- Department of Neurosurgery, Field of Oncology, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan
- Department of Molecular and Cellular Pathology, Field of Oncology, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan
Department of Neurosurgery, Field of Oncology, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan
DOI:10.4103/2152-7806.102348Copyright: © 2012 Shinsato Y. 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: Shinsato Y, Hanada T, Kisanuki T, Yonezawa H, Yunoue S, Yoshioka T, Hanaya R, Tokimura H, Hirano H, Arita K. Primary malignant melanoma in the pineal region treated without chemotherapy. Surg Neurol Int 13-Oct-2012;3:123
How to cite this URL: Shinsato Y, Hanada T, Kisanuki T, Yonezawa H, Yunoue S, Yoshioka T, Hanaya R, Tokimura H, Hirano H, Arita K. Primary malignant melanoma in the pineal region treated without chemotherapy. Surg Neurol Int 13-Oct-2012;3:123. Available from: http://sni.wpengine.com/surgicalint_articles/primary-malignant-melanoma-in-the-pineal-region-treated-without-chemotherapy/
Background:Primary pineal malignant melanomas are uncommon intracranial tumor. Here we discuss and review a case of primary pineal malignant melanoma over its feature of imaging studies, pathological findings, and management.
Case Description:A 49-year-old woman receiving renal dialysis underwent computed tomography due to a 4-month history of tinnitus and hearing disturbance. A high-density 35-mm diameter tumor was detected in the pineal region; there was obstructive hydrocephalus. The tumor was heterogeneously hyperintense on T1-weighted magnetic resonance images, iso- and low-mixed intense on T2-weighted images with hemorrhagic components, and very low-intense on T2* images. A tumor was subtotally removed via the occipital transtentorial approach. Histologically, it consisted of densely proliferated spindle-shaped or polygonal cells with rich cytoplasmic melanin. The neoplastic cells manifested cellular pleomorphism, nuclear atypia, and mitosis (3/10 high-power fields) and were immunopositive for HMB45, Melan-A, and S100 protein. The MIB-1 index was 17.4%. Whole-body 18-fluoro-deoxyglucose positron emission tomography did not demonstrate any sites with hyper uptake. Examination of the skin and mucosa identified no lesions suggestive of melanoma. She underwent treatment with the whole brain and extended local boost irradiation. Chemotherapy was not delivered due to renal failure. Follow-up imaging studies showed no recurrence or distant lesions 56 weeks after surgery.
Conclusion:We report a rare case of primary pineal malignant melanoma with prolonged survival of more than 56 weeks after subtotal tumor resection followed by whole-brain and extended local irradiation without chemotherapy. Radiotherapy without chemotherapy might be sufficient for the treatment of this tumor.
Keywords: Central nervous system, malignant melanoma, pineal, primary
Primary melanomas of the central nervous system (CNS) are rare but aggressive neoplasms; 3.6% of these tumors arise in the pineal region.[
To our knowledge, 16 pineal malignant melanomas have been reported since 1899. We now report a patient whose primary pineal malignant melanoma was treated without chemotherapy and discuss the imaging studies, pathological findings, and management of this rare case.
A 49-year-old Japanese woman on renal dialysis had a 4-month history of tinnitus and hearing loss. Computed tomography (CT) showed a high-density 35-mm diameter tumor in the pineal region and obstructive hydrocephalus [
We used the left occipital transtentorial approach with the patient in the three-quarter prone position.[
Hematoxylin and eosin (HE) staining showed the tumor to be composed of anaplastic spindle-shaped and epithelioid cells arranged in nests, fascicles, or sheets; they contained variable amounts of cytoplasmic melanin and manifested significant cellular pleomorphism, nuclear atypia, and mitosis (3/10 high-power fields; HPFs).The tumor cells were immunohistochemically positive for HMB45, Melan-A, and S100 protein. Additionally, the MIB-1 index was 17.4 ± 3.0% [
Photomicrographs of the excised tumor. Hematoxylin and eosin staining showed anaplastic spindled or epithelioid cells arranged in nests, fascicles, or sheets, displaying variable cytoplasmic melanin (a and b, ×100). These tumor cells exhibited significant cellular pleomorphism and nuclear atypia. Mitotic figures were seen occasionally (c, ×400). The tumor cells were positive for HMB45 (d, ×200) and Melan-A (e, ×200). The MIB-1 index was 17.4% (f, ×200)
After surgery, the patient was treated with 36 Gy of whole-brain- and 18 Gy of extended local boost irradiation. Her coexisting renal failure ruled out chemotherapy. A whole-body 18-fluoro-deoxyglucose positron emission tomography (FDG-PET) scan showed no abnormally increased uptake [
The most recent MRI scans acquired 56 weeks after her initial treatment showed no evidence of recurrence or metastatic lesions [
Most reported lesions of primary melanomas of the CNS were found in the spinal canal or the posterior fossa because of the higher concentration of melanocytes in the leptomeninges at the anterior and lateral surface of the spinal cord and ventrolateral to the medulla oblongata.
Primary pineal malignant melanomas are exceedingly rare; only 16 cases have been reported to date and of these, eight were detected by MRI [
CT scans of intracranial malignant melanomas usually show a homogeneously enhanced high-dense mass and MRI shows a characteristic signal. The melanin content of the tumor reflects the presence of free radicals; therefore, a paramagnetic effect responsible for a shortened T1 relaxation time is produced. Consequently, MRI reveals a mass that is hyperintense on T1- and iso/hyperintense on T2-weighted images. The shorter T1 relaxation time correlates with the amount of melanin in the tumor.[
Melanocytic tumors are classified into well-differentiated melanocytomas, intermediate-grade melanocytic tumors, and malignant melanomas. Pathologically, malignant melanomas consist of spindle-shaped or epithelioid cells arranged in loose nests, fascicles, or sheets, with variable amounts of cytoplasmic melanin pigment. These tumors are densely cellular and manifest significant cellular pleomorphism and nuclear atypia associated with a high mitosis rate (mean 5.7/10 HPFs) and high MIB-1 labeling indices (mean 8.1%).[
It is difficult to distinguish primary CNS melanoma from metastatic melanoma on neuroimages alone.[
At present, there is no standardized treatment to address primary pineal malignant melanomas. As patients who did not receive adjuvant treatment survived no longer than 3 months,[
We report a rare case of primary melanoma of the pineal gland with prolonged survival of more than 56 weeks after subtotal tumor resection followed by whole-brain and extended local irradiation without chemotherapy. However, a long-term follow-up was needed to determine whether radiotherapy without chemotherapy is sufficient for the treatment of primary pineal malignant melanomas.
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