Schwannome présacré: à propos d’un cas

Abstract

Introduction : Les schwannomes sont des tumeurs bénignes neurogènes localisées dans la région sacrée dans 1 à 5 % des cas. Les auteurs rapportent un cas de neurinome présacré. Observation : Une patiente de 55 ans a consulté pour des douleurs pelviennes évoluant depuis un an sans signes de compression digestive ou urinaire. Les touchers pelviens avaient objectivé une masse rétrorectale avec une muqueuse rectale saine. L'imagerie avait évoqué le diagnostic de neurinome. L'exploration chirurgicale avait permis d'objectiver une masse tissulaire rétrosacrée qui a bénéficié d'une exérèse en monobloc. L'histologie a conclu à un neurinome bénin. Discussion : Les schwannomes présacrés géants sont des tumeurs rares. Du fait de leur symptomatologie pauvre, leur diagnostic est souvent retardé. L'imagerie, TDM mais surtout IRM, est intéressante dans le diagnostic des neurinomes ainsi que leurs rapports avec les organes de voisinage ; elle permet de déceler également des signes d'agressivité de la tumeur mais ne peut affirmer de façon formelle la nature bénigne ou maligne de la tumeur. Le traitement repose sur l'exérèse ou l'énucléation de la tumeur. Le pronostic est bon, mais entaché par la survenue de récidives. Conclusion : Devant des douleurs pelviennes, le diagnostic de neurinome présacré est rarement posé. L'imagerie (IRM) permet le diagnostic et guide la stratégie thérapeutique. Mots clés Schwannome présacré · TDM-IRM-traitementAbstract Introduction: Presacral schwannomas are rare benign tumors, representing 1% to 5% of all schwannomas. We report a case diagnosed by imaging modalities (CT-MRI) and treated by complete surgical resection. Case report: A 55-year-old female has had a history of abdominal pain since one year without compressive signs. A pelvic retrorectal mass has been discovered clinically. CT and MRI were very suggestive of a presacral schwannoma. Histological examination after complete surgical removal of the mass confirmed the diagnosis of a benign schwannoma. Discussion: Giant presacral schwannomas are rare pelvic tumors. Often asymptomatic, they are diagnosed lately. CT and MRI are very useful in assessing the diagnosis and evaluating the extent of the tumor. Surgery is the therapeutic modality of first choice. Prognosis is good if the tumor is benign but recurrence is frequent. Conclusion: Presacral schwannoma can cause pelvic pain. Imaging modalities (MRI++) often guide surgical resection.

Retrorectal Schwannomas: Atypical Presentation and Controversial Surgical Management

“…Accompanying symptomatology is often nonspecific, including pain or pelvic mass syndrome and signs of compression of neighboring organs such as constipation and low back pain [ 3 ], which is the case with our patient. During Physical examination, signs such as “café au lait” spots, freckling of the axillae, or inguinal regions should be searched since they may be present in VRH syndrome [ 3 ]. Digital rectal examination reveals an intact rectal mucosa with a retrorectal firm, regular, insensitive mass.…”

“…These tumors occur without predominance of sex in the 20- to 50-year age group [ 1 ]. Because of their location, deep schwannomas such as prerectal schwannomas usually reach a large size before becoming symptomatic, and it is estimated that patient can be asymptomatic for 7 years [ 3 , 5 ]. Accompanying symptomatology is often nonspecific, including pain or pelvic mass syndrome and signs of compression of neighboring organs such as constipation and low back pain [ 3 ], which is the case with our patient.…”

“…Because of their location, deep schwannomas such as prerectal schwannomas usually reach a large size before becoming symptomatic, and it is estimated that patient can be asymptomatic for 7 years [ 3 , 5 ]. Accompanying symptomatology is often nonspecific, including pain or pelvic mass syndrome and signs of compression of neighboring organs such as constipation and low back pain [ 3 ], which is the case with our patient. During Physical examination, signs such as “café au lait” spots, freckling of the axillae, or inguinal regions should be searched since they may be present in VRH syndrome [ 3 ].…”

“…Malignant change is uncommon, yet malignant tumors associated with von Recklinghausen's disease have been reported [ 1 ]. Retrorectal schwannomas are extremely rare as they represent only 1 to 5% of all schwannomas [ 3 ]. These tumors are found most commonly in the head, neck, and extremities, while only 0.5–5% occur in the retrorectal space [ 4 ], representing 1 in 250 of all retrorectal tumors [ 5 ].…”

“…These tumors are found most commonly in the head, neck, and extremities, while only 0.5–5% occur in the retrorectal space [ 4 ], representing 1 in 250 of all retrorectal tumors [ 5 ]. They can be asymptomatic and incidentally revealed by a pelvic or a rectal examination; however, they can be revealed by pelvic pain, back pain, lower extremities pain, or constipation [ 3 , 5 ]. Imaging tests can be suggestive of schwannomas and help to identify the exact location of the mass.…”