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Diagnosis and Treatment > Medication

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Kaja Grønning Department of Endocrinology, Akershus University Hospital, Lorenskog, Norway

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Archana Sharma Department of Endocrinology, Akershus University Hospital, Lorenskog, Norway

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Maria Adele Mastroianni Department of Haematology, Akershus University Hospital, Lorenskog, Norway

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Bo Daniel Karlsson Department of Radiology, Akershus University Hospital, Lorenskog, Norway

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Eystein S Husebye Department of Clinical Science and K.G. Jebsen Center of Autoimmune Disorders, University of Bergen, Bergen, Norway
Department of Medicine, Haukeland University Hospital, Bergen, Norway

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Kristian Løvås Department of Clinical Science and K.G. Jebsen Center of Autoimmune Disorders, University of Bergen, Bergen, Norway
Department of Medicine, Haukeland University Hospital, Bergen, Norway

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Ingrid Nermoen Department of Endocrinology, Akershus University Hospital, Lorenskog, Norway
Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway

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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.

Taxonomy:
Clinical Overview, Gland/Organ, Adrenal, Topic, Adrenal, Hormone, ACTH, Cortisol, Condition/ Syndrome, Adrenal insufficiency, Patient Demographics, Age, Geriatric, Gender, Male, Ethnicity, White, Country of Treatment, Norway, Diagnosis and Treatment, Signs and Symptoms, Abdominal discomfort, Anorexia, Arthralgia, Bowel movements - bleeding, Dizziness, Fatigue, Hyponatraemia, Hypotension, Nausea, Pyrexia, Renal failure, Tachycardia, Vomiting, Weight loss, Investigation, ACTH, ACTH stimulation, Adrenal antibodies, Biopsy, Cortisol, Creatinine (serum), CT scan, MRI, Potassium, Sodium, Urine osmolality, Intervention, Fluid repletion, Radiotherapy, Medication, Cortisone acetate, Doxorubicin, Fludrocortisone, Glucocorticoids, Hydrocortisone, Mineralocorticoids, Prednisolone, Rituximab, Sodium chloride, Related Disciplines, Oncology, Radiology/Rheumatology, Urology, Publication Details, Case Report Type, Unique/unexpected symptoms or presentations of a disease
DOI:
https://doi.org/10.1530/EDM-19-0131
Volume/Issue:
Volume 2020: Issue 1
Open access
A León-Suárez Department of Endocrinology and Metabolism, Neuroendocrinology Clinic, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Tlalpan, Mexico

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P Roldán-Sarmiento Department of Endocrinology and Metabolism, Neuroendocrinology Clinic, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Tlalpan, Mexico

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M A Gómez-Sámano Department of Endocrinology and Metabolism, Neuroendocrinology Clinic, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Tlalpan, Mexico

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A Nava-De la Vega Department of Endocrinology and Metabolism, Neuroendocrinology Clinic, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Tlalpan, Mexico

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V M Enríquez-Estrada Department of Endocrinology and Metabolism, Neuroendocrinology Clinic, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Tlalpan, Mexico

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F J Gómez-Pérez Department of Endocrinology and Metabolism, Neuroendocrinology Clinic, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Tlalpan, Mexico

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D Cuevas-Ramos Department of Endocrinology and Metabolism, Neuroendocrinology Clinic, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Tlalpan, Mexico

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Summary

Non-Hodgkin lymphoma (NHL) is a hematological tumor caused by abnormal lymphoid proliferation. NHL can arise in any part of the body, including central nervous system (CNS). However, pituitary involvement is a quite rare presentation. The diffuse large B-cell lymphoma (DLBCL) is the most common subtype when pituitary is infiltrated. Here, we report a case of pituitary infiltration of NHL DLBCL type in a woman with hypopituitarism and an infundibulum-hypophysitis-like image on magnetic resonance imaging (MRI). A female aged 64 years, complained of dyspepsia, fatigue, weight loss and urine volume increment with thirst. Endoscopy and gastric biopsy confirmed diffuse large B-cell lymphoma. Treatment with chemotherapy using R-CHOP was initiated. During her hospitalization, hypotension and polyuria were confirmed. Hormonal evaluation was compatible with central diabetes insipidus and hypopituitarism. Simple T1 sequence of MRI showed thickening of the infundibular stalk with homogeneous enhancement. After lumbar puncture analysis, CNS infiltration was confirmed showing positive atypical lymphocytes. Pituitary and infundibular stalk size normalized after R-CHOP chemotherapy treatment. In conclusion, pituitary infiltration of NHL with infundibular-hypophysitis-like image on MRI is a rare finding. Clinical picture included hypopituitarism and central diabetes insipidus. Diagnosis should be suspected after biochemical analysis and MRI results. Treatment consists of chemotherapy against NHL and hormonal replacement for pituitary dysfunction.

Learning points:

  • Pituitary infiltration by lymphoma can present with signs and symptoms of panhypopituitarism and diabetes insipidus.

  • MRI findings can resemble an autoimmune hypophysitis.

  • Patients can recover pituitary function as well as normalization of MRI after chemotherapy treatment.

Taxonomy:
Clinical Overview, Gland/Organ, Pituitary, Topic, Pituitary, Hormone, Cortisol, FSH, LH, Oestradiol (E2), Prolactin, Thyroxine (T4), Condition/ Syndrome, Adrenal insufficiency, Diabetes insipidus - neurogenic/central, Hypopituitarism, Hypothyroidism, Non-Hodgkin lymphoma, Panhypopituitarism, Pituitary tumour (malignant), Patient Demographics, Age, Adult, Gender, Female, Ethnicity, Hispanic or Latino - Mexican, Mexican American, Chicano, Country of Treatment, Mexico, Diagnosis and Treatment, Signs and Symptoms, Dyspepsia, Fatigue, Hypoadrenalism, Hypogonadism, Hyponatraemia, Hypopituitarism, Hypotension, Hypothyroidism, Nausea, Polydipsia, Polyuria, Vomiting, Investigation, ACTH stimulation, Biopsy, Cortisol (serum), CT scan, Endoscopy, FSH, FT4, Gastric biopsy, Histopathology, LH, MRI, Oestradiol (E2), Prolactin, Sodium, Urine 24-hour volume, Urine osmolality, Intervention, Chemotherapy, Medication, Anthracyclines, Antibiotics, Desmopressin, Dexamethasone, Doxorubicin, Glucocorticoids, Hydrocortisone, Levothyroxine, Norepinephrine, Prednisolone, Rituximab, Related Disciplines, Oncology, Publication Details, Case Report Type, Unique/unexpected symptoms or presentations of a disease
DOI:
https://doi.org/10.1530/EDM-16-0103
Volume/Issue:
Volume 2016: Issue 1
Open access