Journal of Peking University (Health Sciences) ›› 2023, Vol. 55 ›› Issue (2): 276-282. doi: 10.19723/j.issn.1671-167X.2023.02.011

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Mucinous tubular and spindle cell carcinoma of kidney: Clinicopathology and prognosis

Qi SHEN*(),Yi-xiao LIU,Qun HE   

  1. Department of Urology, Peking University First Hospital; Institute of Urology, Peking University; National Urological Cancer Center, Beijing 100034, China
  • Received:2022-10-10 Online:2023-04-18 Published:2023-04-12
  • Contact: Qi SHEN E-mail:13522300373@163.com

Abstract:

Objective: To investigate and summarize the clinicopathological features, immunophenotype, differential diagnosis and prognosis analysis of mucinous tubular and spindle cell carcinoma (MTSCC). Methods: The data of thirteen cases of MTSCC were retrospectively analyzed, the clinical and pathological characteristics and immunohistochemical expression were summarized, and fluorescence in situ hybridization was detected. Results: Among the thirteen patients, four were males and nine females, with a male-to-female ratio of 1 ∶2.25. The average age was 57.1 years, ranging from 39 to 78 years. The maximum diameter of the tumor was 2-12 cm. All cases had no symptoms, and were accidentally discovered, 3 cases underwent partial renal resection, 10 cases underwent radical renal resection, 9 cases were located in the left kidney, and 4 cases were located in the right kidney. Most of the cases showed the classical morphological changes, with 11 cases of nuclear grading [World Health Organization (WHO)/International Society of Urological Pathology (ISUP) grading system] being G2 and 2 cases being G3. There were 6 cases of stage PT1a, 3 cases of PT1b, 2 cases of PT2a, and 1 case of PT2b and 1 case of PT3a. The positive rates of immunohistochemical staining were: vimentin, AE1/AE3, α-methylacyl-CoA racemase (αMACR) and cytokeratin (CK) 8/18, 100% (13/13); CK7, 92.3% (12/13); epithelial membrane antigen (EMA), 92.3% (12/13); CK20, 46.2% (6/13); CD10, 30.8% (4/13); synaptophysin (Syn), 7.7% (1/13); chromogranin A (CgA), CD57, WT1 and Ki-67, 0 (0/13), and fluorescence in situ hybridization showed that no trisomy of chromosomes 7 and 17 were observed in any of the cases. The follow-up period was 6 months to 7 years and 6 months, 2 cases died after lung metastasis (one with ISUP/WHO grade G3, one with necrosis), and the remaining 11 cases had no recurrence and metastasis. Conclusion: MTSCC is a unique type of low-grade malignancy kidney tumor, occurs predominantly in females, widely distributed in age, the current treatment method is surgical resection, and cases with necrosis and high-grade morphology are prone to recurrence and metastasis, although most cases have a good prognosis, but they still need close follow-up after surgery.

Key words: Renal cell carcinoma, Mucinous adenocarcinoma, Clinical pathology, Immunohistoche-mistry, Prognosis

CLC Number: 

  • R737.11

Table 1

Clinicopathological features of the MTSCC cases"

Case no. Gender Age/years Location Nephrectomy Tumor size/cm WHO/ISUP nuclear grade Necorsis T stage Follow-up/months Status
1 F 51 Right Total 7.6 2 No 2a 90 NED
2 F 77 Left Total 2.0 2 Yes 1a 15 Dead (lung metastasis)
3 F 56 Left Partial 2.5 2 No 1a 70 NED
4 M 69 Left Total 5.5 3 No 3a 6 Dead (lung metastasis)
5 M 50 Left Total 6.0 2 No 1b 54 NED
6 F 56 Right Total 7.3 2 No 2a 50 NED
7 F 51 Left Total 6.8 2 No 1b 38 NED
8 M 54 Left Partial 3.5 2 No 1a 27 NED
9 F 78 Left Total 2.5 3 No 1a 20 NED
10 F 50 Right Total 12.0 2 No 2b 14 NED
11 M 67 Left Total 3.6 2 No 1a 8 NED
12 F 39 Right Partial 2.2 2 No 1a 8 NED
13 F 44 Left Total 6.0 2 No 1b 6 NED

