Radiology–pathology conference: primary adrenal lymphoma

Radiology–pathology conference: primary adrenal lymphoma

Clinical Imaging 36 (2012) 156 – 159 Radiology–pathology conference: primary adrenal lymphoma Teerath P. Tanpitukpongse a,⁎, Shahmir Kamalian a , Mic...

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Clinical Imaging 36 (2012) 156 – 159

Radiology–pathology conference: primary adrenal lymphoma Teerath P. Tanpitukpongse a,⁎, Shahmir Kamalian a , Michael Punsoni b , Mala Gupta b , Douglas S. Katz a a

Department of Radiology, Winthrop-University Hospital, Mineola, NY 11501, USA Department of Pathology, Winthrop-University Hospital, Mineola, NY 11501, USA

b

Received 4 August 2011; accepted 25 August 2011

Abstract We present a case of a 62-year-old man with a history of type II diabetes mellitus who presented to our emergency department with back pain and right upper quadrant abdominal pain associated with vomiting and weight loss. A computed tomographic scan of the abdomen and pelvis demonstrated a large adrenal mass, and subsequent biopsy showed primary adrenal lymphoma. © 2012 Elsevier Inc. All rights reserved. Keywords: Adrenal gland; Lymphoma; Diffuse large B-cell; Primary adrenal lymphoma; Computed tomography

1. Introduction Primary adrenal lymphoma is a rare diagnosis with only about 80 reported cases in the literature [1–4]. Primary adrenal lymphoma usually presents with bilateral adrenal involvement [5]. Non-Hodgkin's lymphoma is more common than Hodgkin's lymphoma when there is involvement of the adrenals [5]. In addition, secondary involvement of the adrenal in non-Hodgkin's lymphoma is much more common than primary adrenal lymphoma, occurring in up to 25% of patients [5]. The clinical presentation of patients with adrenal lymphoma is usually nonspecific, including abdominal pain, weight loss, fever, anemia, nausea and vomiting [6]. Adrenal insufficiency is a reported complication of bilateral adrenal lymphoma [6–11]. Here, we report the radiologic and pathologic findings in a patient with primary unilateral adrenal lymphoma. 2. Case presentation A 62-year-old man presented to our emergency department with 8 weeks of back pain and right upper quadrant ⁎ Corresponding author. Tel.: +1 302 743 6043; fax: +1 516 663 8172. E-mail address: [email protected] (T.P. Tanpitukpongse). 0899-7071/$ – see front matter © 2012 Elsevier Inc. All rights reserved. doi:10.1016/j.clinimag.2011.08.022

abdominal pain associated with vomiting and weight loss. His past medical history was notable for type II diabetes mellitus for which he was taking metformin. The patient reported that his back pain was worse when lying supine and that the pain was sharp and constant. The pain was not relieved by acetaminophen. The patient denied any prior surgical history. There was no history of smoking or alcohol use. The family history was noncontributory. On physical examination, his vital signs were only significant for a mildly elevated blood pressure of 151/96. The remainder of the physical examination was unremarkable. Routine laboratory studies were only notable for an elevated serum glucose level of 220. In the emergency department, a nonenhanced computed tomographic (CT) examination of the abdomen and pelvis was initially performed to evaluate the patient's abdominal and back pain, as renal colic was the leading clinical diagnosis. Shortly following this, due to identification of a right adrenal mass, a repeat examination of the abdomen and pelvis was obtained in the early portal venous and nephrogenic phases from the diaphragm to the symphysis pubis at 3-mm intervals using nonionic intravenous contrast. The CT examination demonstrated an enlarged heterogeneous hypoenhancing mass arising from the right adrenal, which displaced the right kidney laterally and posteriorly

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Fig. 1. Axial CT images of the abdomen in a 62-year-old man with back pain, abdominal pain, vomiting and weight loss, performed without (A) and then with intravenous contrast (B and C), demonstrate an enlarged heterogeneous hypoenhancing mass arising from the right adrenal, which encases the right renal artery as well as both renal veins and which abuts the aorta and inferior vena cava. A CT coronal image (D) is also provided, which demonstrates mass effect on the right kidney from this mass.

(Fig. 1). The mass also extended inferiorly in the retroperitoneum into the aortocaval region, displacing the pancreas slightly anteriorly. The mass measured approxi-

mately 11 cm in the craniocaudal dimension, 10 cm in the anteroposterior dimension and 7.7 cm in the transverse dimension. There was encasement of the right renal vessels,

Fig. 2. Microscopic view of the tumor on hematoxylin and eosin staining (A). The permanent section shows large, pleomorphic lymphoid cells with moderate eosinophilic cytoplasm. Immunoperoxidase stains were positive for MUM1 (B) and CD20 (C). Myeloperoxidase immunoperoxidase stain (D) was negative, as well as CD10, BCL6, and chromogranin and synaptophysin (not shown), effectively excluding both follicular and Burkitt's lymphoma, a myeloid lineage tumor and a neuroendocrine tumor. CD3 positivity (E) was seen in fewer than 10% of the total population of cells. Leukocyte common antigen (F) was also positive. A final diagnosis of high grade, B-cell lymphoproliferative disorder was made.

