|Year : 1980 | Volume
| Issue : 3 | Page : 204-6
CNS involvement in mycosis fungoides.
VV Jayakar, GH Tilve, RJ Fernandes, UG Rege, RN Soman
V V Jayakar
|How to cite this article:|
Jayakar V V, Tilve G H, Fernandes R J, Rege U G, Soman R N. CNS involvement in mycosis fungoides. J Postgrad Med 1980;26:204-6
|How to cite this URL:|
Jayakar V V, Tilve G H, Fernandes R J, Rege U G, Soman R N. CNS involvement in mycosis fungoides. J Postgrad Med [serial online] 1980 [cited 2023 Oct 1 ];26:204-6
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Mycosis fungoides is an interesting condition as it intrigues both physicians and dermatologists. It exemplifies cutaneous manifestations of internal malignancy. Like Sezary syndrome, it is a primary cutaneous lymphoma. The present case under discussion has a course much different from the natural history described in standard textbooks.
A. D., a 23 year old man first noticed a swelling over the scalp in the left parietal region at the age of 19 years. Biopsy of this slowly progressive lesion had shown a few characteristic 'Mycosis Fungoides cells'. He was treated with arsenical drops in gradually increasing dosage. The lesion had responded completely to arsenicals.
Three years later, he developed slowly progressive weight loss, but repeated examination did not reveal any fresh lesions. Three months prior to admission to this hospital, the patient developed pain in the back, radiating down the legs, for which he was seen in another hospital and diagnosed to have ankylosing spondylitis. He was put on prednisolone and ibuprofen.
One month prior to the admission, he had developed glands on both sides of the neck. Fifteen days later he developed diplopia, numbness on left side of the face and repeated vomiting and diplopia, but not much of headache. At this stage he was referred to us.
He was a well built and poorly nourished young man. Temperature was normal. Pulse was 100/min., B.P. was 120/80 mm. of Hg. He was pale. No icterus was seen. No pedal edema was detected. Bilateral, discrete, non-tender, firm, cervical lymph nodes were palpable. There was no sternal tenderness. There was congenital naevus on the anterior abdominal wall on the right side. The scalp showed 3 nodular, nontender, 2 cm x 1 cm swellings, probably subaponeurotic in the right posterior parietal region. The liver was palpable 2.5 cm. below the costal margin, soft and non-tender; spleen was not palpable; no lymph nodes or other intra-abdominal masses were felt. Cardiovascular and respiratory system examinations were normal.
C.N.S. examination showed bilateral papilloedema, bilateral 6th nerve palsy L > R, left VIIth and left Vth nerve palsy. Pupils were normal. There was no VIIth nerve palsy, nor any cerebellar signs. Rest of the CNS was normal. ENT examination was negative.
The presumptive diagnosis was mycosis fungoides in tumour stage with systemic dissemination.
Haemoglobin was 9.7 gm%. Retic count was 2.6%. Total WBC count was 6700/c.mm. with N: 52%; L:40% and E:1%. ESR was 35 mm. at the end of 1 hour (Westergren) . Urine, stools and X-Ray chest examinations were normal. Liver function tests, BUN and blood sugar were normal. X-Rays of the skull suggested minimal demineralization of the posterior clinoids. Serum uric acid was 4.5 mg%; serum calcium was 13.4 mg%; serum phosphorus was 4.5 mg% and alkaline phosphatase was 9.0 B.U. Liver biopsy did not reveal any abnormality. Cerebrospinal fluid showed 127 mg% of proteins and 3 lymphocytes per field. No malignant cells were seen. Sugar was 80 mg%. Chlorides were 680 mg%. Bone marrow did not show abnormal cells. Left carotid angiogram showed i) minimal stretching of the anterior cerebral artery suggestive of hydrocephalus. ii) Venous phase showed anterior displacement of torcula and posteriormost portion of sagittal sinus, consistent with an extradural mass in the occipital region.
Aspiration biopsy of the lymph node showed abnormal cells consistent with mycosis fungoides. Lymph node biopsy showed (i) complete effacement architecture; (ii) diffuse proliferation of medium to large sized round cells with dark staining nuclei and moderate eosinophilic cytoplasm forming loose clusters and sheets; (iii) admixture of a few lymphocytes and plasma cells; (iv) cells with rarely encountered atypical features, and (v) occasional foci of necrosis consistent with lymph node involvement in mycosis fungoides.