Figure 1

Histopathology of MTSCC (×100) A-E, hematoxylin-eosin staining. A, the typical features of MTSCC; B, the blue pools of mucin; C, the lymphocytic infiltration; D, the high grade MTSCC with WHO/ISUP nucleolar grade 3; E, the foam cells; F, immunohistochemical staining of CK7 showed the necrosis. MTSCC, mucinous tubular and spindle cell carcinoma. WHO/ISUP, World Health Organization/International Society of Urological Pathology."

Table 2

The results of immunohischemical stains for MTSCC"

Markers +++ ++ + -
CK7 1 7 4 1
αMACR 7 6 0 0
EMA 0 3 9 1
AE1/AE3 5 5 3 0
Vimentin 3 2 8 0
CK8/18 6 5 2 0
CD10 0 0 4 9
CK20 0 0 6 7
Syn 0 0 1 12
CgA 0 0 0 13
CD57 0 0 0 13
WT1 0 0 0 13
Ki-67 0 0 0 13

Figure 2

Immunohistochemical staining of MTSCC (×100) A, CK7; B, αMACR; C, EMA; D, AE1/AE3; E, vimentin; F, CD10; G, CgA; H, Ki-67. Abbreviations as in Table 2."

Figure 3

Fluorescence in situ hybridization analysis in MTSCC A, the FISH with centromeric probe for chromosome 7 displaying nuclei with two hybridization signals in MTSCC; B, the FISH with centromeric probe for chromosome 7 displaying nuclei with three hybridization signals in PRCC. FISH, fluorescence in situ hybridization; MTSCC, mucinous tubular and spindle cell carcinoma; PRCC, papillary renal cell carcinoma."

