Surgically treated solitary giant gluteal and retroperitoneal neurofibroma: a case report.
BACKGROUND
Giant neurofibromas in patients with neurofibromatosis type 1 involve multiple regions and are often difficult to surgically extirpate. However, surgical intervention is the most effective means for improving quality of life. The case reported herein is unique in that it involves a giant neurofibroma, involving the patient's peritoneal and pelvic cavities, retroperitoneal space, and buttock, which was causing compressive displacement of abdominal and pelvic organs. A challenging surgical intervention was required to accomplish near-total resection to relieve organ compression while preserving visceral and genitoanal function.


METHODS
The case reported is of a patient presenting with a solitary giant retroperitoneal neurofibroma that threatened to obliterate both peritoneal and pelvic cavities and protruded conspicuously into the right gluteal region. The enormous dumbbell-shaped mass was surgically removed in three parts. Postoperative pathology studies confirmed a diagnosis of neurofibroma. Follow-up computed tomography images taken three months postoperatively revealed residual tumor in the perianal region. The patient's quality of life had measurably improved on follow-up at eight months.


CONCLUSIONS
Surgical intervention in such extraordinary circumstances of a giant neurofibroma causing compressive displacement of critical organs reduces tumor burden, restores appearance and function of patient's body and internal organs, and improves the patient's quality of life.
Department of Hand Surgery and Microsurgery Center, The First Affiliated Hospital, College of Medicine, ZheJiang University, HangZhou, China.
Xiang-qian
XQ
Shen
Department of Hand Surgery and Microsurgery Center, The First Affiliated Hospital, College of Medicine, ZheJiang University, HangZhou, China.
Hui
H
Shen
Department of Hand Surgery and Microsurgery Center, The First Affiliated Hospital, College of Medicine, ZheJiang University, HangZhou, China.
Shou-cheng
SC
Wu
The Children's Hospital, Zhejiang University School of Medicine, HangZhou, China.
Ying
Y
Lv
Department of Hand Surgery and Microsurgery Center, The First Affiliated Hospital, College of Medicine, ZheJiang University, HangZhou, China.
Hui
H
Lu
Department of Orthopaedics, The First Affiliated Hospital, College of Medicine, ZheJiang University, 79# Qingchun Road, HangZhou, 310003, ZheJiang province, People's Republic of China. linxjco@outlook.com.
Xiang-jin
XJ
Lin
D000005:Abdomen / Q000473:pathology / Q000601:surgery*; D002081:Buttocks / Q000473:pathology / Q000601:surgery*; D005260:Female; D006801:Humans; D008875:Middle Aged; D009455:Neurofibroma / Q000473:pathology / Q000601:surgery*; D010386:Pelvic Neoplasms / Q000473:pathology / Q000601:surgery*; D011379:Prognosis; D011788:Quality of Life; D012186:Retroperitoneal Neoplasms / Q000473:pathology / Q000601:surgery*; D047368:Tumor Burden
Giant neurofibroma; Gluteal; Neurofibromatosis type 1; Retroperitoneal; Surgical treatment