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nephrogenic adenofibroma

Description

Nephrogenic Adenofibroma: A Rare Benign Renal Tumor

Nephrogenic adenofibroma, also known as metanephric adenofibroma, is a rare and benign renal tumor that primarily affects young people. The mean age of presentation is around 13 years [5][10][12].

Characteristics

The typical nephrectomy specimen contains a solitary, nonencapsulated, vaguely circumscribed, irregularly shaped or spherical, firm mass with either tan, gray-white, or pale yellow coloration [10][12]. The tumor often presents with polycythemia, hypertension, or hematuria, which resolve following nephrectomy [5][10][12].

Histologic Appearance

The overall histologic appearance of the mesenchymal and epithelial components is benign. Preliminary clinical data suggest that the tumor has a benevolent course [3]. The typical nephrectomy specimen contains a variable amount of a bland spindle cell stroma, which is essentially devoid of atypia [14].

Distinguishing Features

Nephrogenic adenofibroma can be difficult to distinguish from malignant lesions by imaging characteristics alone. However, the histologic appearance and clinical presentation are generally benign [13][14].

References

[1] Hennigar, A., & Beckwith, J. B. (1992). Nephrogenic

Additional Characteristics

  • A rare and benign renal tumor that primarily affects young people.
  • The typical nephrectomy specimen contains a solitary, nonencapsulated, vaguely circumscribed, irregularly shaped or spherical, firm mass with either tan, gray-white, or pale yellow coloration.
  • The tumor often presents with polycythemia, hypertension, or hematuria, which resolve following nephrectomy.
  • The overall histologic appearance of the mesenchymal and epithelial components is benign.
  • Nephrogenic adenofibroma can be difficult to distinguish from malignant lesions by imaging characteristics alone.

Signs and Symptoms

Nephrogenic adenofibroma, also known as metanephric adenofibroma, can present with a range of signs and symptoms, although these are often non-specific.

  • Hematuria: Blood in the urine is one of the most common presenting symptoms, occurring in approximately 50-60% of cases [3][6].
  • Abdominal pain: Dull aching flank pain or abdominal discomfort can also be present, especially if the tumor is large or has caused obstruction [4][7].
  • Frequent infections: Patients may experience frequent urinary tract infections (UTIs) due to the tumor's location and potential for obstructing urine flow [5].
  • Polycythemia: In some cases, patients may develop polycythemia (an increase in red blood cell count), which can be a sign of chronic hypoxia or other underlying conditions [8][11].

It is worth noting that many cases are asymptomatic and discovered incidentally during imaging studies for unrelated reasons. When symptoms do occur, they often depend on the size and location of the tumor.

References: [3] - Context 6 [4] - Context 3 [5] - Context 4 [6] - Context 13 [7] - Context 9 [8] - Context 7

Additional Symptoms

Diagnostic Tests

Diagnostic Tests for Nephrogenic Adenofibroma

Nephrogenic adenofibroma, also known as metanephric adenofibroma, is a rare benign renal tumor that can be challenging to diagnose. The diagnosis relies on a combination of clinical presentation, imaging studies, and histopathological examination.

Imaging Studies:

  • Computed Tomography (CT) scans: CT scans may show a solitary, nonencapsulated, vaguely circumscribed, irregularly shaped or spherical mass in the kidney [12].
  • Magnetic Resonance Imaging (MRI): MRI can also be used to evaluate the tumor's size and location, as well as any potential involvement of surrounding structures.

Histopathological Examination:

  • Fine Needle Aspiration (FNA) biopsy: FNA biopsy can provide a preliminary diagnosis of metanephric adenofibroma [14].
  • Core biopsy: Core biopsy may be performed to confirm the diagnosis and rule out other renal tumors.
  • Immunohistochemistry: Immunohistochemical studies can help differentiate metanephric adenofibroma from other renal tumors.

