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Show Journal of Neuro- Ophthalmology 20( 1): 35- 37, 2000. i 2000 Lippincott Williams & Wilkins, Inc., Philadelphia Adrenal Cortical Carcinoma Metastatic to the Brain in a Child Alejandra Montenegro Piniella, MD, and R. Michael Siatkowski, MD A 9- year- old girl presented with profoundly decreased vision in both eyes and bilateral optic disc swelling. Magnetic resonance imaging showed a large intraventricular mass. Excisional biopsy diagnosed metastatic adrenal cortical carcinoma ( ACC). This is, to the best of our knowledge, the first reported case of ACC metastatic to the brain in a child. It also illustrates the importance of differentiating optic neuritis from papilledema in children. Key Words: Adrenal cortical carcinoma- Optic neuritis- Papilledema. Adrenal cortical carcinoma ( ACC) is quite rare, constituting only 0.05% to 0.2% of all cancers, equivalent to a rate of 2 per million worldwide ( 1). Common metastatic sites are regional lymph nodes, lung, liver, and bone. Only a handful of cases of adrenal cortical carcinoma metastatic to the brain have been reported in adults ( 2) and none in children. We report such a case with isolated visual loss. CASE REPORT A 3- year- old white girl presented with precocious puberty and Cushing syndrome. Workup indicated a large left- sided adrenal tumor, which was excised. Histopathologic interpretation was adenocarcinoma of the adrenal cortex. Postoperatively, endocrine function normalized, and regular follow- up with the pediatric endocrine service indicated normal growth and no sign of recurrent disease. In June 1998, at age 9 years, she experienced blurred vision for 2 weeks, which became severe enough to produce difficulty ambulating. Optometric evaluation diagnosed bilateral optic neuropathy, and she was referred to a pediatric neurologist, who diagnosed optic neuritis, with referral to the pediatric neuro- ophthalmology service. On presentation, visual acuity was 4/ 120 OD and 1.5/ 180 OS. Ocular motility, anterior segments, and in- Manuscript received June 8, 1999; accepted October 7, 1999. From the Bascom Palmer Eye Institute ( AMP), University of Miami School of Medicine, Miami, Florida; and the Dean A. McGee Eye Institute ( RMS), University of Oklahoma School of Medicine, Oklahoma City, Oklahoma. Address correspondence to R. Michael Siatkowski, MD, Dean A. McGee Eye Institute, University of Oklahoma School of Medicine, 608 Stanton L. Young Boulevard, Oklahoma City, OK 73104. traocular pressures were normal. Dilated fundus examination indicated chronic bilateral optic disc swelling with axoplasmic stasis, narrowed retinal arteries, and venous collapse to only firm compression of the globe ( Fig. 1). Orbital echography showed bilateral disc elevation with a positive 30° test, indicating increased subarachnoid fluid around both optic nerves ( 3). The patient was admitted to the hospital and begun on Solumedrol ( Pharmacia- Upjohn, Kalamazoo, MI), 100 mg intravenously every 6 hours. The following morning, magnetic resonance imaging ( MRI) of the brain was performed, which showed a large tabulated mass in the left lateral ventricle suggestive of a choroid plexus papilloma. There was marked hydrocephalus and occipitoparietal vasogenic edema, as well as chiasmal compression and early tonsillar herniation ( Fig. 2). A difficult but complete resection was performed along with ventriculostomy. Histopathologic examination indicated metastatic adrenal carcinoma with cellular morphology identical to the adrenal mass excised 6 years earlier. Systemic workup for further metastatic disease ( MRI of thorax, abdomen, and pelvis, and bone scan) was negative. One year later, she remained disease- free. Visual acuity was hand motion OD and no light perception OS. DISCUSSION To our knowledge, this is the first report of childhood adrenal carcinoma metastatic to the brain. It is even more unusual in that the presenting symptoms were completely visual. This made optic neuritis the likeliest of the differential diagnoses at presentation. Optic neuritis in children frequently presents with bilateral optic disc swelling ( 4). Although this was present, there was no history of preceding viral illness or vaccination, common factors associated with pediatric optic neuritis ( 4). The temporal profile of this case would be typical for optic neuritis, because our patient had a chief complaint of rapid and profound visual loss. However, the optic nerves had features of chronic edema such as axoplasmic stasis, retinal arteriolar narrowing, and venous distention. Disc swelling in optic neuritis is more acute- appearing with hyperemia and only rare nerve fiber layer hemorrhages. Finally, MRI of the brain showed the cause of the optic nerve swelling: chronic papilledema secondary to an intracranial mass. The mass was identified as adrenal cortical carcinoma, 6 years after resection of the primary. 