- Norton Neuroscience Institute, Norton Healthcare, Louisville, KY 40202, USA
- Leatherman Spine Institute, Norton Healthcare, Louisville, KY 40202, USA
Correspondence Address:
Christopher B. Shields
Norton Neuroscience Institute, Norton Healthcare, Louisville, KY 40202, USA
Leatherman Spine Institute, Norton Healthcare, Louisville, KY 40202, USA
DOI:10.4103/2152-7806.85609
Copyright: © 2011 Shields LBE. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.How to cite this article: E. Shields LB, Glassman SD, Raque GH, Shields CB. Malignant psammomatous melanotic schwannoma of the spine: A component of Carney complex. Surg Neurol Int 30-Sep-2011;2:136
How to cite this URL: E. Shields LB, Glassman SD, Raque GH, Shields CB. Malignant psammomatous melanotic schwannoma of the spine: A component of Carney complex. Surg Neurol Int 30-Sep-2011;2:136. Available from: http://sni.wpengine.com/surgicalint_articles/malignant-psammomatous-melanotic-schwannoma-of-the-spine-a-component-of-carney-complex/
Abstract
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.
Keywords: Carney complex, malignant, melanin, psammoma body, schwannoma, spine
INTRODUCTION
Melanotic schwannomas consist of melanin containing nerve sheath tumors[
Psammomatous melanotic schwannomas (PMS) (predominantly spinal), myxomas (cardiac, mammary, and cutaneous), spotty pigmentation, endocrine overactivity, and cutaneous blue nevi constitute the Carney complex with autosomal dominant inheritance.[
Two cases of malignant spinal PMS are reported with a uniquely aggressive course. The differential diagnosis, surgical treatment, and pathological appearance of this tumor are presented. The hereditary nature mandates an extensive investigation to identify the presence of this tumor in patients and their families.
CASE REPORTS
Case 1
A 65-year-old woman presented with a history of mid-thoracic spinal pain. Neurological and physical examination as well as family history were noncontributory. Erosion of the right T7 and T8 pedicles was observed on computed tomography (CT) scan [
A laminectomy was performed and a circumscribed soft black extradural mass was resected, initially thought to be a melanotic melanoma. Complete tumor resection was not possible; however, margins were resected where possible. Postoperative external beam radiotherapy (XRT) with a boost to the tumor bed was performed. Eight months later, local tumor developed in the T8 vertebral body, necessitating local resection of a tumor with identical histology as the initial diagnosis. The patient died within a short time postoperatively.
Histological examination revealed epithelioid and spindle-shaped cells [
Case 2
A 33-year-old male developed low back pain and right L5 radiculopathy. There was a family history of "cardiac tumors", but no symptoms of the Carney complex were elicited. Lumbar MRI revealed a right L5-S1 epidural mass with transforamenal extraspinal extension.
At surgery, a black tumor in the right L5-S1 neural foramen encased adjacent nerve roots. Frozen section suggested a malignant melanoma and therefore, a wide resection was performed including resection of the L5 nerve root providing grossly clear margins. Eighteen months later, an MRI scan revealed a recurrent tumor in the right sacrum [
DISCUSSION
Carney described a syndrome consisting of myxomas (cardiac, cutaneous, and mammary), spotty pigmentation, endocrine overactivity (Cushing's syndrome and acromegaly),[
PMS may be cured by wide en bloc resection.[
The differential diagnosis of a melanotic schwannoma includes malignant melanoma, pigmented meningioma and neurofibroma, rhabdomyosarcoma, clear cell soft tissue sarcoma,[
Surgery: The optimal surgical approach is complete tumor resection without aggravating the neurological deficit. Successful outcome depends on 1) grade of malignancy, 2) bone metastasis, and 3) visceral metastasis.[ Would a more aggressive initial resection have improved the clinical outcome? Tumor-free margins in highly aggressive tumors are uncommon in spinal surgery as most oncological spine operations are palliative.[ Preoperative percutaneous CT-guided biopsy was not performed prior to surgical intervention as recommended for tumor staging to determine the surgical strategy.[ Pathology: Melanotic schwannomas (also termed pigmented schwannoma, melanogenic schwannoma, or melanotic nerve sheath tumor) are circumscribed black, brown, blue, or gray tumors on gross examination. Microscopic characteristics of this tumor are (1) spindle and epithelioid shaped cells, (2) spindle cells arranged in whorl formation with occasional nuclear palisading,[ Pathogenesis and genetics: Associated cardiac, thyroid, and other systemic manifestations may occur in the same patient as PMS, leading to a poor prognosis. Close evaluation of family members should be undertaken to prevent unanticipated potentially catastrophic events. Our two patients and their families were interviewed for other features of the Carney complex, with a suggestion of cardiac disease being obtained only in Case 2.
CONCLUSION
Malignant PMS are rarely encountered by spine surgeons. Total excision with tumor-free margins is recommended for both conventional and psammomatous types.[
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