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- Primary adrenal lymphoma (PAL) is a particularly rare subset of malignant adrenal neoplasms, accounting for ∼1% of all non-Hodgkin's lymphomas. Reported outcomes of PAL, though limited, are dismal, with a 12-month survival rate of ∼20%. PAL is treated with polychemotherapy and early tissue diagnosis to allow initiation of chemotherapy is associated with improved outcomes. Early and accurate radiological diagnosis of PAL is therefore essential in improving outcomes through informing decisions to biopsy and thereby facilitating timely initiation of chemotherapy. To date, however, imaging features of PAL have not been conclusively defined, and a range of divergent imaging appearances have been reported. Cinematic rendering (CR) is a 3D post-processing technique that simulates the propagation and interaction of photons as they pass through the imaged volume.
Cinematic rendering of primary adrenal lymphoma.
Ahmed TM, Fishman EK, Morris-Wiseman LF, Baraban E, Chu LC.
Curr Probl Diagn Radiol. 2024 Sep-Oct;53(5):641-647. - “Amongst malignant entities, primary adrenal lymphoma is an exceedingly rare diagnosis and accounts for ∼1% of all non-Hodgkin's lymphomas. To date, fewer than 250 cases of PAL have been reported in the English literature. The mean age of presentation of PAL is 65 years; it exhibits a 2:1 male to female ratio. The two most common subtypes of PAL are diffuse large B cell lymphoma (DLBCL) (78%) and peripheral T cell lymphoma (PTCL) (7%). In the majority of cases, PAL involves both adrenals synchronously, with only 30% of cases being unilateral at time of presentation. Clinically, PAL most commonly presents with a range of ‘B symptoms’ including fever, night sweats and weight loss (55%) as well as fatigue (45%) and abdominal pain (35%). Up to 61% of cases may also present with adrenal insufficiency which is more common in patients with bilateral tumors. In a pattern atypical for most adrenal masses, only 1%-7% of PALs are found incidentally, reflecting the highly symptomatic nature of these high-grade lymphomas.”
Cinematic rendering of primary adrenal lymphoma.
Ahmed TM, Fishman EK, Morris-Wiseman LF, Baraban E, Chu LC.
Curr Probl Diagn Radiol. 2024 Sep-Oct;53(5):641-647. - “Given the infiltrative growth pattern of PALs, the adreniform shape of the involved glands is typically maintained. Improved characterization of the varying imaging appearances of PAL could help expedite diagnostics and augment clinical problem solving. To date, however, imaging features of PAL have not been conclusively defined, and a number of divergent imaging appearances have been reported. Considerable overlap also exists between the reported imaging features of PAL and other adrenal masses (i.e. ACC), which further complicates diagnostics.”
Cinematic rendering of primary adrenal lymphoma.
Ahmed TM, Fishman EK, Morris-Wiseman LF, Baraban E, Chu LC.
Curr Probl Diagn Radiol. 2024 Sep-Oct;53(5):641-647. - “Cinematic rendering (CR) is a cutting-edge post processing technology that has greatly enhanced 3D visualization methods. Unlike classical volume rendering, CR utilizes a complex global lighting model that integrates elements of ray tracing and scatter to generate highly photorealistic images. Precise simulation of the interactions of photons passing through the imaged volume results in significantly improved visualization of surface detail and realistic shadowing in rendered images. CR also boasts real-time post processing and windowing capabilities which allows for tailored visualization of regions of interest.”
Cinematic rendering of primary adrenal lymphoma.
Ahmed TM, Fishman EK, Morris-Wiseman LF, Baraban E, Chu LC.
Curr Probl Diagn Radiol. 2024 Sep-Oct;53(5):641-647. - “In our experience, CR allowed for more nuanced appreciation of the internal heterogeneity of these tumors and augmented visualization of cystic components. Given that PAL was not considered as a potential diagnosis based on imaging findings in five out of six cases, it emphasizes the importance of understanding the imaging characteristics associated with PAL. Improved characterization of the imaging features of PAL can inform decisions to pursue adrenal biopsy in cases of suspected PAL, and also rule out PAL and preclude unnecessary biopsy in cases where an alternate diagnosis (i.e ACC) is deemed more likely on imaging.”
