scholarly journals Bilateral Adrenal Incidentalomas, Uncommon Presentation and Very Rare Pathology

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A119-A119
Author(s):  
Mohammad Khair Ahmad Ibraheem Hamad ◽  
Ahmed Osman Saleh ◽  
Emad Naem

Abstract Background: Adrenal incidentaloma is an adrenal mass larger than 1cm accidentally discovered by radiological examination. The incidentalomas should be evaluated for malignancy and functionality. The prevalence of adrenal incidentaloma is 4–10%,1 and around 10–15% of incidentally discovered adrenal masses can be bilateral.2 We present a patient with bilateral adrenal masses due to primary adrenal lymphoma. Clinical Case: 74-year-old gentleman, with past medical history of type-2 Diabetes mellitus, hypertension, presented to our hospital with 2 months history of right-sided flank pain, 20 kg weight loss, loss of appetite and dizziness. Otherwise, no fever, night sweats, bleeding per rectum or urinary symptoms. On physical examination, vital signs were within normal, no remarkable findings other than tenderness over the right flank. Complete blood count and chemistry panel were within normal. Abdominal CT scan was done for evaluation of the abdominal pain which showed two large masses replacing the adrenal glands measure about 10cm in diameter with patchy areas of enhancement. Adrenal insufficiency was suspected based on the symptoms and the CT scan findings. Short syncatin test (high-dose test 250mcg) was done, which showed a cortisol baseline 152nmol/L, 30 minutes 168nmol/L, and 60 minutes 169nmol/L. This was suggestive of adrenal insufficiency, so he was started on hydrocortisone. Screening for pheochromocytoma was done as the patient developed uncontrolled BP readings prior to a scheduled adrenal biopsy. 24-hour urine metanephrines and normetanephrine were negative. CT-guided adrenal mass biopsy showed findings suggestive of large B-cell lymphoma. FDG PET-CT scan showed huge bilateral intense FDG uptake in the adrenal glands with no extra-abdominal manifestations noted. He was started on R-CHOP chemotherapy and after 4cycles, a PET scan showed a significant decrease in the size of previous adrenal masses. Conclusion: Bilateral adrenal incidentaloma should be evaluated the same as unilateral adrenal mass. Although adrenal involvement is common in non-Hodgkin lymphoma, primary adrenal lymphoma is extremely uncommon. References 1.Bovio S, Cataldi A, Reimondo G, Sperone P, Novello S, Berruti A, Borasio P, Fava C, Dogliotti L, Scagliotti GV, Angeli A, Terzolo M. Prevalence of adrenal incidentaloma in a contemporary computerized tomography series. J Endocrinol Invest. 2006 Apr;29(4):298–302. doi: 10.1007/BF03344099. PMID: 16699294. 2.Angeli A, Osella G, Alì A, Terzolo M. Adrenal incidentaloma: an overview of clinical and epidemiological data from the National Italian Study Group. Horm Res. 1997;47(4–6):279–83. doi: 10.1159/000185477. PMID: 9167965.

2020 ◽  
Vol 2020 (4-5) ◽  
Author(s):  
Kylan Pathmanathan ◽  
Venkata Kodali ◽  
Abdulrazak Mohamad

Abstract Approximately 250 cases of primary adrenal lymphoma have been reported. We describe an unusual presentation of this condition. Our patient is a 65-year-old male whom presented to the emergency department with 4 days of severe persistent hiccups. He had lost 26 kg in weight in the preceding 4 months. Computed tomography abdomen revealed large invasive bilateral adrenal masses. Biochemical evaluation confirmed adrenal insufficiency. Hiccups resolved within 24 h of steroid replacement. Adrenal biopsy confirmed a diffuse large B-cell lymphoma. Hypotheses for the aetiology of his hiccups include diaphragmatic irritation and primary adrenal insufficiency. This case is interesting for its rarity involving bilateral adrenal glands, Addison’s disease from the primary adrenal tumour and rapid resolution of hiccups with corticosteroid therapy.


