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

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.

Primary Pituitary Lymphoma As Rare Cause Of A Pituitary Mass And Hypopituitarism In Adulthood

“…Notable, 5 concomitant pituitary adenomas and 2 cases of pituitary cell hyperplasia were diagnosed (19,20,31,37,43,45,46). This association could be explained by the stimulating effect of pituitary hormones on lymphoma cells that is known to express endocrine hormones receptors (57,58), but this association is speculative and remain to be elucidated so far.…”

Acromegaly Caused by Ectopic Growth Hormone Releasing Hormone Secretion: A Review

“…Extracranial tumors constituted 78.7% of the cases and the remaining 27 cases presented with GHRH-secreting tumors within the sellar region are shown in Table 2 . Histopathological evaluation was available for 121 tumors, indicating that 119 of them were neuroendocrine tumors with only 2 exceptions for a pituitary diffuse large B-cell lymphoma (DLBCL) and an adenoid cystic carcinoma of the lung ( 18 , 33 ). Most common histopathological assessment for tumors of the sellar region was mixed gangliocytoma-pituitary adenoma, representing 77.8% of all intracranial cases.…”

“…The efficacy of surgical resection and SRL therapy cannot be righteously compared in this condition, as the second method has usually served as basic treatment in more advanced cases, often with metastatic disease which itself is a factor of worse prognosis ( 36 ). Along with SRL, other supportive methods that may be mentioned are chemotherapy, radiotherapy, immunotherapy, metastases embolization, and other hormonal treatments such as bromocriptine or pegvisomant ( 9 , 18 , 33 , 36 , 40 , 42 , 43 , 46 , 49 , 51 , 73 , 78 , 90 , 96 ). Altogether, 41 patients received one of these adjuvant treatment options, with pituitary radiation being the most frequent modality ( 18 , 19 , 40 , 59 , 64 , 67 , 70 , 77 , 78 , 96 ).…”

“…Along with SRL, other supportive methods that may be mentioned are chemotherapy, radiotherapy, immunotherapy, metastases embolization, and other hormonal treatments such as bromocriptine or pegvisomant ( 9 , 18 , 33 , 36 , 40 , 42 , 43 , 46 , 49 , 51 , 73 , 78 , 90 , 96 ). Altogether, 41 patients received one of these adjuvant treatment options, with pituitary radiation being the most frequent modality ( 18 , 19 , 40 , 59 , 64 , 67 , 70 , 77 , 78 , 96 ). Therapeutic results were available for 115 patients with an overall survival rate of 88.7%.…”

Ockham’s Razor for a Retinal Lesion and Acromegaly and Breaking the Vicious Circle

“…Growth hormone-releasing hormone (GHRH)-secreting tumors are rare causes of acromegaly/gigantism [ 1-3 ]. Neuroendocrine tumors (NETs), predominantly in the lung [ 4-10 ] and pancreas [ 4 , 9 , 11-14 ], represent the vast majority of the cases, but the gastrointestinal tract [ 15 , 16 ], thymus [ 17 , 18 ], phaeochromocytomas [ 19-21 ], mediastinal paragangliomas [ 22 ], teratomas [ 23 ], hypothalamic [ 24 ] or pituitary [ 25 ] gangliocytomas, diffuse B-cell lymphoma [ 26 ], and even a pituitary tumor [ 27 ] have been shown to be the source of GHRH. The majority of GHRH-secreting pancreatic NET patients have the MEN1 syndrome, some starting in childhood causing gigantism [ 3 ].…”