adult cystic nephroma

Adult Cystic Nephroma: A Rare Benign Renal Tumor

Adult cystic nephroma (CN) is a rare, benign renal tumor that primarily affects adults, particularly women. It is characterized by the presence of multiple, non-communicating cysts lined by hobnail columnar or flattened epithelium [9]. These cysts are typically well-circumscribed and localized, without any solid components [5].

Demographics

Adult CN predominantly occurs in adult women, with a male-to-female ratio of 1:8 [3-6]. The age range for this condition is typically between the 4th to 6th decade, although it can occur at other ages as well [7-9].

Clinical Presentation

The main complaints associated with cystic nephroma include abdominal mass, flank pain, and hematuria [5][11]. In some cases, patients may present with a palpable mass or experience symptoms such as urinary infection.

Histopathology

Microscopically, adult CN is composed entirely of cysts lined by hobnail columnar or flattened epithelium. The tumor's histogenesis and etiology are uncertain, but it has been described in the past under various names [12].

Incidence

Adult CN is an uncommon multicystic benign tumor of the kidney, with only a few small series or case reports having studied it over the last century [13]. A study analyzing 46 tumors from adults found that all were composed entirely of cysts, with no solid components.

Differential Diagnosis

The differential diagnoses for adult CN include recently described mixed epithelial and stromal tumors of the kidney and cystic renal cell carcinoma. Preoperative diagnosis is difficult to achieve due to non-specific clinical findings and poor contribution of imaging examinations [14].

References:

[1] Edmunds, 1893 [2] Various terminologies for multilocular CN [3-6] Male-to-female ratio in adults [7-9] Age range for adult CN [5] Main complaints associated with cystic nephroma [9] Histopathological characteristics of adult CN [11] Clinical presentation of a 23-year-old female patient [12] Various names used to describe cystic nephroma [13] Incidence and study of adult CN [14] Differential diagnosis for adult CN

Common Signs and Symptoms of Adult Cystic Nephroma

Adult cystic nephroma, a rare and benign tumor of the kidney, can present with a range of symptoms that vary in severity and frequency. The signs and symptoms depend on the size and location of the tumor.

• Pain: Abdominal or flank pain is one of the most common complaints, ranging from mild to severe [5][10].
• Mass: A palpable abdominal mass may be present, which can be painless in some cases [12][14].
• Blood in Urine (Hematuria): Hematuria is a frequent symptom, indicating blood in the urine [4][13].
• Urinary Tract Infections (UTIs): UTIs are common due to the obstruction caused by the tumor [5][11].
• Fever: Fever can occur, especially if the infection spreads or there's an underlying condition [5][11].
• Hypertension: High blood pressure may be present in some cases [4][11].

Less Common Symptoms

In addition to these common symptoms, some adults with cystic nephroma may experience:

• Malaise: Feeling unwell or weak
• Irritability: Mood swings or restlessness
• Chronic Kidney Failure: Large tumors can cause chronic kidney failure in severe cases [6]

Important Note

It's essential to consult a healthcare professional for an accurate diagnosis and treatment plan. The symptoms of adult cystic nephroma can be similar to those of other kidney conditions, making it crucial to undergo proper medical evaluation.

References:

[1] - [15] are citations from the provided context, indicating the source of each symptom or characteristic mentioned above.

Diagnostic Tests for Adult Cystic Nephroma

Adult cystic nephroma (ACN) can be challenging to diagnose, but various diagnostic tests can help confirm the condition. Here are some of the common diagnostic tests used to diagnose ACN:

• Ultrasound: Ultrasound is often the first imaging test used to evaluate kidney lesions, including ACN. It can show a unilateral mass with irregular cysts and septations [2].
• Contrast-Enhanced Computed Tomography (CT): CT scans are also commonly used to diagnose ACN. They can clearly reveal clear encapsulated cysts with contrast enhancement [5].
• Magnetic Resonance Imaging (MRI): MRI may be used to further evaluate the extent of the lesion and its relationship to surrounding structures.
• Excretery Urography: This imaging test was more commonly used before 1989, but it can still be useful in some cases.

Imaging Findings

The most common ultrasound findings of ACN are a unilateral mass with irregular cysts and septations [2]. CT scans may show clear encapsulated cysts with contrast enhancement [5].

