Primary Adrenal Lymphoma Research Articles

Uncommon presentations of non-Hodgkin's lymphoma

In this issue of Hospital Medicine, a case of a primary adrenal lymphoma (PAL) and a primary cutaneous B cell lymphoma (PCBCL) are presented (p. 759). These extranodal lymphomas are uncommon presentations of B cell non-Hodgkin's lymphoma.

Primary adrenal lymphoma discovered as an incidentaloma: case report and review of the literature

Primary adrenal lymphoma (PAL) discovered at an early stage is extremely rare. We report a case of PAL incidentally discovered by abdominal computed tomography (CT) scan as a 4.5-cm solid mass in the left adrenal gland. The patient was an 80-year-old man, and, from imaging studies, the left adrenal mass was considered to be a non-functioning adrenal cortical cancer. Left adrenalectomy was performed, and the resected tumor proved to be non-Hodgkin's lymphoma of diffuse large B-cell type. Adjuvant chemotherapy was administered, and the patient has been well with no evidence of the disease for 20 months postoperatively.

Primary Bilateral Adrenal Lymphoma

Primary adrenal lymphoma is a rare primary neoplastic disease of the adrenal glands, with up to 65 cases reported in the literature over the past 40 years. The increasing use and sophistication of medical diagnostic imaging has allowed this disease to be diagnosed more frequently premortem, presenting more opportunity for treatment. The true incidence of these neoplasms is not known, nor is it clear why historical autopsy series have not reported this neoplasm more frequently, and why its existence has been documented more recently with advanced diagnostic imaging. This review has presented our new case, and reviewed 5 more in the literature, bringing the total number of cases to over 70. Accumulation of more cases and the experience treating these cases is needed to develop a better picture of diagnostic procedures and treatment regimens that have maximum efficiacy.

Primary Bilateral Adrenal Lymphoma Associated with Idiopathic Thrombocytopenic Purpura

Autoimmune disorders are occasionally associated with malignant lymphoma. To date only one case of primary adrenal lymphoma associated with idiopathic thrombocytopenic purpura (ITP) has ever been reported. This paper reports the case of a 63-year-old man with bilateral adrenal masses whose laboratory data showed decreased platelet count. Despite normal blood pressure, the adrenal tumors endocrinologically appeared to be pheochromocytoma. Core needle biopsy was not done due to thrombocytopenia attributed to concurrent ITP. After intravenous immunoglobulin treatment, splenectomy and bilateral adrenalectomy were performed since the first pathological findings of the frozen specimen suggested the possibility of a poorly-differentiated carcinoma. Immunohistochemical study finally showed the tumors to be diffuse large B-cell lymphoma. The patient underwent a subsequent course of combination chemotherapy and survived 6 years recurrence-free without any need for further treatment other than steroid replacement. The coincidence of adrenal lymphoma and ITP should be considered even if another kind of tumor is suspected, and core needle biopsy should be performed prior to operation, since the specific kind of tumor found alters the therapeutical strategy adopted.

PRIMARY ADRENAL NONHODGKIN'S LYMPHOMA: LONG-TERM SURVIVAL

Primary nonHodgkin's lymphoma of the adrenal gland is a rare neoplasm, with only 27 cases reported in the world literature, and maximum survival is 15 months. To our knowledge we report the first case of long-term survival (8 years) of a patient with primary adrenal nonHodgkin's lymphoma. In contrast to the cases reported the treatment of our patient consisted of adrenalectomy and adjuvant radiation therapy. CASE REPORT A 59-year-old patient presented with a painless mass above the right kidney incidentally discovered on abdominal ultrasound. Abdominal sonography and computerized tomography (CT) showed a right 8 X 4 X 4 cm. inhomogeneous suprarenal mass adherent to the vena cava with contrast enhancement ranging from 60 to 80 Hounsfield units (fig. 1). No other abdominal mass or retroDentoneal1vmDhadenooathv was identified. Excretory urograp6y demonstra& no pa&ological findings. Endocrinological examination was normal. The adrenal tumor was removed via a thoracoabdominal approach above the 10th rib. Because of its adherence the mass had to be dissected sharply from the vena cava. Histological examination, including immunohistochemical analysis, showed malignant centroblastic T cell nonHodgkin's lymphoma (Eel Classification, fig. 2). Postoperative staging procedures, including gastroscopy, barium enema, bone marrow biopsy and chest x-ray, revealed no evidence of metastatic disease. Intraoperative and histological findings did not confirm total extirpation. Therefore, 59 Gy. adjuvant radiotherapy were administered in 4 weeks. At 8-year followup the patient has no evidence of disease. DISCUSSION Secondary involvement of the adrenal gland is found in 25% of autopsy cases of nonHodgkin's lymphoma.' primary adrenal

