scholarly journals A case of high-grade mucinous tubular and spindle cell carcinoma

2020 ◽  
Vol 2020 (2) ◽  
Author(s):  
Koujin Miura ◽  
Yasushi Adachi ◽  
Toshiaki Shirahase ◽  
Yoji Nagashima ◽  
Kazuki Suemune ◽  
...  

Abstract Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare renal cell carcinoma that initially presents as low-grade renal cell carcinoma. However, cases of MTSCC with high-grade histology and poor prognosis have been reported. Here, we report a case of MTSCC with high-grade histological features and metastasis. A 77-year-old woman consulted a hospital following frequent and painful micturition. Computed tomography scan revealed a tumor of the left kidney. First, chemotherapy was performed, with no effects. Therefore, nephrectomy was subsequently performed. Histologically, the tumor showed the features of MTSCC with sarcomatoid component. Metastasis of the tumor into the lymph node was also observed. Although adjuvant chemotherapy was performed after nephrectomy, metastasis to the lungs and bone and local recurrence was observed. The patient is still alive 2 years after nephrectomy with metastasis and recurrence of the tumor. High-grade MTSCC shows a relatively poor prognosis, specifically MTSCC with metastasis upon nephrectomy.

2020 ◽  
Vol 50 (11) ◽  
pp. 1313-1320 ◽  
Author(s):  
Keiichi Ito ◽  
Shuji Mikami ◽  
Naoto Kuroda ◽  
Yoji Nagashima ◽  
Katsunori Tatsugami ◽  
...  

Abstract Objectives In our multicenter study evaluating metastatic papillary renal cell carcinoma (PRCC), 29% of tumors diagnosed as PRCC in collaborative institutes were finally diagnosed as other RCCs under central review. In those tumors, mucinous tubular and spindle cell carcinoma (MTSCC) was the leading histology, followed by unclassified RCC (ucRCC). We focused on those patients with MTSCC or ucRCC. Methods We reviewed the processes for the pathological diagnoses of nine tumors and reviewed their clinical features. Results All of the MTSCCs and ucRCCs were positive for AMACR, which is frequently positive in PRCC. Mucin was demonstrated in 80% of the MTSCCs, and its presence is important for their diagnoses. One MTSCC was diagnosed as a mucin-poor variant. The presence of spindle cells with low-grade nuclei was suggestive of MTSCC, but the diagnosis of high-grade MTSCC was difficult. Four tumors were diagnosed as ucRCC by histological and immunohistochemical findings. Three of the four tumors were suspicious of ucRCC in the initial review due to atypical findings as PRCC. Sunitinib and interferon-α were effective for one MTSCC patient who survived for >5 years. Two MTSCC patients who were Memorial Sloan-Kettering Cancer Center poor risk had unfavorable prognoses. One patient with mucin-poor MTSCC had an indolent clinical course. Two of four ucRCC patients showed durable stable disease with targeted agents (TAs) and survived >3 years. Conclusion Some MTSCC metastases progressed very slowly and poor-risk tumors progressed rapidly. Systemic therapies including TAs showed some efficacies. Some patients who have metastatic ucRCC with microscopic papillary architecture can benefit from TAs.


2017 ◽  
Vol 2017 ◽  
pp. 1-4 ◽  
Author(s):  
I. Sokolakis ◽  
C. Kalogirou ◽  
L. Frey ◽  
M. Oelschläger ◽  
M. Krebs ◽  
...  

Background. Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare type of renal cell carcinoma, whose clinical behaviour and metastatic potential have not been fully elucidated to date. There are only a few metastatic cases in the literature, which all either featured sarcomatoid differentiation or were synchronously metastasised at diagnosis. Case Presentation. We report a case of a 49-year-old male with end-stage kidney disease on dialysis, presenting with multiple osseous metastases of a mucin-poor variant of MTSCC of the kidney, without sarcomatoid differentiation, two years after bilateral nephrectomy for papillary renal cell carcinoma (RCC) at a curable stage. After retrospectively reexamining the initial nephrectomy specimens, the tumour of the right kidney was also diagnosed as a mucin-poor variant of MTSCC, while the tumour of the left kidney was confirmed as a papillary RCC. Conclusions. It is proposed that MTSCC can be associated with end-stage renal disease and that particularly the mucin-poor variant is easily confused with papillary renal cell carcinoma, as happened in this case. Although it is considered as a relatively indolent malign entity, it can metastasise even years after successful primary surgical treatment. This implies, besides accurate diagnosis, that MTSCC patients should be monitored closely in the follow-up period.


2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S155-S155
Author(s):  
F Rajack ◽  
T J Naab

