Blood Res 2017; 52(3):
Published online September 25, 2017
https://doi.org/10.5045/br.2017.52.3.227
© The Korean Society of Hematology
1Department of Hematology, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India.
2Department of Cytopathology, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India.
3Department of Nuclear Medicine, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India.
Correspondence to : Man Updesh Singh Sachdeva. Department of Hematology, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh 160012, India. drmanupdeshpgi@yahoo.co.in, drmanupdesh@gmail.com
Primary adrenal lymphoma (PAL) is a rare and aggressive malignancy and involves bilateral adrenals in 60–75% of cases [1,2]. The most common PALs are diffuse large B-cell lymphomas (-80%), followed by peripheral T cell lymphoma (7%) [2,3,4]. Since the majority of intraocular lymphomas (IOL) are of B-cell origin, T-cell type IOLs are very rare and often secondary to cutaneous or adult T-cell lymphoma [5]. Except for a single case report showing choroidal involvement, intraocular metastasis by PAL is extremely rare [6].
To the best to our knowledge, only 4 cases of T-cell type PAL have been reported in literature, but none of the cases had bone marrow involvement (Table 1) [4,7,8,9]. The median age at diagnosis was 40 years (range, 31–70 yrs). The most common presentation is weight loss and most of the cases have poor outcome in spite of intensive chemotherapy.
In conclusion, we describe the first case of primary adrenal T-cell lymphoma with ocular and bone marrow metastasis in a young adult. In the absence of clinical evidence to suspect a primary adrenal pathology, our case reinforces the importance of thorough radiologic workup, especially 18F-FDG PET/CT, in the evaluation of occult lymphomas. In addition, we also emphasize the role of morphological evaluation combined with immunophenotyping for definite diagnosis in lymphomas.
Microscopic findings, immunohistochemical staining, and whole-body fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) of primary adrenal T-cell lymphoma.
Table 1 Review of clinical and radiologic findings in patients with primary adrenal T-cell lymphoma.
Abbreviations: CHOP, Cyclophosphamide 750 mg/m2 per day, vincristine 2 mg per cycle, doxorubicin 50 mg/m2 per cycle, prednisone 60 mg per day; CNS, central nervous system; CSF, cerebrospinal fluid; IVC, Inferior vena cava; NA, not available.
Blood Res 2017; 52(3): 227-229
Published online September 25, 2017 https://doi.org/10.5045/br.2017.52.3.227
Copyright © The Korean Society of Hematology.
Karthik Bommannan1, Man Updesh Singh Sachdeva1*, Aravind Sekar2, Rajender Kumar3, and Pranab Dey2
1Department of Hematology, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India.
2Department of Cytopathology, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India.
3Department of Nuclear Medicine, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India.
Correspondence to:Man Updesh Singh Sachdeva. Department of Hematology, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh 160012, India. drmanupdeshpgi@yahoo.co.in, drmanupdesh@gmail.com
Primary adrenal lymphoma (PAL) is a rare and aggressive malignancy and involves bilateral adrenals in 60–75% of cases [1,2]. The most common PALs are diffuse large B-cell lymphomas (-80%), followed by peripheral T cell lymphoma (7%) [2,3,4]. Since the majority of intraocular lymphomas (IOL) are of B-cell origin, T-cell type IOLs are very rare and often secondary to cutaneous or adult T-cell lymphoma [5]. Except for a single case report showing choroidal involvement, intraocular metastasis by PAL is extremely rare [6].
To the best to our knowledge, only 4 cases of T-cell type PAL have been reported in literature, but none of the cases had bone marrow involvement (Table 1) [4,7,8,9]. The median age at diagnosis was 40 years (range, 31–70 yrs). The most common presentation is weight loss and most of the cases have poor outcome in spite of intensive chemotherapy.
In conclusion, we describe the first case of primary adrenal T-cell lymphoma with ocular and bone marrow metastasis in a young adult. In the absence of clinical evidence to suspect a primary adrenal pathology, our case reinforces the importance of thorough radiologic workup, especially 18F-FDG PET/CT, in the evaluation of occult lymphomas. In addition, we also emphasize the role of morphological evaluation combined with immunophenotyping for definite diagnosis in lymphomas.
Microscopic findings, immunohistochemical staining, and whole-body fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) of primary adrenal T-cell lymphoma.
Table 1 . Review of clinical and radiologic findings in patients with primary adrenal T-cell lymphoma..
Abbreviations: CHOP, Cyclophosphamide 750 mg/m2 per day, vincristine 2 mg per cycle, doxorubicin 50 mg/m2 per cycle, prednisone 60 mg per day; CNS, central nervous system; CSF, cerebrospinal fluid; IVC, Inferior vena cava; NA, not available..
Microscopic findings, immunohistochemical staining, and whole-body fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) of primary adrenal T-cell lymphoma.