Abdominopelvic neurofibromatosis with malignant peripheral nerve sheath tumor
Presentation
Pelvic pain and concern for a mass.
Patient Data
Lobulated, mildly enhancing confluent masses in the right subdiaphragmatic/perihepatic space extending behind the IVC/caudate lobe. Smaller areas of fusiform low-density thickening in the lower thoracic intercostal space. Low attenuation masses arising from the lumbar neural foramina extending into the psoas muscles. Numerous other small low attenuation masses in the presacral space branching into the upper right thigh/gluteal tissues, also along the right prostate margin.
Large, irregular enhancing extraperitoneal mass in the left pelvis extending along the sidewall and involving the iliacus/iliopsoas muscles.
Case Discussion
This case presented as an unknown case with a suspected pelvic mass, but with further questioning, the patient had cutaneous manifestations of neurofibromatosis. Viewing the unusual abdominopelvic findings with this lens, it becomes clear - there is a large, plexiform neurofibroma in the right subdiaphragmatic space, numerous other neurofibromas with some plexiform branching into the upper right thigh, and finally the left pelvic mass which appears more aggressive and irregular than these other processes. This raises concern of malignant transformation - e.g. a malignant peripheral nerve sheath tumor, and requires further evaluation with biopsy.
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