Reticulosarcoma of the eye treated with cyclophosphamide

AM Gokhle; AJ Desai

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Year : 1966 | Volume : 14 | Issue : 3 | Page : 124-127

Reticulosarcoma of the eye treated with cyclophosphamide

AM Gokhle, AJ Desai
Department of Ophthalmology and Haematology, Bai Yamunabai Nair Hospital, Bornbay, India

Date of Web Publication

16-Jan-2008

Correspondence Address:
A M Gokhle
Department of Ophthalmology and Haematology, Bai Yamunabai Nair Hospital, Bornbay
India

Source of Support: None, Conflict of Interest: None

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How to cite this article:
Gokhle A M, Desai A J. Reticulosarcoma of the eye treated with cyclophosphamide. Indian J Ophthalmol 1966;14:124-7

How to cite this URL:
Gokhle A M, Desai A J. Reticulosarcoma of the eye treated with cyclophosphamide. Indian J Ophthalmol [serial online] 1966 [cited 2021 Mar 3];14:124-7. Available from: https://www.ijo.in/text.asp?1966/14/3/124/38639

Primary involvement of the eyes in lymphomas is uncommon. A case of reticulosarcoma of the eye responding dramatically to intravenous cyclophosphamide (endoxan) is reported below.

Case Report

Mr. L. J., a 51-year old Christian farmer from Goa, was seen for the first time on 12th Dec. 1964. He gave a history of an insect bite on the left upper eye lid three months ago. A month later, he observed watering from the eyes and a rapidly growing swelling of the left eye lids. He was not able to see with the left eye at all. Two weeks later, the right eye lids were found to be involved. No systemic manifestations were noted. [Figure - 1].

On examination, the left eye was closed with a marked swelling of both the eye lids. It was hard, nontender, nodular and the overlying skin was red and stretched, but freely moving. On separating the lids, a marked swelling of the conjunctiva was noted. Cornea could be barely seen.

The vision of the right eye was normal. A hard swelling of the right lower eye-lid was also seen. The cornea and conjunctiva were normal. The right fundus was normal.

Small discrete lymph nodes were palpable in the posterior cervical region on both sides. and the tonsils were markedly enlarged. Epitrochlear nodes on the right side were massively enlarged and on the left side they were just palpable. Other lymph nodes, liver and spleen were not palpably enlarged. The other systems were normal.

Investigations

i) Hemoglobin: 13.5 gm. %, Packed cell volume 45 ml % Mean corpusuar hemoglobin concentration 33%; white blood cells: 9800 per cmm. Polymophs: 78% Lymphocytes: 12%; Eosinophils : 1%; Monocytes : 9%.

ii) Chest X-Ray did not show any enlargement of mediastinal lymph nodes.

iii) Biopsy from the tonsil and left eye lid showed a similar histology which was diagnostic of reticulosarcoma. [Figure - 2],[Figure - 3],[Figure - 4]

The patient was treated with 200 mg. of cyclophosphamide intravenously daily for twenty days. Within three days of starting the treatment he felt better and the swelling of the eyes was markedly diminished a fortnight later. By the 20th day no swelling of the lids was perceptible and the tonsils had regressed in size completely. Epitrochlear glands could just be felt. No ill effects of the drug were noticed. Eosinophil count was normal before giving the cyclophosphamide. It was noticed to be higher on the second day following cyclophosphamide therapy and has remained high till today. Stools were examined for parasites but none were found. The patient denied any history of asthma and had no symptoms suggestive of tropical eosinophilia. Besides, chest X-Ray was normal. It is thought that the high eosinophil count resulted from the cyclophosphamide therapy. [Figure - 5]

He discontinued the treatment on his own by middle of February '65. He was readmitted on 15th of April with a recurrence of the cervical, epitrochlear, and tonsillar lymphadenopathy [Figure - 6]

No swelling of the eye lids was observed. A white necrotic patch was seen on the right tonsil. Liver and spleen were not palpable. A repeat chest X-Ray did not reveal any mediastinal lymph nodes. Blood counts were as follows: Hemoglobin 14 gms: Packed cell volume 45 ml %; white blood cells 9000 cmm. with 18% eosinophils, 50%, polymorphs, and 32% lymphocytes. He was treated intravenously again with cyclophosphamide 200 mgm. for 20 days and again responded very well with regression of lymph adenopathy. He is now on a maintenance therapy of cyclophosphamide 50 mgm. three times a day orally.

Discussion

From primitive multipotent mesenchymal cells are developed fully differentiated connective tissue cells and tissue elements whose basic structure is a sponge like reticulum. From these are developed lymphoid, hoemic, and phagocytic cells which are bound together by fine fibres of reticulin. Hyperplasia of this system is pleomorphic and may give different clinical forms which are grouped together under the generic name of 'Reticuloses or malignant Lyphomas'. Out of this the following five may affect the eye lids--(1) Lypmphoma, (2) Lymphosarcoma, (3) Giant follicular adenopathy, (4) Reticulosarcoma and rarely (5) Hodgkin's disease.

(I) Lvmphoma has a structure approaching that of normal tissue. It is benign. It consists of dense mass of lymphocytes with occasional mitotic figures.

(2) Lymphosarcoma is a highly invasive tumour of lymphocytes and lymphoblasts. This and lymphoma may be associated with Leukemia. It is locally invasive. Multiple metastasis on pleuricentric dissemination of the disease may occur, some time or other involving all lymphoid tissue of the body. Leukaemia depends upon malignant cells entering the blood stream in demonstrable numbers. It shows lymphocytes with many mitoses.

(3) In Giant follicular lymph adenopathy similar neoplasia of multicentric origin occur throughout the body, sometimes benign but usually of low malignancy. Tonsils and lymphoid tissue of gastro-intestinal tract are not affected. Lymph nodes show lymphoid tissue with large follicles and germinal centres.

(4) In Reticulosarcoma reticular cells predominate. Any variant of reticular cell may be involved, but in a particular tumor, the type of cell is uniform. Fine reticulum is present throughout. It is locally invasive and spreads by lymphatics and blood stream. These nodules may ulcerate.

(5) Hodgkin's desease shows considerable pleomorphism with preponderance of lymphoid and reticular elements. It rarely affects the eyelids.

Involvement of eyelids is uncommon in the first two. Primary involvement of the eyes without simultaneous systemic disease is very rare. Of 21 cases of lymphomatoid disease in the region of the eye, Mc Carvic (1943) found that in 17 the periocular lymphoblastoma was primary; while in 4 it appeared after the generalized disease had become apparent. Of these 21 tumors. 10 involved the eyelids and 20 the orbit. Gall and Mallory (1942) found only one case of lymphosarcoma of eye in their large series of 618 cases of malignant lymphomas. When the eyelids are involved, lymphosarcoma or reticulosarcoma are the usual causes.

The tumor forms an ill defined mass in the lids and may have a variable involvement of all the four lids. Orbit may be encroached upon. Usually the overlying skin is free. There may be a very rapid spread of the disease in case of reticulosarcoma and may prove fatal in a few months.

The treatment on the whole is unsatisfactory, though, the tumor seems to be a little less malignant in this area. Partial or rarely a complete remission is known to occur due to radio therapy. Recurrence rate is very high. Cyclophosphamide to our knowledge is not tried in this situation. The dramatic response in our patient has been very encouraging. Cyclophosphamide is selectively concentrated by the malignant cells and hence a long term maintenance therapy is possible. Besides the absence of recurrence at the same site is very noteworthy and so is the response of the recurrence of growth to the drug.

We are grateful to Dr. C. K. Deshnande. Prof. of Pathology for the histopathological studies.

Summary