Para-adrenal high-grade fibrosarcoma in a young woman with neurofibromatosis type 1: a case report

Abstract Background Neurofibromatosis type 1 (NF1) is a tumor-predisposition syndrome associated with an increased risk of benign and malignant neoplasms. Primary adrenal or para-adrenal mesenchymal malignancies are exceptionally rare and may mimic more common adrenal lesions on imaging. Case presentation A 26-year-old Turkish woman with NF1 was evaluated after routine abdominal ultrasonography raised suspicion of a right adrenal-region mass. Contrast-enhanced magnetic resonance imaging demonstrated a well-circumscribed 62 × 55 mm nonfunctional lesion that was initially interpreted as an adrenal adenoma. Because a clear intraoperative plane between the mass and the adrenal gland could not be identified, the lesion was removed en bloc with the right adrenal gland by laparoscopy. Histopathological examination demonstrated an FNCLCC Grade 3 high-grade fibrosarcoma adjacent to the adrenal gland with negative surgical margins. After multidisciplinary review by Urology and Medical Oncology, close oncological surveillance was recommended. Conclusions Para-adrenal high-grade fibrosarcoma should be considered among the rare differential diagnoses of adrenal-region masses, particularly in patients with tumor-predisposition syndromes such as NF1. Complete oncological resection and multidisciplinary follow-up are essential.