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Show journal of Nctuv • 0; I/ II/ I « / » K% M/ ( 6( 3): 204- 207, 735) 6. © 1996 Ijppincott- Raven Publishers, Philadelphia Cavernous Sinus Syndrome in Hodgkin's Disease Scott E. Kasner, M. D., Steven L. Galetta, M. D., and David J. Vaughn, M. D. Hodgkin's disease has not been reported to produce an isolated cavernous sinus syndrome, although this phenomenon is well- described in non- Hodgkin's lymphoma. We review the 16 cases of cavernous sinus syndrome caused by non- Hodgkin's lymphoma and report two patients with Hodgkin's disease in clinical remission who developed recurrent disease in the cavernous sinus. MR1 revealed a mass lesion in the left cavernous sinus in each patient. Corticosteroids and radiation therapy were effective palliative measures. In both patients, recurrence in the cavernous sinus preceded other systemic evidence of recurrent Hodgkin's disease. Key Words: Cavernous sinus syndrome- Hodgkin's disease. Central nervous system involvement by systemic lymphoma is uncommon but may include meningeal infiltration, mass lesions, and paraneoplastic processes. Sixteen patients have been described in whom non- Hodgkin's lymphoma manifested as a cavernous sinus syndrome ( 1- 10). Hodgkin's disease has been reported to cause numerous neurologic disturbances, but has not previously been reported to produce an isolated cavernous sinus syndrome. A single report described a patient who was diagnosed with Tolosa- Hunt syndrome and subsequently developed Hodgkin's disease one year later ( 11). Here we describe two patients with Hodgkin's disease in remission who presented with recurrence in the cavernous sinus. CASE REPORTS Manuscript received July 12, 1995; accepted August 4, 1995. From the Departments of Neurology ( S. E. K., S. L. G.) and Medicine ( D. J. V.), Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, U. S. A. Address correspondence and reprint requests to Scott E. Kasner, Department of Neurology, Hospital of the University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA 19104, U. S. A. Case 1 A 28- year- old man noted pressure and swelling in his neck. Examination revealed bilateral supraclavicular lymphadenopathy. Biopsy of a right neck node demonstrated nodular sclerosing Hodgkin's disease. Chest CT showed moderate lymphadenopathy in the superior and anterior mediastinum. There were no abnormal lymph nodes on abdominal CT. A staging laparotomy revealed a normal spleen. The bone marrow was normal. He was diagnosed with pathological state IIA Hodgkin's disease and treated with definitive radiation therapy. Approximately three months later, he developed progressive peripheral lymphadenopathy, and a biopsy of a cervical node demonstrated recurrent Hodgkin's disease. He subsequently was found to have lymphadenopathy in the abdomen and pelvis, as well as involvement of the bone marrow. He was treated with combination chemotherapy resulting in clinical remission of his lymphoma. 204 CAVERNOUS SINUS SYNDROME IN HODGKIN'S DISEASE 205 He remained well for several months until he experienced pain in the left eye without any other symptom. During the next three days, he developed left eye dryness and left facial numbness. Neuro- ophthalmologic examination demonstrated normal visual acuity and normal visual fields. Pupils were symmetrically reactive without an afferent pupillary defect. Funduscopic examination was normal. There was a marked left abduction deficit. Motility of the right eye was normal. There was decreased sensation to light touch and cold in the distribution of the ophthalmic branch of the left fifth cranial nerve ( V[). The remainder of the neurologic examination was normal. He was diagnosed with a left cavernous sinus syndrome. MRI of the brain revealed a large left cavernous sinus mass with involvement of Meckel's cave ( Fig. 1A). Cerebrospinal fluid ( CSF) cytology was normal. He received whole- brain radiation therapy and corticosteroids. His symptoms completely resolved during the next month, although he continued to have a moderate esophoria. A subsequent MRI revealed a marked decrease in the size of the left cavernous sinus mass ( Fig. IB). The patient later developed recurrent disease in his chest and spine, FIG. 1. Patient 1. A: T1- weighted, gadolinium-enhanced MRI demonstrating abnormal enlargement and enhancement within the left cavernous sinus at the time of presentation. B: After palliative therapy, there is a marked reduction of the mass. and ultimately died. A postmortem examination was declined. Case 2 A 19- year- old woman presented with a four- year history of fluctuating cervical lymphadenopathy. Biopsy of a left axillary lymph node revealed nodular sclerosing Hodgkin's disease. CT revealed diffuse