A Unique Case of Sclerosing Mediastinal B Cell Lymphoma

  • Vijay Kaul Bhagwan Mahaveer Cancer Hospital and Research Centre, Jaipur, India
  • Neha Sethi Bhagwan Mahaveer Cancer Hospital and Research Centre, Jaipur, India
  • C L Pande Bhagwan Mahaveer Cancer Hospital and Research Centre, Jaipur, India
  • Anjali Sharma Bhagwan Mahaveer Cancer Hospital and Research Centre, Jaipur, India
Keywords: NHL, mediastinum, sclerosing

Abstract

Primary mediastinal B cell lymphoma is a rare subtype of diffuse large B cell Lymphoma (DLBCL) arising from putative thymic B cell origin. It accounts for 2-4 % of NHL. The present case was a 55 years old male presented with sudden onset of cough. Chest x ray and CT thorax showed presence of mildly enhancing solid mass in anterior mediastinum. Fine needle aspiration cytology (FNAC) from the mediastinal mass showed presence of poorly differentiated malignant neoplasm. Trucut biopsy from the same showed presence of tumour with foci of hyalinization. On immunohistochemistry tumour cells expressed CD20, CD 30 and CD 23.  Hence the diagnosis of NHL of mediastinal sclerosing B cell type was made. The present case was one of the rare cases of Non Hodgkin Lymphoma (NHL) with an unusual presentation. Primary mediastinal B cell Lymphoma (PMBCL) with sclerosis is a distinct subtype of NHL with unique clinicopathological aspects and aggressive behavior. Prompt recognition and aggressive treatment may lead to relatively longer survival of patients.

Author Biographies

Vijay Kaul, Bhagwan Mahaveer Cancer Hospital and Research Centre, Jaipur, India
Dept of Pathology
Neha Sethi, Bhagwan Mahaveer Cancer Hospital and Research Centre, Jaipur, India
Dept of Pathology
C L Pande, Bhagwan Mahaveer Cancer Hospital and Research Centre, Jaipur, India
Dept of Pathology
Anjali Sharma, Bhagwan Mahaveer Cancer Hospital and Research Centre, Jaipur, India
Dept of Pathology

References

1. Wilson WH, Grossbard ML, Pittaluga S. Dose adjusted EPOCH chemotherapy for untreated B Cell lymphomas, a pharmacodynamics approach with high efficacy. Blood 2002;99:2685-93.
2. Lichtenstein AK, Levine A, Taylor CR. Primary mediastinal lymphoma in adults. Am J Med. 1980;68(4):509–514.
3. Swerdlow SH, Campo E, Harris NL. WHO classification of tumours of hematopoietic and lymphoid tissue, Lyon, France: IARC Press: 2008: 194-195.
4. Hofmann WJ, Momburg F, Moller P. Thymic medullary cells expressing B lymphocyte antigens. Hum pathol, 1988; 19; 1280-1287.
5. Barth TF, Leithauser F, Joos S, Bentz M, Moller P. Mediastinal (thymic) large B Cell Lymphoma where do we stand? Lancet Oncol, 2002; 3; 229-234.
6. Shemmari SA, Sankarnarayan SP, Krishan Y. Primary Mediastinal large cell lymphoma. Clinical features, prognostic factors and survival with RCHOP in Arab patients in the PET Scan era. Lung India. 2014 Jul-Sep; 31(3):228-231.
7. Paulli M, Strater J, Gianelli U, Rousset MT, Gambacorta M, Orlandi E, et al. Mediastinal B cell lymphoma, a study of its histomorphological spectrum based on 109 cases. Hum pathol, 1999; 30; 178-187.
8. Hoeller S, Zihler D, Zlobec I. BOB 1, CD79a, Cyclin E are the most appropriate markers to discriminate classical Hodkins Lymphoma from PMLBCL. Histopathology. 2010; 56(2):217-228.
9. Melzner I, Bucur AJ, Bruderlein S, Dorsch K, Hasel C, Barth F. Biallelic mutation of SOCS-1 impairs JAK 2 degradation and sustains phosphor –JAK 2 action in the MedB-1 mediastinal Lymphoma line. Blood. 2005: 105:2535-2542.
10. Kanavaros P, Gaulard P, Charlotte F, Martin N, Ducos C, Lebezu M, et al. Discordant expression of immunoglobulin and its associated molecule mb -1/CD 79a is frequently found in mediastinal large Bcell lymphomas. Am J Pathol. 1995; 146:735-741.
11. Hoeller S, Bergman CC. Grey zone Lymphoma: Lymphoma with intermediate features. AdvHematol 2012; Article id 460801, 7 pages doi:10.1155/2012/460801.
12. Johnson PW, Davies AJ. Primary mediastinal B Cell Lymphoma. Hematology Am Soc; 2008; 349-358.
13. Klimo P, Connors JM. MACOP-B chemotherapy for the treatment of diffuse large cell lymphoma.Ann Intern Med 1985 May; 102(5):596-602.
Published
2018-11-29
Section
Case Report