo Clinic in Arizona, Florida and Minnesota between 2008 and 2020 was performed. Demographics, presentation, evaluation, surgical approach, postoperative complications, pathology and recurrence rates were reviewed. RESULTS: Seventy-three patients were identified (81% female) with a mean age of 45 years. Thirty-nine patients (53%) were symptomatic, most commonly with pelvic pain (26 patients). Digital rectal examination identified a palpable mass in 68%. Mean tumour size was 6 cm. Resection was primarily performed through a posterior approach (77%, n = 56), followed by a transabdominal approach (18%, n = 13) and a combined approach (5%, n = 4). Six patients underwent a minimally invasive resection (laparoscopic/robotic). Coccygectomy or distal sacrectomy was performed in 41 patients (56%). Complete resection was achieved in 94% of patients. Thirty-day morbidity occurred in 18% and was most commonly wound related; there was no mortality. Malignancy was identified in six patients (8%). For the 30 patients with follow-up greater than 1 year, the median follow-up was 39 months (range 1.0-11.1 years). Local recurrence was identified in three patients and distant metastatic disease in one patient. CONCLUSION: The rate of malignancy in presacral tailgut cysts based on this current review was 8%. Overall recurrence was 5% at a median of 24 months.

Currarino syndrome: a comprehensive genetic review of a rare congenital disorder.

BACKGROUND: The triad of a presacral mass, sacral agenesis and an anorectal anomaly constitutes the rare Currarino syndrome (CS), which is caused by dorsal-ventral patterning defects during embryonic development. The major causative CS gene is MNX1, encoding a homeobox protein. MAIN BODY: In the majority of patients, CS occurs as an autosomal dominant trait; however, a female predominance observed, implies that CS may underlie an additional mode(s) of inheritance. Often, the diagnosis of CS is established solely by clinical findings, impacting a detailed analysis of the disease. Our combined data, evaluating more than 60 studies reporting patients with CS-associated mutations, revealed a slightly higher incidence rate in females with a female-to-male ratio of 1.39:1. Overall, MNX1 mutation analysis was successful in only 57.4% of all CS patients investigated, with no mutation detected in 7.7% of the familial and 68% of the sporadic patients. Our studies failed to detect the presence of an expressed MNX1 isoform that might explain at least some of these mutation-negative cases. CONCLUSION: Aside from MNX1, other genes or regulatory regions may contribute to CS and we discuss several cytogenetic studies and whole-exome sequencing data that have implicated further loci/genes in its etiology.

Laparoscopic resection of a neuroendocrine tumor that almost fully replaced tailgut cysts: a case report.

BACKGROUND: Neuroendocrine tumors (NETs) originate from neuroendocrine cells, which are found throughout the body. NETs occur principally in the gastrointestinal tract (approximately 65%) and bronchopulmonary tract (approximately 25%) but rarely occur in the presacral space. Aside from primary and metastatic lesions, there have been reports of NETs occurring in the presacral space arising from tailgut cysts, teratomas, and imperforate anus. We herein report a rare case of laparoscopic resection of a NET in the presacral space, which almost fully replaced tailgut cysts. CASE PRESENTATION: A 68-year-old woman was referred to our hospital for surgery of a right inguinal hernia, but preoperative computed tomography revealed an asymptomatic 43-mm mass in the presacral space. Magnetic resonance imaging showed a multilocular solid mass with clear boundaries and a slightly high signal intensity on T1- and T2-weighted images. Positron emission tomography showed 18F-fluorodeoxyglucose uptake. Thus, we suspected a malignant tumor and performed laparoscopic resection to obtain a definitive diagnosis. Macroscopically, the tumor was 43 mm in size with clear boundaries, and the cut surface was a gray-white solid component. Histopathological findings revealed that the tumor was composed of relatively uniform cells with fine chromatin, with round to oval nuclei arranged in solid, trabecular, or rosette-like growth patterns. Small cysts lined with stratified squamous epithelium and columnar epithelium were observed along with solid components of the tumor, which is a feature of tailgut cysts. Therefore, the final diagnosis was NET Grade 1 arising from tailgut cysts. No recurrence was observed within 1 year after surgery. CONCLUSIONS: We performed en bloc laparoscopic resection of a NET arising from tailgut cysts in the presacral space without injury. In cases of a solid lesion in the presacral space, not only the primary disease but also the pathological condition with tissue transformation and replacement should be considered, as in this case.

Currarino syndrome in an elderly man: Multimodality imaging findings.

