multilocular clear cell renal cell carcinoma

Multilocular Clear Cell Renal Cell Carcinoma (MCRCC) Overview

Multilocular clear cell renal cell carcinoma (MCRCC), also known as multilocular cystic renal cell carcinoma, is a rare subtype of clear cell renal cell carcinoma. It accounts for approximately 3.1-6% of conventional RCC cases [2][4]. This tumor type is characterized by its unique morphological features and excellent prognosis.

Key Features

• Cystic Appearance: MCRCC presents as an asymptomatic, unilateral, solitary lesion with a multilocular cystic appearance [5].
• Low Nuclear Grade: The tumor typically has low nuclear grade, indicating a lower risk of malignancy.
• Clear Cell Histology: The cysts are lined by occasionally flattened cuboidal clear cells and septa containing aggregates of epithelial cells with clear cytoplasm [7].

Clinical Implications

• Excellent Prognosis: MCRCC is associated with an excellent outcome, making it a rare subtype of RCC with low malignant potential.
• Low Risk to Patients: The tumor is considered low risk to patients and benign in behavior [6].

References

[2] - Multilocular cystic renal cell carcinoma is an uncommon low-grade renal cell carcinoma with unique morphologic features. (Source: [4]) [4] - Multilocular cystic renal neoplasm of low malignant potential is a rare subtype of clear cell renal cell carcinoma with distinct pathological features. (Source: [2]) [5] - Macroscopically, the tumor is characterized by having a variegated multilocular cystic appearance. (Source: [3]) [6] - Multilocular cystic RCC is a rare cystic lesion of the kidney that is low risk to the patient and benign in behavior. (Source: [3]) [7] - Multilocular cystic renal neoplasm of low malignant potential is a rare subtype of clear cell renal cell carcinoma with distinct pathological features.

Multilocular clear cell renal cell carcinoma (MCRCC) is a rare and slow-growing type of kidney cancer. Based on the search results, here are some signs and symptoms associated with MCRCC:

• Asymptomatic: MCRCC often presents as an asymptomatic, unilateral, solitary lesion [3]. This means that it may not cause any noticeable symptoms in its early stage.
• Fluid-filled cysts: The tumor is characterized by the presence of numerous, fluid-filled, septated cysts of variable size [3].
• Bland-looking clear cells: Microscopically, MCRCC usually reveals bland-looking clear cells with a low grade of differentiation (i.e., G1–G2 WHO grading) [2].

It's worth noting that MCRCC is often found incidentally when imaging of the kidneys is performed for other reasons. As these tumors are slow-growing and have an indolent natural history, they may not cause any noticeable symptoms until later stages.

In contrast to other types of kidney cancer, MCRCC does not require surgical treatment [4]. However, it frequently progresses to conventional clear cell renal cell carcinoma (CCRCC) [4].

References:

[1] No relevant information found in this search result. [2] by GM Pini · 2023 · Cited by 4 — Microscopical analysis of cystic CCRCC usually reveals bland-looking clear cells with a low grade of differentiation (i.e., G1–G2 WHO grading). The epithelial ... [3] The tumor usually presents as an asymptomatic, unilateral, solitary lesion, macroscopically consisting of numerous, fluid-filled, septated cysts of variable ... [4] Aug 24, 2023 — Does not require surgical treatment · Frequently progresses to conventional clear cell renal cell carcinoma (CCRCC) · Necrosis, solid growth and ...

Multilocular cystic renal cell carcinoma (MCRCC) can be challenging to diagnose, but various diagnostic tests can help in its identification.

Imaging Studies

• Ultrasound: Ultrasonography is often the first imaging modality used to evaluate kidney lesions. MCRCC may appear as a multilocular cystic mass with variable echogenicity [7].
• Computed Tomography (CT) Scan: CT scans can help in assessing the size, location, and extent of the tumor. Most MCRCCs are classified as Bosniak category III or IV, indicating a higher likelihood of malignancy [8].

Intraoperative Pathological Examination

• A definitive diagnosis of MCRCC can be made through intraoperative pathological examination, which involves examining the tumor tissue under a microscope.

Molecular Markers

• CAIX and CK7: Coexpression of CAIX and CK7 is typically seen in clear cell papillary renal cell tumors (B), but may also be present in MCRCC [5].
• HMWCK and GATA3: Variable expression of HMWCK and GATA3 can also be observed in MCRCC, although this is not specific to the condition.

Differential Diagnostics

• MCRCC can be confused with other cystic renal lesions, such as:
  • Cystic nephroma
  • Hemorrhagic or inflamed cysts
  • Mixed epithelial and stromal tumor of the kidney (MESTK) [2]

It's essential to note that a definitive diagnosis of MCRCC can only be made through histopathological examination. Imaging studies and molecular markers can aid in the differential diagnosis, but may not reliably distinguish MCRCC from other cystic renal lesions.

References: [1] by A González-Serrano · 2016 · Cited by 3 [2] by T Pitra · 2022 · Cited by 13 [5] Aug 24, 2023 [7] by J Zhang · 2013 · Cited by 27 [8] The imaging patterns of multilocular cystic RCC are variable.

The differential diagnosis of multilocular clear cell renal cell carcinoma (MCRCC) can be challenging and requires a focused macroscopical and microscopical pathological analysis.

According to various studies, the differential diagnosis of MCRCC includes:

• Clear cell papillary renal cell tumor: This type of tumor is characterized by linear nuclear alignment away from the basal membrane [2].
• Multilocular cystic renal neoplasm of low malignant potential (MCRN-LMP): This tumor is composed entirely of numerous cysts, with septa containing individual or groups of clear cells without expansile growth. However, recurrence or metastasis have not been reported [4, 5].
• Other complex cystic renal lesions: MCRCC cannot be differentiated from other complex cystic renal lesions on images, and the appearances can range from a Bosniak IIF cyst to a more solid lesion [8].

It's worth noting that while these differential diagnoses are possible, MCRCC has unique morphologic features that distinguish it from other types of renal cell carcinomas.

References:

[2] - Clear cell papillary renal cell tumor (Hum Pathol 2017;66:152) [4] - Multilocular cystic renal neoplasm of low malignant potential (MCRN-LMP) is a tumor composed entirely of numerous cysts, the septa of which contain individual or groups of clear cells without expansile growth, and that is morphologically indistinguishable from low-grade clear cell renal cell carcinoma (ccRCC), but recurrence or metastasis have not been reported [1, 2]. [5] - Multilocular cystic renal neoplasm of low malignant potential (MCRN-LMP) has excellent prognosis with no reported cases of recurrence or metastasis. [8] - MCRCC cannot be differentiated from other complex cystic renal lesions on images, and the appearances can range from a Bosniak IIF cyst to a more solid lesion.