Indian Journal of Pathology and Microbiology
Home About us Instructions Submission Subscribe Advertise Contact e-Alerts Ahead Of Print Login 
Users Online: 2116
Print this page  Email this page Bookmark this page Small font sizeDefault font sizeIncrease font size


 
  Table of Contents    
CASE REPORT  
Year : 2012  |  Volume : 55  |  Issue : 4  |  Page : 549-551
Primary intravascular large B-cell lymphoma of pituitary


1 Department of Radiology, Regional Cancer Centre, Thiruvananthapuram, Kerala, India
2 Department of Pathology, Regional Cancer Centre, Thiruvananthapuram, Kerala, India

Click here for correspondence address and email

Date of Web Publication4-Mar-2013
 

   Abstract 

A 68-year-old retired nurse, who was a known hypertensive on medication, presented with prolonged fever of 2-month duration without any clinical evidence of infection. On examination she had altered mental status. She also had other nonspecific complaints such as sleep disturbances, loss of weight, etc. On investigation, she was found to have anemia, thrombocytopenia, raised erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and lactate dehydrogenase (LDH) values. She also had electrolyte imbalance. Radiological evaluation of brain showed mass lesion in the sella turcica, suggestive of pituitary adenoma. Biochemical evaluation showed hypopituitarism. Trans-sphenoidal biopsy was done. Based on histopathological and immunohistochemical findings a diagnosis of intravascular large B-cell lymphoma (IVLBCL) of pituitary was made. Our patient's condition deteriorated rapidly and she succumbed to her illness before therapy could be initiated. We are reporting this case because of the rare subtype of large B-cell lymphoma presenting at an extremely unusual primary site.

Keywords: Intravascular large B-cell lymphoma, lymphoma, pituitary

How to cite this article:
Anila K R, Nair RA, Koshy SM, Jacob PM. Primary intravascular large B-cell lymphoma of pituitary. Indian J Pathol Microbiol 2012;55:549-51

How to cite this URL:
Anila K R, Nair RA, Koshy SM, Jacob PM. Primary intravascular large B-cell lymphoma of pituitary. Indian J Pathol Microbiol [serial online] 2012 [cited 2023 Sep 25];55:549-51. Available from: https://www.ijpmonline.org/text.asp?2012/55/4/549/107811



   Introduction Top


Intravascular large B-cell lymphoma (IVLBCL) is considered to be a rare type of extra-nodal large B-cell lymphoma in the World Health Organization (WHO) classification. It is characterized by the selective growth of lymphoma cells within the lumina of capillaries with exception of larger arteries and veins. [1] The first case of IVLBCL was described by Pfleger and Tappeiner [2] in 1959. Two clinical variants have been described, Western and Asian forms. The neurologic and dermatologic signs described as specific to IVLBCL in Western countries are due to occlusion of capillaries by neoplastic cells in these organs. In addition, a considerable number of patients with IVLBCL present with nonspecific clinical manifestations including fever, malaise, pancytopenia, and hepatosplenomegaly associated with hemophagocytic syndrome. These cases are considered as Asian variant of IVLBCL because of their relatively high prevalence in Asian countries. [3]


   Case Report Top


A 68-year-old woman presented with altered sensorium and fever for the past 2 months. She also had nonspecific complaints of fatigue, sleep disturbance, polyuria, and polydypsia for the past two months. Her laboratory investigation showed anemia and thrombocytopenia. Her erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and lactate dehydrogenase (LDH) values were raised. Biochemical evaluation showed hypopitutarism with decreased cortisol (4.8 μg/dl), leutinising hormone (0.12 miu/ml), follicle stimulating hormone (1.55 miu/ml), and thyroid stimulating hormone (0.97 miu/l) levels. Her serum prolactin value was within normal limits. She also had electrolyte imbalance with hyponatremia and hypomagnesaemia.

