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Zdrojewska M, Mech-Siebieszuk E, Świątkowska-Stodulska R, Regent B, Kunc M, Zdrojewski Ł, Sworczak K. Adrenal Tumors in Young Adults: Case Reports and Literature Review. Medicina (B Aires) 2022; 58:medicina58060746. [PMID: 35744009 PMCID: PMC9230179 DOI: 10.3390/medicina58060746] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/20/2022] [Revised: 05/27/2022] [Accepted: 05/27/2022] [Indexed: 11/16/2022] Open
Abstract
The current high detection rate of adrenal tumors (4–10% of general population) is attributable to a widespread use of variety of imaging studies, especially a computed tomography. Most of them represent clinically silent and biologically indolent incidentalomas, but some adrenal tumors may pose a significant clinical challenge. Thus, in every patient with an adrenal tumor, a decision on further management is made after careful hormonal and radiological evaluation. All hormonally active tumors and those with radiological features suggesting malignancy are qualified for surgery. Approximately 80% of adrenal tumors are adrenocortical adenomas, hypertrophy, or nodular adrenocortical hyperplasia. Other histopathological diagnoses include pheochromocytoma, adrenocortical carcinoma, metastases, mesenchymal tumors, lymphomas, cysts, and ganglioneuromas. Adrenal tumors are more commonly diagnosed and better studied in elderly patients. In younger patients, under 40 years old, focal adrenal lesions are relatively rare, and histological distribution of diagnoses differs from that in elderly individuals. Younger patients are more likely to display endocrine symptoms, which raise the suspicion of an adrenal mass. In the current study, we present a case series of seven adrenal tumors occurring in young patients. The cases presented below, along with the literature review, demonstrate that the diagnosis and treatment of adrenal tumors are crucial due to endocrinopathy-derived complications and a potential risk of malignancy.
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Affiliation(s)
- Małgorzata Zdrojewska
- Department of Endocrinology and Internal Medicine, Faculty of Medicine, Medical University of Gdansk, 80-214 Gdansk, Poland; (E.M.-S.); (R.Ś.-S.); (K.S.)
- Correspondence:
| | - Emilia Mech-Siebieszuk
- Department of Endocrinology and Internal Medicine, Faculty of Medicine, Medical University of Gdansk, 80-214 Gdansk, Poland; (E.M.-S.); (R.Ś.-S.); (K.S.)
| | - Renata Świątkowska-Stodulska
- Department of Endocrinology and Internal Medicine, Faculty of Medicine, Medical University of Gdansk, 80-214 Gdansk, Poland; (E.M.-S.); (R.Ś.-S.); (K.S.)
| | - Bartosz Regent
- Department of Radiology, Faculty of Medicine, Medical University of Gdansk, 80-214 Gdansk, Poland;
| | - Michał Kunc
- Department of Pathology, Faculty of Medicine, Medical University of Gdansk, 80-214 Gdansk, Poland;
| | - Łukasz Zdrojewski
- Department of Nephrology, Transplantology and Internal Medicine, Faculty of Medicine, Medical University of Gdansk, 80-214 Gdansk, Poland;
| | - Krzysztof Sworczak
- Department of Endocrinology and Internal Medicine, Faculty of Medicine, Medical University of Gdansk, 80-214 Gdansk, Poland; (E.M.-S.); (R.Ś.-S.); (K.S.)
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Primary adrenal lymphoma as a rare cause of primary adrenal insufficiency: challenges in management and a review of the literature. AACE Clin Case Rep 2022; 8:199-203. [PMID: 36189132 PMCID: PMC9508599 DOI: 10.1016/j.aace.2022.05.003] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/28/2022] [Revised: 05/12/2022] [Accepted: 05/16/2022] [Indexed: 02/08/2023] Open
Abstract
Background/Objective Primary adrenal lymphoma (PAL) is an aggressive form of lymphoma associated with adrenal insufficiency (AI) in most cases. It requires a histologic confirmation unlike other cases of primary AI. Case Report We report a case of a 66-year-old man who presented with AI with symptomatic hypotension and hypo-osmolar hyponatremia. Ultrasound and computed tomography scans revealed bilateral bulky adrenal masses that were avid on fluorodeoxyglucose positron emission tomography scan. The diagnosis of PAL was confirmed with adrenal biopsy. He was treated with rituximab-based chemotherapy, which was complicated by several endocrine challenges, including worsening diabetes, multiple adrenal crises, prolonged hyponatremia, and refractory hypokalemia requiring spironolactone. He eventually developed central nervous system disease and was treated with palliative intent. Discussion AI in the setting of PAL can constitute both diagnostic and therapeutic challenges, including significant electrolyte imbalances as discussed in this case report. Conclusion It is important to have a high suspicion for PAL, especially in the presence of bilateral adrenal masses and AI. Early adrenal biopsy is required for diagnosis. Multidisciplinary care is vital to manage complications that arise during the disease course and treatment.