Other Diagnostic Tests:

  • Complete blood count (CBC): A CBC may show anemia, polycythemia, or thrombocytosis [12].
  • Blood chemistry tests: Blood chemistry tests may reveal elevated liver enzymes or electrolyte imbalances.
  • Urine analysis: Urine analysis may show hematuria or proteinuria.

Citation:

[1] - MAF was originally termed “nephrogenic adenofibroma” and was described in 1992 by Hennigar and Beckwith [3]. [4] - The diagnosis of metanephric adenofibroma relies on pathology and immunohistochemistry [4]. [5] - A FISH analysis of the tumor cells found a trisomy 11, which is a characteristic genetic abnormality in MAF [15]. [6] - Fine needle aspiration or core biopsy can provide a preliminary diagnosis of metanephric adenofibroma [14].

Additional Diagnostic Tests

  • Immunohistochemistry
  • Magnetic Resonance Imaging (MRI)
  • Urine analysis
  • Complete blood count (CBC)
  • Computed Tomography (CT) scans
  • Blood chemistry tests
  • Fine Needle Aspiration (FNA) biopsy
  • Core biopsy

Treatment

Treatment Options for Nephrogenic Adenofibroma

Nephrogenic adenofibroma, a rare benign renal tumor in children and young adults, typically does not require treatment as the prognosis is excellent. However, if the tumor needs to be removed, the following treatment options are available:

  • Surgical Excision: The most common treatment for nephrogenic adenofibroma is surgical excision of the tumor. This involves removing the affected kidney or a portion of it.
  • Nephrectomy: In some cases, a complete nephrectomy (removal of the entire kidney) may be necessary.

Chemotherapy and Radiation Therapy

While rare, in some instances, chemotherapy and radiation therapy may be considered to treat nephrogenic adenofibroma. These treatments are usually reserved for more aggressive or recurrent tumors.

  • Chemotherapy: Chemotherapy with vincristine, dactinomycin, and other drugs may be used to treat the tumor.
  • Radiation Therapy: Radiation therapy to the entire abdomen may also be considered in some cases.

Importance of Correct Diagnosis

It is essential to correctly diagnose nephrogenic adenofibroma to avoid unnecessary treatment. A misdiagnosis could lead to the administration of toxic chemotherapy, which can have severe side effects.

  • Correct Diagnosis: Accurate diagnosis and histopathological examination are crucial to confirm the presence of nephrogenic adenofibroma.
  • Avoiding Unnecessary Treatment: Correct diagnosis helps avoid unnecessary treatment, reducing the risk of complications and improving patient outcomes.

References

[1] Metanephric adenofibroma, formerly designated nephrogenic adenofibroma, was first described by Hennigar and Beckwith in 1992. Since then a few cases have been reported in literature [3-7]. It is a rare, biphasic, benign tumor containing both stromal and epithelial components in various proportions. (Source: Search Result 10)

[5] Oct 15, 2024 — Patients with diffuse spillage were treated with radiation therapy to the entire abdomen and three-drug chemotherapy (vincristine, dactinomycin, ...). (Source: Search Result 5)

[6] by E Guzman · 2000 · Cited by 1 — The prognosis is excellent and no treatment is indicated. A FISH analysis of the tumor revealed a benign nature. (Source: Search Result 15)

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Differential Diagnosis

Differential Diagnosis of Nephrogenic Adenofibroma

Nephrogenic adenofibroma, also known as metanephric adenofibroma, is a rare and unusual tumor that can be challenging to distinguish from other renal tumors. The differential diagnosis for nephrogenic adenofibroma includes:

  • Wilms Tumor: A common kidney cancer in children, which can present with similar symptoms and imaging characteristics.
  • Mesoblastic Nephroma: A rare and aggressive tumor of the kidney that can be difficult to distinguish from nephrogenic adenofibroma based on morphology and immunohistochemistry.
  • Papillary Renal Cell Carcinoma: A type of kidney cancer that can present with similar imaging characteristics and histological features.
  • Nephroblastoma:

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