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Orchiectomy for suspected microscopic tumor in patients with anti-Ma2-associated encephalitis

From the Department of Neurology (R.M.M., M.R.R., J.E.R., J.D.), Division Neuro-oncology, University of Pennsylvania, Philadelphia; Department of Neurology (R.V.), University Hospital Gasthuisberg, Leuven, Belgium; Department of Neurology (A.G.-M.), Clínica Puerta de Hierro, Madrid, Spain; Department of Neurology (T.Y.), University of Tokyo Hospital, Japan; New Jersey Neuroscience Institute (J.C.L.), JFK Medical Center, Edison; Division of Medical Oncology (B.T.), British Columbia Cancer Agency, Vancouver, Canada; and Department of Neurology (E.J.D.), Indiana University Medical Center, Indianapolis.

Address correspondence and reprint requests to Dr. Josep Dalmau, Division Neuro-oncology, Department of Neurology, 3 W. Gates, University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA 19104; e-mail: josep.dalmau{at}uphs.upenn.edu

Objective: To report the presence of microscopic neoplasms of the testis in men with anti-Ma2-associated encephalitis (Ma2-encephalitis) and to discuss the clinical implications.

Methods: Orchiectomy specimens were examined using immunohistochemistry with Ma2 and Oct4 antibodies.

Results: Among 25 patients with Ma2-encephalitis younger than 50 years, 19 had germ-cell tumors, and 6 had no evidence of cancer. These 6 patients underwent orchiectomy because they fulfilled five criteria: 1) demonstration of anti-Ma2 antibodies in association with MRI or clinical features compatible with Ma2-encephalitis, 2) life-threatening or progressive neurologic deficits, 3) age < 50 years, 4) absence of other tumors, and 5) new testicular enlargement or risk factors for germ-cell tumors, mainly cryptorchidism or ultrasound evidence of testicular microcalcifications. All orchiectomy specimens showed intratubular-germ cell neoplasms unclassified type (IGCNU) and other abnormalities including microcalcifications, atrophy, fibrosis, inflammatory infiltrates, or hypospermatogenesis. Ma2 was expressed by neoplastic cells in three of three patients examined. Even though most patients had severe neurologic deficits at the time of orchiectomy (median progression of symptoms, 10 months), 4 had partial improvement and prolonged stabilization (8 to 84 months, median 22.5 months) and two did not improve after the procedure.

Conclusions: In young men with Ma2-encephalitis, 1) the disorder should be attributed to a germ-cell neoplasm of the testis unless another Ma2-expressing tumor is found, 2) negative tumor markers, ultrasound, body CT, or PET do not exclude an intratubular germ-cell neoplasm of the testis, and 3) if no tumor is found, the presence of the five indicated criteria should prompt consideration of orchiectomy.

Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the March 20 issue to find the title link for this article.

Editorial, see page 887

This article was previously published in electronic format as an Expedited E-Pub on December 6, 2006, at www.neurology.org.

Supported in part by RO1CA089054 (J.D.).

Disclosure: The authors report no conflicts of interest.

An abstract of this article was presented at the 58th annual meeting of the American Academy of Neurology; San Diego, CA; April 5, 2006. Video clips of the presentation and commentary by Dr. K. Jaeckle are available at the AAN Web site: http://www.marathonmultimedia.com/aan_library/master2.php?ud=56136; "view sessions."

Received August 14, 2006. Accepted in final form October 31, 2006.

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