Mucinous tubular and spindle cell carcinoma and post-neuroblastoma carcinoma: newly recognised entities in the renal cell carcinoma family

Pathology ◽  
2003 ◽  
Vol 35 (6) ◽  
pp. 499-504 ◽  
Author(s):  
John N. Eble
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Spindle Cell ◽  
Spindle Cell Carcinoma

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 ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Poor Prognosis ◽  
Spindle Cell ◽  
Left Kidney ◽  
Spindle Cell Carcinoma ◽  
Low Grade ◽  
High Grade ◽  
Tomography Scan

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.

scholarly journals Combined Papillary Renal Cell Carcinoma with Neuroendocrine Differentiation and Mucinous Tubular and Spindle Cell Carcinoma

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Gang Wang ◽  
Ren Yuan ◽  
Tracy Tucker ◽  
Allan B. Gates ◽  
Christopher D. Bellamy ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Spindle Cell ◽  
Neuroendocrine Differentiation ◽  
Papillary Renal Cell Carcinoma ◽  
Spindle Cell Carcinoma ◽  
Unique Case ◽  
Primary Neoplasm

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.

scholarly journals ALK Rearrangement-Associated Renal Cell Carcinoma Morphologically Mimicking Mucinous Tubular And Spindle Cell Carcinoma: A Case Report

2021 ◽  
Author(s):  
Keita Kai ◽  
Shohei Tobu ◽  
Shinichi Kido ◽  
Shuji Mikami ◽  
Kengo Takeuchi ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Case Report ◽  
Cell Carcinoma ◽  
Tumor Cells ◽  
Renal Cell ◽  
Spindle Cell ◽  
Spindle Cell Carcinoma ◽  
Renal Tumors ◽  
Alk Rearrangement ◽  
Anaplastic Lymphoma

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.

Comparative study of CT and MRI appearances in mucinous tubular and spindle cell carcinoma and papillary renal cell carcinoma

2021 ◽  
pp. 20210548
Author(s):  
Dajun lu ◽  
Weibiao Yuan ◽  
Qingqiang Zhu ◽  
Jing Ye ◽  
Wenrong Zhu ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Spindle Cell ◽  
Papillary Renal Cell Carcinoma ◽  
Spindle Cell Carcinoma ◽  
Ct Imaging ◽  
Imaging Features ◽  
Unenhanced Ct ◽  
Ct And Mri

Objective: To explore the feasibility of CT and MRI in differentiating mucinous tubular and spindle cell carcinoma (MTSCC) and papillary renal cell carcinoma (PRCC). Methods: 23 patients with MTSCC and 38 patients with PRCC were studied retrospectively. CT and MRI were undertaken to investigate differences in tumour characteristics. Results: 23 patients with MTSCC and 38 patients with PRCC (included 15 cases Type 1,and 23 cases Type 2), tumours (mean diameter 3.7 ± 1.6 cm vs 4.6 ± 1.7 cm, p < 0.05), cystic components (5 vs 32, p < 0.01), calcifications (3 vs 11, p > 0.05), haemorrhage (1 vs 22, p < 0.01), tumour boundaries (1 vs 37, p < 0.01), and homogeneous enhancement (20 vs 11, p < 0.01). The density of MTSCC was lower than that of PRCC, normal renal cortex (p < 0.05), except for the medulla(p > 0.05). MTSCC and PRCC tumour enhancement were lower than that for normal cortex and medulla during all enhanced phases (p < 0.05). Enhancement was higher with PRCC than with MTSCC tumours during all phases (p < 0.05). On MRI, nine cases of MTSCC and 19 cases of PRCC, tumour showed homogeneous (9 vs 3, p < 0.01), heterogeneous (0 vs 16, p < 0.01), hyperintense on T1WI (0 vs 15, p < 0.01), slightly hyperintense on T2WI (9 vs 1, p < 0.01), hypointense on T2WI (0 vs 15, p < 0.05) , relatively high signal intensity was seen on DWI (9 vs 15, p > 0.05), respectively. Conclusion: CT imaging features of MTSCC include isodense or hypodense mass on unenhanced CT, with unclear boundaries; however, PRCC showed mild hyperdensity, easily have cystic components. The degree enhancement of MTSCC is lower than that for PRCC. On MR, MTSCC was slightly hyperintense on T2WI, whereas PRCC was hypointense. Advances in knowledge: 1.CT imaging features of MTSCC include isodense or hypodense mass on unenhanced CT, with unclear boundaries. 2. CT imaging features of PRCC include mild hyperdensity on unenhanced CT, easily have cystic components. 3. On enhanced CT, the degree enhancement of MTSCC is lower than that for PRCC. On MR, MTSCC was slightly hyperintense on T2WI whereas PRCC was heterogeneously hypointense on T2WI.