Currarino syndrome is a rare congenital disorder characterized by the triad of anorectal anomalies, sacrococcygeal dysgenesis and presacral mass. Because of the anorectal anomalies, the extrinsic compression due to the presacral mass and neurologic deficits, patients usually present with gastrointestinal symptoms, most commonly chronic constipation. Most cases of Currarino syndromes are diagnosed in childhood, at birth or in the pre-birth period and, even if adult presentation has been reported in few sporadic case reports, the diagnosis in the late stages of life remains extremely rare. In this paper, we describe the imaging findings of an elderly man with a past medical history of megacolon surgically treated in his childhood, who was diagnosed with Currarino syndrome at the age of 72.

Neurosurgical management of Currarino syndrome: A case series and review of literature.

BACKGROUND: The Currarino syndrome (CS), defined by the triad of anorectal malformations, sacral bone deformities, and presacral masses, is rare. There are few surgical series that discuss conservative management versus the surgical approaches to these lesions. Here, we describe utilizing a combined anterior and posterior approach for resecting these lesions in four patients. METHODS: Four patients with CS were treated with two-stage approaches performed by a multidisciplinary team, including pediatric neurosurgery and general surgery. The first anterior laparoscopic approach mobilized the presacral mass from its ventral attachments. The second posterior procedure detethered the spinal cord, repaired the dural defect, and facilitated removal of the presacral mass. RESULTS: Gross total resection of all four presacral masses was accomplished without intraoperative complication; all patients clinically improved. CONCLUSION: The CS is characterized by a large presacral mass. Here, one must rule out malignancy and also consider diagnosis/resection due to the risks for malignant transformation. The operative approach we described in four patients utilized standard anterior mobilization of the mass, followed by posterior detethering, dural repair, and ultimate resection.

Differentiating presacral masses in anorectal malformations and isolated sacrococcygeal teratomas.

PURPOSE: Presacral masses associated with anorectal malformations (ARM) are most frequently dermoid or teratomas. Sacrococcygeal teratoma (SCT), in isolation, is a different condition. There are limited data comparing the two. The aim of this study was to compare presacral masses associated with ARM and isolated SCTs. METHODS: A retrospective review was performed from 1979 to 2018. Patients with presacral masses and associated ARM (n = 39) were identified and compared to patients with isolated SCTs (n = 32). RESULTS: Those with presacral mass and ARM had a lower proportion of immature and mixed teratomas by pathology and a longer time to mass resection. They had higher percentages of sacral anomalies (76% versus 9%), tethered cord (50% versus 6%), and other anomalies (51% versus 9%). For long-term bowel function, patients with presacral mass and ARM were more likely to be fecally incontinent and require enemas (59% versus 11%). Regarding isolated SCTs, 4% recurred with a time to recurrence of 7 months. CONCLUSIONS: Compared to patients with isolated SCT, patients with presacral mass and ARM had lower grade tumors. In addition, they had a higher proportion of sacral defects and other congenital defects, as well as worse bowel function outcomes.

Anal duplication: is surgery indicated? A report of three cases and review of the literature.

AIM OF THE STUDY: Anal canal duplications (ACDs) are extremely rare with only approximately 90 cases described in the literature. We report on three additional patients. METHODS: Cases were analyzed to evaluate presenting symptoms, physical exam and MRI findings. A comprehensive literature review was performed to compare our patients to previously described cases. IRB approval was obtained for this study (19-0394). MAIN RESULTS: The first female patient presented with an asymptomatic ACD at 2 years old. The second patient was a 13-year-old female with perianal drainage that was initially mistaken for a fistula-in-ano and ultimately found to have an ACD associated with a dermoid cyst. Both posterior midline duplications shared a common wall with the rectum, but did not communicate with it. The ACDs and dermoid cyst were successfully excised through a posterior sagittal approach with no postoperative complications. Histology demonstrated the presence of both squamous epithelium and transitional anal epithelium in each case. The third patient was 8 months old and had a tethered cord, hemisacrum, presacral mass, and anal duplication that was initially undiagnosed. These results corroborate patterns identified in other reports of ACDs with over 90% being female and in the posterior midline. The majority are asymptomatic, but may present with symptoms of local or even systemic infection. CONCLUSION: An opening in the midline posterior to the anus should raise clinical suspicion for anal canal duplication. An associated presacral mass must be ruled out. Complete excision through a posterior sagittal approach is recommended upon diagnosis to avoid symptomatic presentations. The key part of the operation is the separation of the ACD from the posterior rectal wall.

Presacral masses and sacrococcygeal teratomas in patients with and without anorectal malformations: A single institution comparative study.