Cinematic rendering of primary adrenal lymphoma.
Ahmed TM, Fishman EK, Morris-Wiseman LF, Baraban E, Chu LC.
Curr Probl Diagn Radiol. 2024 Sep-Oct;53(5):641-647. - “Transcutaneous adrenal biopsy is associated with a risk of morbidity and mortality and violates a fundamental principle of ACC surgery where en bloc resection is pursued to prevent breach of the tumor capsule. Though rare, cases of needle track seeding of ACC following biopsy have been reported. Simultaneously, timely adrenal biopsy is necessary in cases of suspected PAL because surgical resection, as performed for ACC, substantially delays initiation of tailored chemotherapy. These diagnostic considerations and risks underscore the unique clinical opportunities that improved characterization of PAL at imaging can provide to clinicians during work-up of patients with adrenal masses.”
Cinematic rendering of primary adrenal lymphoma.
Ahmed TM, Fishman EK, Morris-Wiseman LF, Baraban E, Chu LC.
Curr Probl Diagn Radiol. 2024 Sep-Oct;53(5):641-647. - “This case series showcases the diverse CT and CR imaging features of PALs and highlights the capability of CR to enhance visualization of PAL. Familiarity with the imaging features of PALs and how they may subtly differ from more common adrenal masses is essential in facilitating early diagnosis and informing decisions to biopsy. Through generating photorealistic images, CR can potentially assist in the nuanced characterization of these masses, which may improve clinical decision-making..”
Cinematic rendering of primary adrenal lymphoma.
Ahmed TM, Fishman EK, Morris-Wiseman LF, Baraban E, Chu LC.
Curr Probl Diagn Radiol. 2024 Sep-Oct;53(5):641-647.
- ‘Adrenal lymphoma is rare and may be primary (less common) or secondary (more common). The most common adrenal lymphoma subtype is non-Hodgkin lymphoma. Adrenal lymphoma is frequently bilateral, with up to 75% of primary adrenal lymphomas involving both glands. It may occur as focal or multifocal adrenal masses, but it typically occurs as diffuse adrenal enlargement from secondary lymphoma associated with lymphadenopathy and splenomegaly. At CT, these tumors have homogeneous soft-tissue attenuation with mild enhancement..”
Adrenal Neoplasms: Lessons from Adrenal Multidisciplinary Tumor Boards
Ryan Chung et al.
RadioGraphics 2023; 43(7):e220191 July 2023
- “A gland involvement in systemic lymphomas is not rare, but primary adrenal lymphomas (PAL) are very rare with occasional case reports in the literature. It is pre- dominantly a disease of the elderly and typically presents with bilateral adrenal involvement in 70% of the cases. There is a male predominance with a male to female ratio of 2.2:1. PALs represent less than 1% of all non-Hodgkin lymphomas, and patients mostly present with non-specific clinical findings such as fever, abdominal pain, and weight loss. Especially in patients with bilateral involvement, adrenal insufficiency may be the first presenting symptom. There is no direct correlation between the tumor size and the risk of adrenal insufficiency.”
Cross‐sectional imaging features of unusual adrenal lesions: a radiopathological correlation
Karaosmanoglu AD et al.
Abdominal Radiology (2021) 46:3974–3994
- “Adrenal gland involvement in systemic lymphomas is not rare, but primary adrenal lymphomas (PAL) are very rare with occasional case reports in the literature [66]. It is pre- dominantly a disease of the elderly and typically presents with bilateral adrenal involvement in 70% of the cases. There is a male predominance with a male to female ratio of 2.2:1. PALs represent less than 1% of all non-Hodgkin lymphomas, and patients mostly present with non-specific clinical findings such as fever, abdominal pain, and weight loss. Especially in patients with bilateral involvement, adrenal insufficiency may be the first presenting symptom. There is no direct correlation between the tumor size and the risk of adrenal insufficiency.”