2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
Jakob Holm ◽  
Leif Breum ◽  
Katrine Stenfeldt ◽  
Mette Friberg Hitz

Lymphoma may occasionally involve the adrenal glands, but primary adrenal lymphoma (PAL) is very rare and only few cases have been reported. We present a case of a 60-year-old, otherwise healthy, woman, with bilateral PAL presenting with adrenal insufficiency. The patient responded initially upon administration of large doses of intravenously hydrocortisone with total remission of symptoms. An abdominal computerized tomography scan demonstrated bilateral adrenal lesions but did not demonstrate any other pathology. Since metastatic malignant disease was suspected a positron-emission-tomography scan was performed only showing significant uptake in the adrenal glands. Endocrine evaluation did not reveal abnormal function of any hormonal system and the patient was scheduled for bilateral adrenalectomy. However the clinical condition deteriorated rapidly and the patient was readmitted to hospital before surgery was performed. A new computerized tomography scan showed rapid progression of disease with further enlargement of the adrenal masses and both pulmonary and hepatic metastasis. Needle biopsy was performed but the patient refused further treatment and died before a diagnosis was obtained. The immuneohistochemical diagnosis was large B-cell lymphoma. This case should remind clinicians that PAL may be a cause of bilateral adrenal incidentaloma especially if the patient presents with adrenal insufficiency.


Author(s):  
Kaja Grønning ◽  
Archana Sharma ◽  
Maria Adele Mastroianni ◽  
Bo Daniel Karlsson ◽  
Eystein S Husebye ◽  
...  

Summary Primary adrenal lymphoma (PAL) is a rare cause of adrenal insufficiency. More than 90% is of B-cell origin. The condition is bilateral in up to 75% of cases, with adrenal insufficiency in two of three patients. We report two cases of adrenal insufficiency presenting at the age of 70 and 79 years, respectively. Both patients had negative 21-hydroxylase antibodies with bilateral adrenal lesions on CT. Biopsy showed B-cell lymphoma. One of the patients experienced intermittent disease regression on replacement dosage of glucocorticoids. Learning points: Primary adrenal lymphoma (PAL) is a rare cause of adrenal insufficiency. Bilateral adrenal masses of unknown origin or in individuals with suspected extra-adrenal malignancy should be biopsied quickly when pheochromocytoma is excluded biochemically. Steroid treatment before biopsy may affect diagnosis. Adrenal insufficiency with negative 21-hydroxylase antibodies should be evaluated radiologically.


2020 ◽  
Vol 5 (1) ◽  
Author(s):  
Tara S Kim ◽  
Kwan Cheng ◽  
Radhika Jaiswal ◽  
Pranisha Gautam-Goyal ◽  
Alyson K Myers

Abstract Adrenal incidentalomas, masses noted on imaging performed for other purposes, are common, with 10% to 15% presenting as bilateral adrenal masses. These cases can be challenging as the differential diagnosis is broad, including metastatic disease, primary adrenal lymphoma (PAL), or infection, and often requiring a biopsy if initial biochemical workup is unrevealing. We present here a relevant case description, laboratory and radiologic imaging studies, and discussion of literature. A 62-year-old Korean woman presented with altered mental status and fevers. She was found to have bilateral adrenal incidentalomas and retained acupuncture needles. Adrenal workup did not show biochemical evidence of hormonal excess. Infectious workup was unrevealing, as was a metal/toxin workup due to retained acupuncture needles. Fevers and episodes of hypotension persisted which prevented the patient from obtaining an adrenal biopsy. Bone marrow biopsy was obtained for pancytopenia and revealed B-cell lymphoma with large cell morphology and few histiocytes with hemophagocytosis, raising concern for lymphoma-induced hemophagocytic lymphohistiocytosis (HLH). PAL associated with HLH was highly suspected in our patient, given the large (7 cm) bilateral adrenal masses and bone marrow biopsy findings of lymphoma. The patient was treated for diffuse large B-cell lymphoma, with clinical improvement. PAL is a rare but aggressive lymphoma with few reported cases. It should be considered in the differential for both unilateral and bilateral adrenal masses. An early diagnosis is crucial as the main treatment is chemotherapy rather than surgery and it confers a significant survival benefit.


