scholarly journals Primary adrenal lymphoma as a cause of adrenal insufficiency, a report of two cases

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.


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.


2015 ◽  
Vol 7 (3) ◽  
pp. 65-68 ◽  
Author(s):  
Rajeev Parameswaran ◽  
Dedrick Kok Hang Chan ◽  
Poon Li Mei Michelle ◽  
Shi Wang

ABSTRACT Background Primary adrenal diffuse large B-cell lymphoma are aggressive high grade lymphomas affecting elderly gentlemen and mostly present with bilateral enlarged adrenal masses associated with B symptoms, adrenal insufficiency and elevated lactate dehydrogenase. When imaged with CT or ultrasound, these lesions usually appear as heterogeneous complex large masses with low density. They appear metabolically active on PET scan. Confirmatory diagnosis is established by image guided biopsy or surgical excision. Treatment is usually with rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP) or CHOP like regimes. Surgery has very limited role, if any in the management of this condition. The prognosis is generally poor with only about a third of patients achieving partial or complete remission following treatment. A concise review of the literature (PubMed database; 1990–2014) on the clinical management of primary adrenal lymphoma along with a case example is discussed. How to cite this article Parameswaran R, Chan DKH, Michelle PLM, Wang S. Primary Adrenal Diffuse Large B-cell Lymphoma: A Mini Review. World J Endoc Surg 2015;7(3):65-68.


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.


2018 ◽  
Vol 63 (4) ◽  
pp. 125-131 ◽  
Author(s):  
Jo Anne Lim ◽  
Peng Shyan Wong ◽  
Kar Nim Leong ◽  
Kar Loon Wong ◽  
Ting Soo Chow

We report a 72-year-old patient who presented with an ulcerated palatal mass, weight loss and adrenal insufficiency. Repeated biopsies from the mass revealed actinomycosis with no features of malignancy, while computed tomography scanning revealed a left maxillary sinus mass with invasive features and bilateral large adrenal masses. Blood and urine investigations showed adrenal insufficiency. The patient was treated as actinomycosis with adrenal involvement and was given intravenous penicillin and intravenous hydrocortisone. However, his condition did not improve and new signs appeared, that of left facial swelling and lymphadenopathy. A repeat biopsy of the palatal and adrenal masses revealed B-cell lymphoma. This case highlights the possibility that actinomycosis and lymphoma may share similar clinical presentations and may coexist. Either may mask and/or mimic the other, thus causing a delay in diagnosis. We describe the clinical progress and review the related literature. Interestingly, 9 out of the 12 reported cases of concomitant actinomycosis and malignancy (including this case) involve haematological malignancy. A high index of suspicion and treatment response reassessment is important in the management of either rare clinical entity.


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.


2017 ◽  
Vol 06 (02) ◽  
Author(s):  
Zheng Lei ◽  
Wang Qi fei ◽  
Jiang Tao ◽  
Li Quan lin ◽  
Wang Ke nan ◽  
...  

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