Cross‐sectional imaging features of unusual adrenal lesions: a radiopathological correlation
Ali Devrim Karaosmanoglu et al.
Abdominal Radiology (2021) 46:3974–3994 - “On CT and MRI, PALs characteristically appears as solid masses of variable size in both adrenal glands. Adreniform shapes of the glands may or may not be preserved. The masses characteristically enhance heterogeneously on post-contrast images, and necrotic or hemorrhagic foci may be detected within the lesions. Calcifications are uncommon. Despite the commonality of bilateral involvement, unilateral disease may also be occasionally detected. On MRI, PALS may appear heterogeneous on T2W images and may be often initially diagnosed as metastasis or a primary adrenal tumor. Progressive enhancement was also reported in MRI. Histopathological diagnosis is almost always necessary; however, in patients with large bilateral adrenal solid masses with no known history of malignant disease, PAL may be considered in the differential diagnosis.”
Cross‐sectional imaging features of unusual adrenal lesions: a radiopathological correlation
Ali Devrim Karaosmanoglu et al.
Abdominal Radiology (2021) 46:3974–3994 - "Collision tumors are infrequently described entities and, by definition, refer to independently coexisting neoplasms that have different biological and genetic features. These coexisting neoplasms are sharply demarcated, and they do not show any tissue admixture . Although they are rare, collision tumors may also be encountered in the adrenal glands. Several adrenal collision tumors (ACT) have been reported, including adenoma or pheochromocytoma, adenoma and metastasis, myelolipoma and lymphoma, adrenocortical carcinoma and metastases, or myelolipoma have all been reported.”
Cross‐sectional imaging features of unusual adrenal lesions: a radiopathological correlation
Ali Devrim Karaosmanoglu et al.
Abdominal Radiology (2021) 46:3974–3994
- “Primary adrenal lymphoma without lymphadenopathy or other extra-nodal disease is rare .Secondary adrenal involvement is seen in about 4% of diffuse non- Hodgkin’s lymphoma (NHL), and 43% of these cases are bilateral. About two-thirds of patients with bilateral adrenal involvement had adrenal insufficiency.”
Bilateral adrenal abnormalities: imaging review of different entities
Meshal Ali Alshahrani et al.
Abdom Radiol (2019) 44:154–179 - ”Adrenal lymphoma tend to grow in an infiltrative manner, maintaining the adreniform appearance. However, it may progress and have heterogeneous enhancement with necrotic or cystic components, which would pose a diagnostic challenge. On CT, an adrenal lymphomatous mass can be homogeneous with washout features similar to other malignancies. Prior to treatment, calcification is rare.”
Bilateral adrenal abnormalities: imaging review of different entities
Meshal Ali Alshahrani et al.
Abdom Radiol (2019) 44:154–179 - ”In unwell patients with hypoperfusion status, the adrenal glands demonstrate symmetric bilateral intense enhancement with attenuation values matching the great vessels such as abdominal aorta or inferior vena cava (IVC). IVC slit-like flattening, increased renal enhancement and diffuse bowel wall thickening are another supportive diagnostic imaging clues of hypoperfusion complex. This phenomenon is related to a sympathetic response to shock to preserve the perfusion of vital organs including the adrenals.”
Bilateral adrenal abnormalities: imaging review of different entities
Meshal Ali Alshahrani et al.
