EBV-positive mucocutaneous ulcer affecting the bowel
Abstract
Epstein-Barr virus (EBV)-positive mucocutaneous ulcer (EBVMCU) was included in the latest edition of the WHO classification of tumors of hematopoietic and lymphoid tissues as a distinct clinicopathological entity. The disease is characterized by well demarcated indurated ulcers occurring in cutaneous and mucosal sites including the gastrointestinal tract. Herein, we report two new cases of this peculiar disease. The first case presents as large bowel obstruction mimicking malignancy, the second as acute abdomen due to small bowel perforation. Histology discloses characteristic B cell blasts with Hodgkin/Reed-Sternberg cell like-features, which are positive for EBV on EBER in situ-hybridization. Clinical implications and differential diagnosis, including distinction from classical Hodgkin lymphoma and lymphomatoid granulomatosis are discussed.
Keywords
Full Text:
Read as PDFReferences
Dojcinov SD, Venkataraman G, Raffeld M, Pittaluga S, Jaffe ES. EBV positive mucocutaneous ulcer--a study of 26 cases associated with various sources of immunosuppression. Am J Surg Pathol. 2010; 34: 405-17.
Gaulard P, Swerdlow S, Harris N, Sundström C, Jaffe E. EBV-positive mucocutaneous ulcer. In: Swerdlow S, al. e, editors. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon: IARC Press; 2017. p. 307-8.
Roberts TK, Chen X, Liao JJ. Diagnostic and therapeutic challenges of EBV-positive mucocutaneous ulcer: a case report and systematic review of the literature. Exp Hematol Oncol. 2016 Apr 27; 5:13.
Zanelli M, Mengoli MC, Valli R, Froio E, Bisagni A, Zizzo M, et al. Primary classic Hodgkin lymphoma of the ileum and Epstein-Barr virus mucocutaneous ulcer of the colon: two entities compared. Virchows Arch. 2019; 474: 117-23.
Ikeda T, Gion Y, Yoshino T, Sato Y. A review of EBV-positive mucocutaneous ulcers focusing on clinical and pathological aspects. J Clin Exp Hematop. 2019; 59: 64-71.
Hart M, Thakral B, Yohe S, Balfour H, Singh C, Spwars M, et al. EBV-positive Mucocutaneous Ulcer in Organ Transplant Recipients - A Localizes Indolent Posttransplant Lymphoproliferative Disorder. Am J Surg Pathol. 2014; 38: 1522-9.
Juan A, Lobaton T, Tapia G, Manosa M, Cabre E, Domenech E. Epstein-Barr virus-positive mucocutaneous ulcer in Crohn's disease. A condition to consider in immunosuppressed IBD patients. Dig Liver Dis. 2017; 49: 934-7.
Hamanaka S, Nakagawa T, Ota S, Iida M, Ohta Y, Isshiki Y, et al. Immunomodulator-associated Epstein-Barr virus-positive mucocutaneous ulcer in a patient with refractory Crohn's disease. Clin J Gastroenterol. 2019; 12: 330-5.
Satou A, Banno S, Hanamura I, Takahashi E, Takahara T, Nobata H, et al. EBV-positive mucocutaneous ulcer arising in rheumatoid arthritis patients treated with methotrexate: Single center series of nine cases. Pathol Int. 2019; 69: 21-28.
Prieto-Torres L, Eraña I, Gil-Redondo R, Gómez de la Riva I, Manso R, Pajares R, et al. The Spectrum of EBV-Positive Mucocutaneous Ulcer: A Study of 9 Cases. Am J Surg Pathol. 2019; 43: 201-10.
Pittaluga S, Wilson W, Jaffe ES. Lymphomatoid granulomatosis. In: al. SSe, editor. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon: IARC Press; 2017. p. 312-4.
Pereira AAC, Ferreira CB, Hanemann JAC, Paranaiba LMR, Pereira PPI, Rodrigues-Fernandes CI, et al. Oral Manifestation of Lymphomatoid Granulomatosis. Head Neck Pathol. 2019; 13: 270-6.
Refbacks
- There are currently no refbacks.
Copyright 2015-2017, Hellenic Society of Pathology
ISSN: 2459-3443