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We report a case and review the recent literature describing 36 patients with both Lhermitte-Duclos disease (LDD) and Cowden disease (CD). Lhermitte-Duclos disease, or dysplastic gangliocytoma, is a benign hamartomatous condition involving the cerebellum. The presenting symptoms are usually headaches, gait ataxia, and symptoms of lower cranial nerve involvement. Cowden disease is a rare autosomal dominant disease that usually presents with multiple mucocutaneous lesions. Patients with CD are prone to multiple systemic malignancies, the most common of which is breast cancer. Recent studies have demonstrated an association between LDD and CD.
A 44-year-old woman with a previous history of breast cancer, multiple benign skin lesions, Hashimoto's thyroiditis, and chronic headaches presented with exacerbation of her headaches during the previous year. Magnetic resonance imaging of the brain revealed a right cerebellar nonenhancing mass and an acquired tonsillar herniation.
The patient underwent resection of the right cerebellar mass, posterior fossa decompression, C1 and C2 laminectomies, and a duraplasty. Pathologic examination confirmed LDD. The patient recovered well after surgery, with immediate improvement of her headaches.
The association between LDD and CD has been under-recognized and under-reported. Recognition of this association has direct clinical relevance, because diligent monitoring of individuals with LDD and CD may lead to the early detection of systemic malignancies.
Although the benefits of radiotherapy for pituitary adenomas are well-documented, post-irradiation sarcomas of the sella are rarely seen, with only 20 cases (mainly of fibrosarcoma) reported in the medical literature.
We describe a case of post-irradiation sarcoma five years after surgery followed by external-beam irradiation for an extensive and locally invasive growth hormone-secreting tumor. The patient was subsequently given pegvisomant, an antagonist of growth hormone receptor, to control symptoms of growth hormone excess.
The patient underwent transsphenoidal resection of the recurrent tumor, followed by adjuvant chemotherapy. This led to significant relief in the patient's symptoms including radiological evidence of tumor shrinkage, but the tumor regrew when, owing to dose-limiting toxicity, chemotherapy was stopped.
Post-irradiation sarcomas of the pituitary are well-recognized but rare. They should be suspected in patients following sellar irradiation who show abrupt onset of new symptoms and appropriate radiological findings, and such tumors may respond to cytotoxic chemotherapy.
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