BACKGROUND: Despite variability at presentation, presacral masses in patients with and without anorectal malformations (ARM) appear histologically similar. The purpose of this study was to identify differences in oncologic outcomes between these two groups. METHODS: A retrospective review was performed utilizing our institutional cancer and colorectal and pelvic reconstruction databases for patients with presacral masses and sacrococcygeal teratomas between 1990 and 2017. Data captured included age at surgical resection, type of ARM, tumor location within the pelvis, tumor histopathology, tumor size, adjuvant chemotherapy, recurrence, and follow-up. RESULTS: Forty-six patients comprised our cohort, of whom 12 had an ARM. The median age was older at resection for those with an ARM (1.4 years; range 1 day to 29.4 years) compared to those without an ARM (9 days; range 0 days to 6.9 years) (p = 0.01). The mean tumor size was 2.5 cm in patients with an ARM compared to 6.0 cm in patients without an ARM (p = 0.036). All patients with ARM had exclusively intrapelvic tumors, and histopathology included mature teratoma (8), yolk sac tumor (1), lipoma (1), and unknown (2). Tumor location for patients with sacral and presacral masses without ARM included exclusively extrapelvic (10), primarily extrapelvic with large intrapelvic component (7), primarily intrapelvic with extrapelvic component (1), exclusively intrapelvic (8), and unknown (8). Histopathology for patients with presacral masses without ARM included mature teratoma (20), immature teratoma (7), yolk sac tumor (3), ganglioneuroma (1), neuroblastoma (1), benign epithelial cyst (1), and unknown (1). Tumor recurrence rate was similar between patients with ARM (n = 3, 25%) and those without an ARM (n = 5, 15%) (p = 0.41). The 5-year event free survival was 65% (95% CI: 25%-87%) in the group with ARM and 81% (95% CI: 60%-92%) in the group without ARM (p = 0.44). CONCLUSION: Sacral and presacral masses in patients with ARM are resected at a later age and are more likely to be intrapelvic. They appear histologically similar and have similar rates of recurrence and malignancy when compared to patients without ARM. LEVEL OF EVIDENCE: III TYPE OF STUDY: Retrospective comparative study.

Laparoscopic Resection of An Extragastrointestinal Stromal Tumor in the Presacral Area.

STUDY OBJECTIVE: To show a surgical video in which a retroperitoneal extragastrointestinal stromal tumor was resected with the laparoscopic approach in the presacral area. DESIGN: A case report (Canadian Task Force classification III). The local ethics committee waived the requirement for approval. SETTING: A very small proportion of extragastrointestinal stromal tumors (EGISTs) is located in the retroperitoneal region. There are insufficient data on the clinical and pathologic features and the long-term follow-up of these tumors [1]. There are a few cases reported in the presacral region in the literature. The gold standard treatment for EGISTs is complete surgical excision of the mass. If it is possible, en bloc excision with its surrounding tissue is very important [2]. PATIENT: A 53-year-old woman. The patient provided informed consent to use images and videos of the procedure. INTERVENTIONS: This is a step-by-step illustration for resection of a retroperitoneal EGIST in the presacral area. The patient was a 53-year-old, gravida 3, para 3 white woman. She presented with abdominal pain for 5 months. Magnetic resonance imaging showed a 4-cm diameter solid mass localized at the presacral area. Laboratory test results for tumor biomarkers were as follows: cancer antigen (CA) 125 = 40 U/mL (normal values <35 U/mL), CA 19-9 = 29 U/mL (normal values <37 U/mL), carcinoembryonic antigen = 2.1 ng/mL (normal values <3 ng/mL), and CA 15.3 = 18 U/mL (normal values <35 U/mL). Because of malignancy suspicion, gastroscopy and colonoscopy were performed and revealed no abnormality. The Papanicolaou smear and endometrial biopsy results were negative. After preparation of the patient, laparoscopy was performed. After placement of an 11-mm umbilical port and three 5-mm abdominal ports, the pelvis and abdomen were explored and revealed a 4-cm retroperitoneal mass in the presacral area. The peritoneum overlying the presacral mass was incised and the retroperitoneum explored. Given the proximity to the mass, left ureterolysis was performed. The mass was located on the left internal iliac vein and dissected carefully. The retroperitoneal attachments were resected, and the presacral mass was removed without any complications. In order not to widen the abdominal incisions, a posterior colpotomy was performed in the cul-de-sac at equal distances from the uterosacral ligaments. An Endobag (Covidien, Dublin, Ireland) was introduced through the 5-mm port site at the posterior colpotomy. The specimen was removed in the Endobag through posterior colpotomy. MEASUREMENTS AND MAIN RESULTS: The procedure was performed without any complications. The patient had an uneventful postoperative course and was discharged home on postoperative day 2. Pathology revealed a 4-cm retroperitoneal EGIST with negative margins. Immunohistochemistry measurements revealed hematoxylin-eosin; CD117; S100 positivity; and CD34, CD68, desmin, and DOG1 negativity. The cell type was mixed (spindle and epithelioid type). The mitotic rate was 1 to 2/50 high-power fields. The patient has been disease free since the completion of surgery. CONCLUSION: Laparoscopic complete resection of the retroperitoneal EGIST in the presacral area is successfully performed in this patient. The patient presented by us is an example showing that minimally invasive procedures can be used in the treatment of this type of tumor in the presacral area. The key point in this operation is to control the relationship of vascular structures and a ureter with a tumor in every step to avoid any injury.

