Primary B-cell pituitary lymphoma of the Burkitt type: case report of the rare clinic entity with typical clinical presentation

Czech version

Authors: Kozáková D.macháleková K.brtko P. ^{1 *,2 *,1}; P. Szépe ²; P. Vaňuga ¹; M. Pura ¹
Authors‘ workplace: Department of Endocrinology, the National Institute of Endocrinology and Diabetology, Ľubochňa, Slovakia ¹; Institute of Pathology, Jessenius Faculty of Medicine, Comenius University, Martin, Slovakia ²
Published in: Čas. Lék. čes. 2008; 147: 569-573
Category: Case Report

Overview

Primary CNS lymphomas (PCNSLs) constitute 3% of all intracranial neoplasms. From these, primary pituitary lymphomas (PPLs) represent extremely rare clinical entity. Nearly all of PCNSLs are non-Hodgkin diffuse large B-cell lymphomas. We present a 60-year-old female with right-sided third cranial nerve palsy, mild bitemporal visual field deficit, severe cephalea, and polyuria-polydipsia. Hypopituitarism with hyperprolactinemia was confirmed; brain imaging revealed a 16 mm-diameter sellar mass with suprasellar extension. A presumptive diagnosis of pituitary adenoma was established. The patient underwent a neurosurgical intervention. Histopathological examination and immunophenotyping (cytokeratin–, CD45⁺, CD79⁺, bcl-2⁻) verified high-grade B-cell non-Hodgkin lymphoma of the Burkitt type. Systemic work-up showed no other foci of lymphoma, the patient’s HIV status was negative, Epstein-Barr virus status was not disclosed. Although PPL can be undistinguishable from pituitary adenoma at imaging, one should consider lymphoma when evaluating an invasive sellar mass that is iso- to hypointense on T2-weighted magnetic resonance images, particularly when the patient is immunocompromised or old and presents with diabetes insipidus, cranial nerve palsy and fever of unknown origin in addition to the expected finding of hypopituitarism.

Key words:
primary pituitary lymphoma; high-grade B-cell lymphoma; Burkitt lymphoma; hypopituitarism; diabetes insipidus; cranial nerve palsy; fever of unknown origin; magnetic resonance imaging.

Sources

1. Flowers, A.: Brain tumors in the older person. Cancer Causes Control., 2000, 7, s. 523–538.

2. Clayton, R. N.: Sporadic pituitary tumours: from epidemiology to use of database. Baillieres Clin. Endocrinol. Metab., 1999, 13, s. 451-460.

3. Gsponer, J., De Tribolet, N., Déruaz, J. P. et al.: Diagnosis, treatment, and outcome of pituitary tumors and other abnormal intrasellar masses. Retrospective analysis of 353 patients. Medicine, 1999, 78, s. 236–269.

4. Eby, N. L., Grufferman, S., Flannelly, C. M., Schold Jr., S., C., Vogel, F. S., Burger, P. C.: Increasing incidence of primary brain lymphoma in the US. Cancer, 1988, 62, s. 2461–2465.

5. Da Silva, A. N., Lopes, M. B., Schiff, D.: Rare pathological variants and presentations of primary central nervous system lymphomas. Neurosurg. Focus, 2006, 21, s. E7, [online]. American Association of Neurological Surgeons, 2006 [cit. 2008-01-04]. Dostupné na: <http://thejns.org/doi/pdfplus/ 10.3171/foc.2006.21.5.8>.

6. Freda, P. U., Post, K. D.: Differential diagnosis of sellar masses. Endocrinol. Metab. Clin. North. Am., 1999, 28, s. 81–117.

7. Giustina, A., Gola, M., Doga, M., Rosei, E. A.: Clinical review 136: Primary lymphoma of the pituitary: An emerging clinical entity. J. Clin. Endocrinol. Metab., 2001, 86, s. 4567–4575.

8. Monabati, A., Rakei, S. M., Kumar, P. V. et al.: Primary Burkitt lymphoma of the brain in an immunocompetent patient. Case report. J. Neurosurg., 2002, 96, s. 1127–1129.

9. Shehu, B. B.: Primary central nervous system Burkitt’s lymphoma presenting with proptosis. Ann. Trop. Paediatr., 2003, 23, s. 319–320.

10. Spath-Schwalbe, E., Genvresse, I., Stein, H. et al.: Primary cerebral highly-malignant B-cell lymphoma of the Burkitt type. Dtsch. Med. Wochenschr., 1999, 124, s. 451–455.

11. Toren, A., Mandel, M., Shahar, E., et al.: Primary central nervous system Burkitt’s lymphoma presenting as Guillain-Barre syndrome. Med. Pediatr. Oncol., 1994, 23, s. 372–375.

12. Fine, H. A., Mayer, R. J.: Primary central nervous system lymphoma. Ann. Intern. Med., 1993, 119, s. 1093–1104.

13. Snider, W. D., Simpson, D. M., Aronyk, K. E., Nielsen, S. L.: Primary lymphoma of the central nervous system associated with acquired immune-deficiency syndrome (Letter)., N. Engl. J. Med., 1983, 308, s. 45.