1 Eble JN, Sauter G, Epstein JI, et al. World Health Organization classification of tumours: Pathology and genetics of tumours of the urinary system and male genital organs[R]. Lyon, France: International Agency for Research on Cancer, 2004.
2 Moch H, Humphrey P, Ulbright T, et al. World Health Organization classification of tumours of the urinary system and male genital organs[R]. Lyon, France: International Agency for Research on Cancer, 2016.
3 MacLennan GT , Farrow GM , Bostwick DG . Low-grade collecting duct carcinoma of the kidney: Report of 13 cases of low-grade mucinous tubulocystic renal carcinoma of possible collecting duct origin[J]. Urology, 1997, 50 (5): 679- 684.
doi: 10.1016/S0090-4295(97)00335-X
4 Ged Y , Chen YB , Knezevic A , et al. Mucinous tubular and spindle-cell carcinoma of the kidney: Clinical features, genomic profiles, and treatment outcomes[J]. Clin Genitourin Cancer, 2019, 17 (4): 268- 274.
doi: 10.1016/j.clgc.2019.04.006
5 Xu X , Zhong J , Zhou X , et al. Mucinous tubular and spindle cell carcinoma of the kidney: A study of clinical, imaging features and treatment outcomes[J]. Front Oncol, 2022, 12, 865263.
doi: 10.3389/fonc.2022.865263
6 Ferlicot S , Allory Y , Comperat E , et al. Mucinous tubular and spindle cell carcinoma: A report of 15 cases and a review of the literature[J]. Virchows Arch, 2005, 447 (6): 978- 983.
doi: 10.1007/s00428-005-0036-x
7 Rakozy C , Schmahl GE , Bogner S , et al. Low-grade tubular-mucinous renal neoplasms: Morphologic, immunohistochemical, and genetic features[J]. Mod Pathol, 2002, 15 (11): 1162- 1171.
doi: 10.1097/01.MP.0000031709.40712.46
8 Nathany S , Monappa V . Mucinous tubular and spindle cell carcinoma: A review of histopathology and clinical and prognostic implications[J]. Arch Pathol Lab Med, 2020, 144 (1): 115- 118.
doi: 10.5858/arpa.2017-0506-RS
9 Xiao L , Xiao W , Guo Y , et al. Huge mucinous tubular and spindle cell carcinoma of the kidney: A case report[J]. Urol Case Rep, 2021, 40, 101914.
10 Bajpai M , Pooja S , Tyagi M , et al. Mucinous spindle and tubular renal cell cancer: A rare variant of renal cell cancer[J]. J Cancer Res Ther, 2022, 18 (4): 1168- 1170.
doi: 10.4103/jcrt.jcrt_99_21
11 Kuroda N , Hes O , Michal M , et al. Mucinous tubular and spindle cell carcinoma with Fuhrman nuclear grade 3: A histological, immunohistochemical, ultrastructural and FISH study[J]. Histol Histopathol, 2008, 23 (12): 1517- 1523.
12 Fuchizawa H , Kijima T , Takada-Owada A , et al. Metastatic mucinous tubular and spindle cell carcinoma of the kidney responding to nivolumab plus ipilimumab[J]. IJU Case Rep, 2021, 4 (5): 333- 337.
doi: 10.1002/iju5.12342
13 Arafah M , Zaidi SN . Mucinous tubular and spindle cell carcinoma of the kidney with sarcomatoid transformation[J]. Saudi J Kidney Dis Transplant, 2013, 24 (3): 557- 560.
doi: 10.4103/1319-2442.111066
14 Bulimbasic S , Ljubanovic D , Sima R , et al. Aggressive high-grade mucinous tubular and spindle cell carcinoma[J]. Hum Pathol, 2009, 40 (6): 906- 907.
doi: 10.1016/j.humpath.2009.03.004
15 Pillay N , Ramdial PK , Cooper K , et al. Mucinous tubular and spindle cell carcinoma with aggressive histomorphology: A sarco-matoid variant[J]. Hum Pathol, 2008, 39 (6): 966- 969.
doi: 10.1016/j.humpath.2007.10.006
16 Simon RA , di Sant'agnese PA , Palapattu GS , et al. Mucinous tubular and spindle cell carcinoma of the kidney with sarcomatoid differentiation[J]. Int J Clin Exp Pathol, 2008, 1 (2): 180- 184.
17 Dhillon J , Amin MB , Selbs E , et al. Mucinous tubular and spindle cell carcinoma of the kidney with sarcomatoid change[J]. Am J Surg Pathol, 2009, 33 (1): 44- 49.
doi: 10.1097/PAS.0b013e3181829ed5
18 Fine SW , Argani P , DeMarzo AM , et al. Expanding the histologic spectrum of mucinous tubular and spindle cell carcinoma of the kidney[J]. Am J Surg Pathol, 2006, 30, 1554- 1560.
doi: 10.1097/01.pas.0000213271.15221.e3