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although the right renal artery and vein remained patent. There was also encasement of the proximal portion of the left renal vein, which remained patent. The mass displaced the inferior vena cava anteriorly and compressed it. Computed-tomography-guided fine needle aspiration of the right adrenal mass was performed. The specimen was comprised of a population of large, atypical lymphoid cells, which were two to three times the size of a mature lymphocyte, with open chromatin and rare prominent nucleoli. Cores were subsequently processed for cell block. The permanent section showed large, pleomorphic lymphoid cells with moderate eosinophilic cytoplasm (Fig. 2). A small focus of necrosis was also seen. Immunoperoxidase stains performed on the permanent section showed that the large, atypical cells were strongly positive for leukocyte common antigen (CD45), CD20 (indicating a clonal B cell population) and MUM1. CD3 positivity (indicating small reactive T lymphocytes) was seen in fewer than 10% of small reactive T lymphocytes. Significant negative immunoperoxidase stains included CD10, BCL6, myeloperoxidase, and chromogranin and synaptophysin, effectively excluding follicular and Burkitt's lymphomas, a myeloid lineage tumor and a neuroendocrine tumor. A final diagnosis of high-grade, B-cell lymphoproliferative disorder was made. Further laboratory analysis revealed that the adrenal mass was nonfunctioning. The mass was however unresectable, and the patient was treated with six cycles of systemic chemotherapy using rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP) and radiation therapy. The patient did well on the chemotherapy regimen. On follow-up positron emission tomographic (PET) imaging performed 5 months after the patient's initial presentation, there was no evidence of recurrent or residual disease.

3. Discussion Adrenal lesions are commonly found on cross-sectional imaging examinations, and the vast majority of these lesions are incidental, benign adrenal adenomas. However, the diagnosis of primary adrenal lymphoma is relatively rare, although there have been at least 80 cases reported in the English literature [1,5,12]. Primary adrenal lymphoma is bilateral in the majority of cases, and non-Hodgkin's lymphoma is more common than Hodgkin's lymphoma [13]. The most common subtype is diffuse large B-cell lymphoma, which expresses antigens specific to B lymphocytes, including CD20, CD74, CDw75 and CD79a [1]. Other subtypes include mixed large and small cell, small noncleaved cell and undifferentiated lymphoma [5]. Primary adrenal lymphoma is more common in older males, with a male-to-female ratio of 2:1 and a mean age at presentation of 65 years [14]. Patients with adrenal lymphoma usually present with nonspecific signs including fever, malaise, weight loss and anemia [6]. In addition, this entity may also be associated

with primary adrenal insufficiency, if there is bilateral adrenal disease, with additional symptoms of anorexia, orthostatic hypotension, hyponatremia and skin pigmentation [6–11]. While metastatic disease to the adrenals is very common, such as from breast, lung and gastrointestinal tract malignancies, as well as from melanoma, the findings of bilateral adrenal masses or adrenal insufficiency should raise suspicion for another process [6]. These include primary lymphoma, pheochromocytoma, adrenocortical carcinoma, neuroblastoma (in the pediatric population), infection or adenoma [6]. The reported presentation of adrenal lymphoma on crosssectional imaging examinations is that of a large, welldefined soft-tissue mass that replaces the adrenal with homogeneous or slightly inhomogeneous enhancement [12]. Adrenal lymphoma can infiltrate adjacent structures such as the inferior vena cava and the kidneys [12]. Computed tomography shows a suprarenal soft-tissue mass that is homogeneous and slightly hypodense with mild enhancement [12]. A complex mass with a variable density on CT can also be seen [5]. A cystic appearance of primary adrenal lymphoma due to necrosis has also been reported [5]. On T1-weighted magnetic resonance (MR) imaging, adrenal lymphomas are hypointense. On T2-weighted imaging, they usually display heterogeneous hyperintensity. The MR signal characteristics of primary adrenal lymphoma, however, may overlap with other benign entities, complicating the diagnosis [13]. Hahn et al. reported a case of central nervous system involvement from primary adrenal lymphoma, with multifocal lesions [15]. Cases of primary adrenal lymphoma presenting as Addison's disease have also been reported [14,16]. In addition, the role of PET/CT for providing functional and attenuation information in order to characterize adrenal masses as benign versus malignant has been explored [5,12,17]. In one case, 18 fluorodeoxyglucose uptake was noted in both adrenal glands without uptake elsewhere, which assisted in the diagnosis [5]. The treatment for patients with adrenal lymphoma includes a combination of surgery, chemotherapy and radiation therapy. Currently, the standard chemotherapy regimen is R-CHOP, which has been found to be more efficacious than CHOP alone [18]. Poor prognostic factors for primary adrenal lymphoma are advanced age, large tumor size, elevated lactate dehydrogenase level, bilateral adrenal involvement and adrenal insufficiency [18]. In conclusion, our patient presented with a large right adrenal mass that was diagnosed as a relatively rare primary unilateral adrenal lymphoma. References [1] Kim KM, Yoon DH, Lee SG, Lim SN, Sug LJ, Huh J, et al. A case of primary adrenal diffuse large B-cell lymphoma achieving complete remission with rituximab-CHOP chemotherapy. J Korean Med Sci 2009;24:525–8.

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