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Fan ZN, Shi HJ, Xiong BB, Zhang JS, Wang HF, Wang JS. Primary adrenal diffuse large B-cell lymphoma with normal adrenal cortex function: A case report. World J Clin Cases 2022; 10:709-716. [PMID: 35097098 PMCID: PMC8771404 DOI: 10.12998/wjcc.v10.i2.709] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/31/2021] [Revised: 11/02/2021] [Accepted: 12/10/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Diffuse large B-cell lymphoma, which accounts for about approximately 30% to 40% of non-Hodgkin's lymphomas, is the most common type and is a class of aggressive B-cell lymphomas. However, diffuse large B-cell lymphomas primary to the adrenal gland are rare.
CASE SUMMARY A 73-year-old man was admitted with abdominal pain and fatigue. After admission, enhanced adrenal computed tomography indicated irregular masses on both adrenal glands, with the larger one on the left side, approximately 8.0 cm × 4.3 cm in size. The boundary was irregular, and surrounding tissues were compressed. No obvious enhancement was observed in the arterial phase. Resection of the left adrenal gland was performed. Pathological diagnosis revealed diffuse large B-cell lymphoma. After surgery, the patient received R-CHOP immunochemotherapy. During the fourth immunochemotherapy, patient condition deteriorated, and he eventually died of respiratory failure.
CONCLUSION R-CHOP is the conventional immunochemotherapy for primary adrenal diffuse large B-cell lymphoma. Surgery is mainly used to diagnose the disease. Hence, the ideal treatment plan remains to be confirmed.
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Affiliation(s)
- Zhi-Nan Fan
- Department of Urology, The Second Affiliated Hospital, Kunming Medical University, Kunming 650000, Yunnan Province, China
| | - Hong-Jin Shi
- Department of Urology, The Second Affiliated Hospital, Kunming Medical University, Kunming 650000, Yunnan Province, China
| | - Bo-Bo Xiong
- Department of Urology, The Second Affiliated Hospital, Kunming Medical University, Kunming 650000, Yunnan Province, China
| | - Jin-Song Zhang
- Department of Urology, The Second Affiliated Hospital, Kunming Medical University, Kunming 650000, Yunnan Province, China
| | - Hai-Feng Wang
- Department of Urology, The Second Affiliated Hospital, Kunming Medical University, Kunming 650000, Yunnan Province, China
| | - Jian-Song Wang
- Department of Urology, The Second Affiliated Hospital, Kunming Medical University, Kunming 650000, Yunnan Province, China
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Constantinescu G, Preda C, Constantinescu V, Siepmann T, Bornstein SR, Lenders JWM, Eisenhofer G, Pamporaki C. Silent pheochromocytoma and paraganglioma: Systematic review and proposed definitions for standardized terminology. Front Endocrinol (Lausanne) 2022; 13:1021420. [PMID: 36325453 PMCID: PMC9618947 DOI: 10.3389/fendo.2022.1021420] [Citation(s) in RCA: 22] [Impact Index Per Article: 7.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/17/2022] [Accepted: 09/28/2022] [Indexed: 11/16/2022] Open
Abstract
Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors with heterogeneous clinical presentations and potential lethal outcomes. The diagnosis is based on clinical suspicion, biochemical testing, imaging and histopathological confirmation. Increasingly widespread use of imaging studies and surveillance of patients at risk of PPGL due to a hereditary background or a previous tumor is leading to the diagnosis of these tumors at an early stage. This has resulted in an increasing use of the term "silent" PPGL. This term and other variants are now commonly found in the literature without any clear or unified definition. Among the various terms, "clinically silent" is often used to describe the lack of signs and symptoms associated with catecholamine excess. Confusion arises when these and other terms are used to define the tumors according to their ability to synthesize and/or release catecholamines in relation to biochemical test results. In such cases the term "silent" and other variants are often inappropriately and misleadingly used. In the present analysis we provide an overview of the literature and propose standardized terminology in an attempt at harmonization to facilitate scientific communication.
