{"entity":{"id":"mucinous-tubular-spindle-cell-carcinoma","kind":"cancer","name":"Mucinous tubular and spindle cell carcinoma of the kidney","aka":["MTSCC","Mucinous tubular and spindle cell renal cell carcinoma","Low-grade collecting duct carcinoma (historical)"],"tldr":"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.","summary":"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).\n\nHow 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.\n\nHow common: no registry share was found in the sources read.\n\nTreatment: 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.","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Renal_cell_carcinoma","links":[{"label":"Moch 2022, European Urology: the 2022 WHO classification of urinary and male genital tumours, part A (renal, penile, testicular)","url":"https://doi.org/10.1016/j.eururo.2022.06.016"},{"label":"Cancer Discovery 2016: biallelic Hippo pathway alteration in mucinous tubular and spindle cell carcinoma","url":"https://doi.org/10.1158/2159-8290.cd-16-0267"},{"label":"Am J Surg Pathol 2018: VSTM2A overexpression as a marker of mucinous tubular and spindle cell carcinoma","url":"https://doi.org/10.1097/pas.0000000000001150"}],"tags":["subtype-page","wave4","kidney","rare"],"related":["rcc","papillary-rcc","eosinophilic-solid-cystic-renal-cell-carcinoma","clear-cell-papillary-renal-cell-tumour"],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":["nephrectomy"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"genitourinary","burden":"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.","subtypes":[],"biomarkers":["Hippo pathway alterations (PTPN14, NF2) and nuclear YAP1","VSTM2A and IRX5 expression (research markers)","Mucinous stroma with tubules and spindle cells","WHO/ISUP grade (usually low)"],"standardOfCare":[{"setting":"All stages","approach":"Partial or radical nephrectomy; advanced disease on the renal cell carcinoma page without a dedicated standard.","refs":["rcc","nephrectomy"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"rcc"},"route":"/cancers/mucinous-tubular-spindle-cell-carcinoma/","neighbours":{"cancer":[{"id":"clear-cell-papillary-renal-cell-tumour","kind":"cancer","name":"Clear cell papillary renal cell tumour","route":"/cancers/clear-cell-papillary-renal-cell-tumour/"},{"id":"eosinophilic-solid-cystic-renal-cell-carcinoma","kind":"cancer","name":"Eosinophilic solid and cystic renal cell carcinoma","route":"/cancers/eosinophilic-solid-cystic-renal-cell-carcinoma/"},{"id":"papillary-rcc","kind":"cancer","name":"Papillary renal cell carcinoma","route":"/cancers/papillary-rcc/"},{"id":"rcc","kind":"cancer","name":"Renal cell carcinoma","route":"/cancers/rcc/"}],"term":[{"id":"nephrectomy","kind":"term","name":"Nephrectomy","route":"/terms/nephrectomy/"}]}}