학술논문
EBV-Positive Inflammatory Follicular Dendritic Cell Sarcoma of the Spleen: Report of an Aggressive Form With Molecular Characterization.
Document Type
Academic Journal
Author
Baber A; Department of Internal Medicine, Assistance Publique-Hôpitaux de Paris, Cochin Hospital, University of Paris, Paris, France.; Legendre P; Department of Internal Medicine, Assistance Publique-Hôpitaux de Paris, Cochin Hospital, University of Paris, Paris, France.; Palmic P; Department of Pathology, Assistance Publique-Hôpitaux de Paris, Cochin Hospital, University of Paris, Paris, France.; Lupo-Mansuet A; Department of Pathology, Assistance Publique-Hôpitaux de Paris, Cochin Hospital, University of Paris, Paris, France.; Burroni B; Department of Pathology, Assistance Publique-Hôpitaux de Paris, Cochin Hospital, University of Paris, Paris, France.; Azoulay C; Department of Internal Medicine, Assistance Publique-Hôpitaux de Paris, Cochin Hospital, University of Paris, Paris, France.; Szwebel TA; Department of Internal Medicine, Assistance Publique-Hôpitaux de Paris, Cochin Hospital, University of Paris, Paris, France.; Costedoat-Chalumeau N; Department of Internal Medicine, Assistance Publique-Hôpitaux de Paris, Cochin Hospital, University of Paris, Paris, France.; Leroy K; Department of Medical Biology, Genomic Medicine and Physiology, Assistance Publique-Hôpitaux de Paris, European Hospital Georges Pompidou, University of Paris, Paris, France.; Blons H; Department of Medical Biology, Genomic Medicine and Physiology, Assistance Publique-Hôpitaux de Paris, European Hospital Georges Pompidou, University of Paris, Paris, France.; Blay JY; Léon Bérard Oncology Center, Claude Bernard Lyon 1 University, Lyon, France.; Boudou-Rouquette P; Department of Oncology, Assistance Publique-Hôpitaux de Paris, Cochin Hospital, University of Paris, Paris, France.; Terrier B; Department of Internal Medicine, Assistance Publique-Hôpitaux de Paris, Cochin Hospital, University of Paris, Paris, France.
Source
Publisher: Sage Publications Country of Publication: United States NLM ID: 9314927 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1940-2465 (Electronic) Linking ISSN: 10668969 NLM ISO Abbreviation: Int J Surg Pathol Subsets: MEDLINE
Subject
Language
English
Abstract
EBV-positive inflammatory follicular dendritic cell sarcoma (EBV+ inflammatory FDCS) is a rare neoplasm almost exclusively located in the spleen or liver. It is characterized by a proliferation of EBV-positive spindle-shaped cells bearing follicular dendritic cell markers, associated with an abundant lymphoplasmacytic infiltrate. EBV+ inflammatory FDCS is often asymptomatic or responsible for mild symptoms. It usually displays an indolent course and its prognosis is excellent after tumor removal, although relapsing and metastatic forms exist. Herein, we describe an aggressive form of splenic EBV+ inflammatory FDCS in a 79-year-old woman presenting with abdominal pain, deterioration of general health status, major inflammatory syndrome, and symptomatic hypercalcemia. A splenectomy was performed leading to a rapid improvement in her clinical condition and normalization of laboratory abnormalities. Unfortunately, her symptoms and laboratory abnormalities reappeared 4 months later. Computed tomography showed a mass in the splenectomy site and multiple liver and peritoneal nodules. Further analyses were performed on tumor tissue and showed positive phospho-ERK staining of tumoral cells indicating activation of MAPK pathway. Inactivating mutations were found on CDKN2A and NF1 genes. Subsequently, the patient's condition deteriorated rapidly. Since interleukin-6 levels were dramatically increased, tocilizumab was used but only had a transient effect on the patient's symptoms and inflammatory syndrome. Antitumor agent gemcitabine was initiated but her clinical condition continued to deteriorate and the patient died 2 weeks later. The management of aggressive forms of EBV+ inflammatory FDCS remains challenging. However, since these tumors seem to display genetic alterations, better characterization could lead to molecular targeted therapies.
Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.