19 Reuter VE , Argani P , Zhou M , et al. Best practices recommendations in the application of immunohistochemistry in the kidney tumors: Report from the International Society of Urologic Pathology[J]. Am J Surg Pathol, 2014, 38 (8): e35- e49.
doi: 10.1097/PAS.0000000000000258
20 Paner GP , Srigley JR , Radhakrishnan A , et al. Immunohistochemical analysis of mucinous tubular and spindle cell carcinoma and papillary renal cell carcinoma of the kidney: Significant immunophenotypic overlap warrants diagnostic caution[J]. Am J Surg Pathol, 2006, 30 (1): 13- 19.
doi: 10.1097/01.pas.0000180443.94645.50
21 Uchida S , Suzuki K , Uno M , et al. Mucin-poor and aggressive mucinous tubular and spindle cell carcinoma of the kidney: Two case reports[J]. Mol Clin Oncol, 2017, 7 (5): 777- 782.
doi: 10.3892/mco.2017.1400
22 Kuroda N , Nakamura S , Miyazaki E , et al. Low-grade tubular-mucinous renal neoplasm with neuroendocrine differentiation: A histological, immunohistochemical and ultrastructural study[J]. Pathol Int, 2004, 54 (3): 201- 207.
doi: 10.1111/j.1440-1827.2004.01608.x
23 Peckova K , Martinek P , Sperga M , et al. Mucinous spindle and tubular renal cell carcinoma: Analysis of chromosomal aberration pattern of low-grade, high grade, and an overlapping morphologic variant with papillary renal[J]. Ann Diagn Pathol, 2015, 19 (4): 226- 231.
doi: 10.1016/j.anndiagpath.2015.04.004
24 Kuroda N , Naroda T , Tamura M , et al. High-grade mucinous tubular and spindle cell carcinoma: Comparative genomic hybridization study[J]. Ann Diagn Pathol, 2011, 15 (6): 472- 475.
doi: 10.1016/j.anndiagpath.2010.08.003
25 Sadimin ET , Chen YB , Wang L , et al. Chromosomal abnormalities of high-grade mucinous tubular and spindle cell carcinoma of the kidney[J]. Histopathology, 2017, 71 (5): 719- 724.
doi: 10.1111/his.13298
26 Mehra R , Vats P , Cieslik M , et al. Bi-allelic alteration and dysregulation of the Hippo pathway in mucinous tubular and spindle cell carcinoma of the kidney[J]. Cancer Discov, 2016, 6 (11): 1258- 1266.
doi: 10.1158/2159-8290.CD-16-0267
27 Ren Q , Wang L , Al-Ahmadie HA , et al. Distinct genomic copy number alterations distinguish mucinous tubular and spindle cell carcinoma of the kidney from papillary renal cell carcinoma with overlapping histologic features[J]. Am J Surg Pathol, 2018, 42 (6): 767- 777.
doi: 10.1097/PAS.0000000000001038
28 邹子归, 王玉红, 周晋星, 等. 肾黏液样小管状和梭形细胞癌临床病理分析及全外显子组测序分析[J]. 中华病理学杂志, 2021, 50 (7): 762- 767.
doi: 10.3760/cma.j.cn112151-20200922-00731
29 Xu H , Li W , Zhu C , et al. Proteomic profiling identifies novel diagnostic biomarkers and molecular subtypes for mucinous tubular and spindle cell carcinoma of the kidney[J]. J Pathol, 2022, 257 (1): 53- 67.
doi: 10.1002/path.5869
30 Bharti JN , Choudhary GR , Madduri VKS , et al. Mucinous tubular and spindle cell carcinoma: A difficult diagnosis[J]. Urol Ann, 2021, 13 (2): 180- 182.
doi: 10.4103/UA.UA_44_20
31 Thway K , du Parcq J , Larkin JM , et al. Metastatic renal muci-nous tubular and spindle cell carcinoma. Atypical behavior of a rare, morphologically bland tumor[J]. Ann Diagn Pathol, 2012, 16 (5): 407- 410.
doi: 10.1016/j.anndiagpath.2011.04.001
32 Cossu-Rocca P , Eble JN , Delahunt B , et al. Renal mucinous tubular and spindle carcinoma lacks the gains of chromosomes 7 and 17 and losses of chromosome Y that are prevalent in papillary renal cell carcinoma[J]. Mod Pathol, 2006, 19 (4): 488- 493.
doi: 10.1038/modpathol.3800565
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[5] . [J]. Journal of Peking University(Health Sciences), 2010, 42(6): 739 -745 .
[6] . [J]. Journal of Peking University(Health Sciences), 2007, 39(5): 507 -510 .
[7] . [J]. Journal of Peking University(Health Sciences), 2000, 32(4): 300 .
[8] . [J]. Journal of Peking University(Health Sciences), 2008, 40(2): 146 -150 .
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