lymphadenopathy in the chest, but no infra-diaphragmatic nodes were identified. Staging laparotomy revealed a normal spleen, but lymphoma was found in a celiac node. The bone marrow was normal. She subsequently developed intermittent fevers of unknown origin. She was diagnosed with stage IIIB Hodgkin's disease and treated with combination chemotherapy. She had clinical and radiologic remission of her lymphoma. Four months later, a routine chest film revealed recurrent lymphadenopathy. She was treated with salvage chemotherapy without response. She then received salvage radiation therapy with clinical remission. Two months later, she developed low back pain and was found to have relapsed lymphoma involving the sacrum. The pain resolved with palliative radiation therapy. She remained in remission for one month until she developed diplopia and headache. Neuro-ophthalmologic examination demonstrated normal acuity, visual fields, and pupils. Funduscopic examination was normal. There was reduced abduction of the left eye. Facial sensation and strength were normal. The remainder of the neurologic examination was normal except for longstanding hy-poactive deep tendon reflexes. MRI of the brain revealed a mass adjacent to the left cavernous sinus and diffuse meningeal enhancement ( Fig. 2). CSF studies including cytology were normal. She was treated with whole- brain radiation therapy and corticosteroids, and her diplopia resolved. A subsequent MRI showed minimal resolution of the left cavernous sinus mass. However, she continued to have active disease and died several months later. A postmortem examination was declined. DISCUSSION Neurologic deficits are rare in Hodgkin's disease, occurring in only about 0.5% of patients ( 12), even though the central and peripheral nervous systems may be pathologically involved in nearly 15% ( 13). Non- Hodgkin's lymphoma invades the nervous system more aggressively and more frequently, with nearly 30% of patients developing / Neum- OpMliulmul, Vol. 16, No. 3, 29.96 206 S. E. KASNER ET Ah. may occur at any site, and the neurologic findings will depend on the location of the tumor. The most common presenting symptoms of Hodgkin's disease in the central nervous system are cranial nerve palsies, headache, focal weakness, and seizures ( 12). Usually there is concurrent disease in either the lymph nodes or the bone marrow, although patients have been described in whom the neurologic deficits preceded the detection of systemic disease. In a report by Lascelles and Burston ( 14), a 36- year- old woman presented initially with dysphagia and dysarthria and later developed complete left ophthalmoplegia. At autopsy, Hodgkin's lymphoma was found infiltrating cranial nerves III- XII and had spread into the cavernous sinus bilaterally. The pituitary gland was also infiltrated. Widespread Hodgkin's disease within the brain was discovered in parallel with diffuse systemic disease involving the lungs and peripheral lymph nodes. A cavernous sinus syndrome occurring in isolation has not been reported as the initial manifestation of either new or recurrent Hodgkin's disease. Sakamoto et al. reported a 38- year- old man with a right cavernous sinus syndrome ( 11). Obstruction of the right superior orbital vein within the cavernous sinus was demonstrated by orbital venography. His symptoms resolved with corticosteroid treatment, and the diagnosis of Tolosa- Hunt syndrome was made on the basis of the findings and the clinical response to steroids. One year later, he developed left cervical and axillary lymphadenopathy and was found to have Hodgkin's disease. Although the Tolosa- Hunt syndrome characteristically responds rapidly to steroids, it is not a pathological diagnosis and should be considered only as a diagnosis of exclusion ( 2). Other causes of the cavernous sinus syndrome, TABLE 1. Cavernous sinus syndrome in non- Hodgkin's lymphoma ( NHL) Reference Crocker 1966 ( 1) Fowler 1975 ( 2) Mitsumoto 1980 ( 3) Kori 1982 ( 4) Koh 1983 ( 5) Slamovits 1983 ( 6) Julien 1984 ( 7) Post 1985 ( 8) Delpassand 1988 ( 9) Cyhan 1994 ( 10) Age ( yr) 44 61 43 79 57 46 70 45 40 5 Right/ left left right right right right right right right right right right right right left right bilateral Histology undifferentiated malignant lymphoma malignant lymphoma diffuse histiocytic malignant lymphoma malignant lymphoma malignant lymphoma malignant lymphoma malignant lymphoma malignant lymphoma diffuse poorly differentiated NHL histiocytic malignant lymphoma lymphosarcoma, centroblastic- type lymphosarcoma, high grade malignancy malignant lymphoma large cell immunoblastic small, noncleaved NHL FIG. 2. Patient 2. T1- weighted, gadolinium- enhanced MRI demonstrating abnormal enlargement and enhancement within the left