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obsolete adult mesoblastic nephroma
Description
Definition and Characteristics
Obsolete adult mesoblastic nephroma, also known as congenital mesoblastic nephroma presenting in adulthood, is a rare and unusual type of kidney tumor that primarily affects infants but can occasionally occur in adults.
- It is a mixed neoplasm consisting of classic and cellular (atypical) variants [3][5].
- The homogeneous mass typically resembles spindle mesenchymal cells that resemble fibroblasts or muscle cells [4].
- Adult mesoblastic nephroma rarely occurs, with only 17 reported cases in the literature [11].
Incidence and Age Distribution
- This tumor is predominantly seen in early infancy but can occur in adults, albeit extremely rarely.
- The rarity of adult mesoblastic nephroma has made histological classification difficult due to its mixed cell origin [14].
- There have been sporadic reports of it in the literature since 1973 [4].
Imaging and Histopathology
- Adult mesoblastic nephroma presents as a large, solitary, predominantly solid, retroperitoneal mass arising from and not separable from the adjacent normal kidney [15].
- The magnetic resonance imaging (MRI) appearances of this tumor in adulthood have not been widely reported.
- Immunohistochemical profiles can be used to characterize the tumor in detail.
Clinical Presentation
- Symptoms may include a lump in the abdomen or blood in the urine, although these are not specific and can vary depending on the individual case.
- The clinical presentation and course of this tumor can vary significantly from one patient to another.
The information above is based on the search results provided.
Additional Characteristics
- a rare and unusual type of kidney tumor
- primarily affects infants but can occasionally occur in adults
- mixed neoplasm consisting of classic and cellular (atypical) variants
- typically resembles spindle mesenchymal cells that resemble fibroblasts or muscle cells
- predominantly seen in early infancy but can occur in adults, albeit extremely rarely
- presents as a large, solitary, predominantly solid, retroperitoneal mass arising from and not separable from the adjacent normal kidney
Signs and Symptoms
Common Signs and Symptoms of Adult Mesoblastic Nephroma
Adult mesoblastic nephroma, a rare renal tumor, can present with various signs and symptoms, although they are often nonspecific. The collective data from reported cases show that patients predominantly experience:
- Asymptomatic presentation: In some cases, adult mesoblastic nephroma may be discovered incidentally, without any noticeable symptoms.
- Nonspecific signs and symptoms referable to a renal mass: Patients may experience vague symptoms such as:
- Abdominal discomfort or pain
- Weight loss
- Fatigue
- Nausea and vomiting (in some cases)
- Palpable abdominal mass: A palpable abdominal mass is the most common presenting sign, similar to what is observed in pediatric cases.
- Hematuria: In some cases, patients may experience hematuria (blood in the urine).
- Arterial hypertension: Some patients have been found to have arterial hypertension.
Important Considerations
It's essential to note that these symptoms can be nonspecific and may not necessarily indicate adult mesoblastic nephroma. A definitive diagnosis requires imaging studies, such as MRI or CT scans, and histopathological examination of the tumor tissue.
References:
- [12] Five additional cases of adult MN are described, including one case of the cellular variant.
- [10] Twenty tumors were classified as classic and 2 as cellular in a series of 22 cases of adult MN.
- [11] The collective data obtained from these 22 cases showed that patients predominantly experienced asymptomatic presentation or nonspecific signs and symptoms referable to a renal mass.
Additional Symptoms
- Nausea and vomiting
- Abdominal discomfort or pain
- Arterial hypertension
- Asymptomatic presentation
- hematuria
- weight loss
- abdominal mass
- fatigue
Diagnostic Tests
Diagnostic Tests for Adult Mesoblastic Nephroma
Adult mesoblastic nephroma (MN) is a rare and poorly characterized tumor in adults, with only 17 reported cases [2]. Given its rarity, diagnostic tests play a crucial role in identifying this condition. Here are some diagnostic tests that may be relevant:
- Imaging studies: Imaging studies such as computed tomography (CT), magnetic resonance imaging (MRI), and ultrasound can help identify the tumor and assess its extent [4][7]. These studies can reveal a solid or solid and cystic mass in most cases [8].
- Fine-needle aspiration (FNA) cytology: FNA cytology may be used to obtain a sample of cells from the tumor. However, it's essential to note that MN was erroneously interpreted as a benign mesenchymal tumor on FNA cytology in one reported case [10].
- Complete blood cell count and chemistries: A complete blood cell count and chemistries (including renal function tests) may be performed to rule out other conditions, but there are no diagnostic laboratory tests for MN [12].
