Pelvic mass in a case of suspected Neurofibromatosis
Posted in Pictures on 17 November 2014
Published by Gabriel Díaz
Gabriel Díaz (Gastrointestinal Surgery Specialist)
Hospital Clínic, Barcelona, Spain
A 30-year-old male with suspected family history of Neurofibromatosis (mother had several tumors resected, by their knowledge they were neurofibromas) presented with non cutaneous tumors at the neck and thorax.
A MRI was ordered to amplify the study, it described 4 main lesions, one at T6-T7 with intra-vertebral component, at T11-T12 with intradural portion that produces a compression of 75% of the medullary canal, one depending of the third trigeminal branch and a mass at the pelvis. There was no evidence of vestibular tumors. The patient did not suffer pain or neurological symptoms.
The tumors resection was performed at different surgical times. The first was the one located at T11-T12 (posterior approach) as it was causing medullary stenosis and in a second procedure the one located at T6-T7 (thoracotomy).
These surgeries were performed uneventfully with good recovery of the patient. The pathology examination was compatible with Schwannomas, a fact that modifies the initial clinical diagnosis. After counseling, the patient was recommended to perform genetic testing for estimation of the risk for his family members and children.
The third surgery will be to treat the pelvic mass, by MRI measures 11x12x10.8 cm and produced displacement of the surrounding organs without signs of infiltration (FIG. 1). It is an heterogeneous tumor with cystic component and necrotic zones that arises suspicion of sarcomatous degeneration.