Surgical management of malignant melanotic nerve sheath tumors: an institutional experience and systematic review of the literature
Departments ofNeurosurgery and
Search for other papers by Abdul Karim Ghaith in
jns
Google Scholar
PubMed
Departments ofNeurosurgery and
Search for other papers by Sarah E. Johnson in
jns
Google Scholar
PubMed
Departments ofNeurosurgery and
Search for other papers by Victor Gabriel El-Hajj in
jns
Google Scholar
PubMed
Search for other papers by Oluwaseun O. Akinduro in
jns
Google Scholar
PubMed
Departments ofNeurosurgery and
Search for other papers by Marc Ghanem in
jns
Google Scholar
PubMed
Search for other papers by Gaetano De Biase in
jns
Google Scholar
PubMed
Search for other papers by Loizos Michaelides in
jns
Google Scholar
PubMed
Departments ofNeurosurgery and
Search for other papers by Antonio Bon Nieves in
jns
Google Scholar
PubMed
Search for other papers by W. Richard Marsh in
jns
Google Scholar
PubMed
Search for other papers by Bradford L. Currier in
jns
Google Scholar
PubMed
Search for other papers by John L. Atkinson in
jns
Google Scholar
PubMed
Search for other papers by Robert J. Spinner in
jns
Google Scholar
PubMed
Departments ofNeurosurgery and
Search for other papers by Mohamad Bydon in
jns
Google Scholar
PubMed
OBJECTIVE
Malignant melanotic nerve sheath tumors are rare tumors characterized by neoplastic melanin-producing Schwann cells. In this study, the authors report their institution’s experience in treating spinal and peripheral malignant melanotic nerve sheath tumors and compare their results with the literature.
开云体育世界杯赔率
数据收集从8个病人surgical treatment for malignant melanotic nerve sheath tumors between 1996 and 2023 at Mayo Clinic and 63 patients from the literature. Time-to-event analyses were performed for the combined group of 71 cases to evaluate the risk of recurrence, metastasis, and death based on tumor location and type of treatment received. Unpaired 2-sample t-tests and Fisher’s exact tests were used to determine statistical significance between groups.
RESULTS
在1996年至2023年之间,恶性患者8米elanotic nerve sheath tumors underwent surgery at the authors’ institution, while 63 patients were identified in the literature. The authors’ patients and those in the literature had the same mean age at diagnosis (43 years). At the authors’ institution, 5 patients (63%) experienced metastasis, 6 patients (75%) experienced long-term recurrence, and 5 patients (62.5%) died. In the literature, most patients (60.3%) were males, with a peak incidence between the 4th and 5th decades of life. Nineteen patients (31.1%) were diagnosed with Carney complex. Nerve root tumors accounted for most presentations (n = 39, 61.9%). Moreover, 24 patients (38.1%) had intradural lesions, with 54.2% (n = 13) being intramedullary and 45.8% (n = 11) extramedullary. Most patients underwent gross-total resection (GTR) (n = 41, 66.1%), followed by subtotal resection (STR) (n = 12, 19.4%), STR with radiation therapy (9.7%), and GTR with radiation therapy (4.8%). Sixteen patients (27.6%) experienced metastasis, 23 (39.7%) experienced recurrence, and 13 (22%) died. Kaplan-Meier analyses showed no significant differences among treatment approaches in terms of recurrence-free, metastasis-free, and overall survival (p > 0.05). Similar results were obtained when looking at the differences with respect to intradural versus nerve root location of the tumor (p > 0.05).
CONCLUSIONS
Malignant melanotic nerve sheath tumors are rare tumors with a high potential for malignancy. They carry a dismal prognosis, with a pooled local recurrence rate of 42%, distant metastasis rate of 27%, and mortality rate of 26%. The findings from this study suggest a trend favoring the use of GTR alone or STR with radiation therapy over STR alone. Mortality was similar regardless, which highlights the need for the development of effective treatment options to improve survival in patients with melanotic schwannomas.
ABBREVIATIONS
GTR = gross-total resection ; STR = subtotal resection .
开云体育平台上