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Clinically asymptomatic pituitary adenoma manifesting as pituitary apoplexy and fatal third-ventricular hemorrhage
CASE STUDIES 9. MinarsN, SilvermanJF, Escobar MR, et al: Fatal cytomegalicinclusion disease: associated skin manifestationsin a renal transplant patient. Arch Dermatol 113:1569, 1977 10. NakonecznaI, Kay S: Fatal disseminatedcytomegallcinclusiondisease in an adult presentingwith a lesionof the gastrointestinaltract. Amj Clin Pathol 47:124, 1967 11. RobsonGS, MackayIR: Generalizedcytomegalovirusinfectionin a patient with lupoid hepatitis. Anat Ann Med 18:147, 1969
12. EvansDJ, WilliamsED: Cytomegalicinclusiondisease in an adult.J Clin Pathol 21:311, 1968 13. WongT, WarnerNE: Cytomegalovirusinclusiondiseasein adults. Arch Pathol 74:403, 1962 14. GoodmanMD, Porter DD: C)'tomegalovirusvasculitiswith fatal colonic hemorrhage. Arch Pathol 96:281, 1973 15. BusselJ, Danou F, Ferchal F, et ah Cytomegalovirusinfectionin malignant blood diseases: clinicaland laboratorydata in 29 patients. Nouv Rev Fr tlematol 20:67, 1978
CLINICALLY ASYMPTOMATIC PITUITARY ADENOMA MANIFESTING AS PITUITARY APOPLEXY AND FATAL THIRD-VENTRICULAR HEMORRHAGE UMA P. KALYANARAMAN,gIBBS, MSc*
A 56-year-old woman with an asymptomatk pituitary adenoma had a fatal third-ventricular hemorrhage of sudden onset. A suprasellar mass with hemorrhage and rupture into the third ventricle was found at autopsy. Histologically, the tumor cells from the suprasellar mass and the third-ventricular hemorrhage were diaguostic of a pituitary adenoma. The pathogenesis of the thirdveutricular hemorrhage from a pituitary adenoma and the need to consider pituitary apoplexy in the differential diagnosis of thirdventricular hemorrhage are discussed. On the basis of a review of the literature, it is felt that computerized tomographic scanning, although most helpful in diaguosis of sellar lesions, does not replace skull radiographs and sellar tomography in the htvestigation of saspected pituitary apoplexy. Hum Patho113 :1141-114 3 , 1982. Pituitary apoplexy is defined as a hemorrltage of sudden onset of infarction in a pituitary gland, t Rarely the gland may be normal, lint often it is enlarged owing to tumor, z Clasically, symptonas consist of sudden onset or worsening of pre-existing headache, sudden deterioration of vision, ocular palsies, confusion, drowsiness, and coma. Brougham et al. z and others 3"4 have reviewed tim condition in detail. A case of an asymptomatic pituitary a d e n o m a m a n i f e s t i n g as a fatal t h i r d - v e n t r i c u l a r h e m o r r h a g e is presented. Tiffs mode of clinical presentation of pituitary apoplexy has not been reported previously.