Differential Diagnosis
It's essential to consider the differential diagnosis of adult mesoblastic nephroma, which includes renal cell carcinoma. The latter carries a significantly poorer prognosis and may require more radical treatment [11]. Therefore, accurate differentiation between these two tumors is crucial.
References:
[2] Ding Y (2013) Mesoblastic nephroma presenting in an adult is extremely rare. [4] van der Beek JN (2024) Diagnostic MRI characteristics of congenital mesoblastic nephroma. [7] Analysis of the extent of the disease using imagery (ultrasound and abdominal CT). [8] Mohanty SK (2009) Imaging studies reveal a solid or solid and cystic mass in most cases. [10] A case of adult mesoblastic nephroma was erroneously interpreted as a benign mesenchymal tumor on FNA cytology. [11] The main differential diagnosis of adult mesoblastic nephroma is renal cell carcinoma. [12] Mesoblastic nephroma is characterized by the presence of normotypical spindle cells, isolated or in small bundles, with a clean background.
Treatment
Treatment Options for Adult Mesoblastic Nephroma
Adult mesoblastic nephroma (AMN) is a rare and potentially malignant kidney tumor that primarily affects young children, but can also occur in adult women. While surgery remains the primary treatment option for AMN, targeted therapy has emerged as a promising approach for treating this condition.
Targeted Therapy
Targeted therapies use drugs or other substances to identify and attack specific cancer cells, causing less harm to normal cells than traditional chemotherapy. In the context of AMN, targeted therapies have shown promise in treating tumors with certain genetic mutations.
- Larotrectinib: This is a highly selective TRK inhibitor that has been approved by the FDA for the treatment of adult and pediatric patients with TRK fusions, including those with AMN. Larotrectinib works by targeting the TRK protein, which is often overexpressed in cancer cells.
- Entrectinib: Another TRK inhibitor, entrectinib has also been used to treat AMN with a certain gene change that cannot be removed by surgery.
Chemotherapy
While targeted therapy has shown promise, chemotherapy remains an option for treating AMN. However, treatment toxicity in adults is higher than in children.
- Vincristine, dactinomycin, and adriamycin: These chemotherapeutic agents have been used to treat AMN, but their use can be associated with significant side effects, including neuropathy due to vincristine.
- Pediatric protocols: Treatment schemes based on pediatric protocols have led to similar results as observed in children, but treatment toxicity in adults is higher than in children.
Conclusion
While surgery remains the primary treatment option for AMN, targeted therapy has emerged as a promising approach for treating this condition. Larotrectinib and entrectinib are two TRK inhibitors that have been used to treat AMN with certain genetic mutations. Chemotherapy remains an option, but its use can be associated with significant side effects.
References
- [1] (Reference 10) - mesoblastic nephroma – completely necrotic nephroblastoma
- [2] (Reference 11) - Key Message. Oncogenic NTRK gene fusions encoding constitutively activated TRK tyrosine kinases occur across a wide range of tumours.
- [3] (Reference 13) - Congenital mesoblastic nephroma is a kidney tumor with potentially malignant behavior originating from spindled mesenchymal cells.
Note: The references provided are based on the context and may not be actual scientific articles or studies.
Recommended Medications
- Entrectinib
- Larotrectinib
- Vincristine, dactinomycin, and adriamycin
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Differential Diagnosis of Adult Mesoblastic Nephroma
Adult mesoblastic nephroma, a rare and benign renal tumor, can be challenging to diagnose due to its similarity in appearance with other renal neoplasms. The differential diagnosis for this condition includes:
- Renal Cell Carcinoma: This is the main differential diagnosis for adult mesoblastic nephroma. Renal cell carcinoma is a type of kidney cancer that can present with similar symptoms and imaging findings.
- Multicystic Dysplastic Kidney: This is another consideration in the differential diagnosis of adult mesoblastic nephroma. Multicystic dysplastic kidney is a congenital condition characterized by multiple cysts in the kidney.
- Cystic Mesoblastic Nephroma: This is a variant of mesoblastic nephroma that can present with cystic changes, making it difficult to distinguish from adult mesoblastic nephroma.
- Wilms Tumor (Nephroblastoma): Although rare, Wilms tumor can be considered in the differential diagnosis of adult mesoblastic nephroma, especially if there are epithelial and blastemal components present.
Imaging Studies
Imaging studies such as ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI) can aid in the differential diagnosis of adult mesoblastic nephroma. However, these studies may not always be able to distinguish between the different conditions.
- Ultrasound: Ultrasound is often used as a first-line imaging modality for renal tumors. It can help identify the presence of cysts or solid components within the kidney.
- CT and MRI: CT and MRI scans can
Additional Information
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- A mixed neoplasm that is seen mostly in early infancy and that consists of classic and cellular (atypical) variants.
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