title: Primary bilateral adrenal non-Hodgkin's Burkitt-like lymphoma: a rare cause of primary adrenal insufficiency. Case report and literature review.
abstract: OBJECTIVE
  Primary bilateral adrenal non-Hodgkin's lymphoma is an extremely rare entity. Only 44 cases have been reported in the literature. The most common presenting symptoms are abdominal pain, fever, asthenia, constipation, weight loss or typical symptoms of adrenal insufficiency, hypertension, darkening of skin, orthostatic hypotension or an addisonian crisis.
  
  
  METHODS
  The case is presented of a 57-year-old man suffering from primary bilateral adrenal lymphoma with symptoms of adrenal insufficiency syndrome associated with bilateral, stabbing lumbar pain and a palpable mass on the left side. Laboratory tests revealed a considerable increase in lactate dehydrogenase levels, adrenal insufficiency, and high corticotropin levels.
  
  
  RESULTS
  Abdominal CT scan showed two large adrenal masses. A CT-guided fine needle aspiration biopsy revealed a large B-cell non-Hodgkin's lymphoma. Combination chemotherapy according to the CHOP protocol with cyclophosphamide, doxorubicin, vincristine and prednisolone was initiated, which caused a slight reduction in size of the two adrenal masses. The patient underwent a bilateral adrenalectomy with almost complete excision of the tumors. Replacement therapy with cortisone acetate was initiated postoperatively. Adjuvant combination chemotherapy according to the CHOP protocol was started but renal failure gradually emerged and the treatment could not be completed. The patient died seven months after surgery due to acute bronchial pneumonia and progression of disease.
  
  
  CONCLUSIONS
  Primary bilateral adrenal non-Hodgkin's lymphoma mainly affects adult men. Diagnosis is based on histological examination. Whether associated or not with radiotherapy, chemotherapy is the most recommended treatment. Surgery, where possible, seems to lead to an increase in survival rates, but it is not possible to draw any definite conclusions on its effectiveness as yet.
authors.affiliation: Istituto di Clinica Chirurgica Generale e Terapia Chirurgica, Università degli Studi di Trieste, Trieste, Italy.
authors.forename: Chiara
authors.initials: C
authors.lastname: Dobrinja
authors.forename: Giuliano
authors.initials: G
authors.lastname: Trevisan
authors.forename: Gennaro
authors.initials: G
authors.lastname: Liguori
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000150:complications* / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000628:therapy; D000309:Adrenal Insufficiency / Q000175:diagnosis* / Q000209:etiology*; D000315:Adrenalectomy; D000324:Adrenocorticotropic Hormone / Q000097:blood; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use; D015415:Biomarkers / Q000097:blood; D044963:Biopsy, Fine-Needle; D017024:Chemotherapy, Adjuvant; D003520:Cyclophosphamide / Q000008:administration & dosage; D018450:Disease Progression; D004317:Doxorubicin / Q000008:administration & dosage; D017809:Fatal Outcome; D006801:Humans; D007770:L-Lactate Dehydrogenase / Q000097:blood; D016393:Lymphoma, B-Cell / Q000150:complications* / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000628:therapy; D008297:Male; D008875:Middle Aged; D020360:Neoadjuvant Therapy / Q000379:methods; D011241:Prednisone / Q000008:administration & dosage; D014057:Tomography, X-Ray Computed; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D015415:Biomarkers; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D000324:Adrenocorticotropic Hormone; D007770:L-Lactate Dehydrogenase; D011241:Prednisone