Pediatric extraspinal sacrococcygeal ependymoma (ESE): an Italian AIEOP experience of six cases and literature review

Background: Primary pediatric extraspinal sacrococcygeal ependymoma (ESE) is a very rare disease, poorly described in literature, whose diagnostic, therapeutic, and follow-up approach is still controversial. Methods: We describe six cases of pediatric ESE treated at Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) centers in Italy since 1983, with a review of the literature. Results: All six patients had primary sacrococcygeal disease (two presacral and four subcutaneous) with median age of 10 years. Three patients were males, and two of them are metastatic at diagnosis; 3/6 had myxopapillary ependymoma grade I and 3/6 had classic ependymoma grade II. Five patients underwent surgical resection with complete removal only in one case with coccygectomy. Adjuvant chemoradiotherapy was administered to one metastatic patient obtaining a complete remission. Two patients relapsed at 3 and 8 years from diagnosis: they were treated with salvage chemotherapy (high-dose sequential chemotherapy with myeloablative regimen in one case), surgery, and radiotherapy achieving complete remission (CR). All six patients are in complete continuous remission (CCR) at a median follow-up of 12.8 years. Conclusions: Pediatric patients with this peculiar disease need to be referred to specialized pediatric cancer centers that can provide multidisciplinary treatment after a centralized pathology review. Our experience highlights the role of chemotherapy and radiotherapy in adjuvant and relapse setting. The final prognosis is relatively optimistic, but with a careful follow-up due to the high risk of recurrence

A patient with neurofibroma (schwannoma) in peri-sacral: A case report

Schwannomas are benign tumors of the nerve sheath and are usually single encapsulated and slow growing in peripheral or sympathetic nervous system. In this report a 49 year-old man is presented with one year complain of abdominal pain and intermittent hematospermia. The CT scan of the abdomen showed a 60 × 65 × 60 mm mass in anterior pelvic cavity with deviation to the sacral bone, originated from nerve. Several examinations revealed neurofibroma. Due to the large size of the tumor and it's position to the pelvic nerves, to remove the mass the patient only underwent laparotomy with partial resection. Pathology tests confirmed Ancient Schwannoma with degenerative changes. Radiotherapy was done with the aim of reducing the size of the rest of tumor. In our case, schwannoma was diagnosed incidentally. The size of the tumor indicated a relatively long period from the time that tumor was generated until the time of diagnosis. Despite using paraclinical findings, a definite diagnosis of the disease was made by histopathological tests. © 2015, Mazandaran University of Medical Sciences. All rights reserved

Sacral tumours on MRI : a pictorial essay

Tumours of the sacrum can be primary or secondary. Since the sacrum is rich in haematopoietic bone marrow, bone metastases are the most frequent aetiologies. However, tumours can arise from all components of the sacrum and primary bone tumours should be considered in case of a solitary lesion and absence of oncologic history. As the clinical signs are usually non-specific, magnetic resonance imaging has become an indispensable tool in narrowing the differential diagnosis and determining the therapeutic approach. This pictorial essay illustrates specific features of the most common sacral tumours on magnetic resonance (MR) imaging

Presacral Myelolipoma

Many reports have described adrenal myelolipomas but there have been only a few reports of extra-adrenal myelolipomas. We describe a 74-year-old woman who came to our observa- tion for MRI of the lumbar spine for typical lumbar back pain. In addition to signs of mild scoliosis and spondylo disc arthrosis, MR imaging revealed a presacral mass showing a heterogeneously high signal in all pulse sequences and almost completely suppressed on inversion recovery sequences for fat tissue. CT imaging confirmed the fatty nature of the lesion and no signs of bone involvement. These findings were most consistent with a diagnosis of a rare presacral myelolipoma as confirmed at histopathologic analysis. This work reports a case of one of the rarest presacral masses, empha- sizing the role of imaging in the differential diagnosis of other presacral masses

A qualitative study of the development of a multidisciplinary case conference review methodology to reduce involved margins in pelvic exenteration surgery for recurrent rectal cancer

Aim Pelvic exenteration surgery remains the only curative option for recurrent rectal cancer. Microscopically involved surgical margins (R1) are associated with a higher risk of local recurrence and decreased survival. Our study aimed to develop a post hoc multidisciplinary case conference review and investigate its potential for identifying areas for improvement. Method Results Patients who underwent pelvic exenteration surgery for recurrent rectal cancer with R1 resections at a tertiary referral centre between April 2014 and January 2016 were retrospectively reviewed from a prospectively maintained database. Patients with non-rectal cancers or who underwent palliative surgery were excluded. Cases, imaging and histopathology were evaluated by a dedicated panel including colorectal surgeons, an abdominal radiologist and a gastrointestinal pathologist. R1 resections were reported in 32 of 110 pelvic exenterations. Patients with other tumours were excluded and one patient had a palliative resection. Nine male patients with 11 exenterations were included with a median age of 56 years. All patients had positive soft tissue margins, and one patient also had an involved bony margin. Failures were due to (interdisciplinary) communication problems, specific management of tumour biology (multifocality, spiculated tumours), which can lead to radiological undercalling, and inadequate surgical technical planning. In hindsight, surgery would have been withheld from one patient. Conclusion A retrospective multidisciplinary case evaluation of pelvic exenteration patients with involved surgical margins led to a list of recommendations which included the need to plan for wider surgical soft tissue resections and improvement in interdisciplinary communication. Lessons learned may increase clear margin rates in future resections