dead after clinical and electroencephalographic evalnations supported irreversible central nervous system damage. On admission, a computed tomographic (CT) scan with contrast e n h a n c e m e n t (fig. 1, left) revealed a Immorrhagic mass lesion occupying the anterior third ventricle, with dilatation of the ventricular system. T h e impression was of colloid cyst or another type of ueoplasm with lmmorrhage. Autopsy Findings. General autopsy findings were unrenmrkable. Gross examination of tim brain revealed severe brain edema with bilateral uncal herniation and minimal subaraclmoid hemorrlmge. A grossly necrotic suprasellar nmss approximately 2.5 cm in diameter was seen (fig. 1, right). Coronal sections of the brain after fixation showed a dilated third ventricle distended with lmmorrhage and extending slightly into the lateral ventricles. Microscopically, the suprasellar mass showed a papillary ttmmr composed of c o h u n n a r cells with eosinophilic cystoplasm and perivascular rosette fornmtion (fig. 2, left). Sections from the third-ventricular hemorrhage showed cells similar to those in the suprasellar mass, a r r a n g e d in similar perivascular rosettes (fig. 2, rigkt). T h e histologic appearance of the suprasellar t u m o r and of the cells from the hemorrhagic mass in the ttfird ventricle was characteristic of pituitary a d e n o m a with papillary features. DISCUSSION
REPORT OF A CASE A 54-year-okl white woman was admitted to St. Francis Hospital in coma. Prior to admission, a spinal tap at a n o t h e r hospital showed u n i f o r m l y blood-stained cerebrospinal fluid. T h e patient's medical history was sketchy and indicated tlmt she might have been unconscious for 24 hours. She had complained o f a "severe headache" the day prior to admission and was found unconscious the next morning. Her history was not significant except for atrial fibrillation and surgery for diverticulosis of the colon. She had no history of headache or visnal problem. On admission, the patient was comatose a n d on a respirator. Physical examination revealed generalized flaccidity, and no corneal, oculocephalic, or deep t e n d o n reflexes. T i m pupils were dilated and tixed. Respiratory support was discontintted, and tim patient was pronotmced * Assistant Professor of Pathology (Neuropathology), Universit)' of Illinois at Peoria; and Neurot)athologist, St. Francis Hospital, Peoria. Accepted for publication April 12, 1982. Slides fi-ont this case were presented at the diagnostic slide scnfinar of the American Association of Neuropathologists, hekl in New Orleans, June 1980. Address correspondence attd reprint requests to Dr. Kalyanaraman, Department of l'athology, Peoria School of Medicine, One Illini Drive, I'O Box 1649, Peoria, IL 61656.
T h e pituitary gland is an organ with complex functions and anatomic relationships. It is not unusual for a t u m o r arising from the pituitary to manifest in different ways, especially as "pituitary apoplexy." Rare cases or pituitary apoplexy m a n i f e s t i n g as acute p y o g e n i c m e n i n g i t i s with hemiparalysis) internal carotid artery occlusion,6 a n d middle cerebral artery thrombosis 7 emphasize the variations in clinical presentation. T h e outcome of pituitary apoplexy is usually death, but timely diagnosis and surgical intervention lmve significantly reduced tile mortality. 2 Henderson's review of 338 patients with pituitary adenomas from the Cushing series* revealed 21 largely cystic tumors with hemorrhage. T h a t series and Bakay's review of 300 cases of pituitary adenomas from tim Olivecrona series 9 failed to reveal any case nmnifesting as third-ventricular hemorrlmge. T h e pathogenesis of pituitary apoplexy is not well understood. Atheromatous emboli, ~~anticoagulant therapy, tt and roentgen therapy 12 have all been incriminated. With regard to its manifestation as third-ventricular hemorrlmge, I would postulate that when hemorrlmge occurs suddenly in a pituitary adenonm it results in an expansion the pituitary's size. T h e resulting increase in intracapsular pressure within the adenoma may produce acute compression of neighboring structures. T h e floor of the third ventricle may be already thinned by a long-standing pituitary
0046-81771821120011141 $00.60 (~ W. B. Saunders Co.
