Abstract
Melanotic schwannomas (MS) are tumors of Schwann cell origin characterized by cytoplasmic deposition of melanin. The authors present the case of a 61-year-old man who experienced progressive weakness of the lower extremities over 2 years. This was followed by acute deterioration, which prompted his presentation. MRI of the spine revealed an intradural, extramedullary lesion at the level of T7 with severe spinal cord compression. More caudally, there was involvement of the lumbar spine with drop metastases to the conus medullaris and cauda equina. The patient underwent thoracic laminectomy for tumor resection. A diagnosis of MS was made based on histologic morphology, immunohistochemical profile, and ultrastructural findings. In this report, the authors describe a MS with expression of CD117 (c-kit) and review the literature pertaining to this condition.
Similar content being viewed by others
References
JP Earls HA Robles HP McAdams et al. (1994) ArticleTitleGeneral case of the day. Malignant melanotic schwannoma of the eighth cranial nerve Radiographics 14 1425–1427
JB Gregorios SM Chou J Bay (1982) ArticleTitleMelanotic schwannoma of the spinal cord Neurosurgery 11 57–60
RM Killeen CL Davy SC Bauserman (1988) ArticleTitleMelanocytic schwannoma Cancer 62 174–183
F Leger C Vital J Rivel et al. (1996) ArticleTitlePsammomatous melanotic schwannoma of a spinal nerve root. Relationship with the Carney complex Pathol Res Pract 192 1142–114
J Handley D Carson J Sloan et al. (1992) ArticleTitleMultiple lentigines, myxoid tumours and endocrine overactivity; four cases of Carney’s complex Br J Dermatol 126 367–371
CM Thornton J Handley EA Bingham et al. (1992) ArticleTitlePsammomatous melanotic schwannoma arising in the dermis in a patient with Carney’s complex Histopathology 20 71–73
CA Utiger JT Headington (1993) ArticleTitlePsammomatous melanotic schwannoma. A new cutaneous marker for Carney’s complex. Arch Dermatol 129 202–204
N Acciarri R Padovani L Riccioni (1999) ArticleTitleIntramedullary melanotic schwannoma. Report of a case and review of the literature Br J Neurosurg 13 322–325
J Sola-Perez M Perez-Guillermo Bas-Bernal A et al. (1994) ArticleTitleMelanocytic schwannoma: the cytologic aspect in fine-needle aspiration cytology (FNAC): report of a case located in the spinal cord Diagn Cytopathol 11 291–296
C Magni P Yapo J Mocaer et al. (1996) ArticleTitlePrimary intramedullary melanoma. Apropos of a case. J Neuroradiol 23 41–45
AV Vallat-Decouvelaere M Wassef G Lot et al. (1999) ArticleTitleSpinal melanotic schwannoma: a tumour with poor prognosis Histopathology 35 558–566
JA Carney (1990) ArticleTitlePsammomatous melanotic schwannoma. A distinctive, heritable tumor with special associations, including cardiac myxoma and the Cushing syndrome. Am J Surg Pathol 14 206–222
RC Janzer M Makek (1983) ArticleTitleIntraoral malignant melanotic schwannoma. Ultrastructural evidence for melanogenesis by Schwann’s cells. Arch Pathol Lab Med 107 298–301
M Roytta J Elfversson H Kalimo (1988) ArticleTitleIntraspinal pigmented schwannoma with malignant progression Acta Neurochir (Wien) 95 147–154
P Hollinger N Godoy M Sturzenegger (1999) ArticleTitleMagnetic resonance imaging findings in isolated spinal psammomatous melanotic schwannoma J Neurol 246 1100–1102
TJ Cummings K Liu LK Jordan SuffixIII et al. (2000) ArticleTitleFine-needle aspiration diagnosis of psammomatous melanotic schwannoma Diagn Cytopathol 23 55–58
JA Carney H Gordon PC Carpenter et al. (1985) ArticleTitleThe complex of myxomas, spotty pigmentation, and endocrine overactivity Medicine (Baltimore) 64 270–283
JA Carney JA Ferreiro (1996) ArticleTitleThe epithelioid blue nevus. A multicentric familial tumor with important associations, including cardiac myxoma and psammomatous melanotic schwannoma. Am J Surg Pathol 20 259–272
JA Carney (1995) ArticleTitleThe Carney complex (myxomas, spotty pigmentation, endocrine overactivity, and schwannomas) Dermatol Clin 13 19–26
RM Lowman VA Livolsi (1980) ArticleTitlePigmented (melanotic) schwannomas of the spinal canal Cancer 46 391–397
RT Miller H Sarikaya A Sos (1986) ArticleTitleMelanotic schwannoma of the acoustic nerve Arch Pathol Lab Med 110 153–154
JA Carney (1995) ArticleTitleCarney complex: the complex of myxomas, spotty pigmentation, endocrine overactivity, and schwannomas Semin Dermatol 14 90–98
PC Gibson K Cooper (2002) ArticleTitleCD117 (KIT): a diverse protein with selective applications in surgical pathology Adv Anat Pathol 9 65–69
RP Mennemeyer KO Hallman SP Hammar et al. (1979) ArticleTitleMelanotic schwannoma. Clinical and ultrastructural studies of three cases with evidence of intracellular melanin synthesis. Am J Surg Pathol 3 3–10
BA Noubari I Chiaramonte G Magro et al. (1998) ArticleTitleSpinal malignant melanotic schwannoma. Case report. J Neurosurg Sci 42 245–249
T Nakai H Rappaport (1963) ArticleTitleA study of the histogenesis of experimental melanotic tumors resembling cellular blue nevi: the evidence in support of their neurogenic origin Am J Pathol 43 175–199
