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Tumor Discovery Ureteral IgG4-related disease in urology

resembling a tumor, characterized by active contrast agent involving 65 patients with IgG4-RD of the genitourinary
accumulation along the thickened ureteral wall and the system, Teng et al. discovered that, for patients with
presence of enlarged regional lymph nodes . Similar IgG4-RD affecting the kidney, the mean IgG4 serum level
[12]
radiological images obtained through multi-slice CT was 9510 mg/L (4295 – 15050 mg/L), while for those with
(MSCT) of abdominal organs are presented in the clinical ureteral IgG4-RD, the mean serum IgG4 was 7920 mg/L
observation by Williams et al. , which also reported the (3058 – 38150 mg/L). In cases of renal IgG4-RD combined
[16]
CT progression of the mass, noting a 1.6-fold increase with retroperitoneal fibrosis, mean serum IgG4 levels were
in size over a 6-week observation period. In addition, maximal, remaining at 14100 mg/L (3200 – 22000 mg/L).
through the evaluation of positron emission tomography Teng et al. also reported an established direct correlation
(PET) with 18 F-fluorodeoxyglucose (FDG), Williams between the level of blood serum creatinine and the level
et al. demonstrated high metabolic activity in the of serum IgG4 . In our observation, the examination of
[10]
[16]
lesion identified through MSCT, as well as in the regional serum IgG4 was performed after surgical treatment and
lymph nodes. Analyzing FDG PET-CT results, Ueki et al. histological examination. However, we noted a serum
also reported elevated metabolic activity in the ureteral IgG4 increase to 149 mg/dL after the primary source was
formation, with up to an 8-fold excess accumulation removed.
[15]
of the radiopharmaceutical . In our observation, we The pre-operative diagnosis of IgG4-RD is exceptionally
encountered challenges in interpreting CT results, as challenging due to non-specific clinical manifestations, such
the patient presented with an extensive ureteral tumor as lumbar and abdominal pain, coupled with a radiological
exhibiting signs of active contrast agent accumulation image that is frequently misinterpreted as indicative of a
and an increase in regional lymph nodes. The obtained malignant neoplasm in the ureter. The determination of
radiological results did not permit the exclusion of a serum IgG4 is extremely rare in practice. For this reason,
malignant neoplasm of the ureter. urologists often misdiagnose these diseases as malignant
The question of performing a tumor biopsy for neoplasms, leading to the inadvertent treatment of patients
morphological confirmation of the diagnosis remains with nephroterectomy. Early diagnosis is imperative to
debatable and unresolved. According to Ueki et al., prevent overtreatment.
endoscopic biopsy of lesions should always be performed Treatment strategies for IgG4-associated disease are not
in controversial clinical situations. In such complicated well-developed, mainly relying on expert opinions and the
clinical scenarios, where the outcome of the disease results of retrospective studies. While the first prospective
may be affected, Ueki et al. suggest performing a biopsy studies have been published in recent years, they are mostly
through laparoscopic access or resection of the tumorous small and non-randomized .
[17]
ureter with an urgent morphological study . At the same
[15]
time, the question of endoscopic and intraoperative biopsy Glucocorticosteroids (GCS) serve as the primary first-
remains open, as, in the case of a malignant process, it line treatment for IgG4, with most patients responding
may lead to its further spread. In our pre-operative clinical well to induction therapy. In cases where there is a high
risk of recurrence, identified by risk factors such as
observation, the patient underwent ureteroscopy with a
biopsy of the pathologically modified parts of the ureter. multiorgan damage, elevated concentrations of IgG4
However, morphological examination revealed fragments and IgE, and peripheral blood eosinophilia, additional
of the ureteral wall represented by fibrous tissue with immunosuppressive drugs (azathioprine, methotrexate,
tacrolimus, and cyclophosphamide) may complement the
signs of chronic inflammation but without reliable signs initial GCS therapy. However, their effectiveness has not been
of tumor growth. Therefore, pre-operative biopsy is not convincingly proven. Genetically engineered biologics are
always informative and may not reveal specific signs of usually prescribed when conventional immunosuppressive
IgG4-RD.
drugs prove ineffective. Ebbo et al. used rituximab in
According to established criteria, an important diagnostic 33 patients diagnosed with IgG4-associated disease ,
[18]
parameter for identifying IgG4-related ureteral disease is an achieving a 93.5% clinical response rate and allowing
elevation in serum IgG4 beyond specified reference values discontinuation of GCS in half of the cases. However,
(>135 mg/dL) . In this regard, in contentious clinical within an average of 19 ± 11 months post-treatment,
[6]
scenarios, most authors deem it necessary to examine disease recurrence was observed in 41.9% of patients.
serum IgG4 levels. In the clinical observation by Ueki et al., Maintenance therapy demonstrated prolonged disease-
the median serum IgG4 level was 384 mg/dL (206 – 965 mg/ free survival (P = 0.02). However, it should be noted that
dL). Zhong et al. reported a serum IgG4 increase exceeding rituximab therapy is often associated with the development
3 times the reference values . In an analysis of a survey of severe infections and hypogammaglobulinemia . The
[19]
[13]
Volume 2 Issue 3 (2023) 5 https://doi.org/10.36922/td.1766