ll41
HUMAN PATHOLOGY~VOLUME
13, NUMBER 12
December 1982
Figure I (top row). Left, computed tomographic scan with contrast, showing third-ventricular hemorrhage. Right, base of the brain, showing the necrotic suprasellar mass (arrow). Figure 2 (bottom row). Left, histologic section of the suprasellar mass, showing perivascular arrangement of tumor cells. (Hematoxylin-eosin stain, x470.) Right, histologic section of the cells from the third-ventricular hemorrhage, showing a similar arrangement of cells with the erythrocytes in the background. (Hematoxylin-eosin stain, x470.) a d e n o m a e x p a n d i n g in the suprasellar direction. A s u d d e n expansion o f the t u m o r size by h e m o r r h a g e can result in its r u p t u r e into the third ventricle, causing h e m o r r l m g e there. Colloid cyst, ependynloma, and choroid plexus papilloma are the o t h e r common tumors arising from the third ventricle, in a d d i t i o n to a r a r e s u p r a s e l l a r anti t h i r d -
1142
ventricular extension o f a craniopharyngionta. All these tumors are capable o f bleeding and can manifest as thirdventricular Itemorrhage. T h e presence o f a suprasellar mass with histologic features o f pituitary a d e n o m a and the presence o f similar t u m o r ceils in the third-ventrictd,tr h e m o r r h a g e helped establish the diagnosis in the present
CASE STUDIES case. In cases o f papillary t u m o r o f the third ventricle with h e m o r r h a g e a n d necrosis, diagnosis f r o m biopsy m a y be difficult nnless the possibility o f pituitary a d e n o m a is k e p t in m i n d . E l e c t r o n microscopic study nmy be h e l p f u l in establishing a d e f i n i t e diagnosis in such cases. R e c e n t r e p o r t s ~3-ts have e m p h a s i z e d the v a l n e o f c o m p u t e r i z e d t o m o g r a p h y in the diagnosis o f pituitary a p o plexy. O n e r e p o r t t~ e m p h a s i z e d the i m p o r t a n c e o f skull r a d i o g r a p h y in a d d i t i o n to c o m p u t e r i z e d t o m o g r a p h y in the diagnosis. T h e a u t h o r s p o i n t e d out that, in a d d i t i o n to t h e i r two cases o f pituitary a p o p l e x y with sellar e n l a r g e m e n t , 52 o f 54 cases r e p o r t e d in the literature had skull findings o n plain r a d i o g r a p h y that w e r e consistent with pituitary ttnnor. Post et al. ta e m p h a s i z e d the value o f C T scans in the diagnosis o f pituitary a p o p l e x y because o f tile ability o f these scans to detect frank h e m o r r h a g e f r o m necrosis d u e to infarction. H o w e v e r , in all t h e i r cases sellar c h a n g e s a p p a r e n t on plain r a d i o g r a p h y w e r e consistent with pituitary t u m o r . T h i s is also t r u e o f the cases r e p o r t e d by F u j i m o t o et al. tz U n f o r t u n a t e l y , because o f the acute o n s e t o f c o m a in o u r patient, only a C T scala was p e r f o r m e d as tile first diagnostic p r o c e d u r e , a n d plain r a d i o g r a p h s o f tile skull w e r e n e v e r obtained. C o n s i d e r i n g the large size o f the pituitary t u m o r at autopsy, it is likely that plain r a d i o g r a p h s o f tile patient's skull would have revealed, b e f o r e autopsy, h e n t o r r h a g e into the third ventricle f r o m a pituitary tuntor. It may b e c o m e i m p o r t a n t that patients with h e m o r r h a g e d o c u m e n t e d by C T scan in t h e s u p r a s e l l a r a r e a should have plain r a d i o g r a p h i c stt, dies o f the skull a n d tom o g r a p h i c studies o f the sella to avoid missing p r i m a r y sellar t u m o r s with suprasellar extensions. T i l e possibility o f a pituitary a d e n o m a also shotdd be c o n s i d e r e d in tile d i f f e r ential diagnosis o f a t h i r d - v e n t r i c u l a r t n m o r with h e m o r rhage, especially ill biopsy material. ACKNOWLEDGMENTS T i l e a t t t h o r thanks Dr. J o h n H e n d e r s o n a n d Dr. Patrick Elwood for access to the clinical material, Dr. G e o r g e
Zwicky for p e r m i s s i o n to r e p r o d u c e the C T scala, and Dr. J o h n King for access to the autopsy material. Dr. V. A m b r u s h n l a c h e r o f the A r m e d Forces Institnte o f Pathology c o n c u r r e d in the diagnosis.
