Primary adrenal T-cell lymphoma in a young adult presented with pseudo-hypopyon: a case report and literature review.

TO THE EDITOR: A 26-year-old Indian man presented with weight loss for 6 months and blurred vision on his right eye for 2 weeks. He seemed to be pale, but had no lymphadenopathy or hepatosplenomegaly. Initial complete blood count showed a hemoglobin level of 10.7 g/dL, white blood cell count of 1.4×109/L (neutrophils 44%, lymphocytes 43%, eosinophils 3%, and monocytes 10%), and platelet count of 40×109/L. Ophthalmic evaluation revealed a white meniscus in the anterior chamber of the right eye. The corresponding aqueous humor aspirate showed infilatration of atypical lymphoid cells (Fig. 1). Whole body fluorine-18-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (18F-FDG PET/CT) did not reveal any FDG avid intra-ocular/intra-orbital lesions. However, there were FDG avid soft tissue masses in both the adrenal glands (4.8×9.3×6.2 cm lesion with maximum standardized uptake value (SUVmax) of 22.7 in the right adrenal gland and 4.6×9.2×5.9 cm lesion with SUVmax of 17.6 in the left adrenal) suggesting a lymphomatous pathology (Fig. 1). CT guided fine needle aspiration from the adrenal glands revealed the infiltration of atypical lymphoid cells, which were CD3 positive and CD20 negative by immunocytochemistry. Bone marrow aspiration showed increased number (about 83%) of 15–20 µm sized atypical lymphocytes (Fig. 1). In flowcytometric analysis, these cells showed expression of CD45, CD2, CD3, and CD8, whereas no expression of CD1a, CD4, CD5, CD7, CD34, CD16, CD56, CD57, TCRαβ and TCRγδ. These lymphoid cells had clear cytoplasm, imparting a ‘fried-egg’ pattern of arrangement in bone marrow biopsy. On immunohistochemistry (IHC), these lymphoid cells were positive for CD8 and negative for CD3, CD5, CD4, and CD56. Negative staining for CD34 by IHC highlighted vascular proliferation and intra-sinusoidal infiltration (Fig. 1). The patient was diagnosed with CD8-positive extranodal T-cell lymphoma, but the patient died on 2 days of admission, prior to initiating therapy.

Fig. 1

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. (A) Aqueous humor aspiration showed infiltration of atypical lymphoid cells. (B) Bone marrow (BM) aspiration showed infiltration of atypical lymphoid cells with irregular nuclear membrane, coarse chromatin, basophilic cytoplasm and fine azurophilic granules. (C) BM biopsy showed infiltration of lymphoma cells with “fried egg” pattern. (D) FDG avid lesions in the adrenal glands. (E) Diffuse FDG uptake in the BM. (F) Absence of FDG avid intra-orbital and intra-ocular mass lesions. (G) Adrenal aspiration showed lymphoma infiltration. (H) CD34 immunohistochemistry (IHC) of the BM biopsy highlighting intra-sinusoidal pattern. (I) CD8 positivity in the BM lymphoid infiltrate. (J, K) Diffuse and intense FDG uptake in both the adrenal glands (A, B, & G: May-Grünwald Giemsa stain; C: Hematoxylin and eosin stain).

Primary adrenal lymphoma (PAL) is a rare and aggressive malignancy and involves bilateral adrenals in 60–75% of cases [12]. The most common PALs are diffuse large B-cell lymphomas (-80%), followed by peripheral T cell lymphoma (7%) [234]. 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) [4789]. 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.

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.

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.