# Mucinous tubular and spindle cell carcinoma of the kidney

Source: https://onco.cc/cancers/mucinous-tubular-spindle-cell-carcinoma/  
OnCo record `mucinous-tubular-spindle-cell-carcinoma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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).

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.

## Fields

- Kind: Cancer
- Last checked: 2026-09-24
- Also known as: MTSCC; Mucinous tubular and spindle cell renal cell carcinoma; Low-grade collecting duct carcinoma (historical)
- Tags: subtype-page; wave4; kidney; rare
- 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.
- 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)

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/mucinous-tubular-spindle-cell-carcinoma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/mucinous-tubular-spindle-cell-carcinoma/#overview
- What it is (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/mucinous-tubular-spindle-cell-carcinoma/#what-it-is
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/mucinous-tubular-spindle-cell-carcinoma/#finding-it [4 biomarkers]
- Treating it (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/mucinous-tubular-spindle-cell-carcinoma/#treating-it [1 setting]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/mucinous-tubular-spindle-cell-carcinoma/#evidence
- The science (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/mucinous-tubular-spindle-cell-carcinoma/#science
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/mucinous-tubular-spindle-cell-carcinoma/where-you-are/
- Living with it (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/mucinous-tubular-spindle-cell-carcinoma/#living-with-it [6 questions]
- What is coming (own page): Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/mucinous-tubular-spindle-cell-carcinoma/coming/
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/mucinous-tubular-spindle-cell-carcinoma/data/ [1 connected records]

## Standard of care

- All stages: Partial or radical nephrectomy; advanced disease on the renal cell carcinoma page without a dedicated standard. ([Renal cell carcinoma](https://onco.cc/cancers/rcc/), [Nephrectomy](https://onco.cc/terms/nephrectomy/))

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Renal_cell_carcinoma
- Moch 2022, European Urology: the 2022 WHO classification of urinary and male genital tumours, part A (renal, penile, testicular): https://doi.org/10.1016/j.eururo.2022.06.016
- Cancer Discovery 2016: biallelic Hippo pathway alteration in mucinous tubular and spindle cell carcinoma: https://doi.org/10.1158/2159-8290.cd-16-0267
- Am J Surg Pathol 2018: VSTM2A overexpression as a marker of mucinous tubular and spindle cell carcinoma: https://doi.org/10.1097/pas.0000000000001150

## Connected records

- cancers: [Clear cell papillary renal cell tumour](https://onco.cc/cancers/clear-cell-papillary-renal-cell-tumour/), [Eosinophilic solid and cystic renal cell carcinoma](https://onco.cc/cancers/eosinophilic-solid-cystic-renal-cell-carcinoma/), [Papillary renal cell carcinoma](https://onco.cc/cancers/papillary-rcc/), [Renal cell carcinoma](https://onco.cc/cancers/rcc/)
- terms: [Nephrectomy](https://onco.cc/terms/nephrectomy/)

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JSON: https://onco.cc/api/v1/entities/mucinous-tubular-spindle-cell-carcinoma.json