REFERENCES 1. Conomy JP, Ferguson TH, BrodkayJS, et al: Spontaneous infarction in pituitary tumors: neurologlc and therapeutic aspects. Neurology 25:580, 1975 2. Brougham M, Hensner AP, Adams RD: Acute degenerative changes in adenomas of the pituitary body, with special reference to pituitary apoplexy. J Neurosurg 7:421, 1950 3. Lopez IA: l'itultary apoplexy. J Oslo City itosp 20:17, 1970 4. Rovit RI., Fein JM: Pituitary apoplexy: a review and reappraisal. J Neurosurg 37:280, 1972 5. Shuangshoti S. Prichayudh P: Spontaneous massive bemorrhagic necrosis of chromophobc adenomas in two patients with features of acute pyogenic meningitis and hemiparalysis in one. J Med Assoc Thai 62:324, 1979 6. Rosenbanm "FJ, tlouser O, Wayne EL: Pituitary apoplexy prodocing internal carotid artery occlusion. J Neurosurg 47:599, 1977 7. Schuitker MT, Lelmbert tlB: Apoplexy in a pituitary chromopllobe adenoma producing the syndrome of mkldle cerebral artery thrombosis: a case report. J Neurosurg 9:210, 1952 8. ilenderson WR: The pituitary adenoma: a follow-up study of the surgical results in 338 cases (Dr. ttarvey Cushiug series). Br J Surg 26:81 !, 1939 9. Bakay L: The results of 300 pituitary adenoma operations (Olivecrona series).J Neurosurg 7:240, 1950 10. Sussmart E. Porro RS: Pituitary apoplexy: the role ofatheromatous emboli. Stroke 5:318, 1974 11. Nourizadeh AL, Pitts FW: llemorrlmge into pituitary adenoma during anticoagulant therapy. JAMA 193:623, 1965 12. Weisberg LA: Pituitary apoplexy: association of degenerative change in pituitary adenolna with radiotherapy and detection by cerehral computed tomography. Am J Med 63:109, 1977 13. l'ost MJD, l)avid NT, Glaser JS, et al: l'ituitary apoplexy: diagnosis by computed tomography. Radiology 134:665, 1980 14. Fitzpatrick D, Telis G, McGarry EE, et al: l'ituitary apoplexy: the ioaportauce of skull roentgenograms and computerized tornograpby in diagnosis. JAMA 244:59, 1980 15. Fujimoto M, Yoshiuo E, Ueguchi T, et al: Fluid blood density level demonstrated by computerized tomography in pinfitary apoplexy: report of two cases. J Neurosurg 55:143, 1981
GANGLIONEUROMA CAUSING ACUTE APPENDICITIS MEIIDI ZARABI, MD, AM~ J. PARKER LABACll, MD
A case ofganglioneuroma of the appendb: causing occlusion of the lumen and acute necrotizing appendicitis in a 27-year-old mart is reported, and the literature is reviewed. Neoplastic diseases of the appendbc are uncommon but may manifest as acute appendicitis. The authors have found only three previous reports of acute appendicitis caused by a ganglioneuroma. Hum Pathol 1 3 : 1 1 4 3 1146, 1982. A c u t e appendicitis was r e g i s t e r e d as a surgical entity in 18897 Since that time, a c c u m u l a t e d data have r e v e a l e d a variety of etiologic factors, principally fecaliths, that obstruct the l u m e n a n d cause acute i n f l a m m a t i o n , vascular c o m p r o m i s e , a n d necrosis. Neoplastic diseases o f the appendix are rare, the most common being carcinoid, adenocarcinoma, and primary lymphoma. These tumors may cause occlusion o f the l u m e n a n d i n f l a m m a t i o n o f the
Acc6pted for publication April 12, 1982. Presented at Kansas City Society of Pathologists meeting, December 1981. Address correspondence and reprint requests to Dr. Zarabi, Assistant Professor, Department of Pathology, University of Missouri-Kansas City School of Medicine, 2301 Holmes St, Kansas City, MO 64108.
wall. G a n g l i o n e u r o m a is the least c o m m o n o f the p r i m a r y t u m o r s h a v i n g these effects. REPORT OF A CASE A 27-year-old white m a n was seen ill the e m e r g e n c y r o o m o f T r u m a n Medical C e n t e r with a two-day history o f a b d o m i n a l pain, which started in the periumbilical r e g i o n attd later becante localized in tile right lower q u a d r a n t . H e was in acute distress, with an oral t e m p e r a t u r e o f 99~ a n d a pulse rate o f 96 beats/min. T h e r e was r e b o u n d t e n d e r n e s s at M c B u r n e y ' s point. T i l e leukocyte c o u n t was 12,200/ul, with 52 p e r cent p o l y m o r p h o n u c l e a r leukocytes, 35 p e r cent lymphocytes, 9 p e r c e n t b a n d cells, and 4 p e r c e n t m o n o c y t e s . H e m o g l o b i n was 16.8 g/dl. A n a b d o m i n a l r a d i o g r a p h r e v e a l e d gaseous distention o f the colon. An a p p e n d e c t o m y was p e r f o r m e d , a n d t h e patient was disc h a r g e d f o u r days later. T h e a p p e n d i x was 9 cm in l e n g t h and 1 cm in d i a m e t e r . T h e serosa r e v e a l e d h y p e r e m i a a n d a f i b r i n o p u r u l e n t e x u date o v e r the distal half. T h e p r o x i m a l ltalf a p p e a r e d n o r real, and the c e n t e r disclosed a firm, pale-gray internal mass 2 cm in d i a m e t e r . Sections r e v e a l e d that this o b s t r u c t e d the l u m e n (fig. 1). T h e only a b n o r m a l histologic f i n d i n g in the
0046-8177182/120011143 $00.80 9 W. B. Saunders Co.
