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Sellar mass with vision loss and hypopituitarism
Journal of Clinical Neuroscience 19 (2012) 1282
Contents lists available at SciVerse ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Images in Neuroscience: Question
Sellar mass with vision loss and hypopituitarism Nicholas F. Marko a,⇑, Richard A. Prayson b, Robert J. Weil a,c a
Department of Neurosurgery, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA Department of Pathology, Cleveland Clinic, Cleveland, OH, USA c Brain Tumor and Neuro-Oncology Center, Neurological Institute, Cleveland Clinic, Cleveland, OH, USA b
1. Clinical background A 38-year-old woman developed secondary amenorrhea 1 year prior to presentation to our clinic. Her health was good except for generalized anxiety, for which she took lorazepam as needed, and a gastric bypass surgery 5 years earlier, which had led to a maintained 40 kg weight loss. Initial evaluation, including hormone testing, failed to reveal a cause. She was started on oral contraceptive medication, with estrogen and progesterone, but she continued to be amenorrheic. Evaluation 10 months later by an endocrinologist was remarkable only for a prolactin (PRL) level of 54.1 ng/mL (normal, < 20 ng/mL). An MRI revealed a sellar mass measuring 12 mm 10 mm, consistent with a macroadenoma. Formal testing of both visual acuity and visual fields was normal. She was commenced on bromocriptine, which lowered the PRL to the normal range. Two months later, the patient presented with new onset of headache, photophobia, nausea, cold intolerance, fatigue, and difficulty with reading. There was no excessive urination or thirst. Her physical examination was remarkable for bitemporal hemianopsia, superior more than inferior, and visual acuity of 20/50 in the right eye and 20/100 in the left; there was no meningismus. A brain CT scan did not reveal acute blood. Repeat MRI (Fig. 1) demonstrated an enlarged mass in the sellar and suprasellar regions, 18 mm 15 mm 11 mm (lateral, superior, and antero-posterior dimensions respectively) with optic compression. The optic nerves were thickened and the third cranial nerves enhanced (Fig. 1). There was no hemorrhage. A repeat PRL test was normal. Dynamic pituitary testing revealed that the patient had hypothyroidism and adrenal insufficiency; levothyroxine and hydrocortisone were administered, with alleviation of her cold intolerance and fatigue. Her serum sodium levels were normal. Given the unusual presentation, further evaluation was pursued: CT scans of the chest, abdomen, and pelvis and mammography were normal. Laboratory studies for erythrocyte sedimentation rate, C-reactive protein, alpha fetoprotein, syphilis, angiotensin converting enzyme levels, and the human immunodeficiency virus were negative or normal. Lumbar puncture was performed: the cerebrospinal fluid
DOI of answer: http://dx.doi.org/10.1016/j.jocn.2011.05.032
⇑ Corresponding author.
E-mail address: [email protected] (N.F. Marko). 0967-5868/$ - see front matter Ó 2011 Published by Elsevier Ltd. doi:http://dx.doi.org/10.1016/j.jocn.2011.05.031
(CSF) showed an elevated lymphocyte count (21 white cells per microliter, mainly small lymphocytes) but the CSF cytology was negative for abnormal cells; the protein level was normal and there were no oligoclonal bands. The patient underwent trans sphenoidal surgery and a subtotal resection of a firm, fibrous, gritty, and adherent yellow mass (Fig. 1). A normal pituitary gland was not identified. 2. What is the most likely diagnosis? A. B. C. D.
Pituitary adenoma. Physiological enlargement of the pituitary. Pituitary sarcoidosis. Metastatic tumor involvement of the pituitary gland.
Answer on page 1331.
Fig. 1. Representative (A) axial, (B) sagittal, and (C) coronal T1-weighted, gadolinium-enhanced MRI showing a sellar mass. (A) optic nerve (left arrow); and 3rd cranial nerve (right arrow); and (B) optic nerve (arrow). enhancement. (D, E) Stained microscopic sections of the sellar lesion (hematoxylin and eosin, 200).
