Targeting PD-L1 After Adjuvant Radiation in Subtotally Resected Primary Pineal Melanoma: A Case Report and Literature Review

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  • a Department of Radiation Oncology,
  • | b Division of Neurosurgery, and
  • | c Division of Medical Oncology, The University of Arizona, Tucson, Arizona.

Primary melanoma of the pineal gland is a rare disease entity with an overall poor prognosis. Limited data exist to appropriately guide treatment decisions. Historical case reports have showed some success using a combination of surgical resection, radiotherapy, and chemotherapy, but long-term survival has been exceedingly rare. This report presents a female patient with a primary pineal melanoma who underwent subtotal resection followed by adjuvant focal radiation to the residual tumor. Immunohistochemistry identified a strong positivity for PD-L1 (70%). After radiation, systemic therapy with pembrolizumab was initiated with the plan to treat until progression. She has now completed 33 cycles of pembrolizumab without interruptions, complications, or disease progression. At the time of writing, the patient has had an excellent clinical outcome, with a durable near-complete response of >138 weeks. To our knowledge, this is the first patient with a pineal melanoma to be managed by targeting PD-L1. Furthermore, she has achieved the second longest overall survival and the longest progression-free survival reported in the literature.

Submitted March 25, 2019; accepted for publication June 25, 2019.

Disclosures: The authors have disclosed that they have not received any financial considerations from any person or organization to support the preparation, analysis, results, or discussion of this article.

Correspondence: Justin Famoso, MD, Department of Radiation Oncology, The University of Arizona, 3838 North Campbell Avenue, Building 2, Tucson, AZ 85719. Email:
  • 1.

    Wendel C, Kaech DL, Woodtli M. Primary malignant melanoma in the pineal region: case report and literature review. J Neurol Surg A Cent Eur Neurosurg 2018;79:344352.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 2.

    Rubino GJ, King WA, Quinn B, et al.. Primary pineal melanoma: case report. Neurosurgery 1993;33:511515; discussion 515.

  • 3.

    Yamane K, Shima T, Okada Y, et al.. Primary pineal melanoma with long-term survival: case report. Surg Neurol 1994;42:433437.

  • 4.

    Suzuki T, Yasumoto Y, Kumami K, et al.. Primary pineal melanocytic tumor. Case report. J Neurosurg 2001;94:523527.

  • 5.

    Arantes M, Castro AF, Romão H, et al.. Primary pineal malignant melanoma: case report and literature review. Clin Neurol Neurosurg 2011;113:5964.

  • 6.

    Cedeño Diaz OM, Leal RG, La Cruz Pelea C. Primary pineal malignant melanoma. Clin Pract 2011;1:e31.

  • 7.

    Azimi P, Mohmmadi HR, Refiezadeh M. Primary pineal melanoma presenting with leptomeningeal spreading in a 22-year-old woman: a case report. J Med Case Reports 2012;6:165.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 8.

    Shinsato Y, Hanada T, Kisanuki T, et al.. Primary malignant melanoma in the pineal region treated without chemotherapy. Surg Neurol Int 2012;3:123.

  • 9.

    Biswas A, Chaudhari PB, M SK, et al.. Primary pineal malignant melanoma - illustrated review. Turk Neurosurg 2015;25:201209.

  • 10.

    Park JH, Hong YK. Primary malignant melanoma in the pineal region. J Korean Neurosurg Soc 2014;56:504508.

  • 11.

    Arlant PA, Grunnet ML, Heilbrun MP. Primary malignant melanoma of the pineal region. Surg Neurol 1977;7:121123.

  • 12.

    Carlson BR, Glick AD, Cushman AR. Primary malignant melanoma of pineal region. J Tenn Med Assoc 1987;80:597599.

  • 13.

    Bookland M, Anderson WS, Biser-Rohrbaugh A, et al.. Primary pineal malignant melanoma. Pediatr Neurosurg 2007;43:303308.

  • 14.

    Barron J, Morris-Larkin C, Finch T, et al.. Long survival of primary pineal melanoma with radiation treatment only. Can J Neurol Sci 2007;34:251253.

  • 15.

    Martin-Blondel G, Rousseau A, Boch AL, et al.. Primary pineal melanoma with leptomeningeal spreading: case report and review of the literature. Clin Neuropathol 2009;28:387394.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 16.

    Somers KE, Almast J, Biemiller RA, et al.. Diagnosis of primary CNS melanoma with neuroimaging. J Clin Oncol 2013;31:e911.

  • 17.

    Jetschke K, Viehweger H, Freesmeyer M, et al.. Primary pineal malignant melanoma with B-Raf V600E mutation: a case report and brief review of the literature. Acta Neurochir (Wien) 2015;157:12671270.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 18.

    Masoomian B, Shields JA, Shields CL. Overview of BAP1 cancer predisposition syndrome and the relationship to uveal melanoma. J Curr Ophthalmol 2018;30:102109.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 19.

    Murali R, Wiesner T, Scolyer RA. Tumours associated with BAP1 mutations. Pathology 2013;45:116126.

  • 20.

    Harbour JW, Onken MD, Roberson EDO, et al.. Frequent mutation of BAP1 in metastasizing uveal melanomas. Science 2010;330:14101413.

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