| CASE REPORT |
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| Year : 2011 | Volume
: 2
| Issue : 1 | Page : 136 |
Malignant psammomatous melanotic schwannoma of the spine: A component of Carney complex
Lisa B.E Shields1, Steven D Glassman2, George H Raque3, Christopher B Shields3
1 Norton Neuroscience Institute, Norton Healthcare, Louisville, KY 40202, USA
2 Leatherman Spine Institute, Norton Healthcare, Louisville, KY 40202, USA
3 Norton Neuroscience Institute, Norton Healthcare, Louisville, KY 40202;Leatherman Spine Institute, Norton Healthcare, Louisville, KY 40202, USA
Correspondence Address:
Christopher B Shields
Norton Neuroscience Institute, Norton Healthcare, Louisville, KY 40202;Leatherman Spine Institute, Norton Healthcare, Louisville, KY 40202
USA
© 2011 Shields et al; This is an open-access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
DOI: 10.4103/2152-7806.85609 PMID: 22059131
Background : Psammomatous melanotic schwannomas (PMS) of the spine may be a component of the Carney complex in 50% of cases and is inherited in an autosomal dominant manner. Most PMS are benign and frequently associated with lentiginous pigmentation; cardiac, cutaneous, or breast myxomas; endocrine overactivity; and cutaneous blue nevi. These tumors are characterized by melanin containing cells having ultrastructural characteristics of Schwann cells.
Case Description : Two patients had spinal PMS that were surgically resected with adjacent local radiotherapy, followed by local recurrence and metastasis. The aggressive nature of this tumor is reported.
Conclusion : Spinal PMS are rarely malignant with local recurrence and distal metastases. Inquiry into the patient's and family members' hereditary background for the Carney complex is important to avoid overlooking potential lethal associated abnormalities.
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