1143
12. EvansDJ, WilliamsED: Cytomegalicinclusiondisease in an adult.J Clin Pathol 21:311, 1968 13. WongT, WarnerNE: Cytomegalovirusinclusiondiseasein adults. Arch Pathol 74:403, 1962 14. GoodmanMD, Porter DD: C)'tomegalovirusvasculitiswith fatal colonic hemorrhage. Arch Pathol 96:281, 1973 15. BusselJ, Danou F, Ferchal F, et ah Cytomegalovirusinfectionin malignant blood diseases: clinicaland laboratorydata in 29 patients. Nouv Rev Fr tlematol 20:67, 1978
CLINICALLY ASYMPTOMATIC PITUITARY ADENOMA MANIFESTING AS PITUITARY APOPLEXY AND FATAL THIRD-VENTRICULAR HEMORRHAGE UMA P. KALYANARAMAN,gIBBS, MSc*
A 56-year-old woman with an asymptomatk pituitary adenoma had a fatal third-ventricular hemorrhage of sudden onset. A suprasellar mass with hemorrhage and rupture into the third ventricle was found at autopsy. Histologically, the tumor cells from the suprasellar mass and the third-ventricular hemorrhage were diaguostic of a pituitary adenoma. The pathogenesis of the thirdveutricular hemorrhage from a pituitary adenoma and the need to consider pituitary apoplexy in the differential diagnosis of thirdventricular hemorrhage are discussed. On the basis of a review of the literature, it is felt that computerized tomographic scanning, although most helpful in diaguosis of sellar lesions, does not replace skull radiographs and sellar tomography in the htvestigation of saspected pituitary apoplexy. Hum Patho113 :1141-114 3 , 1982. Pituitary apoplexy is defined as a hemorrltage of sudden onset of infarction in a pituitary gland, t Rarely the gland may be normal, lint often it is enlarged owing to tumor, z Clasically, symptonas consist of sudden onset or worsening of pre-existing headache, sudden deterioration of vision, ocular palsies, confusion, drowsiness, and coma. Brougham et al. z and others 3"4 have reviewed tim condition in detail. A case of an asymptomatic pituitary a d e n o m a m a n i f e s t i n g as a fatal t h i r d - v e n t r i c u l a r h e m o r r h a g e is presented. Tiffs mode of clinical presentation of pituitary apoplexy has not been reported previously.
dead after clinical and electroencephalographic evalnations supported irreversible central nervous system damage. On admission, a computed tomographic (CT) scan with contrast e n h a n c e m e n t (fig. 1, left) revealed a Immorrhagic mass lesion occupying the anterior third ventricle, with dilatation of the ventricular system. T h e impression was of colloid cyst or another type of ueoplasm with lmmorrhage. Autopsy Findings. General autopsy findings were unrenmrkable. Gross examination of tim brain revealed severe brain edema with bilateral uncal herniation and minimal subaraclmoid hemorrlmge. A grossly necrotic suprasellar nmss approximately 2.5 cm in diameter was seen (fig. 1, right). Coronal sections of the brain after fixation showed a dilated third ventricle distended with lmmorrhage and extending slightly into the lateral ventricles. Microscopically, the suprasellar mass showed a papillary ttmmr composed of c o h u n n a r cells with eosinophilic cystoplasm and perivascular rosette fornmtion (fig. 2, left). Sections from the third-ventricular hemorrhage showed cells similar to those in the suprasellar mass, a r r a n g e d in similar perivascular rosettes (fig. 2, rigkt). T h e histologic appearance of the suprasellar t u m o r and of the cells from the hemorrhagic mass in the ttfird ventricle was characteristic of pituitary a d e n o m a with papillary features. DISCUSSION
REPORT OF A CASE A 54-year-okl white woman was admitted to St. Francis Hospital in coma. Prior to admission, a spinal tap at a n o t h e r hospital showed u n i f o r m l y blood-stained cerebrospinal fluid. T h e patient's medical history was sketchy and indicated tlmt she might have been unconscious for 24 hours. She had complained o f a "severe headache" the day prior to admission and was found unconscious the next morning. Her history was not significant except for atrial fibrillation and surgery for diverticulosis of the colon. She had no history of headache or visnal problem. On admission, the patient was comatose a n d on a respirator. Physical examination revealed generalized flaccidity, and no corneal, oculocephalic, or deep t e n d o n reflexes. T i m pupils were dilated and tixed. Respiratory support was discontintted, and tim patient was pronotmced * Assistant Professor of Pathology (Neuropathology), Universit)' of Illinois at Peoria; and Neurot)athologist, St. Francis Hospital, Peoria. Accepted for publication April 12, 1982. Slides fi-ont this case were presented at the diagnostic slide scnfinar of the American Association of Neuropathologists, hekl in New Orleans, June 1980. Address correspondence attd reprint requests to Dr. Kalyanaraman, Department of l'athology, Peoria School of Medicine, One Illini Drive, I'O Box 1649, Peoria, IL 61656.