scholarly journals Difficulty in differential diagnosis for renal cancer with microscopic papillary architecture: overlapped pathological features among papillary renal cell carcinoma (RCC), mutinous tubular and spindle cell carcinoma, and unclassified RCC. Lessons from a Japanese multicenter study

2020 ◽  
Vol 50 (11) ◽  
pp. 1313-1320 ◽  
Author(s):  
Keiichi Ito ◽  
Shuji Mikami ◽  
Naoto Kuroda ◽  
Yoji Nagashima ◽  
Katsunori Tatsugami ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Multicenter Study ◽  
Renal Cell ◽  
Spindle Cell ◽  
Papillary Renal Cell Carcinoma ◽  
Spindle Cell Carcinoma ◽  
Cancer Center ◽  
Low Grade ◽  
Poor Risk

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 &gt;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 &gt;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.

scholarly journals Mucin-Poor Mucinous Tubular and Spindle Cell Carcinoma of the Kidney Presented with Multiple Metastases Two Years after Nephrectomy: An Atypical Behaviour of a Rare, Indolent Tumour

2017 ◽  
Vol 2017 ◽  
pp. 1-4 ◽  
Author(s):  
I. Sokolakis ◽  
C. Kalogirou ◽  
L. Frey ◽  
M. Oelschläger ◽  
M. Krebs ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Spindle Cell ◽  
Left Kidney ◽  
Papillary Renal Cell Carcinoma ◽  
Spindle Cell Carcinoma ◽  
Rare Type ◽  
End Stage ◽  
Sarcomatoid Differentiation

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.

scholarly journals Combination of Nivolumab Plus Ipilimumab for Mucinous Tubular and Spindle Cell Carcinoma of the Kidney with Bone Metastases: The First Case Report

2021 ◽  
Author(s):  
Nobuki Furubayashi ◽  
Takahito Negishi ◽  
Kenichi Taguchi ◽  
Akihiro Miura ◽  
Yoshiaki Sato ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
T Cells ◽  
Combination Therapy ◽  
Bone Metastases ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Spindle Cell ◽  
Renal Tumor ◽  
Spindle Cell Carcinoma ◽  
Tubular Components

Abstract Background: Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare subtype of renal cell carcinoma (RCC) and generally considered a low-grade renal epithelial neoplasm composed of tubules, spindle cells, and extracellular mucin. MTSCC with distant metastases has been reported, with some cases showing a poor prognosis. While only a few case reports regarding the treatment of metastatic MTSCC have been reported, targeted agents and monotherapy of immune checkpoint inhibitors have shown some efficacy. To our knowledge, this is the first reported case of metastatic MTSCC of the kidney treated with combination therapy of nivolumab plus ipilimumab.Case presentation: A 26-year-old man consulted our hospital after a 72-mm tumor was detected at the upper pole of the left kidney with multiple osteolytic bone metastases by computed tomography (CT). A CT-guided biopsy of the renal tumor and bone metastases resulted in a diagnosis of MTSCC of the kidney with bone metastases (cT2aN0M1) and an ‘intermediate risk’ according to the International Metastatic Renal Cell Carcinoma Database Consortium criteria. He therefore received combination therapy of nivolumab plus ipilimumab therapy. After 4 cycles of combination nivolumab plus ipilimumab and 9 cycles of nivolumab monotherapy, he underwent cytoreductive nephrectomy because the tumor had shrunk, and sclerotic changes in the bone metastases were noted. In the excised specimen, the programmed cell death ligand 1 expression was higher in the spindle components than in the tubular components, but CD4- and CD8-positve T cells showed greater infiltration in the tubular components than in the spindle components. Furthermore, CD-4- and CD-8-positive T cells in both components of the resected specimen showed greater infiltration than they had at the pretreatment biopsy of the renal tumor and bone metastases.Conclusions: Combination immunotherapy of nivolumab and ipilimumab may be an effective treatment option for metastatic MTSCC of the kidney.

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