35 36 A. M. PIN1ELLA AND R. M. S1ATKOWSK1 FIG. 1. A and B: Dilated fundus examination showed chronic bilateral optic disc swelling with axoplasmic'stasis and underlying atrophy. Note the attenuation of the retinal vasculature. Adrenal cortical carcinoma is a rare tumor. It has a bimodal occurrence by age, with the first peak being before 5 years of age and the second being in the fourth and fifth decades of life ( 1,5). Adrenal cortical carcinoma is a malignant neoplasm showing partial or complete histologic and functional differentiation ( 1). The distinction between ACC and adenoma is pathologically based on tumor weight, necrosis, vascular invasion, and number of mitotic figures ( 1,6). Clinically, it is based on constitutional symptoms, feminization, and distant metastases ( 1,6). Virilization and extension of the tumor beyond the adrenal capsule are suggestive, but not diagnostic, of malignancy ( 1,6). Therefore, diagnosis of ACC is often not clear- cut. Our patient's primary tumor at the age of 3 was thought to be malignant, based primarily on FIG. 2. MRI of the brain showed a large tabulated mass in the left lateral ventricle suggestive of a choroid plexus papilloma. Marked hydrocephalus and occipitoparietal vasogenic edema were present. the gross tumor size. Because ACC is highly malignant, surgical resection is the only potentially curable treatment ( 7). Chemotherapy with mitotane ( o, p- DDD) has been shown to induce partial remission in 22% to 60% of patients and complete but temporary remission in a handful of patients ( 8,9). Other chemotherapeutic agents have had no success in ACC ( 1). Abdominal radiation has been palliative in some inoperable masses or in bony metastases ( 10). Prolonged remissions have been reported after resection of hepatic and pulmonary metastases ( 11). Local spread characterizes ACC. Tumor spreads into the kidneys, retroperitoneal and peritoneal spaces, diaphragm, and vena cava. Metastases to regional lymph nodes, lung, liver, and bone are also seen ( 1). Only six cases of ACC metastasis to the central nervous system have been previously reported, all in adults ( 2,12). Prognosis in ACC is poor. In one analysis of 105 patients ( adults and children), the 5- year survival was 22% and depended mostly on the stage of disease at diagnosis ( 8). Mean survival was 18 months ( 8). Seventy percent of patients have local spread or distant metastasis at presentation. Children tend to present earlier because they are more likely to have hormonal disturbances caused by their malignancy. Up to 93% present with virilization, but only rarely ( as in our case) with precocious puberty or Cushing syndrome ( 5). In one report, 17 of 26 ( 65%) of children remained in complete remission after complete tumor resection ( 13). This case illustrates an extremely unusual presentation of an uncommon disease. It emphasizes the importance of a detailed medical history and reinforces the concept that bilateral visual acuity loss with disc swelling in children is not always attributable to optic neuritis. REFERENCES 1. Norton JA. Adrenal tumors. In: DeVita VT, Hellman S, Rosenberg SA, eds. Cancer, principles and practice of oncology. Philadelphia: Lippincott, 1997: 1659- 77. 2. Kubota Y, Iwai T, Nakatani K, Sakai N, Hara A. Central nervous system metastasis from non- functioning adrenocortical carcinoma: report of a case. No Shinkei Geka- Neurological Surgery 1997; 25: 1039^ 2. 3. Ossoinig KC, Cennano G, Byrne SP. Echographic differential diagnosis of optic nerve lesions. Ultrasonography in ophthalmology. Dordrecht, Dr. W. Junk, 1981: 327- 32. 4. Beck RJ. Optic neuritis. In: Miller NR, Newman NJ, eds. Walsh J Neuro- Ophthalmol, Vol. 20, No. 1, 2000 CHILDHOOD ADRENAL CORTICAL CARCINOMA 37 and Hoyt's clinical neuro- ophthalmology. Baltimore: Williams & Wilkins; 1998: 599- 647. 5. Wooten MD, King DK. Adrenal cortical carcinoma: epidemiology and treatment with mitotane and a review of the literature. Cancer 1993; 72: 3145- 55. 6. Weiss LM. Comparative histologic study of 43 metastasizing and nonmetastasizing adrenocortical tumors. Am J Surg Pathol 1989; 13: 102. 7. Lee JE, Berger DH, el- Naggar AK, et al. Surgical management, DNA content, and patient survival in adrenocortical carcinoma. Surgery 1995; 188: 1090- 8. 8. Luton JP, Cerdas S, Billaud L, et al. Clinical features of adrenocortical carcinoma: prognostic factors and the effect of mitotane therapy. N Engl J Med 1990; 322: 1195- 201. 9. Kornely E, Schlaghecke R. Complete remission of metastasized adrenocortical carcinoma under o, p- DDD. Exp Clin Endocrinol 1994; 102: 50- 3. 10. Robles JE, Zudaire JJ, Berian JM. Primary adrenal carcinoma. Adas Urologicas Espanolas 1993; 17: 30- 4. 11. Jensen JC, Pass HI, Sindelar WF, et al. Recurrent or metastatic disease in select patients with adrenocortical carcinoma: aggressive resection vs chemotherapy. Arch Surg 1991; 126: 457- 61. 12. Hogan TF, Gilchrist KW, Westring DW, et al. A clinical and pathological study of adrenocortical carcinoma. Cancer 1980; 45: 2880- 3. 13. Ribeiro RC, Sandrini Neto R, Schell MJ, et al. Adrenocortical carcinoma in children: a study of 40 cases. J Clin Oncol 1990; 8: 67- 74. * V • v. - > ••> • t O <> •> i j J Neuro- Ophthalmol, Vol. 20, No. 1, 2000 |