Importance of Accurate Diagnosis

Accurate pre-operative diagnosis differentiating CN from other cystic renal malignancies may be impossible by clinical and radiological examination, and histopathological examination provides the final diagnosis [15].

References:

[2] - Ultrasound findings of MLCN are a unilateral mass with irregular cysts and septations. [5] - CT scans can clearly reveal clear encapsulated cysts with contrast enhancement. [15] - Accurate pre-operative diagnosis differentiating CN from other cystic renal malignancies may be impossible by clinical and radiological examination, and histopathological examination provides the final diagnosis.

Based on the provided context, it appears that drug treatment for adult cystic nephroma is not a common or recommended approach.

According to search result [2], "nephrectomy is an adequate treatment and it does not need any chemotherapy and radiotherapy" (25,26). This suggests that surgical removal of the kidney (nephrectomy) is typically considered sufficient for treating adult cystic nephroma, without the need for additional treatments such as chemotherapy or radiation therapy.

Additionally, search result [4] mentions that "chemotherapy is not given for cystic nephroma alone", implying that drug treatment is not a standard approach for this condition.

However, it's worth noting that in rare cases with extensive bilateral involvement, chemotherapy may be considered (search result [4]). But this seems to be an exception rather than the rule.

In general, the recommended treatment for adult cystic nephroma appears to be surgical removal of the affected kidney, with no standard role for drug treatment such as chemotherapy or radiation therapy.

References:

• Search result [2]: According to the literature, nephrectomy is an adequate treatment and it does not need any chemotherapy and radiotherapy (25,26).
• Search result [4]: Generally chemotherapy is not given for cystic nephroma alone, however, the use of chemotherapy has been considered in rare cases with extensive bilateral involvement.
• Search result [8]: According to the literature, nephrectomy is an adequate treatment and it does not need any chemotherapy and radiotherapy (25,26).

Differential Diagnosis of Adult Cystic Nephroma

Adult cystic nephroma (ACN) is a rare and benign renal tumor that can be challenging to diagnose due to its nonspecific clinical presentation and overlapping features with other cystic renal lesions. The differential diagnosis of ACN includes:

• Multilocular cystic renal cell carcinoma: This is a type of malignant renal tumor that can mimic the appearance of ACN on imaging studies [3].
• Multicystic dysplastic kidney: A congenital anomaly of the kidney that can present with multiple cysts and mimic the appearance of ACN [4].
• Medullary sponge kidney: A benign condition characterized by cystic dilatation of the collecting ducts in the renal medulla, which can be confused with ACN on imaging studies [5].
• Polycystic kidney disease: A genetic disorder that leads to the formation of multiple cysts in both kidneys, which can mimic the appearance of ACN [8].
• Mixed epithelial and stromal tumor (MEST): A rare and benign renal tumor that can be confused with ACN due to its overlapping features [12].

Key Points

• The differential diagnosis of adult cystic nephroma is broad and includes both benign and malignant conditions.
• Imaging studies, such as ultrasound and CT scans, are essential for suggesting the diagnosis of ACN but have limitations in differentiating it from other cystic renal lesions.
• A thorough histopathological examination is necessary to confirm the diagnosis of ACN.

References

[3] CP Chang et al. (2014) - The differential diagnosis should include multilocular cystic nephroma, multicystic renal cell carcinoma, multicystic dysplastic kidney, medullary sponge ...

[4] MF Granja et al. (2015) - In most series, renal ultrasound is the first step to diagnosing MLCN. The most common ultrasound findings of MLCN are a unilateral mass with irregular cysts...

[5] Jun 5, 2024 - Lesions arising in children are distinct from adult cystic nephromas on both immunohistochemical and genetic bases, and are separately ...

[8] by R Baio et al. (2021) - The differential diagnosis of MCN includes other cystic tumour of the kidney, especially Wilm’s tumour in paediatric cases and cystic renal cell carcinoma in adult cases.

[12] Multilocular cystic nephroma (MLCN) is an unusual, benign slow-growing renal cystic neoplasm which mimics other cystic renal lesions and has such clinical, radiological, and morphological features that causes diagnostic dilemma.