DK Burns FG Silva KA Forde et al. (1983) ArticleTitlePrimary melanocytic schwannoma of the stomach. Evidence of dual melanocytic and schwannian differentiation in an extra-axial site in a patient without neurofibromatosis. Cancer 52 1432–1441
P Napoli P De Domenico (1987) ArticleTitlePrimary melanotic schwannoma of the spinal canal Appl Pathol 5 253–260
D Dastur G Sinh S Pandys (1967) ArticleTitleMelanotic tumor of the acoustic nerve: case report J Neurosurg 27 166–170
C Lumsden (1971) The study by tissue culture tumors of the nervous system D Russel L Rubinstein (Eds) Pathology of Tumors of the Nervous System Williams and Wilkins Baltimore 334
IM Turnbull MI Tom (1963) ArticleTitlePigmented meningioma J Neurosurg 20 76–80
P Masson (1967) ArticleTitleMelanogenic system: nevi and melanomas Pathol Ann 2 351–397
YS Fu GI Kaye R Lattes (1975) ArticleTitlePrimary malignant melanocytic tumors of the sympathetic ganglia, with an ultrastructural study of one Cancer 36 2029–2041
TI Mandybur (1974) ArticleTitleMelanotic nerve sheath tumors J Neurosurg 41 187–192
JJ Hodson (1961) ArticleTitleA morphological study of dermal melanocytes and cytocrine activity in relation to certain Schwann cells and epithelial cells J Pathol Bacteriol 82 267–272
P Masson (1932) ArticleTitleExperimental and spontaneous schwannomas (peripheral gliomas) Am J Pathol 8 367–416
JA Weston (1970) ArticleTitleThe migration and differentiation of neural crest cells Adv Morphol 8 41–114
OA Jensen P Bretlau (1990) ArticleTitleMelanotic schwannoma of the orbit Immunohistochemical and ultrastructural study of a case and survey of the literature. Apmis 98 713–723
KW Gratz M Makek HF Sailer (1991) ArticleTitleMalignant melanotic schwannoma of the oral cavity Int J Oral Maxillofac Surg 20 236–238
R Radin RA Kempf (1995) ArticleTitleCarney complex: report of three cases Radiology 196 383–386
D Rowlands C Edwards F Collins (1987) ArticleTitleMalignant melanotic schwannoma of the bronchus J Clin Pathol 40 1449–1455
P Cras C Ceuterick-de Groote M Van Vyve et al. (1990) ArticleTitleMalignant pigmented spinal nerve root schwannoma metastasizing in the brain and viscera Clin Neuropathol 9 290–294
Hisaoka M H Ohta J Haratake et al. (1991) ArticleTitleMelanocytic schwannoma in the spinal canal Acta Pathol Jpn 41 685–688
H Krichen MS Daghfous A Mrabet et al. (1993) ArticleTitleExtended melanocytic tumor of the cervical spine Apropos of a case of melanotic schwannoma. Ann Pathol 13 184–187
C Gontier MF Le Cam-Savin A. Martin et al. (1995) ArticleTitle[Spinal melanocytic schwannoma with malignant course Microscopic and ultrastructural study of a case]. Arch Anat Cytol Pathol 43 160–163
AE Abbott SuffixJr. RC Hill MA Flynn et al. (1990) ArticleTitleMelanotic schwannoma of the sympathetic ganglia: pathological and clinical characteristics Ann Thorac Surg 49 1006–1008
C Miracco MC Montesco R Santopietro et al. (1996) ArticleTitleProliferative activity, angiogenesis, and necrosis in peripheral nerve sheath tumors: a quantitative evaluation for prognosis Mod Pathol 9 1108–1117
SG O’Brien F Guilhot RA Larson et al. (2003) ArticleTitleImatinib compared with interferon and low-dose cytarabine for newly diagnosed chronic-phase chronic myeloid leukemia N Engl J Med 348 994–1004
E Buchdunger J Zimmermann H Mett et al. (1996) ArticleTitleInhibition of the Abl protein-tyrosine kinase in vitro and in vivo by a 2-phenylaminopyrimidine derivative Cancer Res 56 100–104
E Buchdunger CL Cioffi N Law et al. (2000) ArticleTitleAbl protein-tyrosine kinase inhibitor STI571 inhibits in vitro signal transduction mediated by c-kit and platelet-derived growth factor receptors J Pharmacol Exp Ther 295 139–145
BJ Druker S Tamura E Buchdunger et al. (1996) ArticleTitleEffects of a selective inhibitor of the Abl tyrosine kinase on the growth of Bcr-Abl positive cells Nat Med 2 561–566
MC Heinrich DJ Griffith BJ Druker et al. (2000) ArticleTitleInhibition of c-kit receptor tyrosine kinase activity by STI 571, a selective tyrosine kinase inhibitor Blood 96 925–932
T Schindler W Bornmann P Pellicena et al. (2000) ArticleTitleStructural mechanism for STI-571 inhibition of abelson tyrosine kinase Science 289 1938–1942
JJ Ryan KA Klein TJ Neuberger et al. (1994) ArticleTitleRole for the stem cell factor/KIT complex in Schwann cell neoplasia and mast cell proliferation associated with neurofibromatosis J Neurosci Res 37 415–432
A Badache N Muja GH De Vries (1998) ArticleTitleExpression of Kit in neurofibromin-deficient human Schwann cells: role in Schwann cell hyperplasia associated with type 1 neurofibromatosis Oncogene 17 795–800
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Tawk, R.G., Tan, D., Mechtler, L. et al. Melanotic schwannoma with drop metastases to the caudal spine and high expression of CD117 (c-kit). J Neurooncol 71, 151–156 (2005). https://doi.org/10.1007/s11060-004-9301-0
Issue Date:
DOI: https://doi.org/10.1007/s11060-004-9301-0