Indian Journal of Pathology and Microbiology

: 2022  |  Volume : 65  |  Issue : 3  |  Page : 733--735

A rare case of chromophobe RCC with sarcomatous change associated with squamous differentiation

Manika Khare1, Zai Wade1, Rakesh Sharma2,  
1 Consultant Pathologist, Apex Hospitals, Jaipur, Rajasthan, India
2 Consultant Urologist, Apex Hospitals, Jaipur, Rajasthan, India

Correspondence Address:
Manika Khare
3/293 Malviyanagar, Jaipur, Rajasthan

How to cite this article:
Khare M, Wade Z, Sharma R. A rare case of chromophobe RCC with sarcomatous change associated with squamous differentiation.Indian J Pathol Microbiol 2022;65:733-735

How to cite this URL:
Khare M, Wade Z, Sharma R. A rare case of chromophobe RCC with sarcomatous change associated with squamous differentiation. Indian J Pathol Microbiol [serial online] 2022 [cited 2022 Sep 25 ];65:733-735
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Full Text

Dear Editor,

Chromophobe RCC accounts for less than 5% of RCCs.[1] Sarcomatoid change in the RCC is seen is associated with aggressive behavior and over all poor survival.[2] Squamous differentiation with sarcomatoid change is chromophobe RCC is very rare. We present a case of such rare variant.

A 60 years male came to the urology OPD with complaints of generalized weakness, weight loss since 4 months. Patient also complained of the palpable lump in the abdomen since 2 months. Blood investigations showed anemia with hemoglobin of 11 gm/dl, all other parameters were within normal limits. Urine routine showed hematuria (full field RBCs). CT abdomen was done which showed a cystic exophytic lesion arising from lower and middle pole of left kidney with nodule at peripheral aspect of the lesion and homogeneously enhancing solid component with calcify foci at anterior aspect of the lesion suggestive of RCC. Cystic lesion was measuring 11.2 × 8.8 × 10.1 cm and solid area was measuring 3.5 × 3.4 cm [Figure 1]a. Mild bowel thickening with intramural fat involving ileo caecal junction was also noted, which was suggestive of inflammatory process. The cystic fluid cell block was submitted for the review, which was negative for the malignant cells. Operation of the patient was planned. Per operatively an irregular renal mass was identified. External surface was roughened and was adherent to the intestine and surrounding tissue. Gross specimen of nephrectomy was received for the histopathological examination. Grossly external surface was rough irregular and friable. Cut section showed a gray white mass on the lower pole of the kidney measuring 3 × 3 cm. Along with that a cystic irregular friable mass was identified, present over the middle and the lower pole of the kidney which have breached the capsule and invaded the gerota's fascia [Figure 1]b. The renal pelvis, renal vessels and the ureter were grossly unremarkable. No renal stones were identified. Microscopic examination showed a chromophobe RCC with areas of marked sarcomatous change. These sarcomatous areas are associated with prominent squamous differentiation. Large area of necrosis and calcification were seen. Tumor showed capsular breach and have invaded beyond gerotas fascia [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d. Based on above histomorphological findings diagnosis of chromophobe renal cell carcinoma with sarcomatous change associated with squamous differentiation was given. Immunohistochemistry was applied to further confirm the diagnosis which showed that tumor was positive for PAX 8, CK 7 and CD 117 in both chromophobe and sarcomatoid areas [Figure 3]a, [Figure 3]b, [Figure 3]c, [Figure 3]d. CK 5/6 was positive in the areas showing squamous differentiation. Tumor was negative for CK 20, Inhibin and uroplakin ruling out other differentials.{Figure 1}{Figure 2}{Figure 3}

Sarcomatoid change is thought to occur by the dedifferentiation of the epithelial component and hence, shows the original genomic pattern of the “parent” cells.[3] Maximum sarcomatoid change is seen associated with collecting duct renal cell carcinoma.[4] Akhtar et al. 2 in 1997 first documented the sarcomatoid transformation in CRCC, and thereafter additional reports have been published.[5]

Sarcomatoid transformation can also show heterologous differentiation, which is most commonly associated with conventional RCC.[4] Its incidence is about 1.5% of all sarcomatoid RCCs. Less than 10 cases reported to have the presence of heterologous elements, such as chondrosarcomatous, osteoarcomatous, and rhabdomyosarcomatous components. Squamous differentiation in the sarcomatous component is very rare and very few cases are reported.[6] A composite tumor composed of squamous cell carcinoma with sarcomatoid change are documented in the literature but in this case renal pelvis was absolutely normal grossly and microscopically ruling out transitional cell carcinoma.

Treatment with surgical resection only, does not change the clinical prognosis of the patient as by that time tumor has already metastasized to different sites or locally advanced, with a very short survival rates for these patients regardless of the type of treatment.[7] If left untreated, the sarcomatoid RCC have overall survival of approximately 3.8 to 6.8 months. The aggressiveness of such tumors is highlighted by Cangiano et al.,[8] according to him 84% of metastases have already occurred at the moment of surgery.

Interferon-a, an immune modulating agent with, has been employed in a number of clinical trials and has also been investigated retrospectively in the treatment of sarcomatoid RCC, but now the results does not seems to be so promising.[9] Now doxorubicin and gemcitabine have been drug of choice to the oncologists, after a definitive study performed by Nanus and colleagues in 2004. The development of receptor tyrosine kinase inhibitors that target neoangiogenesis (sunitinib, in particular) has brought a modestly improved clinical response in recent studies.[10] However, the prognosis of pleomorphic renal sarcomas, treated with chemotherapy still continues to be poor.

sRCC is an important entity for all clinicians to be familiar with. The sarcomatoid component may represent a terminally dedifferentiated clone arising from any of the conventional histologic subtypes of RCC or it may arise from a completely separate clone. The squamous differentiation in chromophobe RCC is very rare, hence very less information is available on its effect on prognosis. As per our knowledge only three cases are reported. Surgery in the setting of localized disease is the standard of care, but adjuvant trial participation should be considered because of the high-risk for recurrence, however still associated with poor prognosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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