T h e pituitary gland is an organ with complex functions and anatomic relationships. It is not unusual for a t u m o r arising from the pituitary to manifest in different ways, especially as "pituitary apoplexy." Rare cases or pituitary apoplexy m a n i f e s t i n g as acute p y o g e n i c m e n i n g i t i s with hemiparalysis) internal carotid artery occlusion,6 a n d middle cerebral artery thrombosis 7 emphasize the variations in clinical presentation. T h e outcome of pituitary apoplexy is usually death, but timely diagnosis and surgical intervention lmve significantly reduced tile mortality. 2 Henderson's review of 338 patients with pituitary adenomas from the Cushing series* revealed 21 largely cystic tumors with hemorrhage. T h a t series and Bakay's review of 300 cases of pituitary adenomas from tim Olivecrona series 9 failed to reveal any case nmnifesting as third-ventricular hemorrlmge. T h e pathogenesis of pituitary apoplexy is not well understood. Atheromatous emboli, ~~anticoagulant therapy, tt and roentgen therapy 12 have all been incriminated. With regard to its manifestation as third-ventricular hemorrlmge, I would postulate that when hemorrlmge occurs suddenly in a pituitary adenonm it results in an expansion the pituitary's size. T h e resulting increase in intracapsular pressure within the adenoma may produce acute compression of neighboring structures. T h e floor of the third ventricle may be already thinned by a long-standing pituitary
0046-81771821120011141 $00.60 (~ W. B. Saunders Co.
ll41
HUMAN PATHOLOGY~VOLUME
13, NUMBER 12
December 1982
Figure I (top row). Left, computed tomographic scan with contrast, showing third-ventricular hemorrhage. Right, base of the brain, showing the necrotic suprasellar mass (arrow). Figure 2 (bottom row). Left, histologic section of the suprasellar mass, showing perivascular arrangement of tumor cells. (Hematoxylin-eosin stain, x470.) Right, histologic section of the cells from the third-ventricular hemorrhage, showing a similar arrangement of cells with the erythrocytes in the background. (Hematoxylin-eosin stain, x470.) a d e n o m a e x p a n d i n g in the suprasellar direction. A s u d d e n expansion o f the t u m o r size by h e m o r r h a g e can result in its r u p t u r e into the third ventricle, causing h e m o r r l m g e there. Colloid cyst, ependynloma, and choroid plexus papilloma are the o t h e r common tumors arising from the third ventricle, in a d d i t i o n to a r a r e s u p r a s e l l a r anti t h i r d -
1142
ventricular extension o f a craniopharyngionta. All these tumors are capable o f bleeding and can manifest as thirdventricular Itemorrhage. T h e presence o f a suprasellar mass with histologic features o f pituitary a d e n o m a and the presence o f similar t u m o r ceils in the third-ventrictd,tr h e m o r r h a g e helped establish the diagnosis in the present
CASE STUDIES case. In cases o f papillary t u m o r o f the third ventricle with h e m o r r h a g e a n d necrosis, diagnosis f r o m biopsy m a y be difficult nnless the possibility o f pituitary a d e n o m a is k e p t in m i n d . E l e c t r o n microscopic study nmy be h e l p f u l in establishing a d e f i n i t e diagnosis in such cases. R e c e n t r e p o r t s ~3-ts have e m p h a s i z e d the v a l n e o f c o m p u t e r i z e d t o m o g r a p h y in the diagnosis o f pituitary a p o plexy. O n e r e p o r t t~ e m p h a s i z e d the i m p o r t a n c e o f skull r a d i o g r a p h y in a d d i t i o n to c o m p u t e r i z e d t o m o g r a p h y in the diagnosis. T h e a u t h