Abdominal Pain, Progressive Weakness, and Weight Loss in an 83-Year-Old Man

Case PresentationAn 83-year-old man presented to the emergency room with nonspecific abdominal pain, and a subsequent computed tomography (CT) scan revealed bilateral solid adrenal masses measuring 5.5cm on the right and 2.7cm on the left. The average noncontrast Hounsfield unit of adrenal lesions was 32 HU. One month later, a positron emission tomography–CT scan showed hypermetabolism and interval growth of lesions, measuring 6.5cm on the right and 3.9cm on the left, with extensive hypermetabolic retroperitoneal lymphadenopathy (Fig. 1). Follow-up was delayed by the patient; in the subsequent 4 months, he experienced progressive weakness, impaired ambulation, abdominal pain, persistent nausea and vomiting, and weight loss (20 lbs). Upon hospital admission, his baseline 8 AM cortisol and adrenocorticotropic hormone levels were 6.2 mcg/dL and 357.0 pg/mL, respectively. He underwent adrenocorticotropic hormone–stimulation testing, which showed a peak serum cortisol level of 7.3 mcg/dL. The levels of plasma aldosterone, renin activity, plasma normetanephrine, and plasma metanephrine were <1.0 ng/dL (0.0-30.0), 1.102 ng/mL/h (0.167-5.380), 35.6 pg/mL (0.0-297.2), and <10.0 pg/mL (0.0-88.0), respectively. His dehydroepiandrosterone sulfate level was 13.5 mcg/dL (20.8-226.4). He also developed hypercalcemia, with a parathyroid hormone level of 9.7 pg/mL (15-65) and a 1,25-OH2-vitamin D level of 108.0 pg/mL (19.9-79.3). A repeat CT scan showed further interval growth and confluence of his adrenal lesions, with inferior vena cava displacement and compression of the second part of his duodenum (Fig. 2). An esophagogastroduodenoscopy performed for melena revealed a 3.0-cm friable ulcer in the second part of the duodenum—where the right adrenal mass was compressing—and a mucosal biopsy revealed diffuse neoplastic cells with nuclear pleomorphism, prominent nucleoli, and mitotic figures with positive CD20 immunohistochemical staining (Fig. 3 A and B).Fig. 2View Large Image Figure ViewerDownload Hi-res image Download (PPT)Fig. 3View Large Image Figure ViewerDownload Hi-res image Download (PPT)What is the diagnosis?AnswerBilateral primary adrenal lymphoma (PAL) and associated primary adrenal insufficiency. PAL is very rare, accounting for 1% to 4% of all non-Hodgkin lymphomas.1Ram N. Rashid O. Farooq S. Ulhaq I. Islam N. Primary adrenal non-Hodgkin lymphoma: a case report and review of the literature.J Med Case Rep. 2017; 11: 1-4Crossref PubMed Scopus (7) Google Scholar PAL typically presents with abdominal pain and symptoms of adrenal insufficiency; up to 70% of cases are bilateral.1Ram N. Rashid O. Farooq S. Ulhaq I. Islam N. Primary adrenal non-Hodgkin lymphoma: a case report and review of the literature.J Med Case Rep. 2017; 11: 1-4Crossref PubMed Scopus (7) Google Scholar,2Ozimek A. Diebold J. Linke R. Heyn J. Hallfeldt K. Mussack T. Bilateral primary adrenal non-Hodgkin’s lymphoma and primary adrenocortical carcinoma—review of the literature preoperative differentiation of adrenal tumors.Endocr J. 2008; 55: 625-638Crossref PubMed Scopus (42) Google Scholar PAL can be distinguished biochemically from adrenocortical carcinoma or metastatic pheochromocytoma because these entities do not cause adrenal insufficiency. Moreover, adrenocortical carcinoma typically presents as a large, heterogeneously enhancing unilateral mass, and up to 60% of these have excess steroid production.2Ozimek A. Diebold J. Linke R. Heyn J. Hallfeldt K. Mussack T. Bilateral