Contents lists available at SciVerse ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Images in Neuroscience: Question
Sellar mass with vision loss and hypopituitarism Nicholas F. Marko a,⇑, Richard A. Prayson b, Robert J. Weil a,c a
Department of Neurosurgery, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA Department of Pathology, Cleveland Clinic, Cleveland, OH, USA c Brain Tumor and Neuro-Oncology Center, Neurological Institute, Cleveland Clinic, Cleveland, OH, USA b
1. Clinical background A 38-year-old woman developed secondary amenorrhea 1 year prior to presentation to our clinic. Her health was good except for generalized anxiety, for which she took lorazepam as needed, and a gastric bypass surgery 5 years earlier, which had led to a maintained 40 kg weight loss. Initial evaluation, including hormone testing, failed to reveal a cause. She was started on oral contraceptive medication, with estrogen and progesterone, but she continued to be amenorrheic. Evaluation 10 months later by an endocrinologist was remarkable only for a prolactin (PRL) level of 54.1 ng/mL (normal, < 20 ng/mL). An MRI revealed a sellar mass measuring 12 mm 10 mm, consistent with a macroadenoma. Formal testing of both visual acuity and visual fields was normal. She was commenced on bromocriptine, which lowered the PRL to the normal range. Two months later, the patient presented with new onset of headache, photophobia, nausea, cold intolerance, fatigue, and difficulty with reading. There was no excessive urination or thirst. Her physical examination was remarkable for bitemporal hemianopsia, superior more than inferior, and visual acuity of 20/50 in the right eye and 20/100 in the left; there was no meningismus. A brain CT scan did not reveal acute blood. Repeat MRI (Fig. 1) demonstrated an enlarged mass in the sellar and suprasellar regions, 18 mm 15 mm 11 mm (lateral, superior, and antero-posterior dimensions respectively) with optic compression. The optic nerves were thickened and the third cranial nerves enhanced (Fig. 1). There was no hemorrhage. A repeat PRL test was normal. Dynamic pituitary testing revealed that the patient had hypothyroidism and adrenal insufficiency; levothyroxine and hydrocortisone were administered, with alleviation of her cold intolerance and fatigue. Her serum sodium levels were normal. Given the unusual presentation, further evaluation was pursued: CT scans of the chest, abdomen, and pelvis and mammography were normal. Laboratory studies for erythrocyte sedimentation rate, C-reactive protein, alpha fetoprotein, syphilis, angiotensin converting enzyme levels, and the human immunodeficiency virus were negative or normal. Lumbar puncture was performed: the cerebrospinal fluid
DOI of answer: http://dx.doi.org/10.1016/j.jocn.2011.05.032
⇑ Corresponding author.
E-mail address: [email protected] (N.F. Marko). 0967-5868/$ - see front matter Ó 2011 Published by Elsevier Ltd. doi:http://dx.doi.org/10.1016/j.jocn.2011.05.031
(CSF) showed an elevated lymphocyte count (21 white cells per microliter, mainly small lymphocytes) but the CSF cytology was negative for abnormal cells; the protein level was normal and there were no oligoclonal bands. The patient underwent trans sphenoidal surgery and a subtotal resection of a firm, fibrous, gritty, and adherent yellow mass (Fig. 1). A normal pituitary gland was not identified. 2. What is the most likely diagnosis? A. B. C. D.
Pituitary adenoma. Physiological enlargement of the pituitary. Pituitary sarcoidosis. Metastatic tumor involvement of the pituitary gland.
Answer on page 1331.
Fig. 1. Representative (A) axial, (B) sagittal, and (C) coronal T1-weighted, gadolinium-enhanced MRI showing a sellar mass. (A) optic nerve (left arrow); and 3rd cranial nerve (right arrow); and (B) optic nerve (arrow). enhancement. (D, E) Stained microscopic sections of the sellar lesion (hematoxylin and eosin, 200).