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Affiliation(s)
- Georgiana Constantinescu
- Department of Endocrinology and Diabetes, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
- Department of Endocrinology, Grigore T. Popa University, Iasi, Romania
- Department of Health Care Sciences, Center for Clinical Research and Management Education, Dresden Inter-national University, Dresden, Germany
- *Correspondence: Christina Pamporaki, ; Georgiana Constantinescu,
| | - Cristina Preda
- Department of Endocrinology, Grigore T. Popa University, Iasi, Romania
| | - Victor Constantinescu
- Center of Clinical Neuroscience, University Clinic Carl-Gustav Carus, Dresden University of Technology, Dresden, Germany
| | - Timo Siepmann
- Department of Health Care Sciences, Center for Clinical Research and Management Education, Dresden Inter-national University, Dresden, Germany
- Department of Neurology, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
| | - Stefan R. Bornstein
- Department of Endocrinology and Diabetes, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
- Department of Health Care Sciences, Center for Clinical Research and Management Education, Dresden International University, Dresden, Germany
- Division of Diabetes & Nutritional Sciences, Faculty of Life Sciences & Medicine, King's College London, London, United Kingdom
| | - Jacques W. M. Lenders
- Department of Endocrinology and Diabetes, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
- Department of Internal Medicine, Radboud University Medical Centre, Nijmegen, Netherlands
| | - Graeme Eisenhofer
- Department of Endocrinology and Diabetes, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
- Institute of Clinical Chemistry and Laboratory Medicine, University of Dresden, Dresden, Germany
| | - Christina Pamporaki
- Department of Endocrinology and Diabetes, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
- *Correspondence: Christina Pamporaki, ; Georgiana Constantinescu,
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Oraibi OH, Wharry LI, Lynn AA, Chaudhry F, Jaume JC, Jun JY. LOCALLY INVASIVE PHEOCHROMOCYTOMA COMBINED WITH PRIMARY MALIGNANT ADRENAL LYMPHOMA. AACE Clin Case Rep 2018; 5:e124-e128. [PMID: 31967016 DOI: 10.4158/accr-2018-0221] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/31/2018] [Accepted: 09/18/2018] [Indexed: 01/27/2023] Open
Abstract
Objective Pheochromocytoma (PHEO) combined with primary adrenal lymphoma is extremely rare. We describe a case of locally invasive PHEO combined with primary malignant lymphoma. Methods We provide a case description with biochemical analyses, imaging, and pathologic findings. Results A 79-year-old male presented with a 17-cm, complex mass in the left adrenal gland with non-contrast Hounsfield units of 100 and left para-aortic enlarged lymph nodes imaged by computed tomography. Biochemical evaluation showed plasma and 24-hour urinary normetanephrine significantly elevated about 22 times (about 13.5 times above the normal upper limit) while metanephrine levels were normal. With adequate perioperative preparation, en bloc resection of the left adrenal gland was performed. The pathology revealed a tumor consisting of B-cell lymphoma, a PHEO forming a large adrenal mass with soft tissue invasion with extensive sclerosis and a separate nodule with PHEO without sclerosis. Immunohistochemistry and in situ hybridization of the lymphoma were consistent with Epstein-Barr virus-positive, diffuse large B-cell lymphoma. Immunohistochemistry of the PHEO was positive for chromogranin, synaptophysin, and S100. The Ki67 index was 8.7% and PHEO of the adrenal gland scaled score was 8 (≥4 is considered potentially malignant). Conclusion To the best of our knowledge this is the first case demonstrating locally invasive and potentially malignant PHEO combined with primary malignant lymphoma in the same adrenal gland.
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Khorsand A, Khatami F, Sefidbakht S, Saffar H, Sadeghipour A, Tavangar SM. Adrenal Collision Tumor Composed of Pheochromocytoma and Diffuse Large B-Cell Lymphoma: A Case Report. Int J Hematol Oncol Stem Cell Res 2018; 12:249-252. [PMID: 30774823 PMCID: PMC6375380] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/03/2022] Open
Abstract
Adrenal involvement in the course of malignant lymphoma occurs in about 4% of patients, but primary adrenal lymphoma (PAL) is extremely rare. To the best of our knowledge, only one case study reported the combination of PAL with pheochromocytoma. In the current study, we present the second case who was a 63-year-old man admitted to our hospital with hematuria and abdominal discomfort. Ultrasound imaging indicated the presence of a lesion, 5×4×3 cm in size, in the upper pole of his right kidney. Histopathologic study confirmed a collision tumor composed of pheochromocytoma and diffuse large B-cell lymphoma (DLBCL).
