Year: 2026 | Month: August | Volume: 16 | Issue: 8 | Pages: 166-183
DOI: https://doi.org/10.52403/ijhsr.20260818
Clinical Presentation, Surgical Management and Outcomes in Complex Neurofibromatosis: An Observational Study Done in a Tertiary Care Institute in Eastern India
Tirtha Raj Manna1, Sibaji Dasgupta2
1Resident, 2Assistant Professor, Department of Neurosurgery,
Bangur Institute of Neurosciences (BIN), IPGME&R & SSKM Hospital, Kolkata
West Bengal University of Health Sciences, West Bengal, India.
Corresponding Author: Dr. Tirtha Raj Manna
ABSTRACT
Background: Complex neurofibromatosis defines a subset of disorders characterized by varied clinical presentations and therapeutic challenges. The group includes individuals with Neurofibromatosis Type 1 (NF1), Neurofibromatosis Type 2 (NF2) and schwannomatosis who exhibit diverse clinical manifestations ranging from benign cutaneous tumors to severe neurological complications with systemic involvement.
Methods: The research was a prospective observational study done on 50 patients diagnosed with complex neurofibromatosis who underwent neurosurgical evaluation and treatment at a tertiary care institute in Eastern India over a study period of two years. The research aimed to characterize clinical presentations, surgical management and outcomes in complex neurofibromatosis.
Results: Benign pathology like neurofibromas and schwannomas was the most common diagnosis. Malignant peripheral nerve sheath tumors were detected in 4% of patients, indicating malignant transformation. Gross total resection was achieved in the majority of patients, followed by near total resection and subtotal resection. Motor functions and sensory functions were improved in 56% and 50% of the patients respectively, in the postoperative period which was statistically significant as tested by Wilcoxon signed-rank test (W = 75.0, p < 0.001 for motor functions and W = 113.0, p = 0.007 for sensory function). Statistical analysis with Spearman rank correlation test showed weak (non-significant; p=0.218) positive correlation between tumor size and preoperative pain score. The correlation between extent of tumor resection and size of tumor (Fisher–Freeman–Halton exact test; p=0.0000788) was found to be statistically significant.
Conclusion: This research concluded regarding consideration of tumor size in assessing extent of surgical resection in complex neurofibromatosis. It gave us a detailed understanding of the clinical manifestation, surgical indications and safety of the surgical management of complex neurofibromatosis in a tertiary care institute in Eastern India.
Key words: Neurofibromatosis, schwannomatosis, neurofibroma, complex neurofibromatosis, schwannoma, malignant peripheral nerve sheath tumors