Author(s):  
David Lopes Sousa ◽  
Sandra D. Santos ◽  
João Pina Cabral ◽  
Ricardo Velho ◽  
Ana Sofia Teixeira ◽  
...  

Primary adrenal lymphoma (PAL) is a very rare type of non-Hodgkin’s lymphoma (NHL). Herein, we report a case of NHL of both adrenal glands in a 69-year-old man. The patient was admitted because of a 1-month history of B symptoms and symptomatic hypotension. Biochemical analysis showed normocytic normochromic anaemia, hyponatraemia, hyperkalaemia, and elevated lactate dehydrogenase, C-reactive protein and D-dimers. A computed tomography scan revealed bilateral enlargement of the adrenal glands. There was no evidence of endocrine adrenal dysfunction. The mass in the right adrenal gland was biopsied and histopathology identified a diffuse large B-cell lymphoma of the activated B-cell subtype. A positron emission tomography 18F-fluorodeoxyglucose scan showed intensive hypermetabolic lesions involving both adrenal glands, as well as other locations, with higher uptake in the adrenal glands. Taken together, these findings suggested the diagnosis of PAL. The patient responded favourably to debulking therapy and is currently undergoing chemotherapy.


2019 ◽  
Vol 12 (7) ◽  
pp. e229758
Author(s):  
Muhammad Hassan ◽  
Amrendra Kumar Mandal ◽  
Jasdeep Singh Sidhu ◽  
Luz Maria Cardenas

Extranodal adrenal involvement in non-Hodgkin’s lymphoma is very rare, estimated to be around less than 0.2%. Most common sites involved are stomach, intestine and testis. It is very rare for adrenal tumours to present as primary adrenal insufficiency, with an incidence of around 1.2% in patients diagnosed with adrenal masses. Diffuse large B cell lymphoma (DLBL) originating from the stomach and metastasizing to bilateral adrenal glands is an extremely uncommon occurrence with only three cases found on review of the literature. We present a case of a 62-year-old African–American man who presented with nausea, vomiting, abdominal pain and hypotension, later being diagnosed as DLBL of the gastric antrum metastasized to bilateral adrenal glands. Initial laboratory workup revealed including hormonal analysis and cosyntropin test revealed adrenal insufficiency. The patient later died during the hospitalisation after developing respiratory failure, severe hypotension refractory to vasopressors and severe metabolic acidosis.


2010 ◽  
Vol 2 (1) ◽  
pp. 39-43 ◽  
Author(s):  
Jaya Bhanu Kanwar

ABSTRACT Adrenal lymphoma is a rare diagnosis in subject presenting with bilateral adrenal masses. Adrenal insufficiency is a common complication of this disease. Most cases of primary adrenal lymphoma (PAL) present with adrenal insufficiency and huge bilateral adrenal masses. These are very aggressive tumors with poor prognosis. We, hereby, report two subjects of adrenal lymphoma presenting with bilateral adrenal masses. First case is a 50 years old male presented with features of adrenal insufficiency like anorexia, weight loss, nausea, vomiting, and generalized hyperpigmentation. Routine investigations revealed hyponatremia, normal serum K; serum lactate dehydrogenase was moderately elevated. Primary adrenal insufficiency was confirmed by low basal and cosyntrophin stimulated cortisol. CT abdomen revealed huge bilateral heterogeneous enlargement of adrenal glands. Lymphoma was confirmed by histopathology and immunohistochemistry, diagnosis of PAL was made after further staging. He was treated with Rituximab-CHOP regimen. Second case, 50 years old lady presented with fever, anorexia, weight loss. She had generalized lymphadenopathy. On imaging found to have bilateral adrenal mass, and histopathology from lymph node, breast mass and adrenal mass was suggestive of non-Hodgkin's lymphoma.


