Mucinous tubular and spindle cell carcinoma of the kidney with sarcomatoid change Journal Article


Authors: Dhillon, J.; Amin, M. B.; Selbs, E.; Turi, G. K.; Paner, G. P.; Reuter, V. E.
Article Title: Mucinous tubular and spindle cell carcinoma of the kidney with sarcomatoid change
Abstract: Sarcomatoid change has been well documented in the various subtypes of renal cell carcinoma (RCC) and its presence is known to portend a worse prognosis in RCC. Mucinous tubular and spindle cell carcinoma is a RCC subtype, which is defined as polymorphous histology wherein the spindled epithelial cell is an inherent carcinomatous component. Many of these putatively low-grade tumors have been previously misdiagnosed as unclassified or sarcomatoid papillary RCC. We present 2 examples of hitherto undescribed sarcomatoid change in mucinous tubular and spindle cell carcinoma in a 71-year-old woman and an 80-year-old man who both underwent a radical nephrectomy procedure. In addition to the classic mucinous tubular and spindle cell carcinoma morphology, both cases had a sarcomatoid component characterized by predominantly high-grade spindle cells, solid pleomorphic epithelioid cells, and malignant fibrous histiocytoma-like storiform patterns. Sarcomatoid change comprised 60% and 20% of the tumors, respectively. Unlike the spindle sarcomatoid cells, the inherent spindle cell elements of mucinous tubular and spindle cell carcinoma had distinctively low-grade cytology and occasionally blended with tubular structures and variable mucinous stroma. The sarcomatoid cells were associated with significant necrosis, marked nuclear pleomorphism, mitoses of up to 5/10 high power field, higher proliferation fraction (MIB1), and loss of α-methylacyl-CoA racemase or cytokeratin 7 expression. Cytogenetic analysis in 1 tumor showed loss of chromosomes 14 and 15 and gains of chromosomes 2, 5, 7, 9, 10, 12, 17, 19, 20, 22, and X. Widespread metastasis to lymph nodes, bones and lungs occurred in one patient who succumbed 9 months after nephrectomy. Helpful features in distinguishing spindle cells of sarcomatoid component versus that of the native tumor include the presence of high-grade cytology, expansile growth with loss of typical imperceptible blending with the tubulo-papillary component, extensive necrosis, high mitotic activity, high proliferation fraction, and loss of expression of α-methylacyl-CoA racemase that contrasted the classic areas. Distinction of the sarcomatoid histology from inherent spindle cell component of mucinous tubular and spindle cell carcinoma is important because of its unfavorable prognostic implication.
Keywords: immunohistochemistry; human tissue; aged; aged, 80 and over; genetics; clinical feature; histopathology; anamnesis; case report; metabolism; metastasis; computer assisted tomography; pathology; kidney carcinoma; kidney neoplasms; nephrectomy; kidney tumor; cancer size; carcinoma; cytokeratin 7; amacr; cytogenetic; mucinous tubular and spindle cell carcinoma of the kidney; sarcomatoid; 2 methylacyl coenzyme a racemase; autosome; mucinous tubular carcinoma; spindle cell carcinoma
Journal Title: American Journal of Surgical Pathology
Volume: 33
Issue: 1
ISSN: 0147-5185
Publisher: Lippincott Williams & Wilkins  
Date Published: 2009-01-01
Start Page: 44
End Page: 49
Language: English
DOI: 10.1097/PAS.0b013e3181829ed5
PUBMED: 18941398
PROVIDER: scopus
DOI/URL:
Notes: --- - "Cited By (since 1996): 9" - "Export Date: 30 November 2010" - "CODEN: AJSPD" - "Source: Scopus"
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  1. Victor Reuter
    1228 Reuter