Clinically silent primary adrenal lymphoma: A case report and review of the literature

Primary adrenal lymphoma (PAL) is extremely uncommon. We describe a case of clinically silent non-Hodgkin's B-cell lymphoma of diffuse large cell type with exclusive left adrenal localization. The tumor was discovered by computed tomography (CT) as a 2.5-cm dense mass and diagnosed at autopsy. Literature concerning this unusual neoplasm is reviewed. During the early stage, particularly when the lesion is small, PAL is likely to be missed. This unusual entity should be included in the differential diagnosis of adrenal masses so that early diagnosis may be made and intervention might dramatically affect the clinical outcome.

Clinically silent primary adrenal lymphoma: A case report and review of the literature

Primary adrenal lymphoma (PAL) is extremely uncommon. We describe a case of clinically silent non-Hodgkin's B-cell lymphoma of diffuse large cell type with exclusive left adrenal localization. The tumor was discovered by computed tomography (CT) as a 2.5-cm dense mass and diagnosed at autopsy. Literature concerning this unusual neoplasm is reviewed. During the early stage, particularly when the lesion is small, PAL is likely to be missed. This unusual entity should be included in the differential diagnosis of adrenal masses so that early diagnosis may be made and intervention might dramatically affect the clinical outcome. Am. J. Hematol. 58:130–136, 1998. © 1998 Wiley-Liss, Inc.

Primary Bilateral Adrenal Lymphoma

We report a case of primary bilateral adrenal non-Hodgkins lymphoma (NHL), with hypercalcemia at the time of presentation. Medical literature review showed that this is an extremely rare site for lymphoma, but presents a distinct clinical entity with many features that differ from NHL overall, extranodal NHL, and NHL in other endocrine organs. The diagnosis is frequently not suspected antemortem, and long delays between the onset of symptoms and the diagnosis are frequent. Adrenal insufficiency is also common, and may be a lethal complication of these tumors. Response to therapy and survival are extremely poor.

Primary Adrenal Lymphoma Associated with Adrenal Insufficiency: A Distinct Clinical Entity

We describe a case of a 42 year old male who presented with Addison's disease resulting from primary lymphoma of the adrenals. Our case and a review of the literature indicates that this distinct entity has some unique clinical and radiologic features. In this entity, the lymphoma tends to be extranodal and have a poor prognosis. In addition, the computed tomography (CT) images have the unique appearance of enlargement of the adrenal gland with maintenance of the adreniform shape. We suggest that primary adrenal lymphoma is a distinct clinical entity and should be considered in patients with an elevated serum lactate dehydrogenase, characteristic CT findings and Addison's disease.

Idiopathic Thrombocytopenic Purpura as the Presenting Feature of a Primary Bilateral Adrenal non Hodgkin's Lymphoma

Primary lymphomas arising in the adrenal gland are extremely rare. The presenting symptoms are nonspecific and may be related to lymphoma or to associated adrenal insufficiency. In this report we describe the case of a 61 year old woman with idiopathic thrombocytopenic purpura and primary bilateral non Hodgkin's lymphoma of the adrenals.

MR imaging of primary adrenal lymphoma

We describe a patient with non-Hodgkin's B-cell lymphoma of diffuse large cell type, which involved both adrenal glands without adrenocortical insufficiency. Magnetic resonance showed bilateral adrenal tumors with some enhancing septa.

CT of Primary Adrenal Lymphoma

We present a case of primary adrenal lymphoma with CT appearance of a cystic mass containing mural calcification. Sonographic findings are correlated. Comparison of imaging features is made with the few previously reported cases of this rare entity.

Primary Bilateral Adrenal Lymphomas in 3 Cases

Primary Bilateral Adrenal Lymphomas in 3 Cases

Primary adrenal lymphoma

A case of non-Hodgkin's lymphoma (large cell immunoblastic sarcoma) arising as a primary malignancy in the adrenal gland is reported and the literature concerning this unusual tumor is reviewed. Although extremely rare, it is possible to make the diagnosis preoperatively. Therefore, primary adrenal lymphoma should be included in the differential diagnosis of a suprarenal mass.