The posterior approach for low retrorectal tumors in adults

Background and aims: Retrorectal tumors are uncommon in adults and arise in different tissues in the presacral space. The aim of this study is to evaluate early complete surgical resection by a perineal approach as the therapy-of-choice for tumors under the sacral promontory. Patients and methods: We evaluated the posterior approach, especially intersphincteric and parasacrococcygeal excisions, in terms of resectability, morbidity, risk of recurrence, and anal function. The records of all patients who underwent a posterior surgical procedure in our institution for low-lying retrorectal tumors between 1994 and 2003 were reviewed. Results: Sixteen patients (13 women and three men) were included in this study. The age range was 21 to 57years (median of 37years). Pathological findings included ten tailgut cysts, three teratomas, one leiomyoma, one dermoid cyst, and one schwannoma. Complete tumor resection was obtained in 15 patients. There was one case with a microscopic residual tumor. No postoperative mortality was seen, and a minor complication occurred in one patient. There was no anal dysfunction. The postoperative course was uneventful, with only one tumor recurrence at 5months. The median follow up was 60months (ranging from 18 to 132months). Conclusion: In this study, the posterior approach allows complete resection of low retrorectal tumors, with low morbidity, no incontinence, nearly no recurrence, and no mortalit

Transanal total mesorectal excision with triangle rules: a road map to prevent injuries

[Extract] Transanal total mesorectal excision (TaTME) is presented as a promising new surgical technique for the approach to the distal rectum, providing the ability to perform a highquality resection, with technical advantages over the laparoscopic approach. However, the new surgical anatomy of the TaTME resection complicates surgical understanding and increases the risks of inadvertent injuries to important anatomic structures...info:eu-repo/semantics/publishedVersio

Tumores presacros. Reporte de serie de casos

Introducción: Los tumores presacros son un raro y diverso grupo de lesiones que se originan en el espacio presacro, infrecuente y de difícil diagnóstico. El objetivo de este trabajo es describir una serie de casos con respecto a las características clínicas, diagnóstico y manejo. Metodología: Recolección de pacientes quienes fueron diagnosticados con tumores presacros desde el año 1988 a 2013. Revisamos la clínica, tiempo de evolución, métodos diagnósticos, el abordaje quirúrgico, patología y complicaciones. Resultados: Nueve pacientes fueron incluidos en este trabajo. 7 de los 9 pacientes presentaron como síntoma principal el dolor. El tiempo de evolución medio fue de 9.8 meses. Diagnóstico fue realizado con tacto rectal en todos los pacientes y confirmado con tomografía axial pélvica 9 pacientes y resonancia magnética nuclear pélvica en 5 pacientes. La patología: teratoma quístico maduro (n = 3), cordoma (no: 1), quiste epidermoide (no=1), fibrohistiocitoma de bajo grado (no =1), fibromatosis (no =1), angiomixoma agresivo (no =1). El abordaje quirúrgico principal fue la vía posterior de (kraske) en 6 pacientes y en 2 por laparotomía. 1 paciente no se llevó a cirugía. La resección completa se obtuvo en un 87%. No se presentó complicaciones tempranas postoperatorias. Complicaciones tardías en 1 caso (12%) por obstrucción intestinal. Se presentó 1 caso de recurrencia por resección parcial tumoral. Conclusión: Los tumores presacros son una patología infrecuente a nivel mundial, siendo un reto para el cirujano su diagnóstico y manejo.Background: Presacral tumors are a rare and diverse group of lesions that originate in the presacral space. Given its rarity, it is difficult to reach an early diagnosis of tumors presenting as difficult to manage. The aim of this paper is to describe a series of cases with respect to clinical features, diagnosis and management. Methodology: Collection of patients who were diagnosed with presacral tumors since 1988-2013. We reviewed the clinical, evolution, diagnosis, surgical approach, pathology and complications. Results: Nine patients were included in this study. 7 of the 9 patients had primary symptom is pain. The average evolution time was 9.8 months. Diagnosis was made with digital rectal examination in all patients and confirmed in 9 patients pelvic CT and pelvic MRI in 5 patients. Pathology: mature cystic teratoma (n = 3), chordoma (no: 1), epidermoid cyst (no = 1), low-grade fibrous histiocytoma (no = 1), fibromatosis ( no = 1), aggressive angiomyxoma (no = 1). The main surgical approach was the posterior (Kraske) in 6 patients and in 2 by laparotomy. 1 patient had no surgery took. Complete resection was obtained in 87%. No early postoperative complications present. Late complications in 1 patient (12 %) for intestinal obstruction. Was only 1 case of partial resection tumor recurrence. Conclusion: Presacral tumors are an uncommon worldwide, being difficult to diagnosis and management.Hospital Militar Central, Universidad Del Rosari