IgG4-related disease mimicking gynecologic malignancy
Immunoglobulin G4 (IgG4) related disease is a systemic disease that causes fibrosis, tumor-like nodules, and lymphoid hyperplasia with infiltration of IgG4 positive plasma cells. It can manifest in many organ systems; however, there are few cases that report gynecologic organ involvement. It is cruc...
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Format: | Article |
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Elsevier
2023-02-01
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Series: | Gynecologic Oncology Reports |
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Online Access: | http://www.sciencedirect.com/science/article/pii/S2352578923000061 |
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author | Rachel R. Pacyna Nicole A. Cipriani Melvy S. Mathew Josephine S. Kim |
author_facet | Rachel R. Pacyna Nicole A. Cipriani Melvy S. Mathew Josephine S. Kim |
author_sort | Rachel R. Pacyna |
collection | DOAJ |
description | Immunoglobulin G4 (IgG4) related disease is a systemic disease that causes fibrosis, tumor-like nodules, and lymphoid hyperplasia with infiltration of IgG4 positive plasma cells. It can manifest in many organ systems; however, there are few cases that report gynecologic organ involvement. It is crucial to correctly diagnose IgG4-related disease versus malignancy because the former is treated with glucocorticoids or rituximab. In this case report, we describe two patients in which IgG4-related disease mimics gynecologic cancer. In the first case, an 85 year old woman presented with diffuse lymphadenopathy and a uterine mass concerning for malignancy. Biopsies were negative for carcinoma. Inguinal lymph node biopsy demonstrated IgG4 positive plasma cells and the patient was treated with rituximab therapy given concurrent severe rheumatoid arthritis. In the second case, a 35 year old woman under surveillance for Stage IB2 squamous cell carcinoma of the cervix (status post definitive chemoradiation therapy) presented with fluorodeoxyglucose (FDG) avid paraaortic lymph nodes on positron emission tomography (PET) imaging with subsequent negative paraaortic lymph node biopsies. Serial imaging and biopsies remained inconclusive despite ongoing diffuse lymphadenopathy and clinical concern for recurrence. Supraclavicular lymph node excision was performed which demonstrated lymphoid hyperplasia with increased IgG4 plasma cells and no evidence of carcinoma, supporting the diagnosis of IgG4-related disease. The patient was treated with high dose steroids with clinical improvement and resolution of abnormal imaging findings. We demonstrate that IgG4-related disease can present with FDG-avid lesions on PET imaging and lymphadenopathy that mimics primary or recurrent gynecologic malignancy. While rare, we conclude the IgG4-related disease is an important differential diagnosis to consider in the workup of primary or recurrent gynecologic malignancy and highlights the value of PET imaging to identify unusual patterns of lymphadenopathy and guide histologic confirmation of disease. |
first_indexed | 2024-04-10T15:52:10Z |
format | Article |
id | doaj.art-d1d3b15b830c47ebbc3f402e5eb2d0bb |
institution | Directory Open Access Journal |
issn | 2352-5789 |
language | English |
last_indexed | 2024-04-10T15:52:10Z |
publishDate | 2023-02-01 |
publisher | Elsevier |
record_format | Article |
series | Gynecologic Oncology Reports |
spelling | doaj.art-d1d3b15b830c47ebbc3f402e5eb2d0bb2023-02-11T04:15:49ZengElsevierGynecologic Oncology Reports2352-57892023-02-0145101137IgG4-related disease mimicking gynecologic malignancyRachel R. Pacyna0Nicole A. Cipriani1Melvy S. Mathew2Josephine S. Kim3Pritzker School of Medicine, The University of Chicago, 5841 S Maryland Ave, Chicago, IL 60637, USA; Corresponding author at: 5841 S Maryland Ave, Chicago, IL 60637, USA.Department of Anatomic Pathology Informatics, The University of Chicago, 5841 S Maryland Ave, Chicago, IL 60637, USADepartment of Radiology/Abdominal Imaging Section, The University of Chicago, 5841 S Maryland Ave, Chicago, IL 60637, USADepartment of Obstetrics and Gynecology/Section of Gynecologic Oncology at The University of Chicago, 5841 S Maryland Ave, Chicago, IL 60637, USAImmunoglobulin G4 (IgG4) related disease is a systemic disease that causes fibrosis, tumor-like nodules, and lymphoid hyperplasia with infiltration of IgG4 positive plasma cells. It can manifest in many organ systems; however, there are few cases that report gynecologic organ involvement. It is crucial to correctly diagnose IgG4-related disease versus malignancy because the former is treated with glucocorticoids or rituximab. In this case report, we describe two patients in which IgG4-related disease mimics gynecologic cancer. In the first case, an 85 year old woman presented with diffuse lymphadenopathy and a uterine mass concerning for malignancy. Biopsies were negative for carcinoma. Inguinal lymph node biopsy demonstrated IgG4 positive plasma cells and the patient was treated with rituximab therapy given concurrent severe rheumatoid arthritis. In the second case, a 35 year old woman under surveillance for Stage IB2 squamous cell carcinoma of the cervix (status post definitive chemoradiation therapy) presented with fluorodeoxyglucose (FDG) avid paraaortic lymph nodes on positron emission tomography (PET) imaging with subsequent negative paraaortic lymph node biopsies. Serial imaging and biopsies remained inconclusive despite ongoing diffuse lymphadenopathy and clinical concern for recurrence. Supraclavicular lymph node excision was performed which demonstrated lymphoid hyperplasia with increased IgG4 plasma cells and no evidence of carcinoma, supporting the diagnosis of IgG4-related disease. The patient was treated with high dose steroids with clinical improvement and resolution of abnormal imaging findings. We demonstrate that IgG4-related disease can present with FDG-avid lesions on PET imaging and lymphadenopathy that mimics primary or recurrent gynecologic malignancy. While rare, we conclude the IgG4-related disease is an important differential diagnosis to consider in the workup of primary or recurrent gynecologic malignancy and highlights the value of PET imaging to identify unusual patterns of lymphadenopathy and guide histologic confirmation of disease.http://www.sciencedirect.com/science/article/pii/S2352578923000061Cervical cancerFluorodeoxyglucose-positron emission tomographyIgG4-related diseaseImmunoglobulin related diseaseUterine massLymphadenopathy |
spellingShingle | Rachel R. Pacyna Nicole A. Cipriani Melvy S. Mathew Josephine S. Kim IgG4-related disease mimicking gynecologic malignancy Gynecologic Oncology Reports Cervical cancer Fluorodeoxyglucose-positron emission tomography IgG4-related disease Immunoglobulin related disease Uterine mass Lymphadenopathy |
title | IgG4-related disease mimicking gynecologic malignancy |
title_full | IgG4-related disease mimicking gynecologic malignancy |
title_fullStr | IgG4-related disease mimicking gynecologic malignancy |
title_full_unstemmed | IgG4-related disease mimicking gynecologic malignancy |
title_short | IgG4-related disease mimicking gynecologic malignancy |
title_sort | igg4 related disease mimicking gynecologic malignancy |
topic | Cervical cancer Fluorodeoxyglucose-positron emission tomography IgG4-related disease Immunoglobulin related disease Uterine mass Lymphadenopathy |
url | http://www.sciencedirect.com/science/article/pii/S2352578923000061 |
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