Malignant Peripheral Nerve Sheath Tumor: MRI and CT Findings

Abstract

This is a case report of a 56-year-old male with hypertension who presented with urinary retention and bowel incontinence. CT and MRI of the abdomen and pelvis showed a large complex cystic and solid enhancing mass in the right presacral space. Pathology biopsy result showed malignant peripheral nerve sheath tumor (MPNST) with extensive necrosis. The diagnosis of MPNST is extremely difficult due to the lack of (1) conclusive immunohistochemistry or unique chromosomal anomaly, (2) universal distinctive histopathology, and (3) clinical criteria. The clinical, radiologic, and histologic presentation of MPNST is important in its diagnosis. A rare case of MPNST that produced urinary retention and bowel incontinence is presented that may aid clinicians in the diagnosis of this rare clinical entity. Motor weakness, central enhancement, and immunohistochemistry may assist in the diagnosis of MPNST and differentiation between benign peripheral nerve sheath tumor (BPNST) and MPNST.

Malignant peripheral nerve sheath tumours in magnetic resonance imaging: primary and recurrent tumour appearance, post-treatment changes, and metastases

“…A reason for this may be the earlier detection of the recurrent tumours within the MRI follow-up scans, which are routinely performed at intervals of 3-12 months. The mean diameter of primary MPNST is reported to range from 1 to 20 cm (median size of 8-12 cm) [5,19,20].…”

“…In this study we mainly focused on the MRI appearance of both primary and recurrent MPNST. MPNST presents with high signal intensity in T2 STIR and PD FS and low signal intensity in T1 sequences [5,19,21]. Furthermore, MPNST is reported to appear in different shapes with mostly heterogeneous appearance and well-defined margins [5,19,20].…”

“…MPNST presents with high signal intensity in T2 STIR and PD FS and low signal intensity in T1 sequences [5,19,21]. Furthermore, MPNST is reported to appear in different shapes with mostly heterogeneous appearance and well-defined margins [5,19,20]. MPNST may also present as ovoid with uniform enhancement [19,20].…”

Ressecção De Tumor Maligno Da Bainha Do Nervo Periférico Retroperitoneal: Relato De Caso

“…A imuno-histoquímica pode auxiliar na diferenciação entre o tumor maligno e benigno e apresenta positividade de 50-90% para a proteína S-100, 50% para a proteína básica da mielina, 40% para leu-7 e, frequentemente, mostra positividade para p53, correlacionando-se à agressividade da doença (7)(8) . O prognóstico, em geral, é reservado com taxa de sobrevida de cinco anos, variando entre 34-64% e elevado risco de recorrência (4)(5)(6)(7)(8)(9)(10) . Os fatores de mau prognóstico são localização, dimensão, mais de 5 cm, diferenciação histológica, margens cirúrgicas positivas e presença de NF1 (4)(5) .…”

“…Os fatores de mau prognóstico são localização, dimensão, mais de 5 cm, diferenciação histológica, margens cirúrgicas positivas e presença de NF1 (4)(5) . Em relação ao tratamento, visto que os TMBNP são usualmente resistentes à radioterapia e quimioterapia, a ressecção total deve ser tentada em todos os casos de tumores da parte periférica do sistema nervoso, independente do suposto diagnóstico e das dimensões do tumor, pois se associa a um melhor prognóstico em termos funcionais e sobrevida global (4)(5)(6)(7)(8)(9)(10) . O presente estudo tem como objetivos relatar o caso raro de uma paciente de 44 anos, portadora de TMBNP, sem história prévia de radioterapia e NF1, submetida à ressecção cirúrgica da massa tumoral gigante retroperitoneal e alertar sobre as características clínicas e laboratoriais desse tumor para possibilidade de um diagnóstico precoce por métodos clínicos e melhorar o prognóstico e cura.…”