Her HIV, HbsAg, and HCV status were negative. An ultrasound examination of abdomen was done which failed to pick up any significant abnormality. Computed tomography (CT) and magnetic resonance imaging (MRI) of brain showed a mass lesion in sella measuring 1.3 × 1.3 × 1.1 cm [Figure 1]. There was involvement of the infundibular stalk also. With a radiological diagnosis of pituitary macro adenoma the patient was taken up for trans-sphenoidal biopsy. The tissue from the sellar mass measured 0.5 × 0.2 × 0.2 cm. A bone marrow study was also done.
Figure 1: Mid-sagittal postcontrast images of the brain showing an enhancing pituitary mass lesion with enhancing altered signal intensity replacing the fatty marrow in the adjacent clivus suggestive of involvement

Click here to view


Sections from sellar mass showed pituitary tissue with tumor cells lodged mainly within the lumina of small vessels. The tumor cells were large with prominent nucleoli [Figure 2]. Immunohistochemistry showed tumor cells to be positive for CD20, CD5, MUM-1, and bcl2 [Figure 3]a-d. Tumor cells were negative for CD10 and bcl6. A CD31 immunostaining was done to highlight the intravascular location of tumor cells [Figure 4]. With the histopathological and immunohistochemical findings a diagnosis of intravascular large B-cell lymphoma of pituitary was given. Bone marrow biopsy was studied with hematoxylin and eosin (H and E) and CD20 immunostaining which failed to reveal involvement by IVLBCL.
Figure 2: Section from pituitary shows tumor cells within vascular lumens (H and E, ×400)

Click here to view
Figure 3: (a) Tumor cells are positive for CD 20 (IHC, ×400). (b) Tumor cells are positive for CD 5 (IHC, ×400). (c) Tumor cells are positive for MUM-1 (IHC, ×400). (d) Tumor cells are positive for Bcl2 (IHC, ×400)

Click here to view
Figure 4: CD 31 staining highlights the intravascular location of tumor cells (IHC ×400)

Click here to view



   Discussion Top


IVLBCL is an unusual subtype of extra-nodal large B-cell lymphoma that generally is a systemic disease with a predilection for skin and CNS involvement. Although there are several case reports and studies of IVLBCL in the literature, according to our knowledge, this is the first reported case of primary IVLBCL of pituitary. Apart from the sellar mass a detailed examination of our patient failed to reveal any lymphadenopathy or organomegaly. A bone marrow study was also normal. We came across two case reports of intravascular lymphoma involving pituitary in the literature. But both these cases were not primary pituitary lymphoma. In one case there was no histopathological confirmation of the involvement of pituitary. In this particular case the patient had IVLBCL of breast along with a sellar mass. On initiation of therapy the sellar mass decreased in size; hence the authors had concluded the pituitary mass to be IVLBCL. [4] In another case report of intravascular lymphoma of natural killer (NK) cell type the authors have demonstated disseminated disease at autopsy wherein pituitary was also involved but the patient had not presented with a sellar mass. [5] Lymphomas of the pituitary are rare. Radiological differentiation from an adenoma of pituitary is virtually impossible. Hence histopathological evaluation is essential for diagnosis.

The symptoms in patients with IVLBCL are mainly due to occlusion of the small vessels in the organs affected by tumor cells. Rare cases have been described in immunosuppressed patients, including patients with AIDS or after transplantation. There has been a case report of IVLBCL in Kaposi sarcoma lesions in a patient with AIDS. [6] Rarely, vascular channels within cutaneous hemangiomas might exhibit IVL. [7] Hematologic findings include pancytopenia, isolated anemia, thrombocytopenia or leucopenia, and autoimmune hemolytic anemia and diffuse intravascular coagulation. In our case the patient had anemia and thrombocytopenia. The bone marrow involvement can be subtle and can be missed if careful screening is not done.

There are two case reports of pituitary lymphoma presenting with PUO and hypopitutarism in the literature. [8],[9] In our case also the patient had fever resistant to treatment for a period of 2 months and biochemical evaluation showed hypopituitarism. Several disease processes can manifest themselves as fever and a sellar mass, including lymphomas. Surgical biopsy is necessary to make a diagnosis and distinguish this process from the more common pituitary adenoma. Intravascular large B-cell lymphoma is often not diagnosed sufficiently early due to the exclusive intravascular growth pattern of the tumor cells. The very poor prognosis in these patients is partly due to frequent delays in diagnosis and initiation of therapy due to their extraordinary presentation. Since vessels of all organs may be affected, many different signs can be observed. The affinity of IVLBCL to vascular structures may be due to defect in homing receptors on the neoplastic cells such as lack of CD29 and CD54. [10]