o r s p o i n t e d out that, in a d d i t i o n to t h e i r two cases o f pituitary a p o p l e x y with sellar e n l a r g e m e n t , 52 o f 54 cases r e p o r t e d in the literature had skull findings o n plain r a d i o g r a p h y that w e r e consistent with pituitary ttnnor. Post et al. ta e m p h a s i z e d the value o f C T scans in the diagnosis o f pituitary a p o p l e x y because o f tile ability o f these scans to detect frank h e m o r r h a g e f r o m necrosis d u e to infarction. H o w e v e r , in all t h e i r cases sellar c h a n g e s a p p a r e n t on plain r a d i o g r a p h y w e r e consistent with pituitary t u m o r . T h i s is also t r u e o f the cases r e p o r t e d by F u j i m o t o et al. tz U n f o r t u n a t e l y , because o f the acute o n s e t o f c o m a in o u r patient, only a C T scala was p e r f o r m e d as tile first diagnostic p r o c e d u r e , a n d plain r a d i o g r a p h s o f tile skull w e r e n e v e r obtained. C o n s i d e r i n g the large size o f the pituitary t u m o r at autopsy, it is likely that plain r a d i o g r a p h s o f tile patient's skull would have revealed, b e f o r e autopsy, h e n t o r r h a g e into the third ventricle f r o m a pituitary tuntor. It may b e c o m e i m p o r t a n t that patients with h e m o r r h a g e d o c u m e n t e d by C T scan in t h e s u p r a s e l l a r a r e a should have plain r a d i o g r a p h i c stt, dies o f the skull a n d tom o g r a p h i c studies o f the sella to avoid missing p r i m a r y sellar t u m o r s with suprasellar extensions. T i l e possibility o f a pituitary a d e n o m a also shotdd be c o n s i d e r e d in tile d i f f e r ential diagnosis o f a t h i r d - v e n t r i c u l a r t n m o r with h e m o r rhage, especially ill biopsy material. ACKNOWLEDGMENTS T i l e a t t t h o r thanks Dr. J o h n H e n d e r s o n a n d Dr. Patrick Elwood for access to the clinical material, Dr. G e o r g e
Zwicky for p e r m i s s i o n to r e p r o d u c e the C T scala, and Dr. J o h n King for access to the autopsy material. Dr. V. A m b r u s h n l a c h e r o f the A r m e d Forces Institnte o f Pathology c o n c u r r e d in the diagnosis.
REFERENCES 1. Conomy JP, Ferguson TH, BrodkayJS, et al: Spontaneous infarction in pituitary tumors: neurologlc and therapeutic aspects. Neurology 25:580, 1975 2. Brougham M, Hensner AP, Adams RD: Acute degenerative changes in adenomas of the pituitary body, with special reference to pituitary apoplexy. J Neurosurg 7:421, 1950 3. Lopez IA: l'itultary apoplexy. J Oslo City itosp 20:17, 1970 4. Rovit RI., Fein JM: Pituitary apoplexy: a review and reappraisal. J Neurosurg 37:280, 1972 5. Shuangshoti S. Prichayudh P: Spontaneous massive bemorrhagic necrosis of chromophobc adenomas in two patients with features of acute pyogenic meningitis and hemiparalysis in one. J Med Assoc Thai 62:324, 1979 6. Rosenbanm "FJ, tlouser O, Wayne EL: Pituitary apoplexy prodocing internal carotid artery occlusion. J Neurosurg 47:599, 1977 7. Schuitker MT, Lelmbert tlB: Apoplexy in a pituitary chromopllobe adenoma producing the syndrome of mkldle cerebral artery thrombosis: a case report. J Neurosurg 9:210, 1952 8. ilenderson WR: The pituitary adenoma: a follow-up study of the surgical results in 338 cases (Dr. ttarvey Cushiug series). Br J Surg 26:81 !, 1939 9. Bakay L: The results of 300 pituitary adenoma operations (Olivecrona series).J Neurosurg 7:240, 1950 10. Sussmart E. Porro RS: Pituitary apoplexy: the role ofatheromatous emboli. Stroke 5:318, 1974 11. Nourizadeh AL, Pitts FW: llemorrlmge