14. Kaufmann, T. J., Lopes, M. B., Laws Jr. et al.: Primary sellar lymphoma: radiologic and pathologic findings in two patients. AJNR Am. J. Neuroradiol., 2002, 23, s. 364–367.

15. Stephens, J. W., Morganstein, D. L., McLaughlin, J. E. et al.: Isolated B-cell lymphoma of the pituitary region: a rare clinical entity. Hosp. Med., 2002, 63, s. 306–307.

16. Huang, Y. Y., Lin, S. F., Dunn, P. et al.: Primary pituitary lymphoma presenting as hypophysitis. Endocr. J., 2005, 52, s. 543–549.

17. Rudnik, A., Larysz, D., Blamek, S. et al.: Primary pituitary lymphoma. Folia Neuropathol., 2007, 45, s. 144–148.

18. Wolfe, S. Q., Hood, B., Barker, J., Benveniste, R. J.: Primary central nervous system lymphoma mimicking pituitary apoplexy: case report. Pituitary, 2008, doi: 10.1007/s11102-008-0084-8.

19. Eichler, A. F., Batchelor, T. T.: Primary central nervous system lymphoma: presentation, diagnosis, and staging. Neurosurg. Focus 2006, 21, s. E16 [online]. American Association of Neurological Surgeons, 2006 [cit. 2008-01-04]. Dostupné na: <http://thejns.org/doi/pdfplus/10.3171/foc.2006. 21.5.16>.

20. Lee, J. H., Lee, H. K., Choi, C. T., Huh, J.: Mucosa-associated lymphoid tissue lymphoma of the pituitary gland: MR imaging features. AJNR Am. J. Neuroradiol., 2002, 23, s. 838–840.

21. Kosák, M., Obenberger, J., Campr, V. et al.: Clinical case seminar: Correct evaluation of the clinical course and radiologic findings leading to the exact presumptive diagnosis of primary hypothalamo-pituitary lymphoma. J. Clin. Endocrinol. Metab. 2008, submitted.

22. Erdag, N., Bhorade, R. M., Alberico, R. A. et al.: Primary lymphoma of the central nervous system: typical and atypical CT and MR imaging appearances. AJR Am. J. Roentgenol., 2001, 176, s. 1319–1326.

23. Onda, K., Wakabayashi, K., Tanaka, R., Takahashi, H.: Intracranial malignant lymphomas: clinicopathological study of 26 autopsy cases. Brain Tumor Pathol., 1999, 16, s. 29–35.

24. Baehring, J. M., Hochberg, F. H.: Central nervous system lymphoma in AIDS and non-AIDS patients. In: Black, P. M., Loeffler, J. S. (eds.). Cancer of the nervous system. Lippincott Williams and Wilkins, 2005, s. 589–603.

25. Hirschmann, J. V.: Fever of unknown origin in adults. Clin. Infect. Dis., 1997, 24, s. 291–300.

26. Dinarello, C. A., Bunn Jr, P. A.: Fever. Semin. Oncol., 1997, 24, s. 288–298.

27. Landman, R. E., Wardlaw, S. L., Connell, R. J. et al.: Pituitary lymphoma presenting as fever of unknown origin. J. Clin. Endocrinol. Metab., 2001, 86, s. 1470–1476.

28. Commins, D. L.: Pathology of primary central nervous system lymphoma. Neurosurg. Focus 2006, 21, s. E2 [online]. American Association of Neurological Surgeons, 2006 [cit. 2008-01-05]. Dostupné na: <http://thejns.org/doi/pdfplus/ 10.3171/foc.2006.21.5.3>.

29. Jaffe, E. S., Harris, N. L., Stein, H., Vardiman, J., W.: Pathology and genetics. Tumours of haematopoietic and and lymphoid tissues. Lyon: WHO classification of tumours, International Agency for Research on Cancer, 2001.

30. Hochberg, F. H., Miller, G., Schooley, R. T. et al.: Central-nervous-system lymphoma related to Epstein-Barr virus. N. Engl. J. Med., 1983, 309, s. 745–748.

Labels

Addictology Allergology and clinical immunology Angiology Audiology Clinical biochemistry Dermatology & STDs Paediatric gastroenterology Paediatric surgery Paediatric cardiology Paediatric neurology Paediatric ENT Paediatric psychiatry Paediatric rheumatology Diabetology Pharmacy Vascular surgery Pain management Dental Hygienist

Galectins in Squamous Cell Carcinomas of the Head and Neck Cancers

Hiatal hernia and Barrett’s oesophagus Impact on symptoms occurrence and complications

Pathogenetically Complicated Case of Osteoporosis in a Young Man

Rationality and Irrationality in the Medicine and in the Life

Article was published in

Journal of Czech Physicians

2008 Issue 11

Download issue PDF