In the ward, he developed some more subcutaneous and subaponeurotic nodules in the occipital and the left frontal regions, severe lower dorsal backache and weakness in both lower limbs, which progressed to paralysis in 24 hours. The physical examination suggested cord compression at D11 level. X-Ray of the dorsal spine showed destruction of the pedicles and fuzzy upper border of the 8th dorsal vertebra. In view of the histological diagnosis of disease, the myelography and ventriculography were postponed and it was decided to give a trial of chemotherapy. In view of previous good response to arsenicals, the patient was treated with arsenical drops and in addition was given injection Cyclophosphamide 500 mg. I.V. every week. After one month his bladder function had returned to normal and he was moving both lower limbs. His power in the lower limbs was of grade III.
Mycosis fungoides is a primary cutaneous,  lymphoma seen between ages of 30 and 60 years. The course is divided in 3 stages: (i) Premycotic stage, when it resembles eczema and other lesions; (ii) Mycotic stage or plaque stage when lesion is raised above the surface like a plaque. It is at this stage that abnormal cells make their appearance in histological preparations. This stage is identified by a characteristic skin biopsy anomaly known as "Pautrier's abscess" seen in the dermis. (iii) Tumour stage-where the tumour tissue breaks down on the surface liberating necrotic material. In this stage, the tumour becomes invasive and infiltrates the internal organs. Local lymph node is the commonest organ, the lungs, liver and kidneys coming next. Brain involvement is unusual. The type which progresses through the three characteristic stages is termed as "Alibert" variety. The rare type known as " 'd' emblee" variety is characterized by direct appearance of tumour stage without going through skin stages.
The unusual features in our case are: (1) appearance of disease at the early age of 19 years. (2) The rare type of presentation viz. " 'd' emblee" variety, as the patient both in primary stage and in relapse presented directly in the tumour stage without undergoing skin stages (3) The brain and spinal cord involvement which itself is rare, but moreover this involvement was seen in absence of invasion of blood, bone marrow and liver. Weber and McGaven in 1967 reported their own case and reviewed 4 other published cases. All of them had diffuse infiltration of brain parenchyma by reticulum cell sarcoma cells (in addition to lungs, liver and kidneys) and resulted in an appearance of diffuse bilateral CNS signs and increased intracranial tension. In our case the patient had all signs explainable on the basis of extradural deposits.
|1||Block, J. B., Edglomb, J., Eisen, A. and van Scot E. J.: Mycosis fungoides: natural history and aspects of its relationship to other malignant lymphomas. Amer. J. Med., 34: 218-235, 1963.|
|2||Cyr, D. P., Geokar, M. C. and Worsley, G. H.: Mycosis fungoides: Arch. Dermatol., 94: 558-573, 1966.|
|3||Domonkos, A. N.: Mycosis fungoides, Malignant Lymphoma and Allied Diseases. In, Andrew's "Diseases of the skin". 6th Edition, W. B. Saunders Co., Philadelphia, London, Toronto, 1971, pp. 823-830. |
|4||Maguire, A.: Treatment of Mycosis fungoides with cyclophosphamide and chlorpromazine. Brit. J. Dermatol., 80: 54 57, 1968. as quoted by Domonkos.|
|5||Samman, P. D.: Mycosis fungoides. In, Chapter on Reticulosis. In, "Textbook of Dermatology". Edited by Rook, A., Wilkinson, D. S., Ebling, F.J.G., Vol. II. Second Edition. Blackwell Scientific Publications. Oxford, London, Edinburgh, Melbourne, 1972. p. 1396.|
|6||van Scott, E. J. and Vonderheld, E. C.: In, "Mycosis Fungoides and Sezary Syndrome in Hematology", (2nd Edition). Edited by Williams, W. J., Beutter, E., Erslev, A. J . , Rundles, R. W . , McGraw-Hill Book Company, New York. `A Blackiston Publication', 1977.|
|7||Weber, M. B. and McGaven, M. H.: Mycosis fungoides involving the brain. Arch. Neurol., 16: 645-650, 1967. |