into pituitary adenoma during anticoagulant therapy. JAMA 193:623, 1965 12. Weisberg LA: Pituitary apoplexy: association of degenerative change in pituitary adenolna with radiotherapy and detection by cerehral computed tomography. Am J Med 63:109, 1977 13. l'ost MJD, l)avid NT, Glaser JS, et al: l'ituitary apoplexy: diagnosis by computed tomography. Radiology 134:665, 1980 14. Fitzpatrick D, Telis G, McGarry EE, et al: l'ituitary apoplexy: the ioaportauce of skull roentgenograms and computerized tornograpby in diagnosis. JAMA 244:59, 1980 15. Fujimoto M, Yoshiuo E, Ueguchi T, et al: Fluid blood density level demonstrated by computerized tomography in pinfitary apoplexy: report of two cases. J Neurosurg 55:143, 1981
GANGLIONEUROMA CAUSING ACUTE APPENDICITIS MEIIDI ZARABI, MD, AM~ J. PARKER LABACll, MD
A case ofganglioneuroma of the appendb: causing occlusion of the lumen and acute necrotizing appendicitis in a 27-year-old mart is reported, and the literature is reviewed. Neoplastic diseases of the appendbc are uncommon but may manifest as acute appendicitis. The authors have found only three previous reports of acute appendicitis caused by a ganglioneuroma. Hum Pathol 1 3 : 1 1 4 3 1146, 1982. A c u t e appendicitis was r e g i s t e r e d as a surgical entity in 18897 Since that time, a c c u m u l a t e d data have r e v e a l e d a variety of etiologic factors, principally fecaliths, that obstruct the l u m e n a n d cause acute i n f l a m m a t i o n , vascular c o m p r o m i s e , a n d necrosis. Neoplastic diseases o f the appendix are rare, the most common being carcinoid, adenocarcinoma, and primary lymphoma. These tumors may cause occlusion o f the l u m e n a n d i n f l a m m a t i o n o f the
Acc6pted for publication April 12, 1982. Presented at Kansas City Society of Pathologists meeting, December 1981. Address correspondence and reprint requests to Dr. Zarabi, Assistant Professor, Department of Pathology, University of Missouri-Kansas City School of Medicine, 2301 Holmes St, Kansas City, MO 64108.
wall. G a n g l i o n e u r o m a is the least c o m m o n o f the p r i m a r y t u m o r s h a v i n g these effects. REPORT OF A CASE A 27-year-old white m a n was seen ill the e m e r g e n c y r o o m o f T r u m a n Medical C e n t e r with a two-day history o f a b d o m i n a l pain, which started in the periumbilical r e g i o n attd later becante localized in tile right lower q u a d r a n t . H e was in acute distress, with an oral t e m p e r a t u r e o f 99~ a n d a pulse rate o f 96 beats/min. T h e r e was r e b o u n d t e n d e r n e s s at M c B u r n e y ' s point. T i l e leukocyte c o u n t was 12,200/ul, with 52 p e r cent p o l y m o r p h o n u c l e a r leukocytes, 35 p e r cent lymphocytes, 9 p e r c e n t b a n d cells, and 4 p e r c e n t m o n o c y t e s . H e m o g l o b i n was 16.8 g/dl. A n a b d o m i n a l r a d i o g r a p h r e v e a l e d gaseous distention o f the colon. An a p p e n d e c t o m y was p e r f o r m e d , a n d t h e patient was disc h a r g e d f o u r days later. T h e a p p e n d i x was 9 cm in l e n g t h and 1 cm in d i a m e t e r . T h e serosa r e v e a l e d h y p e r e m i a a n d a f i b r i n o p u r u l e n t e x u date o v e r the distal half. T h e p r o x i m a l ltalf a p p e a r e d n o r real, and the c e n t e r disclosed a firm, pale-gray internal mass 2 cm in d i a m e t e r . Sections r e v e a l e d that this o b s t r u c t e d the l u m e n (fig. 1). T h e only a b n o r m a l histologic f i n d i n g in the
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