MUCINOUS
TUBULAR AND SPINDLE CELL CARCINOMA OF THE KIDNEY:
�A CASE REPORT OF A RARE TUMOR
�Antonius
Galih Pranesdha Putra1, Taufik Indrawan2
Rumah
Sakit Umum Daerah Dr. Mohamad Soewandhie, Jawa Timur, Indonesia
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ABSTRACT
The objective of this research is to enhance the understanding and
recognition of Mucinous Tubular and Spindle Cell Carcinoma of the Kidney
(MTSCC-K), a rare renal tumor occurring in less than 1% of all renal tumors.
The focus is on improving clinical diagnosis, understanding the tumor's
characteristics, and exploring the prognosis and implications for patients. The
method used in this research is a case study. The results of this research
indicate that Clinical Presentation: MTSCC-K predominantly occurs in women and
often presents with flank pain, similar to other renal tumors. However, it may
lack other typical symptoms such as significant weight loss or urinary problems.
Imaging Characteristics: CT scans may reveal solid masses with patchy
calcification, often protruding outside the renal contour. These specific
imaging features can aid in the differential diagnosis of MTSCC-K Histopathological
Features: MTSCC-K is characterized by small, elongated cords or tubules
arranged densely, with interspersed myxoid stroma. The presence of slender
tubular spindle cell-like structures is a distinctive feature Prognosis:
Patients with MTSCC-K, especially when diagnosed early and treated with radical
nephrectomy, have a comparatively good prognosis compared to other renal cell
carcinomas. The lack of identified metastases in the presented case also
supports a positive prognosis. Implications of this research include: Increased
awareness among doctors and radiologists about the unique clinical and imaging
features of MTSCC-K can lead to faster and more accurate diagnoses. Continued
research into the genetic and molecular aspects of MTSCC-K can pave the way for
personalized therapies, enhancing the overall management of this rare kidney
tumor.
Keywords: mucinous
tubular, spindle cell carcinoma, renal, computed tomography, magnetic resonance
imaging, radical nephrectomy.
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Corresponding Author: Antonius Galih Pranesdha Putra
E-mail: [email protected]
INTRODUCTION
Mucinous tubular and spindle cell carcinoma of the
kidney (MTSCC-K) is a rare tumour newly added to the World Health Organization
(WHO) classification in 2004 (Bajpai et al., 2022). It is believed to be a low-grade malignant tumour
with a good prognosis (Gong et al., 2020). The origin of MTSCC-K is still unclear and
hypothesized to be either the loop of Henle or the collecting duct (Gong et al., 2020). MTSCC-K has a wide range of age distribution (from
13 to 82 years old) and a female predilection, sometimes found accidentally and
asymptomatic (Sun et al., 2014). The most common complaints are gross hematuria,
flank pain, and lumbar mass, depending on the size of the tumour (Alves et al., 2021). Herein, we reported coincidentally finding a patient
with acute flank pain without any urinary problems and found a tumour from an
imaging examination.
Case
����������� A 67-year-old woman presented with
right flank pain without gross hematuria and fever for a month. The Renal
function test results were expected without a history of stone disease. The abdominal computed tomography
(CT) imaging examination revealed a 7,2x7x 5,4 cm well-circumscribed solid mass
with patchy calcification in the central area and protruding outside the renal
contour in the medial-lower pole of the right kidney. The contrast injection
slightly enhanced the lesion at the arterial and venous phases. The tumour part
in the renal was less enhanced than normal renal parenchyma (Figure 1).




Figure 1. (A) Plain CT images
show a solid mass, well-defined margins with patchy calcification in the
central area and protruding outside the renal contour in the medial-lower pole
of the right kidney. (B-E) Contrast-enhanced CT images show that the tumour
part located in the renal was less enhanced compared with normal renal
parenchyma.
The imaging examination by
abdominal MRI revealed a well-circumscribed solid mass with patchy
calcification in the mid to lower pole of the right kidney. On Diffusion
Weighted Imaging (DWI), there is an increase in signal intensity. The
T2-weighted MR image demonstrates the T2-hypointensity of the mass (Figure 2).



Figure 2. It revealed a solid
mass with well-defined margins and calcification in the right kidney's mid to
lower pole (A). On Diffusion Weighted Imaging (DWI), there is an increase in
signal intensity. (B, C) Axial & coronal T2-weighted MR image demonstrates
T2-hypointensity of the mass.
No metastases were identified to
the retroperitoneal lymph node, abdominal organs or lungs. The patient
underwent radical nephrectomy resection of the right renal. No postoperative
therapy was given to the patient. The patient was planned for control 6 months
later.

