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CASE REPORT
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Mucinous spindle and tubular renal cell cancer: A rare variant of renal cell cancer


 Department of Pathology, Santosh Medical College and Hospital, Santosh Deemed to be University, Ghaziabad, NCR Delhi, India

Date of Submission15-Jan-2021
Date of Acceptance30-Apr-2021
Date of Web Publication16-Jun-2022

Correspondence Address:
Malay Bajpai,
Department of Pathology, Santosh Medical College and Hospital, Santosh Deemed to be University, Ghaziabad, NCR Delhi
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcrt.jcrt_99_21

 > Abstract 


In the World Health Organization classification system, mucinous tubular and spindle cell carcinoma of the kidney (MTSCC-K) has been described as a rare pathologic subtype of renal cell cancer. This tumor is supposed to have good prognosis, though follow-up data are limited due to rarity of tumor. MTSCC-K is a rare variant of renal cell carcinoma with female predominance and favorable prognosis. We report our experience with a case of mucinous tubular spindle cell carcinoma involving the kidney. A 61-year-old female presented with a palpable left renal lump of 12 cm in size. Computed axial tomography scan revealed a well-circumscribed mass of 11 cm involving the lower pole of the left kidney. She underwent left open radical nephrectomy. The cut section revealed a well-circumscribed tumor with yellowish-white variegated areas measuring 11 cm × 8 cm × 7 cm. Histopathological diagnosis of mucinous tubular spindle cell carcinoma of the kidney was given, and the patient responded well after the treatment. An accurate diagnosis may have an impact on prognostication of tumor and understanding the biology of the tumor. Thus, the clinicians should be aware of this rare disease for prompt diagnosis.

Keywords: Kidney, mucinous, renal cell carcinoma, spindle cell carcinoma, tubular



How to cite this URL:
Bajpai M, Pooja S, Tyagi M, Pathre A. Mucinous spindle and tubular renal cell cancer: A rare variant of renal cell cancer. J Can Res Ther [Epub ahead of print] [cited 2022 Aug 11]. Available from: https://www.cancerjournal.net/preprintarticle.asp?id=347672




 > Introduction Top


Renal cell carcinoma (RCC) encompasses a family of malignant epithelial tumors arising from the renal tubular epithelium with diverse morphologic features. Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare subtype of RCC.[1] In the 2004 World Health Organization classification system, MTSCC of the kidney (MTSCC-K) has been described as a rare pathologic subtype of renal cell cancer (RCC).[2] It has been described bearing a good prognostic factor, though follow-up data are limited due to rarity of tumor.

We are presenting a case of left renal mass which on excision revealed a similar variant.


 > Materials and Methods Top


Case Report

A 61-year-old female presented with left flank pain for 8 months. Clinically, she had a palpable left renal lump of 12 cm in size. On preliminary ultrasound abdomen, there was a left renal mass of 10-cm size with normal right kidney. Her renal function was found to be normal. Computed axial tomography scan of the abdomen and pelvis revealed a well-circumscribed mass of 11 cm involving the lower pole of the left kidney with no evidence of locoregional infiltration [Figure 1] and [Figure 2]. Chest X-ray revealed no evidence of metastasis. Her performance status was Eastern Cooperative Oncology Group 0. She underwent left open radical nephrectomy by an anterior subcostal incision. Intraoperatively, left renal mass was localized.
Figure 1: Heterogeneously enhancing left renal mass occupying the lower pole

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Figure 2: Coronal reconstruction (a) and sagittal reconstruction (b)

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Pathology

Left radical nephrectomy specimen was 16 cm × 10 cm × 9 cm and weighing 500 g [Figure 3]a. Cut section revealed a well-circumscribed tumor occupying the lower pole of the left kidney. It was a yellowish-white variegated tumor measuring 11 cm × 8 cm × 7 cm [Figure 3]b.
Figure 3: (a) Well-circumscribed mass involving the lower half of the left kidney, (b) cut open specimen revealed a well-circumscribed yellowish-white variegated tumor

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Microscopically, the tumor was well circumscribed and composed of predominantly spindle-shaped cells showing abrupt transition to areas showing tubular differentiation with luminal mucinous material [Figure 4]a, [Figure 4]c and [Figure 4]d. The cells show eosinophilic cytoplasm with minimal nuclear pleomorphism and low mitotic activity [Figure 4]b. Special stains revealed Alcian blue-positive material in background. There was no capsular infiltration. Perinephric fat, renal sinus fat, hilar vessels, and resected end of ureter were free of tumor.
Figure 4: Microscopic sections showed a tumor composed of spindled cells arranged in cords (a, H and E, ×40). Tumor cells show a moderate amount of eosinophilic cytoplasm and round-to-oval nuclei with minimal pleomorphism (b, H and E, ×100; c, H and E, ×200). Focal mucinous stroma is identified (d, H and E, ×200)

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Follow-up

Postoperatively, she was discharged on day 3 after surgery and doing well currently. She is due for first follow-up.


