Primary pituitary diffuse large B-cell lymphoma with somatotroph hyperplasia and acromegaly: case report

J Neurosurg. 2017 May;126(5):1725-1730. doi: 10.3171/2016.5.JNS16828. Epub 2016 Aug 12.

Abstract

Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma and comprises approximately 30% of all lymphomas. Patients typically present with a nonpainful mass in the neck, groin, or abdomen associated with constitutional symptoms. In this report, however, the authors describe a rare case of a 61-year-old woman with hyperprolactinemia, hypothyroidism, and acromegaly (elevation of insulin-like growth factor-1 [IGF-1]) with elevated growth hormone-releasing hormone (GHRH) in whom an MRI demonstrated diffuse enlargement of the pituitary gland. Despite medical treatment, the patient had persistent elevation of IGF-1. She underwent a transsphenoidal biopsy, which yielded a diagnosis of DLBCL with an activated B-cell immunophenotype with somatotroph hyperplasia. After stereo-tactic radiation therapy in combination with chemotherapy, she is currently in remission from her lymphoma and has normalized IGF-1 levels without medical therapy, 8 months after her histopathological diagnosis. This is the only reported case of its kind and displays the importance of a broad differential diagnosis, multidisciplinary evaluation, and critical intraoperative decision-making when treating atypical sellar lesions.

Keywords: ACTH = adrenocorticotropic hormone; B-cell; DLBCL = diffuse large B-cell lymphoma; GHRH = growth hormone–releasing hormone; IGF-1 = insulin-like growth factor-1; NHL = non-Hodgkin lymphoma; PCNSL = primary central nervous system lymphoma; R-CHOP = rituximab, cyclophosphamide, doxorubicin hydrochloride, vincristine sulfate, and prednisone; SIADH = syndrome of inappropriate antidiuretic hormone; TSH = thyroid-stimulating hormone; lymphoma; pituitary surgery; somatotroph hyperplasia.

Publication types

  • Case Reports

MeSH terms

  • Acromegaly / etiology*
  • Acromegaly / pathology
  • Female
  • Humans
  • Hyperplasia
  • Lymphoma, Large B-Cell, Diffuse / complications
  • Lymphoma, Large B-Cell, Diffuse / diagnosis*
  • Lymphoma, Large B-Cell, Diffuse / surgery*
  • Middle Aged
  • Pituitary Neoplasms / complications
  • Pituitary Neoplasms / diagnosis*
  • Pituitary Neoplasms / surgery*
  • Somatotrophs / pathology*