primary adrenal non-Hodgkin’s lymphoma and primary adrenocortical carcinoma—review of the literature preoperative differentiation of adrenal tumors.Endocr J. 2008; 55: 625-638Crossref PubMed Scopus (42) Google Scholar Metastatic pheochromocytoma can present bilaterally, most often affiliated with familial syndromes, such as VHL, RET, SDHB, or other genetic mutations. Classically, pheochromocytoma has marked heterogeneous contrast enhancement.3Nölting S. Bechmann N. Taieb D. et al.Personalized management of pheochromocytoma and paraganglioma.Endocr Rev. 2022; 43: 199-239Crossref PubMed Scopus (32) Google Scholar Lastly, one clue to the ultimate diagnosis was hypercalcemia with an elevated 1,25-OH2-vitamin D level, indicating upregulation of 1α-hydroxylase. The patient was started on steroid replacement and is currently receiving acalabrutinib and RCHOP therapy (rituximab, cyclophosphamide, doxorubicin hydrochloride (hydroxydaunorubicin), vincristine sulfate (Oncovin), and prednisone). This case highlights the importance of early recognition and diagnosis of adrenal insufficiency secondary to adrenal lymphoma.DisclosureThe authors have no multiplicity of interest to disclose. The views expressed in this article are those of the authors and do not reflect the official policy of the Department of Army/Navy/Air Force, Department of Defense, or the U.S. Government. Case PresentationAn 83-year-old man presented to the emergency room with nonspecific abdominal pain, and a subsequent computed tomography (CT) scan revealed bilateral solid adrenal masses measuring 5.5cm on the right and 2.7cm on the left. The average noncontrast Hounsfield unit of adrenal lesions was 32 HU. One month later, a positron emission tomography–CT scan showed hypermetabolism and interval growth of lesions, measuring 6.5cm on the right and 3.9cm on the left, with extensive hypermetabolic retroperitoneal lymphadenopathy (Fig. 1). Follow-up was delayed by the patient; in the subsequent 4 months, he experienced progressive weakness, impaired ambulation, abdominal pain, persistent nausea and vomiting, and weight loss (20 lbs). Upon hospital admission, his baseline 8 AM cortisol and adrenocorticotropic hormone levels were 6.2 mcg/dL and 357.0 pg/mL, respectively. He underwent adrenocorticotropic hormone–stimulation testing, which showed a peak serum cortisol level of 7.3 mcg/dL. The levels of plasma aldosterone, renin activity, plasma normetanephrine, and plasma metanephrine were <1.0 ng/dL (0.0-30.0), 1.102 ng/mL/h (0.167-5.380), 35.6 pg/mL (0.0-297.2), and <10.0 pg/mL (0.0-88.0), respectively. His dehydroepiandrosterone sulfate level was 13.5 mcg/dL (20.8-226.4). He also developed hypercalcemia, with a parathyroid hormone level of 9.7 pg/mL (15-65) and a 1,25-OH2-vitamin D level of 108.0 pg/mL (19.9-79.3). A repeat CT scan showed further interval growth and confluence of his adrenal lesions, with inferior vena cava displacement and compression of the second part of his duodenum (Fig. 2). An esophagogastroduodenoscopy performed for melena revealed a 3.0-cm friable ulcer in the second part of the duodenum—where the right adrenal mass was compressing—and a mucosal biopsy revealed diffuse neoplastic cells with nuclear pleomorphism, prominent nucleoli, and mitotic figures with positive CD20 immunohistochemical staining (Fig. 3 A and B).Fig. 3View Large Image Figure ViewerDownload Hi-res image Download (PPT)What is the diagnosis?AnswerBilateral primary adrenal lymphoma (PAL) and associated primary adrenal insufficiency. PAL is very rare, accounting