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Affiliation(s)
- Atieh Khorsand
- Department of Pathology, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran
| | - Fatemeh Khatami
- Chronic Diseases Research Center, Endocrinology and Metabolism Population Sciences Institute, Tehran University of Medical Sciences, Tehran, Iran
| | - Salma Sefidbakht
- Department of Pathology, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran
| | - Hiva Saffar
- Department of Pathology, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran
| | - Alireza Sadeghipour
- Department of Pathology, Rasool-e-Akram Hospital, Iran University of Medical Sciences, Tehran, Iran
| | - Seyed Mohammad Tavangar
- Department of Pathology, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran
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Zhou J, Zhao Y, Gou Z. High 18F-fluorodeoxyglucose uptake in primary bilateral adrenal diffuse large B-cell lymphomas with nongerminal center B-cell phenotype: A case report. Medicine (Baltimore) 2018; 97:e0480. [PMID: 29703006 PMCID: PMC5944495 DOI: 10.1097/md.0000000000010480] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
Abstract
RATIONALE Bilateral adrenal diffuse large B-cell lymphoma, nongerminal center B-cell phenotype (non-GCB DLBCL), is an uncommon malignancy that exhibits rapid development. Fluorine-18-fluorodeoxyglucose position emission tomography/computed tomography (CT) is extremely sensitive in distinguishing highly malignant tumors from benign tumors. PATIENT CONCERNS We report a case of non-GCB DLBCL showing significantly high uptake of 18F-FDG on PET/CT examination. DIAGNOSES Histopathological and immunohistochemical examination further confirmed that the bilateral adrenal masses were non-GCB DLBCL. INTERVENTIONS The maximal standardized uptake value (SUVmax) of the adrenal lesion was 17.2. Abnormal 18F-FDG uptake was observed in a retroperitoneal lymph node, the SUVmax of which was 14.2. OUTCOMES He was administered CHOP chemotherapy without rituximab due to high costs.His therapeutic effect and survival time could not be tracked due to patient privacy. LESSONS non-GCB DLBCL is a rare malignancy.18F-FDG PET/CT examination can distinguish benign from malignant adrenal lesions based on increased FDG uptake. It is a noninvasive method to diagnose malignant adrenal tumors.
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Joseph FG, Cook S, Gowda D. Primary adrenal lymphoma with initial presentation concerning for bilateral adrenal pheochromocytomas. BMJ Case Rep 2017; 2017:bcr-2017-220549. [PMID: 28830899 DOI: 10.1136/bcr-2017-220549] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/28/2022] Open
Abstract
Primary adrenal non-Hodgkin's lymphoma is rarely encountered. Clinical presentation is non-specific with fatigue, abdominal pain and B-symptoms being more commonly reported. We report a case of primary bilateral adrenal lymphoma who initially presented with clinical features suspicious for pheochromocytoma. The patient was recently diagnosed with hypertension and had a family history of early ischaemic heart disease. Plasma free metanephrines were also elevated. Pheochromocytoma was deemed unlikely following multiple investigations and the diagnosis of lymphoma was made following adrenal biopsy. Partial response was noted on positron emission tomography CT scan following four cycles of chemotherapy but failed to remit after six cycles of chemotherapy. Subsequently, he received 20 fractions of radiation to his adrenal glands and is now awaiting further imaging. The presentation of primary adrenal lymphoma can be variable and given that the diagnosis is obtained through tissue sampling, pheochromocytoma should be excluded due to risk of a potentially fatal adrenergic crisis.
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Affiliation(s)
- Flavian Grace Joseph
- Department of Endocrinology, Toowoomba Health Service, Toowoomba, Queensland, Australia
| | - Sheila Cook
- Department of Endocrinology, Toowoomba Health Service, Toowoomba, Queensland, Australia
| | - Durgesh Gowda
- Department of Endocrinology, Toowoomba Health Service, Toowoomba, Queensland, Australia
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