2019 ◽  
Vol 12 (9) ◽  
pp. e231114
Author(s):  
Raghav Bhargava ◽  
Anthony Dixon

Adrenal lymphoma causing primary hypoadrenalism in the elderly is a very rare finding. We describe a case of an 85-year-old man who was admitted to our hospital. He had a short history of hypotension and unresponsiveness. He was referred to the endocrine team, after a CT scan of his thorax abdomen and pelvis demonstrated significant enlargement of both his adrenal glands with associated lymphadenopathy. This scan was originally done to rule out an occult intra-abdominal malignancy. In addition, a tetracosactide (short Synacthen) test demonstrated adrenal insufficiency. This case demonstrates that adrenal lymphoma should be considered as an important differential when there is evidence of adrenal gland hyperplasia, lymphadenopathy and adrenal insufficiency.


2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Sherin Elsa Mathews ◽  
Jessica Castellanos-Diaz ◽  
Alexis da Silva ◽  
William Troy Donahoo ◽  
Hans Kumar Ghayee ◽  
...  

Abstract Introduction Primary adrenal lymphoma (PAL) is a very rare and aggressive form of Non-Hodgkin’s lymphoma originating from the adrenal glands and with no prior history of lymphoma. It can present with symptoms related to adrenal insufficiency due to tumoral destruction of normal adrenal tissue. Hypercalcemia has also been previously reported as a less common presentation of PAL. We describe here a case of PAL presenting with severe hypercalcemia and adrenal insufficiency. Clinical Case 75 year male presented with 50 pound weight loss, fatigue and anorexia for several months. On admission, patient had altered mental status. Further investigation revealed severe hypercalcemia (corrected calcium for albumin =15mg/dl) and AKI (serum creatine=3.45mg/dl, blood urea nitrogen=89). He was started on IV fluids for the hypercalcemia and AKI. Additional work up revealed suppressed parathyroid hormone (PTH) levels of 9 (12 - 88 pg/mL), high normal PTH-related peptide 22 (14-27), low 25-OHD 7.34 (20-120 ng/ml), high normal 1,25-(OH)2-D 63.0 (19.9-79 pg/mL). His ACTH level was elevated ~159 (7.2-63.3 pg/ml) with low normal morning cortisol levels ~10 mcg/dl. Patient was started on IV stress dose steroids for concerns of adrenal insufficiency and calcitonin was also initiated for the treatment of hypercalcemia. CT of the abdomen showed bilateral adrenal masses, measuring 9.8 x 6.4 x 10.8 cm on the right and 9.3 x 5.2 x 9.6 cm on the left with marked retroperitoneal lymphadenopathy. After ruling out pheochromocytoma with negative plasma and urine metanephrines, biopsy of the adrenal mass revealed a highly aggressive diffuse B large cell lymphoma, with BCL2 and MYC overexpression. Concurrent flow cytometric analysis also identified an abnormal population of medium to large B cells expressing bright CD20, CD19, CD38, CD45 without CD10, CD5, CD23 or overt kappa or lambda expression. His calcium levels improved with hydration and steroids. Chemotherapy was later initiated. Conclusion Primary adrenal lymphoma is rare and aggressive type of NHL. While adrenal insufficiency is expected in this clinical scenario, hypercalcemia from 1,25-(OH)2-D excess is a relatively uncommon presentation. Adrenal insufficiency should be ruled out in bilateral infiltrative adrenal masses. Likely etiologies for hypercalcemia in our case appeared to be secondary to elevated 1,25-(OH)2-D production, humoral hypercalcemia of malignancy and worsened by untreated clinically evident adrenal insufficiency. References (1) Laurent C, Casasnovas O, Martin L, et al. Adrenal lymphoma: presentation, management and prognosis. QJM. 2017 Feb 1;110(2):103-109. doi: 10.1093/qjmed/hcw174 (2) Masood A, Tumyan A, Nussenzveig D R., Wakefield D N., Barb D, Ghayee H K., Maalouf N M. The Diverse Clinical Presentations of Adrenal Lymphoma. AACE Clinical Case Reports: Autumn 2017, Vol. 3, No. 4, pp. e307-e312. doi: 10.4158/EP161595.CR


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