for 1% to 4% of all non-Hodgkin lymphomas.1Ram N. Rashid O. Farooq S. Ulhaq I. Islam N. Primary adrenal non-Hodgkin lymphoma: a case report and review of the literature.J Med Case Rep. 2017; 11: 1-4Crossref PubMed Scopus (7) Google Scholar PAL typically presents with abdominal pain and symptoms of adrenal insufficiency; up to 70% of cases are bilateral.1Ram N. Rashid O. Farooq S. Ulhaq I. Islam N. Primary adrenal non-Hodgkin lymphoma: a case report and review of the literature.J Med Case Rep. 2017; 11: 1-4Crossref PubMed Scopus (7) Google Scholar,2Ozimek A. Diebold J. Linke R. Heyn J. Hallfeldt K. Mussack T. Bilateral primary adrenal non-Hodgkin’s lymphoma and primary adrenocortical carcinoma—review of the literature preoperative differentiation of adrenal tumors.Endocr J. 2008; 55: 625-638Crossref PubMed Scopus (42) Google Scholar PAL can be distinguished biochemically from adrenocortical carcinoma or metastatic pheochromocytoma because these entities do not cause adrenal insufficiency. Moreover, adrenocortical carcinoma typically presents as a large, heterogeneously enhancing unilateral mass, and up to 60% of these have excess steroid production.2Ozimek A. Diebold J. Linke R. Heyn J. Hallfeldt K. Mussack T. Bilateral primary adrenal non-Hodgkin’s lymphoma and primary adrenocortical carcinoma—review of the literature preoperative differentiation of adrenal tumors.Endocr J. 2008; 55: 625-638Crossref PubMed Scopus (42) Google Scholar Metastatic pheochromocytoma can present bilaterally, most often affiliated with familial syndromes, such as VHL, RET, SDHB, or other genetic mutations. Classically, pheochromocytoma has marked heterogeneous contrast enhancement.3Nölting S. Bechmann N. Taieb D. et al.Personalized management of pheochromocytoma and paraganglioma.Endocr Rev. 2022; 43: 199-239Crossref PubMed Scopus (32) Google Scholar Lastly, one clue to the ultimate diagnosis was hypercalcemia with an elevated 1,25-OH2-vitamin D level, indicating upregulation of 1α-hydroxylase. The patient was started on steroid replacement and is currently receiving acalabrutinib and RCHOP therapy (rituximab, cyclophosphamide, doxorubicin hydrochloride (hydroxydaunorubicin), vincristine sulfate (Oncovin), and prednisone). This case highlights the importance of early recognition and diagnosis of adrenal insufficiency secondary to adrenal lymphoma. Case PresentationAn 83-year-old man presented to the emergency room with nonspecific abdominal pain, and a subsequent computed tomography (CT) scan revealed bilateral solid adrenal masses measuring 5.5cm on the right and 2.7cm on the left. The average noncontrast Hounsfield unit of adrenal lesions was 32 HU. One month later, a positron emission tomography–CT scan showed hypermetabolism and interval growth of lesions, measuring 6.5cm on the right and 3.9cm on the left, with extensive hypermetabolic retroperitoneal lymphadenopathy (Fig. 1). Follow-up was delayed by the patient; in the subsequent 4 months, he experienced progressive weakness, impaired ambulation, abdominal pain, persistent nausea and vomiting, and weight loss (20 lbs). Upon hospital admission, his baseline 8 AM cortisol and adrenocorticotropic hormone levels were 6.2 mcg/dL and 357.0 pg/mL, respectively. He underwent adrenocorticotropic hormone–stimulation testing, which showed a peak serum cortisol level of 7.3 mcg/dL. The levels of plasma aldosterone, renin activity, plasma normetanephrine, and plasma metanephrine were <1.0 ng/dL (0.0-30.0), 1.102 ng/mL/h (0.167-5.380), 35.6 pg/mL (0.0-297.2), and <10.0 pg/mL (0.0-88.0), respectively. His dehydroepiandrosterone sulfate level was 13.5 mcg/dL (20.8-226.4). He also developed hypercalcemia, with a parathyroid hormone level of 9.7 pg/mL (15-65) and a 1,25-OH2-vitamin D level of 108.0 pg/mL (19.9-79.3). A repeat CT scan showed further interval growth and confluence of his adrenal lesions, with inferior vena cava displacement and compression of the second part of his duodenum (Fig. 2). An esophagogastroduodenoscopy performed for melena revealed a 3.0-cm friable ulcer in the second part of the duodenum—where the right adrenal mass was compressing—and a mucosal biopsy revealed diffuse neoplastic cells with nuclear pleomorphism, prominent nucleoli, and mitotic figures with positive CD20 immunohistochemical staining (Fig. 3 A and B). An 83-year-old man presented to the emergency room with nonspecific abdominal pain, and a subsequent computed tomography (CT) scan revealed bilateral solid adrenal masses measuring 5.5cm on the right and 2.7cm on the left. The average noncontrast Hounsfield unit of adrenal lesions was 32 HU. One month later, a positron emission tomography–CT scan showed hypermetabolism and interval growth of lesions, measuring 6.5cm on the right and 3.9cm on the left, with extensive hypermetabolic retroperitoneal lymphadenopathy (Fig. 1). Follow-up was delayed by the patient; in the subsequent 4 months, he experienced progressive weakness, impaired ambulation, abdominal pain, persistent nausea and vomiting, and weight loss (20 lbs). Upon hospital admission, his baseline 8 AM cortisol and adrenocorticotropic hormone levels were 6.2 mcg/dL and 357.0 pg/mL, respectively. He underwent adrenocorticotropic hormone–stimulation testing, which showed a peak serum cortisol level of 7.3 mcg/dL. The levels of plasma aldosterone, renin activity, plasma normetanephrine, and plasma metanephrine were <1.0 ng/dL (0.0-30.0), 1.102 ng/mL/h (0.167-5.380), 35.6 pg/mL (0.0-297.2), and <10.0 pg/mL (0.0-88.0), respectively. His dehydroepiandrosterone sulfate level was 13.5 mcg/dL (20.8-226.4). He also developed hypercalcemia, with a parathyroid hormone level of 9.7 pg/mL (15-65) and a 1,25-OH2-vitamin D level of 108.0 pg/mL (19.9-79.3). A repeat CT scan showed further interval growth and confluence of his adrenal lesions, with inferior vena cava displacement and compression of the second part of his duodenum (Fig. 2). An esophagogastroduodenoscopy performed for melena revealed a 3.0-cm friable ulcer in the second part of the duodenum—where the right adrenal mass was compressing—and a mucosal biopsy revealed diffuse neoplastic cells with nuclear pleomorphism, prominent nucleoli, and mitotic figures with positive CD20 immunohistochemical staining (Fig. 3 A and B). What is the diagnosis?AnswerBilateral primary adrenal lymphoma (PAL) and associated primary adrenal insufficiency. PAL is very rare, accounting for 1% to 4% of all non-Hodgkin lymphomas.1Ram N. Rashid O. Farooq S. Ulhaq I. Islam N. Primary adrenal non-Hodgkin lymphoma: a case report and review of the literature.J Med Case Rep. 2017; 11: 1-4Crossref PubMed Scopus (7) Google Scholar PAL typically presents with abdominal pain and symptoms of adrenal insufficiency; up to 70% of cases are bilateral.1Ram N. Rashid O. Farooq S. Ulhaq I. Islam N. Primary adrenal non-Hodgkin lymphoma: a case report and review of the literature.J Med Case Rep. 2017; 11: 1-4Crossref PubMed Scopus (7) Google Scholar,2Ozimek A. Diebold J. Linke R. Heyn J. Hallfeldt K. Mussack T. Bilateral primary adrenal non-Hodgkin’s lymphoma and primary adrenocortical