Giant multiple and bilateral presacral Tarlov cysts mimicking adnexal mass - imaging features.

Perineural or Tarlov cysts (TCs) are nerve root cysts. They are usually incidental findings on MRI and are most frequently observed in the sacral spine. A 49-year-old woman presented with lower abdominal discomfort for several months. Physical examination demonstrated a cyst-like mass. An ultrasonographic examination revealed a cystic mass near the right ovary. MRI (3-Tesla unit) showed multiple, bilateral, and almost symmetric giant TCs with presacral space and endopelvic extension and minime scalloping. TCs originated from the right and left L5, S1, S2, and S3 nerve root sleeves entered the presacral space and extended into the pelvis.

The bottom line: MRI and CT findings of unusual rectal and perirectal pathology.

Although common nonspecific symptoms (i.e., rectal bleeding, pelvic pressure, and change in bowel habits) are associated with rectal cancer, occasionally these are related to a different underlying disease. Over the past few years, considerable progress has been made in imaging of the rectum. Specifically, new magnetic resonance techniques and capabilities provide impressive high-resolution assessment of the rectal wall and enable evaluation and characterization of the perirectal tissues. This paper reviews imaging findings of uncommon causes of rectal and perirectal pathology that may be clinically confounded with rectal cancer. Radiologists need to be aware of uncommon pathologies in this region in order to facilitate optimal management decisions.

Síndrome de Currarino como enfermedad infrecuente dentro del diagnóstico diferencial de la patología anexial oncológica / Currarino Syndrome as a Rare Disease in the Differential Diagnosis of Oncological Adnexal Pathology

El Síndrome de Currarino se define como la presencia de una tríada característica que asocia: estenosis anal, malformación sacrococcígea y masa presacra. La escasa sintomatología, caracterizada además por su inespecificidad, provoca que en muchas ocasiones el diagnóstico se realice durante la edad adulta y pueda confundirse con patologías ginecológicas, de origen predominantemente oncológico. El diagnóstico mediante pruebas de imagen, se realiza fundamentalmente a través de la resonancia magnética nuclear. La extirpación quirúrgica de la masa presacra unida al tratamiento sintomático del resto de la triada son la base terapéutica de esta infrecuente patología. Presentamos el caso de una paciente de 20 años de edad con estreñimiento crónico y dolor abdominal inespecífico. Es enviada al Servicio de Ginecología por la presencia de una masa de gran tamaño que se supone de origen anexial. Tras el estudio exhaustivo de dicha paciente, se llegó a la conclusión diagnóstica de que dicha tumoración corresponde a un meningocele, asociado a agenesia parcial sacra y estenosis anal; tríada que define al Síndrome de Currarino.

Currarino Syndrome is defined as the presence of a characteristic triad that associates anal stricture, sacrococcygeal malformation and presacral mass defines this syndrome. The scarce symptomology, also characterized by nonspecific symptoms, conditions that in many cases the diagnosis is made in adulthood and can be confused with gynecological pathologies. The diagnosis is made by imaging tests, fundamentally the NMR (nuclear magnetic resonance). The main treatment is the surgical excision of the mass presacra together with the symptomatic treatment of the rest of symptoms. We present the case of a 20-year-old patient with chronic constipation and abdominal pain that is referred to Gynecology due to the presence of a mass that is supposed to be adnexal. After the study of this patient is diagnosed a meningocele, associated with partial sacral agenesis and anal stricture, triad that defines Currarino Syndrome.