According to a study by Murase et al. CD5 positivity was associated with a higher prevalence of marrow/blood involvement and thrombocytopenia and a lower frequency of neurologic abnormalities among patients with CD10 negative IVLBCL. [11] However they concluded that there was no significant difference in prognosis between CD5 positive and CD5 negative IVLBCL. In our case CD20, CD5, MUM-1, and bcl2 were positive. Bcl6 and CD10 were negative. According to WHO, almost all cases of CD10 negative IVLBCL are MUM-1 positive. Occasional case of intravascular T-cell or NK-cell lymphoma have been reported. [5] It is better to consider such cases as a different entity separate from IVLBCL.

IVLBCL has a fulminant clinical course. The initiation of an intensive diagnostic work-up is of utmost importance in the diagnosis of this entity since a potentially curative therapeutic option is available, but only if the diagnosis is made at an early stage of the disease. We would also like to highlight that pyrexia of unknown origin (PUO) is a common feature in these cases. Our patient unfortunately succumbed to her disease before therapy could be initiated.

 
   References Top

1.Gatter KC, Warnke RA. Intravascular large B-cell lymphoma. In: Jaffe ES, Harris NL, Stein H, Vardiman JW, editors. World Health Organization: Pathology and Genetics of Tumors of Heamatopoietic and Lymphoid Tissues. Lyon, France: IARC Press; 2001:177-8.  Back to cited text no. 1
    
2.Pfleger VL, Tappeiner J. Zur Kenntnis der systemisierten Endotheliomatose der cutanen Blutgefa ße (Reticuloendotheliose?). Hautarzt 1959;10:363-9.  Back to cited text no. 2
    
3.Murase T, Nakamura S, Kawauchi K, Matsuzaki H, Sakai C, Inaba T, et al. An Asian variant of intravascular large B-cell lymphoma: Clinical, pathological and cytogenetic approaches to diffuse large B-cell lymphoma associated with haemophagocytic syndrome. Br J Haematol 2000;111:826-34.  Back to cited text no. 3
    
4.Yasuda M, Akiyama N, Miyamoto S, Warabi M, Takahama Y, Kitamura M, et al. Primary sellar lymphoma: Intravascular large B-cell lymphoma diagnosed as a double cancer and improved with chemotherapy, and literature review of primary parasellar lymphoma. Pituitary 2010;13:39-47.  Back to cited text no. 4
    
5.Wu H, Said JW, Ames ED, Chen C, McWhorter V, Chen P, et al. First reported cases of intravascular large cell lymphoma of the nk cell type clinical, histologic, immunophenotypic, and molecular Features. Am J Clin Pathol 2005;123:603-11.  Back to cited text no. 5
    
6.Hsiao CH, Su IJ, Hsieh SW, Huang SF, Tsai TF, Chen MY, et al. Epstein-Barr virus-associated intravascular lymphomatosis within Kaposi's sarcoma in an AIDS patient. Am J Surg Pathol 1999;23:482-7.  Back to cited text no. 6
    
7.Rubin MA, Cossman J, Freter CE, Azumi N. Intravascular large cell lymphoma coexisting within hemangiomas of the skin. Am J Surg Pathol 1997;21:860-4.  Back to cited text no. 7
    
8.Landman RE, Wardlaw SL, McConnell RJ, Khandji AG, Bruce JN, Freda PU. Pituitary lymphoma presenting as fever of unknown origin. J Clin Endocrinol Metab 2001;86:1470-6.  Back to cited text no. 8
    
9.Huang YY, Lin SF, Dunn P, Wai YY, Hsueh C, Tsai JS. Primary pituitary lymphoma presenting as hypophysitis. Endocr J 2005;52:543-9.  Back to cited text no. 9
    
10.Ponzoni M, Arrigoni G, Gould VE, Del Curto B, Maggioni M, Scapinello A, et al. Lack of CD 29 (beta 1 integrin) and CD 54 (ICAM-1) adhesion molecules in intravascular lymphomatosis. Hum Pathol 2000;31:220-6.  Back to cited text no. 10
    