carcinoma—review of the literature preoperative differentiation of adrenal tumors.Endocr J. 2008; 55: 625-638Crossref PubMed Scopus (42) Google Scholar PAL can be distinguished biochemically from adrenocortical carcinoma or metastatic pheochromocytoma because these entities do not cause adrenal insufficiency. Moreover, adrenocortical carcinoma typically presents as a large, heterogeneously enhancing unilateral mass, and up to 60% of these have excess steroid production.2Ozimek A. Diebold J. Linke R. Heyn J. Hallfeldt K. Mussack T. Bilateral primary adrenal non-Hodgkin’s lymphoma and primary adrenocortical carcinoma—review of the literature preoperative differentiation of adrenal tumors.Endocr J. 2008; 55: 625-638Crossref PubMed Scopus (42) Google Scholar Metastatic pheochromocytoma can present bilaterally, most often affiliated with familial syndromes, such as VHL, RET, SDHB, or other genetic mutations. Classically, pheochromocytoma has marked heterogeneous contrast enhancement.3Nölting S. Bechmann N. Taieb D. et al.Personalized management of pheochromocytoma and paraganglioma.Endocr Rev. 2022; 43: 199-239Crossref PubMed Scopus (32) Google Scholar Lastly, one clue to the ultimate diagnosis was hypercalcemia with an elevated 1,25-OH2-vitamin D level, indicating upregulation of 1α-hydroxylase. The patient was started on steroid replacement and is currently receiving acalabrutinib and RCHOP therapy (rituximab, cyclophosphamide, doxorubicin hydrochloride (hydroxydaunorubicin), vincristine sulfate (Oncovin), and prednisone). This case highlights the importance of early recognition and diagnosis of adrenal insufficiency secondary to adrenal lymphoma. AnswerBilateral primary adrenal lymphoma (PAL) and associated primary adrenal insufficiency. PAL is very rare, accounting for 1% to 4% of all non-Hodgkin lymphomas.1Ram N. Rashid O. Farooq S. Ulhaq I. Islam N. Primary adrenal non-Hodgkin lymphoma: a case report and review of the literature.J Med Case Rep. 2017; 11: 1-4Crossref PubMed Scopus (7) Google Scholar PAL typically presents with abdominal pain and symptoms of adrenal insufficiency; up to 70% of cases are bilateral.1Ram N. Rashid O. Farooq S. Ulhaq I. Islam N. Primary adrenal non-Hodgkin lymphoma: a case report and review of the literature.J Med Case Rep. 2017; 11: 1-4Crossref PubMed Scopus (7) Google Scholar,2Ozimek A. Diebold J. Linke R. Heyn J. Hallfeldt K. Mussack T. Bilateral primary adrenal non-Hodgkin’s lymphoma and primary adrenocortical carcinoma—review of the literature preoperative differentiation of adrenal tumors.Endocr J. 2008; 55: 625-638Crossref PubMed Scopus (42) Google Scholar PAL can be distinguished biochemically from adrenocortical carcinoma or metastatic pheochromocytoma because these entities do not cause adrenal insufficiency. Moreover, adrenocortical carcinoma typically presents as a large, heterogeneously enhancing unilateral mass, and up to 60% of these have excess steroid production.2Ozimek A. Diebold J. Linke R. Heyn J. Hallfeldt K. Mussack T. Bilateral primary adrenal non-Hodgkin’s lymphoma and primary adrenocortical carcinoma—review of the literature preoperative differentiation of adrenal tumors.Endocr J. 2008; 55: 625-638Crossref PubMed Scopus (42) Google Scholar Metastatic pheochromocytoma can present bilaterally, most often affiliated with familial syndromes, such as VHL, RET, SDHB, or other genetic mutations. Classically, pheochromocytoma has marked heterogeneous contrast enhancement.3Nölting S. Bechmann N. Taieb D. et al.Personalized management of pheochromocytoma and