Brian T O'Neill1,2, Konstantinos Segkos3, Ekkehard M Kasper4, Johanna A Pallotta5. 1. Division of Endocrinology, Diabetes, and Metabolism, Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA. brian.o'neill@joslin.harvard.edu. 2. Section on Integrative Physiology and Metabolism, Joslin Diabetes Center, Harvard Medical School, 1 Joslin Pl, Rm 605, Boston, MA, 02215, USA. brian.o'neill@joslin.harvard.edu. 3. University of Massachusetts School of Medicine, Worcester, MA, USA. 4. Department of Neurosurgery, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA. 5. Division of Endocrinology, Diabetes, and Metabolism, Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA.
Abstract
INTRODUCTION: Primary intracranial and sellar squamous cell carcinoma is an extremely rare entity, usually caused by malignant transformation of epidermoid cysts, or very rarely other non-malignant epithelial cysts. Malignant transformation of a Rathke's cleft cyst has never been described. CASE DESCRIPTION: We present a 49-year-old male patient who presented with a 3-month history of progressive frontotemporal headaches. Imaging revealed a 1.2 cm cystic pituitary mass consistent with a hemorrhagic Rathke's cleft cyst. The patient underwent trans-sphenoidal resection of the pituitary cyst, and pathologic analysis revealed a squamous cell carcinoma lining a Rathke's cleft cyst. Extensive imaging and otorhinolaryngologic evaluation revealed no primary source for metastasis. CONCLUSIONS: We feel this represents the first case of a patient with a pituitary lesion in which presentation and MRI imaging were consistent with Rathke's cleft cyst, yet histology revealed squamous cell carcinoma in situ.
INTRODUCTION: Primary intracranial and sellar squamous cell carcinoma is an extremely rare entity, usually caused by malignant transformation of epidermoid cysts, or very rarely other non-malignant epithelial cysts. Malignant transformation of a Rathke's cleft cyst has never been described. CASE DESCRIPTION: We present a 49-year-old male patient who presented with a 3-month history of progressive frontotemporal headaches. Imaging revealed a 1.2 cm cystic pituitary mass consistent with a hemorrhagic Rathke's cleft cyst. The patient underwent trans-sphenoidal resection of the pituitary cyst, and pathologic analysis revealed a squamous cell carcinoma lining a Rathke's cleft cyst. Extensive imaging and otorhinolaryngologic evaluation revealed no primary source for metastasis. CONCLUSIONS: We feel this represents the first case of a patient with a pituitary lesion in which presentation and MRI imaging were consistent with Rathke's cleft cyst, yet histology revealed squamous cell carcinoma in situ.
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