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Show Journal of Neuro- Ophlhalmology 20( 1): 42^ 14, 2000. 2000 Lippincott Williams & Wilkins, Inc., Philadelphia Spontaneous Resolution of Pituitary Adenoma After Apoplexy Norman J. Schatz, MD, Oliver M. Job, MD, and Joel S. Glaser, MD A patient with typical pituitary apoplexy with documented intralesional hemorrhage and spontaneous resolution is presented. There was no evidence of a tumor at a 3- month follow- up. Key Word: Pituitary apoplexy. CASE REPORT We report a 57 year- old mentally retarded man with a seizure disorder since childhood, which was controlled with 100 mg of lamotrigine a day. A pituitary microadenoma was treated with Ievothyroxine for many years. The patient arrived at Mercy Hospital, Miami, with headache, generalized weakness, and fever. There was no history of diplopia or loss of vision. The patient was agitated, boisterous, and confused; he could not say complete sentences and perseverated in words and actions, neuroophthalmic examination revealed acuity of 20/ 20 OU at near, with illiterate E testing. The patient could mimic finger- counting in all fields, without errors and with good fixation. Both pupils were 4 mm, reactive, and without an afferent pupillary defect. There was a congenital large- angle exotropia, with preferred right fixation. The results of fundus examination revealed normal optic discs and a 0.4 cup- to- disc ratio. Laboratory evaluation showed: sodium, 113 mEq/ L ( normal, 135- 149 mEq/ L); potassium, 4.0 mEq/ L; chloride, 79 mEq/ L ( normal, 95- 110 mEq/ L); serum osmolality, 235 mOsm/ k ( normal, 278- 305 mOsm/ k); TSH values, 0.07 mlU/ mL ( normal, 0.3- 5.0 mlU/ mL); normal T3 and T4; Cortisol of 11.1 while on replacement; ACTH, 8 mg/ L ( normal, 9.0- 52 mg/ L); prolactin, < 1.0 ng/ mL ( normal, 3.6- 16.3 ng/ mL); testosterone, 57 ng/ dL ( normal, 165- 830 ng/ dL); ADH, 1.7 pg/ mL ( normal, 0.0- 8.0 pg/ mL); and somatomedin- C, 46 ng/ mL ( 71- 290 ng/ mL). Magnetic resonance imaging ( MRI) results demonstrated an intrasellar macroadenoma with a hemorrhage occupying the entire gland, with suprasellar extension and some elevation of the chiasm ( Figs. 1A and 2A). A diagnosis of panhypopituitarism was established, Manuscript received January 5, 1999; accepted October 7, 1999. From private practice ( NJS, JSG); and the Department of Ophthalmology ( OMJ), Kantonsspital Luzern, Luzern, Switzerland. Address correspondence to Joel S. Glaser, MD, 4121 Crawford Ave., Miami, FL 33133- 6160. and the patient initially was treated with a bolus of intravenous methylprednisolone. Treatment with fludrocortisone ( 0.1 mg), hydrocortisone acetate ( 25 mg in the morning and 12.5 mg in the evening), Ievothyroxine ( 0.075 mg daily), and testosterone injections ( 200 mg monthly) were started. Hyponatremia was controlled, and the patient clinically improved. His confusion cleared, the headaches resolved, and no other treatment was advised. Results of a repeat MRI scan after 3 months showed complete resolution of the hemorrhage, with no evidence of a residual tumor ( Figs. IB and 2B). DISCUSSION In a large routine autopsy study ( 1), the incidence of pituitary infarction was 3% of all cases and between 10% and 15% in incidental pituitary adenomas. Using magnetic resonance criteria, the actual occurrence of hemorrhage within pituitary adenomas without clinical manifestation is much higher ( 2). The occurrence of symptomatic spontaneous hemorrhage or necrosis in pituitary adenomas constitutes the syndrome of pituitary apoplexy ( PA). In a series of 560 cases with pituitary adenomas, the over- all incidence of PA was 16.6% ( 3). Hemorrhage without clinical signs and symptoms occurred in 7.5%. Major episodes consisting of disturbance of consciousness, hemiparesis, loss of vision, or ocular palsy occurred in 6.8%. Minor attacks including nausea, vomiting, and vertigo was seen in 2.3%. Pituitary adenomas may show gross or microscopic hemorrhage independent of endocrine or neoplastic pattern. In a pathologic review of 320 verified adenomas, with 40% being giant or recurrent large adenomas, evidence of hemorrhage was found in 58 cases ( 18%) ( 4). Actual acute apoplexy occurred in 7 cases, subacute apoplexy in 11 cases, and recent silent hemorrhage in 13 cases. There was evidence of remote silent hemorrhage in 27 cases. Therefore, 40 of 58 cases ( 68%) represent asymptomatic silent