paraganglioma.Endocr Rev. 2022; 43: 199-239Crossref PubMed Scopus (32) Google Scholar Lastly, one clue to the ultimate diagnosis was hypercalcemia with an elevated 1,25-OH2-vitamin D level, indicating upregulation of 1α-hydroxylase. The patient was started on steroid replacement and is currently receiving acalabrutinib and RCHOP therapy (rituximab, cyclophosphamide, doxorubicin hydrochloride (hydroxydaunorubicin), vincristine sulfate (Oncovin), and prednisone). This case highlights the importance of early recognition and diagnosis of adrenal insufficiency secondary to adrenal lymphoma. Bilateral primary adrenal lymphoma (PAL) and associated primary adrenal insufficiency. PAL is very rare, accounting for 1% to 4% of all non-Hodgkin lymphomas.1Ram N. Rashid O. Farooq S. Ulhaq I. Islam N. Primary adrenal non-Hodgkin lymphoma: a case report and review of the literature.J Med Case Rep. 2017; 11: 1-4Crossref PubMed Scopus (7) Google Scholar PAL typically presents with abdominal pain and symptoms of adrenal insufficiency; up to 70% of cases are bilateral.1Ram N. Rashid O. Farooq S. Ulhaq I. Islam N. Primary adrenal non-Hodgkin lymphoma: a case report and review of the literature.J Med Case Rep. 2017; 11: 1-4Crossref PubMed Scopus (7) Google Scholar,2Ozimek A. Diebold J. Linke R. Heyn J. Hallfeldt K. Mussack T. Bilateral primary adrenal non-Hodgkin’s lymphoma and primary adrenocortical carcinoma—review of the literature preoperative differentiation of adrenal tumors.Endocr J. 2008; 55: 625-638Crossref PubMed Scopus (42) Google Scholar PAL can be distinguished biochemically from adrenocortical carcinoma or metastatic pheochromocytoma because these entities do not cause adrenal insufficiency. Moreover, adrenocortical carcinoma typically presents as a large, heterogeneously enhancing unilateral mass, and up to 60% of these have excess steroid production.2Ozimek A. Diebold J. Linke R. Heyn J. Hallfeldt K. Mussack T. Bilateral primary adrenal non-Hodgkin’s lymphoma and primary adrenocortical carcinoma—review of the literature preoperative differentiation of adrenal tumors.Endocr J. 2008; 55: 625-638Crossref PubMed Scopus (42) Google Scholar Metastatic pheochromocytoma can present bilaterally, most often affiliated with familial syndromes, such as VHL, RET, SDHB, or other genetic mutations. Classically, pheochromocytoma has marked heterogeneous contrast enhancement.3Nölting S. Bechmann N. Taieb D. et al.Personalized management of pheochromocytoma and paraganglioma.Endocr Rev. 2022; 43: 199-239Crossref PubMed Scopus (32) Google Scholar Lastly, one clue to the ultimate diagnosis was hypercalcemia with an elevated 1,25-OH2-vitamin D level, indicating upregulation of 1α-hydroxylase. The patient was started on steroid replacement and is currently receiving acalabrutinib and RCHOP therapy (rituximab, cyclophosphamide, doxorubicin hydrochloride (hydroxydaunorubicin), vincristine sulfate (Oncovin), and prednisone). This case highlights the importance of early recognition and diagnosis of adrenal insufficiency secondary to adrenal lymphoma. DisclosureThe authors have no multiplicity of interest to disclose. The views expressed in this article are those of the authors and do not reflect the official policy of the Department of Army/Navy/Air Force, Department of Defense, or the U.S. Government. The authors have no multiplicity of interest to disclose. The views expressed in this article are those of the authors and do not reflect the official policy of the Department of Army/Navy/Air Force, Department of Defense, or the U.S. Government.