|LETTER TO EDITOR
|Year : 2014 | Volume
| Issue : 3 | Page : 395-396
Long term survival after whole brain radiotherapy for brain metastasis in follicular dendritic cell sarcoma
K Dimri, R Trehan, AK Pandey, D Khosla
Department of Radiotherapy, Government Medical College, Chandigarh, India
|Date of Web Publication||10-Dec-2014|
Dr. K Dimri
Department of Radiotherapy, Government Medical College, Chandigarh
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Dimri K, Trehan R, Pandey A K, Khosla D. Long term survival after whole brain radiotherapy for brain metastasis in follicular dendritic cell sarcoma. Indian J Cancer 2014;51:395-6
|How to cite this URL:|
Dimri K, Trehan R, Pandey A K, Khosla D. Long term survival after whole brain radiotherapy for brain metastasis in follicular dendritic cell sarcoma. Indian J Cancer [serial online] 2014 [cited 2020 Jul 7];51:395-6. Available from: http://www.indianjcancer.com/text.asp?2014/51/3/395/146778
We present a rare case of brain metastasis in a patient with follicular dendritic cell sarcoma (FDCS), who was treated with whole brain radiotherapy (WBRT) and is alive and free from disease, 7 years after diagnosis.
A 36-year-old male patient presented with epigastric pain, anorexia and weight loss of 1 month duration (July 2006). Physical examination revealed epigastric fullness and a right sided inguinal lymph node measuring 6-cm in greatest dimension. Fine-needle aspiration from the inguinal lymph node and a biopsy revealed FDCS. Contrast enhanced computed tomographic scan (CECT scan) of the abdomen revealed multiple enlarged retroperitoneal, mesenteric and right inguinal lymph nodes. Further metastatic work-up was non-contributory. Patient was thus started on chemotherapy for disseminated dendritic cell sarcoma of the lymph nodes. He received eight cycles of adriamycin and ifosfamide combination chemotherapy. A subsequent CECT scan showed complete resolution of lymph node masses. The patient was asymptomatic for 1-year when he presented with headache and vomiting. CECT scan of the head was highly suggestive of metastasis in the frontal region [Figure 1]. After a course of steroids, mannitol and neurosurgery consultation for decompression, the patient was taken up for palliative WBRT. He received 30 Gy in 10 daily fractions by parallel opposed portals on a telecobalt machine. He improved symptomatically and a subsequent CECT scan showed near complete regression of the space occupying lesions as well edema. Patient has been on a regular follow-up since then and is largely asymptomatic. His most recent CECT scan of the head (May 2013) is within the normal limits [Figure 2]. The patient is therefore clinically and radiologically free of disease, 7 years after the initial diagnosis and 6 years following radiotherapy for brain metastasis.
|Figure 1: Ill-defined hypo-dense space occupying lesion with necrotic center in the mid frontal region and associated bilateral frontal lobe edema causing partial obliteration of bilateral frontal horns and effacement of frontal lobe sulci and gyri|
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|Figure 2: A normal contrast enhanced computed tomographic brain of the same patient, conducted 6-years after whole brain radiotherapy for brain metastasis in dendritic cell carcinoma|
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| » Discussion|| |
Dendritic cell sarcoma is a rare neoplasm of unknown etiology first reported in 1978.  Since then 462 cases have been reported excluding the present one.  Systemic involvement and distant metastasis are more commonly seen in liver and lungs. Brain involvement is extremely rare and has been reported in four patients only (including the present one). FDCS is the most common histological subtype (343 reported cases). It behaves like an intermediate grade sarcoma with a substantial risk of local recurrence (28%) and distant metastasis (27%).  For localized disease, a radical excision is the preferred treatment and there is no further benefit with adjuvant radiotherapy and chemotherapy. For disseminated/advanced disease (like the present case), chemotherapy is the only option, but the outcome is generally poor. 
Brain metastases develop in 20-40% cancer patients during their course of illness.  The prognosis for such patients is generally poor with a median survival of less than 3 months without any treatment. The mainstay of treatment has been corticosteroids for alleviation of peritumoral edema and WBRT for growth restraint, symptomatic relief and possibly improvement in survival.  The overall response rate to WBRT ranges from 64% to 85% and the median survival approximately 6 months.  Moreover, a recently published Cochrane review on WBRT did not show survival benefit with the usage of different fractionation schedules as against the "standard" 30 Gy in 10 daily fractions, also used in the present case, radio-sensitizers and radiosurgery boost. 
Contrary to the outcomes reported in the literature, there was a complete radiological response in this case and the patient and is alive and free from disease 6 years after WBRT.
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[Figure 1], [Figure 2]