11.Murase T, Yamaguchi M, Suzuki R, Okamoto M, Sato Y, Tamaru J, et al. Intravascular large B-cell lymphoma (IVLBCL): A clinicopathologic study of 96 cases with special reference to the immunophenotypic heterogeneity of CD5. Blood 2007;109:478-85.  Back to cited text no. 11
    

Top
Correspondence Address:
Rekha A Nair
Department of Pathology, Regional Cancer Centre, Thiruvananthapuram, Kerala
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.107811

Rights and Permissions


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

This article has been cited by
1 ICAM1-Negative Intravascular Large B-Cell Lymphoma of the Pituitary Gland: A Case Report and Literature Review
Kumiko Naito, Sawako Suzuki, Chikako Ohwada, Kazuki Ishiwata, Yutaro Ruike, Akiko Ishida, Hanna Deguchi-Horiuchi, Masanori Fujimoto, Hisashi Koide, Emiko Sakaida, Kentaro Horiguchi, Yasuo Iwadate, Ichiro Tatsuno, Naoko Inoshita, Jun-ichiro Ikeda, Tomoaki Tanaka, Koutaro Yokote
AACE Clinical Case Reports. 2021; 7(4): 249
[Pubmed] | [DOI]
2 Intravascular large B-cell lymphoma presenting with reticular telangiectasia on the trunk and panhypopituitarism: an autopsy case
Midori Tokushima, Masaki Tago, Naoko E Katsuki, Shu-ichi Yamashita
BMJ Case Reports. 2021; 14(3): e239422
[Pubmed] | [DOI]
3 Primary central nervous system lymphoma involving the hypothalamic–pituitary axis: a case series and pooled analysis
Dong-Won Shin, Jeong Hoon Kim, Young-Hoon Kim, Young Hyun Cho, Seok Ho Hong
Journal of Neuro-Oncology. 2020; 147(2): 339
[Pubmed] | [DOI]
4 Primary Pituitary Lymphoma As Rare Cause Of A Pituitary Mass And Hypopituitarism In Adulthood
Marina Caputo, Nunzia Prencipe, Alessandro Bisceglia, Chiara Bona, Mauro Maccario, Gianluca Aimaretti, Silvia Grottoli, Valentina Gasco
Endocrine Practice. 2020; 26(11): 1337
[Pubmed] | [DOI]
5 A UNIQUE CASE OF CENTRAL HYPOPITUITARISM AND CENTRAL DIABETES INSIPIDUS CAUSED BY DIFFUSE LARGE B-CELL LYMPHOMA
Jason A. Stegink,Vishal Sehgal,Manige Konig
AACE Clinical Case Reports. 2019; 5(1): e22
[Pubmed] | [DOI]
6 Primary pulmonary intravascular large B-cell lymphoma: A report of three cases and literature review
Yingwei Zhang, Lintao Bi, Yuying Qiu, Tingting Zhao, Mengshu Cao, Jingjing Ding, Fanqing Meng, Hourong Cai
Oncology Letters. 2018;
[Pubmed] | [DOI]
7 Primary pituitary lymphoma: an update of the literature
A. Tarabay,G. Cossu,M. Berhouma,M. Levivier,R. T. Daniel,M. Messerer
Journal of Neuro-Oncology. 2016; 130(3): 383
[Pubmed] | [DOI]
8 Reversible Hypopituitarism Associated with Intravascular Large B-Cell Lymphoma: Case Report of Successful Immunochemotherapy
Yusuke Sawada,Sumiyasu Ishii,Yasuhiko Koga,Taku Tomizawa,Ayako Matsui,Takuya Tomaru,Atsushi Ozawa,Nobuyuki Shibusawa,Tetsurou Satoh,Hiroaki Shimizu,Junko Hirato,Masanobu Yamada
The Tohoku Journal of Experimental Medicine. 2016; 238(3): 197
[Pubmed] | [DOI]



 

Top
 
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
   Introduction
   Case Report
   Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed7224    
    Printed191    
    Emailed1    
    PDF Downloaded84    
    Comments [Add]    
    Cited by others 8    

Recommend this journal