Abdom Radiol (2019) 44:154–179
- Bilateral Adrenal Masses
• pheochromocytoma (40%)
• tuberculosis (27.1%)
• primary adrenal lymphoma (PAL) (10%)
• metastases (5.7%)
• non-functioning adenomas (4.3%)
• primary bilateral macronodular adrenal hyperplasia (4.3%)
• others (8.6%) - Bilateral Adrenal Masses (NEJM)
• adrenal hyperplasia
- micronodular adrenal hyperplasia
- macronodular adrenal hyperplasia
- adrenocorticotropin (ACTH)-independent macronodular adrenocortical hyperplasia (AIMAH)
• adrenal metastases
• adrenal haemorrhage - Bilateral Adrenal Masses (NEJM)
• adrenal involvement with granulomatous diseases
• adrenal lymphoma
• primary pigmented nodular adrenal dysplasia (PPNAD): usually the glands are normal in size, but nodular. In older patients they may be enlarged 1
“Secondary involvement of the adrenal glands with non-Hodgkin lymphoma has been reported to occur in up to 25% of patients during the course of their disease. However, primary adrenal lymphoma is extremely rare and accounts for just 1% of all non- Hodgkin’s lymphoma cases.”
Primary adrenal lymphoma: Radiological; pathological, clinical correlation Zhou L et al. Eur J Radiol. 2012 Mar;8(3):401-5“Primary adrenal lymphoma generally manifests as large, soft- tissue masses, replacing the adrenal glands with the maximal diameters often exceeding 6 cm. In this study, most (7/9) of adrenal lymphomas are round or oval and smooth with well- defined margins, even in the largest lesions as seen in our experience. Despite their large size, imaging often reflects preservation of the native triangular appearance of the normal adrenal gland.”
Primary adrenal lymphoma: Radiological; pathological, clinical correlation Zhou L et al. Eur J Radiol. 2012 Mar;8(3):401-5“some primary adrenal lymphomas may manifest as masses with necrotic or cystic components and heterogeneous, moderate enhancement, as occurred in one of our cases, making it impossible to differentiate from primary adrenal cortical carcinoma, pheochromocytomas, or metastatic disease.”
Primary adrenal lymphoma: Radiological; pathological, clinical correlation Zhou L et al. Eur J Radiol. 2012 Mar;8(3):401-5Primary Adrenal Lymphoma: Facts
• Primary adrenal lymphoma commonly affects elderly men; with a median age of 65 years old
• Bilateral involvement occurred in 73% of the cases in one study
• Approximately 50% of patients actually develop symptoms of adrenal insufficiency, such as pigmented skin and mucous membrane, fatigue, anorexia, and constipationPrimary Adrenal Lymphoma: CT Appearance
• It is characterized by large tumors, exceeding 10 cm in diameter, with a growth pattern of infiltration.
• Masses generally expand and infiltrate the glands, maintaining their triangular appearances.
• some primary adrenal lymphomas may manifest as masses with necrotic or cystic components and heterogeneous, moderate enhancement , as occurred in one of our cases making it impossible to differentiate from primary adrenal cortical carcinoma, pheochromocytomas, or metastatic disease.“Although it is a rare entity, primary lymphoma of the adrenal gland should be considered in the differential diagnosis of bilateral nodular adrenal lesions, particularly when there is evidence of associated adrenal insufficiency. We describe the case of an 83-year-old woman admitted to the emergency department due to a month's history of asthenia, weight loss, anorexia and nausea. Abdominopelvic CT showed bilateral nodular lesions of adrenal glands and a stimulation test with tetracosactide was compatible with primary adrenal insufficiency.”
Diffuse large B-cell lymphoma of the adrenal gland: a rare cause of primary adrenal insufficiency. de Sousa Lages A et al. BMJ Case Rep. 2016 Mar 18;2016“Lymphomatous involvement of an adrenal gland during the course of a lymphoma is common, but a primary presentation of adrenal insufficiency in a patient with lymphoma involving both adrenal glands is rare. We describe a 36-year-old man with non-Hodgkin lymphoma (NHL) who presented with adrenal insufficiency. His evaluation consisted of several imaging modalities, including positron emission tomography-computed tomography (PET-CT) scans, which were helpful in defining the extent of disease prior to treatment and in monitoring the patient's response to treatment. Our case illustrates the importance of preoperative evaluation to exclude a lymphoma, particularly in patients with bilateral renal and/or adrenal masses.”
Adrenal insufficiency as presenting feature of non-Hodgkin lymphoma. Jacobs BL et al. Can J Urol. 2010 Oct;17(5):5411-4.