hemorrhages found on imaging studies ( 4,5). Ostrov et al. ( 6) analyzed the clinical presentations of 12 patients with radiologic findings of intratumoral hemorrhage. In eight of the patients who underwent surgery, hemorrhage could be identified together with adenomatous tissue, except in one case, where no adenomatous 42 RESOLUTION OF PITUITARY ADENOMA AFTER APOPLEXY 43 FIG. 1. T1- weighted MRI. Midline sagittal sections demonstrate ( A) intrasellar hemorrhage with chiasmal displacement. B: After resolution. tissue was found. However, only three of 12 patients had clinical symptoms of PA. The authors concluded that pituitary hemorrhage might be seen without clinical symptoms of pituitary apoplexy. Bills et al. ( 7) reported 37 patients with PA, of which 95% presented with headaches, 69% with vomiting, 78% with ocular paresis, and 64% with visual field defects. Thirty- six of their patients underwent transsphenoidal decompression, although preoperative computed tomography only confirmed hemorrhage in 36% of these patients. Null cell tumors occurred in 50%. Steroid and thyroid replacement was needed in over 80% of patients, desmopressin was required in 11%, and testosterone replacement was needed for 64% of males. Visual acuity and visual fields recovered in at least 90% of patients, and ophthalmoparesis recovered in all. These authors stressed the importance of rapid decompression. Pituitary infarction or hemorrhage is usually considered a medical and neurosurgical emergency. Traditional thinking is that clinical pituitary apoplexy with ophthalmoplegia, sudden onset of headache, loss of vision, and acute panhypopituitarysm should prompt urgent hormonal replacement and surgical decompression ( 8). A prospective study ( 9) to evaluate conservative treatment of PA included 12 patients who presented with sudden headaches, visual impairment, and ophthalmoplegia, all with radiographic evidence of apoplexy. Eleven patients received IV- dexamethasone. Transsphenoidal surgery was performed for four patients whose vision failed to improve or consciousness remained impaired. Among the seven patients who underwent conservative treatment, ophthalmoplegia recovered completely in six, and improved in one. Follow- up MRI scans showed resolution of tumor in four patients and residual masses in three. In the five patients surgically treated, all showed residual tumor. Recurrence was seen in two patients in each group. In another series of 44 untreated patients, 20 of whom had enlarged panhypopituitarism, completely or partially empty sellas were seen on computed tomography scans ( 10). The authors concluded that previous pituitary adenomas had undergone complete or partial necrosis. Ten of the 20 patients with resolution had in fact experienced typical symptoms of pituitary apoplexy. Panhypopituitarism occurred as often in patients with empty sellas as in patients with solid pituitary tumors; however, prolactin levels were significantly lower in patients with empty sellas than in patients with solid tumors. The authors emphasized the spontaneous course of pituitary adenomas when assessing the effects of various treatment protocols. Endocrine remission has been reported after pituitary apoplexy in two patients with pituitary macroadenomas, one with Cushing disease and the other with acromegaly ( 11). The first patient presented with headaches and vomiting, followed by progressive remission of hyper-cortisolism; MRI results disclosed persistence of sellar and suprasellar mass. At transsphenoidal surgery, a hemorrhagic cyst was found, with no evidence of tumor. The second patient had acromegaly; during a luteinizing hormone- releasing hormone- stimulation test, he had an episode of headache, vomiting, and diabetes insipidus, but no visual loss. Computed tomography results disclosed an intrasellar hematoma, which was followed without surgery. Results of follow- up scans showed shrinking and disappearance of the mass and complete endocrine remission. Bjerre et al. ( 12) described 23 patients with acromegaly who were followed untreated for 2 to 13 years and noted that six of the patients had empty sellas or intrasellar cysts, suggesting spontaneous resolution. Plasma growth hormone levels significantly decreased in three of these patients. These authors concluded that adenomas secreting growth hormone might remain stationary for an extended period, and that complete or partial