 > Discussion Top


MTSCC is a rare variant of renal cell carcinoma and recently described subtype with a female predominance and favorable prognosis.[2] MTSCC is a low-grade malignant renal tumor with characteristic histologic, immunohistochemical, and molecular features and hence is considered a distinct entity. Most reported cases have been diagnosed as low-grade malignancies and have had a favorable outcome after surgical removal.[3]

Grossly, MTSCC is usually well circumscribed with a solid gray-to-white appearance on cut surface.[4] Histologically, the tumor is composed of cuboidal cells arranged in microtubules and long cords making abrupt transitions to spindle morphology. These structures are arrayed in a mucinous or myxoid stroma that reacts strongly with Alcian blue. Nuclear atypia and mitoses are rare in both cuboidal and spindle cells. The morphological features of the papillary renal cell carcinoma with sarcomatoid change may have an overlap with the MTSCC, where spindle cells are arranged in parallel bundles with eosinophilic cytoplasm and low-grade nuclei.[5]

Few studies have enquired about the genetic changes in MTSCC. In a study of 5 patients, Rakozy et al. analyzed six tumors with comparative genomic hybridization techniques and revealed consistent losses of chromosomes 1, 4, 6, 8, 9, 13, 14, 15, and 22 in all cases, and these results were corroborated by loss of heterozygosity analyses in all informative cases.[6]

In genetic analysis of 10 cases of MTSCCs, Srigley et al. revealed frequent losses of chromosomes 1, 4q, 6, 8p, 9p, 11q, 13, 14, and 15 and gains for chromosomes 12q, 16q 17, and 20q.[7]

Paolo Cossu-Rocca et al. in genetic analysis of 10 patients of MTSCC concluded that MTSCCs lack the gains of chromosomes 7 and 17 and losses of chromosome Y, whereas these genetic changes are prevalent in papillary renal cell carcinomas. This may be of help in differentiating the classic and sarcomatoid papillary renal cell carcinomas.[7]

MTSCC is considered to be of favorable prognosis. Nafisa et al. reported a case of MTSCC with concurrent retroperitoneal lymph node metastasis and liver metastasis, wherein left radical nephrectomy with lymph node sampling and liver nodule biopsy revealed a similar tumor. Recent genomic and transcriptomic analysis of MTSCC has uncovered recurrent chromosomal losses and frequent biallelic alteration of Hippo pathway genes, resulting in increased YAP1 nuclear expression.[8]


 > Conclusion Top


MTSCC-K is a rare variant of renal cell carcinoma which has a favorable prognosis as compared with other variants. Diagnosis necessitated diligent morphology review, special stains, and immunohistochemical workup.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 > References Top

1.
Adamane SA, Menon S, Prakash G, Bakshi G, Joshi A, Popat P, et al. Mucinous tubular and spindle cell carcinoma of the kidney: A case series with a brief review of the literature. Indian J Cancer 2020;57:267-81.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Lopez-Beltran A, Scarpelli M, Montironi R, Kirkali Z. 2004 who classification of the renal tumors of the adults. Eur Urol 2006;49:798-805.  Back to cited text no. 2
    
3.
Nathany S, Monappa V. Mucinous tubular and spindle cell carcinoma: A review of histopathology and clinical and prognostic implications. Arch Pathol Lab Med 2020;144:115-8.  Back to cited text no. 3
    
4.
Eble JN. Mucinous tubular and spindle cell carcinoma and post-neuroblastoma carcinoma: Newly recognised entities in the renal cell carcinoma family. Pathology 2003;35:499-504.  Back to cited text no. 4
    
5.
Fleming S. Recently recognized epithelial tumours of the kidney. Curr Diagn Pathol 2005;11:162-9.  Back to cited text no. 5
    
6.
Rakozy C, Schmahl GE, Bogner S, Störkel S. Low-grade tubular-mucinous renal neoplasms: Morphologic, immunohistochemical, and genetic features. Mod Pathol 2002;15:1162-71.  Back to cited text no. 6
    
7.
Srigley J, Kapusta L, Reuter V. Phenotypic, molecular, and ultrastructural studies of a novel lowgrade renal epithelial neoplasm possibly related to the loop of Henle. Mod Pathol 2002;15:182.  Back to cited text no. 7
    
8.
Udager A, Dhanasekaran S, Mehra R. Mucinous tubular and spindle cell carcinoma: Case report and review of an uncommon renal tumor. Ajsp 2017;22:297-300.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

 
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