cavernous sinus. Diffuse meningeal enhancement is also present. neurologic signs and symptoms during the course ot their illness ( 13). Delpassand and Kirkpatrick ( 9) reviewed 10 previously reported cases ( 3- 5,7) of cavernous sinus involvement by systemic non- Hodgkin's lymphoma and provided one additional case. The most common clinical findings in these patients were sixth nerve palsy and sharp retroorbital pain. Five additional patients have been reported ( 1,2,6, 8,10). All patients in whom the histopathology was specifically reported were caused by non- Hodgkin's lymphoma ( Table 1). Surprisingly, in the majority ( 13/ 16) of cases, the lymphoma involved the right cavernous sinus. Hodgkin's disease may rarely metastasize to the central nervous system by producing either meningitis or a compressive mass lesion. Meningeal infiltration may cause local injury to the cranial nerves or spinal roots. Compressive lesions most frequently occur around the spinal cord but may involve the brain as well. Intracranial metastases / Neuro- Ophthalmol, Vol. 16, No. 3, 1996 CAVERNOUS SINUS SYNDROME IN HODGKIN'S DISEASE 207 particularly lymphomas, may also respond to steroids. CSF cytology analysis cannot be relied upon to exclude neoplasm since this has been found to be negative for malignancy in all reported cases of lymphoma within the cavernous sinus. It is possible that the patient reported by Sakamoto may have had his first manifestation of Hodgkin's lymphoma within the cavernous sinus, which was then followed by systemic disease one year later. Our patients had known Hodgkin's disease in clinical remission until recurrence in the cavernous sinus. Both had MRI evidence of localized infiltration within the cavernous sinus. Both had normal CSF studies, including cytology. A biopsy of the cavernous sinus was not considered in these patients because they each had a known advanced malignancy and there was no other evidence to suggest thrombosis, infection, or a benign inflammatory process. We treated both patients with corticosteroids and whole- brain radiation therapy and achieved complete resolution of neurological symptoms. No other neurologic disturbances developed subsequently. Our cases are significant for several reasons. The cavernous sinus syndrome can be the sole clinical manifestation of recurrent and possibly new- onset Hodgkin's disease. Although lymphomatous involvement of the brain usually occurs late in the disease, the neurologic signs and symptoms may precede other systemic findings. Moreover, Hodgkin's disease in the cavernous sinus may be effectively palliated with steroids and radiation therapy. REFERENCES 1. Crocker LG, Lang GE. Cavernous sinus thrombosis mimicked by CNS lymphoma. JAMA 1966; 195: 968- 9. 2. Fowler TJ, Earl CJ, McAllister VL, McDonald WI. Tolosa- Hunt syndrome. The danger of an eponym. Br J Ophthalmol 1975; 59: 149- 54. 3. Mitsumoto H, Sweeney PJ. Oculomotor palsy from malignant lymphoma. Cleve Clin Q 1980; 46: 333- 8. 4. Kori SH, Mitsumoto H. Cavernous sinus involvement in lymphoma [ Abstract]. Neurology 1982; 32: A74. 5. Koh CS, Tan CT, Alhady SF. Cavernous sinus syndrome: a manifestation of non- Hodgkin's lymphoma of the ethmoid sinus. Med / Aust 1983; 2: 451- 2. 6. Slamovits TL, Cahill KV, Sibony PA, Dekker A, Johnson BL. Orbital fine- needle aspiration biopsy in patients with cavernous sinus syndrome. / Neurosurg 1983; 59: 1037- 42. 7. Julien U, Ferrer X, Dreuillard J, Phillipe JC, Desbordes P. Cavernous sinus syndrome due to lymphoma. / Neurol Neurosurg Psychiatry 1984; 47: 558- 60. 8. Post MJD, MendezDR, Kline LB, Acker JD, Glaser JS. Metastatic disease to the cavernous sinus: clinical syndrome and CT diagnosis. / Coiuput Assist Toinogr 1985; 9: 115- 20. 9. Delpassand ES, Kirkpatrick JB. Cavernous sinus syndrome as the presentation of malignant lymphoma: case report and review of the literature. Neurosurgery 1988; 23: 501- 4. 10. Ceyhan M, Erdem G, Kanra G, Kaya S, Onerci M. Lymphoma with bilateral cavernous sinus involvement in childhood. Pediatr Neurol 1994; 10: 67- 9. 11. Sakamoto N, Aoyagi Y, Ozaki Y, Kanayama M, Kimula Y, Akabane H. Hodgkin's disease associated with Tolosa- Hunt syndrome [ in Japanese], Riusho Kctsueki jpn j Clin He-matol 1990; 31: 172- 6. 12. Sapozink MD, Kaplan HS. Intracranial Hodgkin's disease. A report of 12 cases and review of the literature. Cancer 1983; 52: 1301- 7. 13. Miller NR. Tumors derived from hematopoeitic cells and tissue. In: Miller NR, ed. Walsh and lloyt's Clinical Neuro- Ophthalmology, 4th ed. Baltimore: Williams & Wilkins, 1982: 1587- 618. 14. Lascelles RG, Burston J. Hodgkin's disease: disease presenting with symptoms of cranial nerve involvement. Arch Neurol 1962; 7: 359- 64. / Neuro- Ophtlmlmol, Vol. 16, No. .3, 1996 |
