|LETTER TO THE EDITOR
|Year : 2016 | Volume
| Issue : 1 | Page : 11-12
Spontaneous regression of orbital lymphoma
AA Khan1, D Jain2, F Siraj2, S Aggarwal3
1 Department of Hematology, Sir Ganga Ram Hospital, New Delhi, India
2 Department of Histopathology, Sir Ganga Ram Hospital, New Delhi, India
3 Department of Medical Oncology, Sir Ganga Ram Hospital, New Delhi, India
|Date of Web Publication||28-Apr-2016|
A A Khan
Department of Hematology, Sir Ganga Ram Hospital, New Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Khan A A, Jain D, Siraj F, Aggarwal S. Spontaneous regression of orbital lymphoma. Indian J Cancer 2016;53:11-2
Spontaneous regression of malignant neoplasms is extremely unusual. We encountered one such patient who presented with right orbital mass, histologically diffuse large B cell type lymphoma, who showed spontaneous regression within a span of 3 weeks after the diagnosis, and at 1-year follow-up, the tumor had not recurred.
An 84-years-old female who had right pthisisbulbi from childhood had been complaining of mild intermittent pain right orbit for last 4 years. Her physical examination was unremarkable except for the right pthisisbulbi. Complete blood counts, liver function tests, kidney function tests, chest X-ray were all within normal limits. However, non-contrast CT scan head revealed small right retro-orbital mass without proptosis. This lesion was biopsied. Histopathological examination showed fibrofattytissue infiltrated by mononuclear cells (chronic inflammatory changes). With impression of pseudotumor orbit, patient received steroids (prednisolone 60 mg/day) for only 1 month. Five months later, she developed proptosis, which was progressive and associated with mild discomfort. Repeat computed tomography (CT) scan head showed right orbital mass occupying whole of the orbit [Figure 1]. On biopsying this lesion, it revealed diffusely invasive tumor, composed of sheets of large atypical lymphoid cells, immunohistochemically positive for CD20 [Figure 2]. An impression of non-Hodgkin lymphoma, diffuse large B cell type was given. Patient was planned for staging workup and treatment; however, patient turned up after 3 weeks with complete disappearance of the orbital swelling without any treatment [Figure 3]. She denied any further investigations and treatment. Patient is doing well at 1-year follow-up without any recurrence.
|Figure 2: Photomicrograph showing a tumor composed of sheets of large atypical lymphoid cells, positive for CD20 (IHC CD20, ×20)|
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|Figure 3: (a) Right orbital mass with proptosis. (b) Spontaneous regression of the mass|
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Complete regression of high-grade non-Hodgkin lymphoma (NHL) is an extremely rare phenomenon. Abe et al., in 2007 reviewed 15 cases of spontaneous regression (SR) in NHL and reported that 3 cases involved were diffuse large B cell Lymphoma (DLBCL) type. Most of the SR cases are localized tumors stage I or IE that has undergone an intervention like excisional biopsy and have been associated with a massive infiltration of T cells in the residual tissue. The leading hypothesis regarding complete SR involves modulation of the host immune system in response to viral or bacterial infection or traumatic intervention. Chang et al., in 2004 reported a 40-years-old female with diffuse large B cell lymphoma of the orbit who showed spontaneous regression at 5 weeks of surgical biopsy and at 6 months follow-up, patient had not shown any sign of relapse.
Information regarding the duration of SR is limited. Wiernik concluded that duration of SR of different hematological malignancies varies and may be triggered by different agents. Spontaneous regression of lymphoma is often of substantial duration, months, or years, and frequently is associated with viral infections. It was documented that one patient with complete SR of DLBCL died of recurrent disease after 13 years.
Treatment of SR cases remains controversial. Some DLBCL cases survive for years  and some relapse early. Because complete SR is an extremely rare phenomenon, there is insufficient evidence that anti-tumor therapy can be omitted. Whichever management plan is decided on, such patients require cautious and long-term follow-up for a long duration.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
| » References|| |
Abe R, Ogawa K, Maruyama Y, Nakamura N, Abe M. Spontaneous regression of diffuse large B-cell lymphoma harbouring Epstein-Barr virus: A case report and review of the literature. J Clin Exp Hematop 2007;47:23-6.
Chang YC, Chang CH, Liu YT, Tsai KB, Liu TC, Lin YN. Spontaneous regression of a large-cell lymphoma in the conjunctiva and orbit. Ophthal Plast Reconstr Surg 2004;20:461-3.
Wiernik PH. Spontaneous regression of hematologic cancers. Natl Cancer Inst Monogr 1976;44:35-8.
Grem JL, Hafez GR, Brandenburg JH, Carbone PP. Spontaneous remission in diffuse large cell lymphoma. Cancer 1986;57:2042-4.
[Figure 1], [Figure 2], [Figure 3]