disappearance occurs, probably as a result of pituitary infarction. Ahmed et al. ( 13) reviewed a series of 13 patients who presented with signs and symptoms of classic PA. Six patients presented with acute symptoms and seven patients had a history of apoplexy antedating admission, by a mean of 887 days. The patients were followed for a mean of 730 days. During this time, two untreated patients developed empty sellas with no evidence of tumor. At the preliminary study, 11 out of 13 patients had evidence of bleed into the tumor, histologically confirmed at surgery; there was evidence of residual tumor in one patient, and an empty sella in one patient. The authors concluded that apoplexy might produce complete or partial adenoma resolution, with or without preservation of endocrine function. FIG. 2. T1- weighted coronal sections demonstrate ( A) phase of acute hemorrhage. B: Partial empty sella and no residual tumor. J Neuro- Opluhalmol, Vol. 20, No. 1, 2000 N. J. SCHATZETAL. Miiller- Jensen and Ludecke ( 14) reviewed a series of 586 surgically treated tumors of the pituitary. Seventy-two cases ( 12.3%) of spontaneous necrosis were found. In only ten cases were there clues of additional rupture of tumor with signs of meningeal reaction. They demonstrate that a relatively benign clinical course with signs of regression is more frequent than generally assumed, and spontaneous necrosis with or without rupture is much more frequent in endocrinologically inactive tumors, including prolactinomas, as opposed to somatostatin somatotrophic hormone or adrenocorticotrophic hormone cell adenomas. We present an example of pituitary apoplexy with panhypopituitarism without detectable visual loss. Spontaneous resolution of the hematoma obviated the necessity for surgical intervention. After successful treatment of pituitary crisis ( hyponatremia, coma, confusion), a conservative course of observation may be considered for patients without visual loss or ophthalmoplegia, when rapid improvement of neuroophthalmic signs occurs, or in patients for whom MRI imaging results demonstrate no tumor tissue and only hematoma. This conservative option is well supported in the literature and should not be ignored in this era of zealous transsphenoidal surgery. REFERENCES 1. Kovacs K. Adenohypophyseal necrosis in routine autopsies. En-dokrinologie 1972; 60: 309- 16. 2. Kurihara, N, Takahashi S, Higano S, Ikeda H, et al. Hemorrhage in pituitary adenoma: correlation of MR imaging with operative findings. Eur Radiol 1998; 8: 971- 6. 3. Wakai S, Fukushima T, Teramoto A, Sano K. Pituitary apoplexy: its incidence and clinical significance. ] Neurosurg 1981; 55: 187- 93. 4. Symon L, Mohanty S. Haemorraghe in pituitary tumors. Acta Neu-rochir 1982; 65: 41- 7. 5. Wilson CB. A decade of pituitary microsurgery; the Herbert 01- ivecrona lecture. J Neurosurg 1984; 61; 814- 33. 6. Ostrov SG, Quencer RM, Hoffman JC, Davis PC, Hasso AN, David NJ. Hemorrhage within pituitary adenomas: how often associated with pituitary apoplexy syndrome? Am J Roentgenol 1989; 153: 153- 60. 7. Bills DC, Meyer FB, Laws ER Jr, et ai. A retrospective analysis of pituitary apoplexy. Neurosurgery 1993; 33: 602- 8. 8. Ebersold MJ, Laws ER Jr, Scheithauer BW, et al. Pituitary apoplexy treated by transsphenoidal surgery: a clinicopathological study. J Neurosurg 1983; 58: 315- 20. 9. Maccagnan P, Macedo CL, Kayath MJ, Norgueria RG, Abucham J. Conservative management of pituitary apoplexy: a prospective study. / Clin Endocrinol Metab 1995; 80: 2190- 7. 10. Lindholm J, Bjerre P, Riishede J, Gyldensted C, Hagen C. Pituitary function in patients with evidence of spontaneous disappearance of a pituitary adenoma. Clin Endocrinol 1983; 18: 599- 603. 11. Miranda M, Barros L, Knopfelmacher M, et al. Pituitary apoplexy followed by endocrine remission. Report of two cases. Arq Neu-ropsiquiatr 1998; 56: 449- 52. 12. Bjerre P, Lindholm J, Videbaek H. The spontaneous course of pituitary adenomas and occurrence of an empty sella in untreated acromegaly. J Clin Endocrinol Metab 1986; 63: 287- 91. 13. Ahmed M, Rifai A, Al- Jurf M, Akhtar M, Woodhouse N. Classical pituitary apoplexy presentation and a follow- up of 13 patients. HormRes 1989; 31: 125- 32. 14. Miiller- Jensen A, Ludecke D. Clinical aspects of spontaneous necrosis of pituitary tumors ( pituitary apoplexy). J Neurol 1981 ; 224: 267- 71. • • . J Neuro- Ophthalmol, Vol. 20. No. 1, 2000 |