Síndrome de Currarino como enfermedad infrecuente dentro del diagnóstico diferencial de la patología anexial oncológica / Currarino Syndrome as a Rare Disease in the Differential Diagnosis of Oncological Adnexal Pathology

El Síndrome de Currarino se define como la presencia de una tríada característica que asocia: estenosis anal, malformación sacrococcígea y masa presacra. La escasa sintomatología, caracterizada además por su inespecificidad, provoca que en muchas ocasiones el diagnóstico se realice durante la edad adulta y pueda confundirse con patologías ginecológicas, de origen predominantemente oncológico. El diagnóstico mediante pruebas de imagen, se realiza fundamentalmente a través de la resonancia magnética nuclear. La extirpación quirúrgica de la masa presacra unida al tratamiento sintomático del resto de la triada son la base terapéutica de esta infrecuente patología. Presentamos el caso de una paciente de 20 años de edad con estreñimiento crónico y dolor abdominal inespecífico. Es enviada al Servicio de Ginecología por la presencia de una masa de gran tamaño que se supone de origen anexial. Tras el estudio exhaustivo de dicha paciente, se llegó a la conclusión diagnóstica de que dicha tumoración corresponde a un meningocele, asociado a agenesia parcial sacra y estenosis anal; tríada que define al Síndrome de Currarino(AU)

Currarino Syndrome is defined as the presence of a characteristic triad that associates anal stricture, sacrococcygeal malformation and presacral mass defines this syndrome. The scarce symptomology, also characterized by nonspecific symptoms, conditions that in many cases the diagnosis is made in adulthood and can be confused with gynecological pathologies. The diagnosis is made by imaging tests, fundamentally the NMR (nuclear magnetic resonance). The main treatment is the surgical excision of the mass presacra together with the symptomatic treatment of the rest of symptoms. We present the case of a 20-year-old patient with chronic constipation and abdominal pain that is referred to Gynecology due to the presence of a mass that is supposed to be adnexal. After the study of this patient is diagnosed a meningocele, associated with partial sacral agenesis and anal stricture, triad that defines Currarino Syndrome(AU)

Extra-adrenal myelolipoma and extramedullary hematopoiesis: Imaging features of two similar benign fat-containing presacral masses that may mimic liposarcoma.

OBJECTIVE: To describe the imaging characteristics of extra-adrenal myelolipoma and mass-forming extramedullary hematopoiesis (EMH) occurring in the presacral region. MATERIALS AND METHODS: Retrospective review between 1980 and 2015 revealed 11 patients with biopsy-proven presacral extra-adrenal myelolipoma and 11 patients with presacral EMH and radiologic imaging studies. RESULTS: All cases of both myelolipoma and EMH directly abutted the anterior sacrum and were centered on the midline. Myelolipomas were round or bilobed (8/11), or were multilobulated in appearance (3/11). On CT, margins could be smooth or ill defined. On MRI, all masses were well-marginated with evidence of a "capsule". All myelolipomas showed evidence of macroscopic fat on both CT and MRI with scattered hazy and nodular soft tissue components. On MRI, all cases showed areas of high T1 signal intermixed with areas of intermediate T1 signal. On T2, all cases showed areas of low and high T2 signal. Enhancement was usually minimal or mild. Most EMH cases had a well-defined round, bilobed or macro-lobulated presacral mass (9/11); 2/11 cases were ill-defined. On CT, when a dominant fatty component was present, the soft tissue components had hazy and nodular features, similar to myelolipoma. On MRI, the masses demonstrated intermediate T1 signal, isointense to skeletal muscle with varying amounts of hyperintense T1 signal. On T2-weighted images, 3 of 4 cases showed areas of mildly hyperintense T2 signal with varying amounts of dark signal. All patients with clinically or biopsy-proven EMH had a hematologic or myeloid disorder. CONCLUSION: Presacral myelolipoma and presacral EMH are two benign fat-containing masses that can present as discrete, often encapsulated, lipomatous masses with varying amounts of non-lipomatous soft tissue components and should be considered in the differential diagnosis of a well-defined fat-containing presacral mass.

Lymph node metastasis of presacral ependymoblastoma in a young child.

Ependymoblastoma presenting outside the brain is rare in children. The overwhelming majority of presacral ependymal tumors are of the benign myxpapillary type. We present a case of ependymoblastoma in the presacral region of a four-year old child. The patient presented with a presacral mass and ipsilateral inguinal lymph node metastasis. Both masses revealed histologic and immunophenotypic features of ependymoblastoma associated with myxoid areas and high Ki-67 proliferation index. The masses were excised and the patient remained well for a year after diagnosis with no other therapy. This report emphasizes ependymoblastoma as rare entity in the differential diagnosis of presacral tumors in children and, up to our knowledge, the first report of inguinal lymph node metastasis of ependymoblastoma.