Degenerated (Ancient) Schwannomas: Unraveling Unusual Locations and Treatment Management
Öz
Background/Aims:Schwannomas, originating from Schwann cells in peripheral nerve sheaths, exhibit diverse clinical manifestations and unpredictable behavior. Among them, ancient schwannomas, with distinctive degenerative features, present in atypical locations and pose diagnostic challenges. This study explores their unique characteristics and implications for diagnosis and management.
Material and Methods: The study was designed as a retrospective analysis of 7 adult patients aged ≥18 years, diagnosed with paraspinal, presacral, sacral, or para-aortic tumors, all confirmed histologically as ancient schwannomas. Data for analysis were collected from patients treated and followed up at Ankara City Hospital between April 2017 and December 2022. Ethical approval and informed consent were obtained before inclusion in the study.
Results: The most common symptoms included the presence of a local lump (71,4%) and localized or radiating pain (57,1%). Notably, 1 patient (14,2%) was incidentally diagnosed during the evaluation process. Surgical intervention played a crucial role in the management of these tumors, with 71,4% of patients undergoing complete resection, while 28,5% underwent subtotal resection. Tumor recurrence occurred in 42,8% of cases, prompting the administration of radiotherapy as part of the treatment strategy. Imaging findings, particularly on MRI, played a crucial role in the accurate identification of ancient schwannomas. These tumors displayed isointensity on T1-weighted images and heterogeneous hyperintensity on T2-weighted images, along with distinctive heterogeneous contrast enhancement.
Conclusion: Ancient schwannomas exhibit unique histological features and distinctive MRI characteristics, differentiating them from other nerve sheath tumors. Early diagnosis and complete surgical resection offer favorable outcomes. Awareness of this entity is essential for managing peripheral nerve sheath tumors effectively.
Anahtar Kelimeler
Schwannoma, Ancient, Benign tumor, neural tumors, Schwann cells.
Kaynakça
- Helbing DL, Schulz A, Morrison H. Pathomechanisms in schwannoma development and progression. Oncogene. 2020 Aug;39(32):5421–9.
- Alotaiby F. Ancient Schwannoma: Case Report of an Unusual Entity in an Unusual Oral Location. Am J Case Rep. 2022 Dec 23;23:e938335.
- Ribeiro da Cunha P, Alves JL, Rocha A. Supra and infratentorial ectopic schwannoma mimicking a meningioma. BMJ Case Rep. 2017 Jan 17;2017:bcr2016216566.
- Röhrich M, Koelsche C, Schrimpf D, Capper D, Sahm F, Kratz A, et al. Methylation-based classification of benign and malignant peripheral nerve sheath tumors. Acta Neuropathol (Berl). 2016 Jun;131(6):877–87.
- Nakarai H, Kato S, Yamato Y, Kodama H, Ohba Y, Sasaki K, et al. Quality of Life and Postoperative Satisfaction in Patients with Benign Extramedullary Spinal Tumors: A Multicenter Study. Spine. 2023 Jul 6;
- Lee SK, Kim JY, Lee YS, Jeong HS. Intramuscular peripheral nerve sheath tumors: schwannoma, ancient schwannoma, and neurofibroma. Skeletal Radiol. 2020 Jun;49(6):967–75.
- Ratnagiri R, Mallikarjun S. Retroperitoneal ancient schwannoma: two cases and review of literature. J Cancer Res Ther. 2014;10(2):368–70.
- Rodriguez FJ, Folpe AL, Giannini C, Perry A. Pathology of Peripheral Nerve Sheath Tumors: Diagnostic Overview and Update on Selected Diagnostic Problems. Acta Neuropathol (Berl). 2012 Mar;123(3):295–319.
- Gakuhara A, Miyazaki Y, Kurokawa Y, Takahashi T, Yamasaki M, Makino T, et al. Laparoscopic distal gastrectomy for gastric cancer with simultaneous resection of para-aortic schwannoma. Asian J Endosc Surg. 2020 Jan;13(1):95–8.
- Antonopoulos C, Lilimpakis C, Karagianni M, Daskalopoulou D, Kyriakou T, Vagianos C. Ancient schwannoma of lumbar spine and review of the literature on paraspinal tumors, the role of preoperative biopsy: a case report. Cases J. 2009 Dec 15;2:9325.