Lymphoma with signet ring morphology: pathology with distinctive differential diagnoses

Main Article Content

Oscar Enrique Sánchez-Castro
Juan José Navarrete-Pérez
Elizabeth Angélica Retana-Flores
Juan Carlos León-Contreras
Javier Alejandro Teco-Cortés

Keywords

Signet-ring, Lymphoma Non-Hodgkin, Lymphoma

Abstract

Background: Signet-ring cell lymphoma is a rare entity that simulates epithelial neoplasms, sarcomas and reactive histiocytes conditions. It represents a morphological variant of non-Hodgkin’s lymphomas, its diagnosis can represent a challenge, therefore it should be considered in complementary studies.


Objective: The aim of this work is to show a case with a very rare morphology and to emphasize the importance of awareness this entity and avoid mistakes in its diagnosis.


Clinical case: We present the case of a 67-year-old man, who developed lymph node growths in the right armpit, neck, right groin, and submandibular region, with apparent involvement of the lungs and spleen; was diagnosed as diffuse large B cell lymphoma with signet-ring morphology, originated in the germinal center. Transmission electron microscopy study was carried out for a more precise characterization of the morphology. Unfortunately, the patient did not return for a follow-up consultation, so he did not start treatment and died 6 months after diagnosis.


Conclusions: Lymphoma with the signet-ring phenotype is rare, and can occur in any type of non-Hodgkin lymphoma; however, this morphology is more commonly associated with carcinomas and, less frequently, with sarcomas, melanomas or reactive histiocytes conditions, therefore should be considered this entity together with the appropriate use of complementary studies for proper diagnosis.

Abstract 148 | PDF (Spanish) Downloads 76

References

 

 Bogusz AM, Tierno B, Brown D, Pihan G. Extreme signet ring cell change in a large B-cell lymphoma of follicular origin. Int J Surg Pathol 2013;21(4):399-403. https://doi.org/10.1177/1066896912474342.

 

Cross PA, Eyden BP, Harris M. Signet ring cell lymphoma of T cell type. J Clin Pathol 1989;42(3):239-45. http://dx.doi.org/10.1136/jcp.42.3.239.

 

Zhang S, Sun J, Fang Y, Nassiri M, Liu L, Zhou J, et al. Signet-ring cell lymphoma: clinicopathologic, immunohistochemical, and fluorescence in situ hybridization studies of 7 cases. Ann Diagn Pathol 2017;26:38-42. https://doi.org/10.1016/j.anndiagpath.2016.10.002.

 

Basir N, Bickle IC, Telisinghe PU, Abdullah MS, Chong VH. Signet Ring Cell Lymphoma of the Small Bowel: A case report. Oman Med J 2012;27(6):491-3. https://doi.org/10.5001/omj.2012.117.

 

Sáenz-de-Chirife AM, Rojas-Bilbao E, Giménez L, Marino L, Celeste F. Signet ring cell lymphoma mimicking mucin-producing carcinoma. Medicina (B Aires) 2004;64(6):521-4. (Spanish).

 

Pappas CT, Johson PC, Sonntag VK. Signet-ring cell lymphoma of the central nervous system. Case report. J Neurosurg 1988;69(5):789-92. https://doi.org/10.3171/jns.1988.69.5.0789.

 

Wu S, Ding W, Sui X, Jiang L, Zhou A, Yu G. Signet Ring B Cell Lymphoma: A Potential Diagnostic Pitfall. Open Med (Wars) 2019;14;343-5. https://doi.org/10.1515/med-2019-0033.

 

Guerrero-Medrano J, Delgado R, Albores-Saavedra J. Signet-ring sinus histiocytosis: a reactive disorder that mimics metastatic adenocarcinoma. Cancer 1997;80(2):277-85. https://doi.org/10.1002/(SICI)1097-0142(19970715)80:2<277::AID-CNCR16>3.0.CO;2-R.

 

Kim H, Dorfman RF, Rappaport H. Signet ring cell lymphoma. A rare morphological and functional expression of nodular (follicular) lymphoma. Am J Surg Pathol 1978;2(2):119-32.

 

Dardick I, Srinivasan R, Al-Jabi M. Signet-ring cell variant of large cell lymphoma. Ultrastruct Pathol 1983;5(2-3):195-200. https://doi.org/10.3109/01913128309141839.

 

Eyden BP, Cross PA, Harris M. The ultrastructure of signet-ring cell non-Hodgking´s lymphoma. Virchows Archiv A Pathol Anat 1990;417(5):395-404. https://doi.org/10.1007/BF01606028.

 

Harris M, Eyden B, Read G. Signet ring cell lymphoma: a rare variant of follicular lymphoma. J Clin Pathol 1981;34(8):884-91. http://dx.doi.org/10.1136/jcp.34.8.884.

 

McCluggage WG, Bharucha H, el-Agnaf M, Toner PG. B cell signet-ring cell lymphoma of bone marrow. J Clin Pathol 1995;48(3):275-8. http://dx.doi.org/10.1136/jcp.48.3.275.

 

Iossifides I, Mackay B, Butler JJ. Signet-ring cell lymphoma. Ultrastruct Pathol 1980;1(4):511-7. https://doi.org/10.3109/01913128009140558.

 

Navas-Palacios JJ, Valdes MD, Lahuerta-Palacios JJ. Signet-ring cell lymphoma. Ultrastructural and immunohistochemical features of three varieties. Cancer 1983;52(9):1613-23. https://doi.org/10.1002/1097-0142(19831101)52:9<1613::AID-CNCR2820520913>3.0.CO;2-V.

 

Vernon SE, Voet RL. Transformation of “signet ring cell” lymphoma to typical nodular, poorly differentiated lymphocytic lymphoma: light microscopic, immunohistochemical and electron microscopic observations. Ultrastruc Pathol 1983;4(2-3):177-86. https://doi.org/10.3109/01913128309140788.

 

Silberman S, Fresco R, Steinecker PH. Signet ring cell lymphoma. A report of a case and review of the literature. Am J Clin Pathol 1984;81(3):358-63. https://doi.org/10.1093/ajcp/81.3.358.

 

Pileri S, Serra L, Govoni E, Martinelli G. Signet ring cell lymphoma: a case report. Histopathology 1981;5(2):165-73. https://doi.org/10.1111/j.1365-2559.1981.tb01775.x.

 

Uccini S, Pescarmona E, Ruco LP, Baroni CD, Monarca B, Modesti A. Immunohistochemical characterization of a B-cell signet ring cell lymphoma. Report of a case. Pathol Res Pract 1988;183(4):497-504. https://doi.org/10.1016/S0344-0338(88)80099-2.

 

Sakai K, Yamasaki N, Notohara K, Ueda Y. Signet ring cell “lymphoma”: mimicking the appearance of signet ring cell carcinoma. Int J Hematol 2016;103(5):481-2. https://doi.org/10.1007/s12185-016-1983-5.