Abstract Introduction/Objective Papillary Renal Cell Carcinoma (PRCC), the 2nd most common RCC, accounts for 10-15% of cases and is usually composed of tubules and papillae with foamy histiocytes in papillary cores. Mucinous tubular and spindle cell carcinoma (MTSC) is composed of tightly packed, elongated, curvilinear tubules with smooth luminal surfaces, separated by mucinous stroma. MTSC is associated with a more favorable prognosis than PRCC. PRCC and MTSC have significant histologic and histochemical overlap including elongated tubules and stromal Alcian blue positive mucin deposits. Methods/Case Report We report a case of a 75 year old female who underwent a robotic assisted partial nephrectomy for resection of a 5.7 x 5.2 x 5.0 cm left upper pole solid renal mass. Spindle cell change with elongated tubules reminiscent of MTSC was present in several blocks; however, the luminal surface was shaggy favoring PRCC. Patchy prominent extracellular Alcian blue positive mucin deposits were also present. PRCC and MTSC both express CK7, AMACR, and EMA. However, absent expression of E-cadherin and strong CD10 expression favored PRCC. Multiple foci of solid spindle cells in a whorled pattern with clear cell change, necrosis, and high grade nuclei bordering on sarcomatoid RCC were present in other blocks. Multiple papillations and psammoma bodies also supported PRCC. A spectrum of spindle cell change was present, ranging from elongated tubules reminiscent of MTSC to whorled foci with high grade nuclei approaching sarcomatoid RCC. Results (if a Case Study enter NA) NA Conclusion Submission of multiple sections and awareness of the protean morphologic features of PRCC are essential in making the correct diagnosis.


Author(s):  
Ayan Kundu ◽  
Anway Sen ◽  
Shouvik Choudhury ◽  
Tapan Kumar Mandal ◽  
Debasish Guha ◽  
...  

Background and aims. Renal cell carcinoma (RCC) seems to be the most aggressive type of genitourinary neoplasm. Down regulation of normal beta-catenin expression contributes to development of RCC, reflecting the role of beta-catenin/Wnt signaling pathway in pathogenesis. This study aims to evaluate the significance of beta-catenin expression and its correlation with the prognostic parameters. Methods. A cross-sectional observational study was carried out in a tertiary care center on 58 RCC cases using variables like histological grade and type, tumor stage, necrosis. Formalin fixed, paraffin-embedded blocks were evaluated for beta-catenin expression by immunohistochemistry using scoring system. Data were analyzed by mean ± SD, χ2 test, Pearson’s correlation test. Results. Membranous score (MS) had a strong negative correlation with tumor stage (r = -0.407, p = 0.044) and grade (r = -0.787, p = <0.001). Mean membranous score difference between low (Stage 1 and 2) vs. high stage (Stage 3 and 4) and low (Grade 1 and 2) vs. high grade (Grade 3 and 4) was statistically significant (p < 0.001). Cytoplasmic score (CS) had positive correlation with tumor stage (r = 0.586; p = 0.002). No significant correlation was evident between cytoplasmic scores and tumor grade, however the mean cytoplasmic score difference between low grade vs. high grade was statistically significant (p < 0.001). Conclusion. Beta-catenin may play a crucial role in the pathogenesis of RCC and has a positive correlation with the biological behavior of this tumor. The important role of beta-catenin as a prognostic parameter and probably a critical evaluator of targeted chemotherapy cannot be overemphasized.


2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Gang Wang ◽  
Ren Yuan ◽  
Tracy Tucker ◽  
Allan B. Gates ◽  
Christopher D. Bellamy ◽  
...  

A unique case of combined papillary renal cell carcinoma (PRCC) and mucinous tubular and spindle cell carcinoma (MTSCC) presenting in a man aged 67 years is reported. The two separate components were distinct on morphological, immunohistochemical (IHC), and genetic grounds, while type 2 PRCC predominated. Three years after the initial diagnosis, the PRCC component metastasized to the lungs where it morphologically mimicked a pulmonary neuroendocrine tumor. Retrospectively focal neuroendocrine differentiation was demonstrated by IHC in the PRCC component of the primary neoplasm.


2016 ◽  
Vol 34 (15_suppl) ◽  
pp. 4564-4564 ◽  
Author(s):  
Heidi Coy ◽  
Michael Douek ◽  
Jonathan Young ◽  
Matthew S. Brown ◽  
Jonathan Goldin ◽  
...  

2021 ◽  
Author(s):  
Keita Kai ◽  
Shohei Tobu ◽  
Shinichi Kido ◽  
Shuji Mikami ◽  
Kengo Takeuchi ◽  
...  

Abstract Background: Anaplastic lymphoma kinase rearrangement-associated renal cell carcinoma (ALK-RCC) is a very rare tumor and no single case report of ALK-RCC that mimics mucinous tubular and spindle cell carcinoma (MTSCC) has been reported.Case presentation: A 42-year-old Japanese woman was admitted to our hospital for the treatment of a left renal tumor measuring approximately 5 × 4 cm in diameter. She underwent a laparoscopic left nephrectomy. Histologically, the tumor formed tubular or focally papillary structures with a small amount of spindle-shaped tumor cells against the background of prominent extracellular mucin. Although the tumor cells were negative for immunohistochemistry (IHC) of alpha-methylacyl-CoA racemase (AMACR) and lymph node metastasis was presented (these are atypical findings for MTSCC), we initially diagnosed the tumor as MTSCC based on its morphological characteristics with mucin deposition. However, an additional IHC analysis revealed that the tumor cells were diffusely positive for ALK-IHC. In addition, TPM3 exon 8 – ALK exon 20 fusion gene was detected by RNA sequencing. The tumor was thus correctly diagnosed as ALK rearrangement-associated renal cell carcinoma (ALK-RCC). Conclusions: Since the use of molecular targeted therapy with an ALK inhibitor for ALK-RCC is promising, the correct pathological diagnosis of ALK-RCC is quite important. We strongly recommend that ALK-IHC be routinely performed for renal tumors with negative AMACR staining that mimic MTSCC.


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