Figure 3. The gross specimen of
the nephrectomy showed a renal mass
Macroscopy: The suitable radical nephrectomy specimen was 12 x 10,5 x 7 cm
and weighed 524 g. The cut section revealed a tumour occupying the lower pole
of the right renal. It was a greyish-white, partly brownish in appearance,
measuring 7 x 6 x 6 cm (Figure 3)
Microscopy: The dominant tumour growth is arranged to form tubules that
anastomose with each other between amorphous materials consisting of
proliferation of anaplastic epithelial cells with rounded oval nuclei, mild
pleomorphic, coarse chromatin, and ample cytoplasm. Areas of necrosis and
bleeding were identified. The tumour grows invasively into the renal parenchyma
without lymphangio or perineural invasion (Figure
4).
Figure 4. Microscopical
photographs of the tumour. Microscopical photographs of the tumour stained by
hematoxylin and eosin staining. (a) 200� magnification of haematoxylin and
eosin stained section of renal tumour. (b) 400x magnification of haematoxylin
and eosin stained section of renal tumour.
The urgency of this research lies in the rarity of
Mucinous Tubular and Spindle Cell Carcinoma of the Kidney (MTSCC-K), occurring
in less than 1% of all renal tumors. Given its low incidence and the need for
specific diagnostic and treatment strategies, urgent research is essential to
improve the understanding of this condition. Early diagnosis and targeted
treatment can significantly impact patient outcomes, making timely research
crucial. This research is novel due to the scarcity of data on MTSCC-K. The
unique combination of clinical, imaging, and histopathological features
associated with this tumor presents an opportunity to contribute new knowledge
to the field of oncology. The detailed examination of its characteristics,
prognosis, and treatment outcomes adds to the limited existing literature,
making this research highly innovative and valuable.
The aim of this research is to develop accurate
diagnostic criteria based on clinical presentation, imaging characteristics,
and histopathological findings, enabling the precise and early identification
of MTSCC-K. Additionally, the study aims to assess the psychosocial impact of
MTSCC-K on patients and develop supportive strategies that enhance their
quality of life during and after treatment. Therefore, the benefits of this
research include early detection and treatment, reducing diagnostic errors, and
enhancing medical knowledge. This study contributes to a broader understanding
of rare kidney tumors, paving the way for further research and potentially
influencing the direction of oncological research in the future.
METHOD
The
research method used in this study is a case study. The data collection
techniques employed in this research include patient medical records, imaging
data, and pathological specimens.
RESULTS AND DISCUSSION
MTSCC is a rare variant called low-grade
collecting duct carcinoma or grouped under renal cell carcinoma (RCC) (Ramya et al.,
2022). This tumor occurs less than 1% of all
kidney tumors. It mainly affects adults, typically occurring around the age of
58, although it can occur in individuals as young as 13 or as old as 81.
Mucinous tubular and spindle cell carcinoma is more common in females, with a
ratio of 3 females to 1 male (Ramya et al.,
2022).
Most patients usually present with
asymptomatic masses and are often found incidentally by abdominal imaging for
unrelated reasons (Nathany &
Monappa, 2020). Some cases might manifest with symptoms
like hematuria, pain in the side, and a noticeable lump in the abdomen (Nathany &
Monappa, 2020). Typically, the tumor is located in the
outer layer of the kidney (renal cortex), and exceptionally rarely, it might
develop in the inner region (renal medulla) as well (Moch et al.,
2016).
This is in line with our patient, who had no
other symptoms besides flank pain, and a tumour was accidentally found during a
lumbosacral MRI. Therefore, in most cases found with no apparent symptoms,
diagnosis of MTSCC-K mainly depends on pathological analysis (Wu et al.,
2013). In our histological findings, dominant
tumours are arranged from tubules that anastomose with each other between
amorphous materials, supporting a picture of MTSCC (Ferro, 2013).
Overall, MTSCC-K shows a lower rate of
malignancy and a better prognosis than other RCC types (Ozturk, 2015). Currently, surgical resection is recognized
as the mainstay of treatment. Since these tumors are typically low in grade,
they tend to respond well to either partial or radical nephrectomy. In general,
radical nephrectomy is the best treatment and no additional treatment after
surgery (Nathany &
Monappa, 2020). Some cases reported in the literature have
shown recurrence, regional lymph node metastases, and distant metastases (Kenney
et al., 2015). These occurrences are associated
with lesions displaying high nuclear grade, sarcomatoid transformation, and
other abnormal histomorphologic characteristics ((Thway
et al., 2012). This is in line with our patients who did not
find any metastases, and we performed nephrectomy to minimize the chance of
recurrence (Mouracade et
al., 2017).
Extremely rare for MTSCC-K to have metastases
to lymph nodes and other organs at the time of diagnosis because of its low
pathological stage (Ursani et
al., 2011). However, MTSCC-K, a type of renal cancer,
requires close follow-up after surgery (Kato et al.,
2009). Follow-up and clinical evaluation are
needed 6 months after surgery, with imaging evaluation one year subsequently if
there is no complaint. Therefore, it is essential to maintain careful
monitoring even after a thorough excision, even though the tumor typically
follows a benign clinical course.
CONCLUSION
MTSCC-K is a malignant tumour classified
as low-grade epithelial, and some cases do not show significant complaints, as
in the case above. Although classified as low grade, MTSCC can recur and
metastasize. Surgical and clean margins are considered a standard localized
renal cell carcinoma treatment. So, high accuracy is expected in making a
diagnosis so that patients get the proper treatment. It is also important to
follow-up and clinical evaluation 6 months after surgery.
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