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Mucinous Tubular and Spindle Cell Carcinoma of the Kidney

Definition

  • Low grade neoplasm of the kidney composed of bland tubules set in a mucinous stroma

Alternate/Historical Names

  • Some were reported as spindle cell sarcomatoid carcinoma
  • Some were reported as low grade collecting duct carcinoma
  • Some were reported as papillary carcinoma (some overlap may exist)

Diagnostic Criteria

  • Composed of elongate tubules, variably packed
    • Curvilinear, branching
    • Range from loose, freely anastomosing spaces to densely packed side by side
    • Generally locally uniform in size, although size may vary from area to area
    • PASd highlights intact basement membrane around most tubules
    • Focal papillary architecture has been reported
  • Spindle cell population merges with and may represent densely packed collapsed tubules
    • Spindle cells resemble the tubular lining cells
    • May vary from predominant to absent
  • Mucinous stroma separates the tubules
    • Varies from abundant to sparse and focally absent
    • Acid mucin stains positive
    • Frequently vacuolated stroma
  • Epithelial and spindle cells are cytologically bland
    • Uniform small oval nuclei
    • Scant pale cytoplasm
    • Focal atypia has been reported in rare cases
  • Occasional findings
    • Collections of foamy macrophages
    • Lymphocytic infiltrate
    • Psammoma bodies
    • Solid foci without detectable stroma
    • Clear cell foci
  • Sarcomatoid change may be seen in rare cases
    • Epithelioid or spindle cells with high grade nuclear atypia
    • May behave aggressively
  • MTSCC may show morphologic and immunophenotypic overlap with papillary renal cell carcinoma (Paner 2006)
    • Features that may be shared
      • Papillary growth
      • Mucin production
      • Foam cells
      • AMACR expression
    • We diagnose borderline cases as papillary carcinoma
      • Urologists are much more familiar with that diagnosis
      • That diagnosis will probably elicit more extensive followup

    John P Higgins MD
    Robert V Rouse MD
    Department of Pathology
    Stanford University School of Medicine
    Stanford CA 94305-5342

    Original posting:: January 24, 2011

Supplemental studies

Immunohistology

Low MW keratin pos
CK7 pos
AMACR/racemase/P504S pos
Synaptophysin, chromogranin pos*
Ulex, vimentin variable
CD10

0-50%

High MW keratin neg
CK20 neg
CD117/KIT neg
*Limited numbers tested

Differential Diagnosis

Mucinous Tubular and Spindle Cell Carcinoma of the Kidney Collecting Duct Carcinoma
Cytologically bland tubules Cytologically highly atypical tubules
Mucinous stroma Desmoplastic stroma
Circumscribed Aggressively invasive

Mucinous Tubular and Spindle Cell Carcinoma of the Kidney Papillary Renal Cell Carcinoma with Low Grade Spindle Cell Foci
Lacks areas of classic papillary carcinoma Areas of classic papillary carcinoma usually present
Mucinous stroma present Mucinous stroma generally absent
Lacks trisomy 7 and 17 Trisomy 7 and/or 17 present

  • Mucinous tubular and spindle cell carcinoma may show morphologic and immunophenotypic overlap with papillary renal cell carcinoma (Paner 2006)
    • Features that may be shared
      • Papillary growth
      • Mucin production
      • Foam cells
      • AMACR expression
    • We diagnose borderline cases as papillary carcinoma
      • Urologists are much more familiar with that diagnosis
      • That diagnosis will probably elicit more extensive followup
    • Genetic studies can separate the two but are rarely indicated

Mucinous Tubular and Spindle Cell Carcinoma of the Kidney Metanephric Adenoma
Mucinous stroma Lacks mucinous stroma
CK7 pos CK7 neg

Mucinous Tubular and Spindle Cell Carcinoma of the Kidney Sarcomatoid Renal Cell Carcinoma
Lacks areas of classic renal cell carcinoma Aresa of classic renal cell carcinoma usually present
Cytologically bland spindle cells Cytologically atypical spindle cells
High grade sarcomatoid foci can arise in mucinous tubular and spindle cell carcinoma, but the uncomplicated MTSCC should not be considered sarcomatoid

Mucinous Tubular and Spindle Cell Carcinoma of the Kidney Renal Carcinoid
Mucinous stroma Lacks mucinous stroma
Lacks neuroendocrine chromatin pattern Stippled chromatin
Tubular and spindled growth patterns Trabecular growth pattern
Some cases of MTSCC have been reported to be positive for neuroendocrine markers

Grading / Staging

Grading

  • Definitionally low grade unless sarcomatoid pattern present

Staging

  • Use TNM for renal cell carcinoma

Clinical

  • Mean age 55
    • Range 17-82
  • M:F = 1:4
  • Association with nephrolithiasis has been reported
  • Generally benign behavior
    • Cases with sarcomatoid areas may behave aggressively

Classification / Lists

Renal epithelial neoplasms

Bibliography

  • Murphy WM, Grignon DJ, Perlman EJ. Tumors of the Kidney, Bladder and Related Urinary Structures, Atlas of Tumor Pathology, AFIP Fourth Series, Fascicle 1, 2004
  • Eble JN, Sauter G, Epstein JI, Sesterhenn IA eds. World Health Organization Classification of Tumors. Pathology and genetics of tumors of the Urinary System and Male Genital Organs. IARC Press: Lyon 2004.
  • Srigley JR, Delahunt B. Uncommon and recently described renal carcinomas. Mod Pathol. 2009 Jun;22 Suppl 2:S2-S23.
  • Ferlicot S, Allory Y, Compérat E, Mege-Lechevalier F, Dimet S, Sibony M, Couturier J, Vieillefond A. Mucinous tubular and spindle cell carcinoma: a report of 15 cases and a review of the literature. Virchows Arch. 2005 Dec;447(6):978-83.
  • Paner GP, Srigley JR, Radhakrishnan A, Cohen C, Skinnider BF, Tickoo SK, Young AN, Amin MB. Immunohistochemical analysis of mucinous tubular and spindle cell carcinoma and papillary renal cell carcinoma of the kidney: significant immunophenotypic overlap warrants diagnostic caution. Am J Surg Pathol. 2006 Jan;30(1):13-9.
  • Fine SW, Argani P, DeMarzo AM, Delahunt B, Sebo TJ, Reuter VE, Epstein JI. Expanding the histologic spectrum of mucinous tubular and spindle cell carcinoma of the kidney. Am J Surg Pathol. 2006 Dec;30(12):1554-60.
  • Dhillon J, Amin MB, Selbs E, Turi GK, Paner GP, Reuter VE. Mucinous tubular and spindle cell carcinoma of the kidney with sarcomatoid change. Am J Surg Pathol. 2009 Jan;33(1):44-9.
  • Pillay N, Ramdial PK, Cooper K, Batuule D. Mucinous tubular and spindle cell carcinoma with aggressive histomorphology--a sarcomatoid variant. Hum Pathol. 2008 Jun;39(6):966-9.
  • Jung SJ, Yoon HK, Chung JI, Ayala AG, Ro JY. Mucinous tubular and spindle cell carcinoma of the kidney with neuroendocrine differentiation: report of two cases. Am J Clin Pathol. 2006 Jan;125(1):99-104.
  • Argani P, Netto GJ, Parwani AV. Papillary renal cell carcinoma with low-grade spindle cell foci: a mimic of mucinous tubular and spindle cell carcinoma. Am J Surg Pathol. 2008 Sep;32(9):1353-9.
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