Exp Clin Endocrinol Diabetes 2013; 121 - P71
DOI: 10.1055/s-0033-1336749

Malignant Prolactinoma – a therapeutic challenge

H Lahner 1, N Unger 2, M Schlamann 3, K Keyvani 4, U Sure 5, D Führer 1
  • 1Universität Duisburg-Essen, Klinik für Endokrinologie und Stoffwechselerkrankungen, Essen, Germany
  • 2Universitätsklinikum der Ruhr-Universität Bochum, Marienhospital Herne, Medizinische Klinik I, Herne, Germany
  • 3Universität Duisburg-Essen, Institut für Diagnostische und Interventionelle Radiologie und Neuroradiologie, Essen, Germany
  • 4Universität Duisburg-Essen, Institut für Pathologie und Neuropathologie, Essen, Germany
  • 5Universität Duisburg-Essen, Klinik für Neurochirurgie, Essen, Germany

Case report: We report a 67 year old male patient, who first presented with visual field defects and 40-fold elevated prolactin (PRL) serum levels in 2003. MRI showed a pituitary macroadenoma of 15 × 17 × 25 mm size with incipient optic nerve compression.

Therapy (1): Cabergoline was started at 1.25 mg/week, visual fields improved and prolactin levels decreased. Subsequent MRI showed a small residual pituitary tumor. In 2006, visual field defects re-appeared and transsphenoidal surgery was performed since hyperprolactinemia did not respond to cabergoline. A second tumor relapse occurred in 2007 and was again treated by transphenoidal surgery followed by gamma-knife therapy. Despite ongoing dopaminergic therapy, PRL levels rose and in 3/2009 the patient reported new onset of neck pain. MRI revealed multiple lesions around the dorsum sellae and along the spine. A malignant epithelial tumor with PRL expression was found on biopsy.

Therapy (2): Fractionated external radiation and radiosurgery were applied in 06/2009 and resulted in regression of metastases. In 04/2010 numerous new intracranial and intraspinal lesions were detected and temozolomide chemotherapy was started. Disease was stable until 03/2012 when further lesions occurred. Therapy was then switched to sunitinib initially resulting in disease stabilization. However, focal neurological signs occurred in 07/2012 and a new parasellar lesion was detected. Based on data showing tumor shrinkage and serum PRL suppression after inhibition of HER2/ErbB2 in prolactinomas, therapy was changed to Lapatinib. Unfortunately, immunohistochemistry showed no expression of HER2/ErbB2 and raising PRL serum levels were observed. In 10/2012 temozolomide therapy was re-induced because of lack of alternatives. This has so far resulted in decrease of PRL levels.

In summary, malignant prolactinoma is a very rare disease with very limited treatment options and thus an urgent need to explore innovative medical therapies.