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Mucinous tubular and spindle cell carcinoma is a rare, usually slow-growing kidney cancer, commoner in women, whose cells form small tubes and spindles in a mucus-rich background. It is driven by loss of the Hippo growth-control pathway rather than the faults of common kidney cancer, and surgery cures most cases.
Mucinous tubular and spindle cell carcinoma is a renal cell carcinoma type recognised since 2004 and kept in the 2022 WHO classification, with tightly packed tubules merging into spindle cells in a mucinous stroma, a female predominance and generally indolent behaviour (Moch 2022). Whole-exome and transcriptome sequencing of 22 tumours found biallelic loss or alteration of Hippo pathway tumour suppressors in 85 percent, PTPN14 (31 percent) and NF2 (22 percent) most often, with SAV1 and HIPK2 in a mutually exclusive pattern, recurrent chromosomal losses, and increased nuclear YAP1 in 90 percent (Cancer Discovery 2016). VSTM2A and IRX5 were then identified as lineage-specific markers, with all 33 tumours showing moderate to high VSTM2A expression by RNA in situ hybridisation against low or absent expression in papillary, clear cell and chromophobe carcinomas (Am J Surg Pathol 2018).
How it differs from its parent: it lacks VHL loss and the papillary trisomies, its main differential is papillary renal cell carcinoma (from which VSTM2A separates it), and it is indolent in the typical form while high-grade and sarcomatoid variants can metastasise.
How common: no registry share was found in the sources read.
Treatment: partial or radical nephrectomy, which is curative for typical tumours; advanced disease is very rare and treated on the renal cell carcinoma page without a dedicated standard.
Rare; the largest molecular series holds 22 tumours (Cancer Discovery 2016) and the marker study 33 (Am J Surg Pathol 2018). No registry share was found in the sources read.
Renal cell carcinoma comes from the kidney's filtering cortex, urothelial cancer from the lining of the collecting system and bladder, and the adrenal on top hosts cortical and medullary (neuroblastoma) tumours.
Same organ: Collecting duct carcinoma of the kidney, Renal medullary carcinoma (SMARCB1-deficient), TFE3-rearranged (translocation) renal cell carcinoma, Fumarate hydratase-deficient renal cell carcinoma (HLRCC-associated), Succinate dehydrogenase-deficient renal cell carcinoma, Eosinophilic solid and cystic renal cell carcinoma, Clear cell papillary renal cell tumour, Urothelial carcinoma of the urethra, Squamous cell carcinoma of the urethra, Adenocarcinoma of the urethra (including clear cell adenocarcinoma), Melanoma of the urethra, Non-muscle-invasive bladder cancer, Muscle-invasive and advanced bladder cancer, Bladder & urothelial cancer, Clear cell renal cell carcinoma, Papillary renal cell carcinoma, Chromophobe renal cell carcinoma, Renal cell carcinoma, Wilms tumour (nephroblastoma), Neuroblastoma (paediatric), Low-risk neuroblastoma (INRG very low and low risk, including stage MS), Intermediate-risk neuroblastoma, High-risk neuroblastoma, Adrenocortical carcinoma, Pheochromocytoma and paraganglioma (PPGL), Urethral cancer, Penile cancer, Localised penile cancer (organ-confined, node-negative), Node-positive and metastatic penile cancer, Localised adrenocortical carcinoma (ENSAT stage I to III, resectable), Advanced and metastatic adrenocortical carcinoma (ENSAT stage IV or unresectable), Hereditary pheochromocytoma and paraganglioma (SDHx, VHL, RET, NF1, MAX and TMEM127), Metastatic pheochromocytoma and paraganglioma
No subtypes recorded beyond the ones named in the family strip above.
Partial or radical nephrectomy; advanced disease on the renal cell carcinoma page without a dedicated standard.
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Query for this cancer: (TITLE:"Mucinous tubular and spindle cell carcinoma of the kidney" OR ABSTRACT:"Mucinous tubular and spindle cell carcinoma of the kidney" OR TITLE:"MTSCC" OR ABSTRACT:"MTSCC" OR TITLE:"Mucinous tubular and spindle cell renal cell carcinoma" OR ABSTRACT:"Mucinous tubular and spindle cell renal cell carcinoma" OR TITLE:"Low-grade collecting duct carcinoma historical" OR ABSTRACT:"Low-grade collecting duct carcinoma historical") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Mucinous tubular and spindle cell carcinoma of the kidney, not a curated reading list.
No targets or pathways are linked to this cancer yet. Browse the gene hub →
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