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Tumor Discovery Ureteral IgG4-related disease in urology

Currently, IgG4-RD is defined by systemic involvement, The non-specific clinical manifestations of IgG4-RD
IgG4-association, and common morphological present a considerable obstacle in achieving an accurate
manifestations, including lymphoplasmacytic infiltration differential diagnosis, given the potential overlap with
and fibrosis. In 2003, a Japanese research group made a infectious, inflammatory, and neoplastic conditions.
pivotal discovery, establishing IgG4-RD as an independent This overlap often results in delayed diagnosis and the
disease with a diverse array of clinical and morphological implementation of inappropriate treatment plans for
expressions. These expressions were previously regarded as affected individuals.
distinct diseases . Here, we present a clinical case of IgG4-RD affecting
[4]
In 2011, diagnostic criteria and a three-level the ureter, initially misinterpreted as urothelial carcinoma
classification for IgG4-RD were proposed for the first and leading to surgical intervention in the patient.
time . The following criteria are considered essential: (i)
[5]
The clinical presentation comprises local or multiple lesions 2. Case presentation
with tumor-like inflammatory infiltration of focal or diffuse Patient P., a 64-year-old individual, sought medical
nature; (ii) the serum IgG4 concentration is elevated above attention at the hospital, presenting complaints of left
135 mg/dL; (iii) a histopathological examination reveals lumbar region pain and a weight loss of 4 kg over a 2-month
lymphoplasmacytic infiltration with fibrosis and obliterative period. A hypervascular tumor, exhibiting a circular shape,
phlebitis and infiltration of IgG4-positive cells (with more was identified during computed tomography (CT) imaging
than 10 cells visible in the field of view at 400× magnification of the abdominal cavity and small pelvis. The tumor was
and a ratio of IgG4/IgG-plasma cells exceeding 40%). To located at the junction of the middle and lower thirds of the
establish a reliable diagnosis of IgG4-RD, it is necessary to left ureter, causing lumen stenosis, with a length measuring
combine clinical criteria with laboratory and histological 111 mm. Extending into the ureteral wall up to 8 mm, the
studies. The presence of clinical and histological criteria tumor resulted in hydronephrosis on the left side. Further
indicates a probable IgG4-RD, while a combination of examination revealed an expansion of the upper third
clinical and laboratory criteria indicate a possible IgG4-RD. of the ureter to 18 mm, and the pelvis of the left kidney
In 2012, the first international nomenclature for this disease measured 27 mm. Additionally, external iliac lymph nodes
was proposed . on the left exhibited enlargement, measuring up to 18 mm
[6]
IgG4-RD affects various organs. Currently, IgG4-RD in diameter, while obturator nodes reached up to 13 mm
has been described in the pancreas, biliary tract, liver, (Figure 1A and B).
stomach, fiber of the retroperitoneal space, mammary, The patient underwent an ureteroscopy, during which a
lacrimal and salivary glands, prostate and thyroid glands, circular constriction of the ureter to 2/3 of its lumen was
and skin [7-9] . observed at the border of the lower and middle sections of the
Within the urinary system, IgG4-RD is infrequently left ureter. This constriction resulted from the proliferation
observed, with a higher prevalence noted in the kidneys of whitish tissue covered with fibrin. The shaft of the
and bladder [10,11] , while occurrences of ureteral lesions are instrument was passed above the constriction to determine
primarily documented through isolated observations [12,13] . the dilatation of the ureter. The length of the constriction
Given that IgG4-RD has only recently been classified as was approximately 100 mm. A biopsy was taken from the
an independent disease, ureteral lesions displaying typical affected segment of the ureter. Microscopic examination of
clinical and morphological characteristics were previously the biopsy revealed small fragments of the ureteral wall with
labeled as “inflammatory pseudotumor” or “idiopathic induced changes, partially covered with urothelium. The
segmental ureteritis.” The first description of such a lesion composition consisted of fibrous tissue with plethoric vessels
dates back to 1978 when Bissada and Finkbeiner first and diffused focal lymphoplasmacytic infiltration, including
published their findings . an admixture of histiocytes and eosinophils. Notably, no
[14]
conclusive signs of tumor growth were identified within the
The clinical manifestations of IgG4-RD are non-specific, examined tissue (Figure S1).
posing difficulties in the differential diagnosis process.
This difficulty extends to distinguishing IgG4-RD from Laboratory tests revealed a decrease in blood hemoglobin
various infectious, inflammatory diseases, and tumors. to 110 g/L, erythrocyte values to 3.39 million/µL, and
The complexity of differentiation contributes to prolonged an increase in blood creatinine to 131 µmol/L and urea
periods from the onset of initial clinical manifestations to to 7.1 mmol/L.
a definitive diagnosis, leading to the potential adoption of The clinical and radiological findings did not permit
incorrect treatment approaches for affected individuals. the exclusion of a malignant neoplasm of the ureter in the

Volume 2 Issue 3 (2023) 2 https://doi.org/10.36922/td.1766