|Year : 2020 | Volume
| Issue : 2 | Page : 93-96
Renal cell carcinoma arising in an open pyelolithotomy scar: A perplexing scenario!
Sandeep Bhoriwal1, Kunal Dhall1, Adarsh Barwad2, Ashutosh Mishra1
1 Department of Surgical Oncology, All India Institute of Medical Sciences (AIIMS), New Delhi, India
2 Department of Pathology, All India Institute of Medical Sciences (AIIMS), New Delhi, India
|Date of Submission||18-Jul-2020|
|Date of Acceptance||25-Sep-2020|
|Date of Web Publication||31-Dec-2020|
Dr. Ashutosh Mishra
Department of Surgical Oncology, All India Institute of Medical Sciences (AIIMS), New Delhi 110029.
Source of Support: None, Conflict of Interest: None
Renal cell carcinoma (RCC) arising in a renal tissue away from the normal kidney is quite rare with only a few cases reported in the literature. The management of such cases is challenging and requires an individualized treatment approach. We report a case of a young male with a prior history of pyelolithotomy who presented with a right paravertebral mass. CECT chest and abdomen revealed a soft-tissue mass near the right kidney with obvious infiltration into posterior abdominal wall muscles and 12th rib erosion. Image-guided biopsy of the mass demonstrated features of metastatic RCC. The patient underwent surgery with en bloc removal of the tumor with 12th rib, infiltrated paraspinal muscles, a small part of the densely adhered right kidney, and overlying skin. Histopathology with recommended IHC panels showed papillary RCC. Because of the above, the multidisciplinary tumor board considered this as a malignancy arising from a pyelolithotomy scar. The patient was planned for follow-up only without any adjuvant treatment. This case highlights the importance of a multidisciplinary team approach and comprehensive planning in such rare and perplexing cases.
Keywords: Papillary RCC, pyelolithotomy, renal cell carcinoma
|How to cite this article:|
Bhoriwal S, Dhall K, Barwad A, Mishra A. Renal cell carcinoma arising in an open pyelolithotomy scar: A perplexing scenario!. J Curr Oncol 2020;3:93-6
| Introduction|| |
Renal cell carcinoma (RCC) accounts for 90% of renal cancers and constitutes 2%–3% of adult malignancies. The development of metastasis in a patient of papillary renal cell carcinoma (PRCC) is well known. But the presentation of metastatic PRCC in the absence of renal primary is a very rare phenomenon. To differentiate, whether such a tumor is metastatic or has arisen de novo in an ectopic renal tissue is a perplexing scenario. Therefore, the individualized treatment approach should be applied to these cases. We report a rare case of a 41-year-old male who presented with a right paravertebral mass which was adjacent to the radiologically normal right kidney but histologically proven to be a metastatic papillary RCC.
| Case Presentation|| |
A 41-year-old male, an industrial worker by occupation, presented to us with a swelling over the right side of his back for the last 3 months. The swelling was gradual in onset, slowly progressive, and painless. There was no history of fever, loss of weight, and appetite. The patient also revealed the history of open right pyelolithotomy done 7 years back for renal lithiasis of which no records were available.
On physical examination, a 3 cm × 3 cm hard, nontender swelling was found in the right subscapular and paravertebral region with restricted craniocaudal mobility. The overlying skin was normal. There was no generalized lymphadenopathy and the rest of the systemic examination was normal. Previous pyelolithotomy transverse scar was present in the right lumbar region [Figure 1]. Ultrasound whole abdomen revealed a 3.6 cm × 2.5 cm, well-defined, lobulated, hypoechoic lesion in the right paraspinal region, cranio-posterior to the right kidney. There was obvious infiltration of posterior abdominal wall muscles. CECT chest and whole abdomen revealed a 4.3 cm × 3.5 cm × 3.3 cm, heterogeneously enhancing soft-tissue mass infiltrating right psoas, erector spinae, and quadratus lumborum (posterior paraspinal muscles) at L2 level with underlying 12th rib erosion. However, the right kidney was seen separately from the mass and in its normal anatomical position. Most of the lesion was extra-abdominal with a small retro-peritoneal component. The contralateral kidney was normal. There were no retroperitoneal lymphadenopathy and other distant metastasis [Figure 2]. There was no evidence of supernumerary kidney or extrarenal RCC. Subsequent MRI of the dorsolumbar spine documented the findings in concordance with CECT and without any marrow edema or cortical destruction of the 12th rib. Whole-body PET-CT showed soft-tissue lesion involving the right paravertebral region with increased FDG uptake at the level of 12th rib.
|Figure 1: Preoperative photograph showing right paravertebral region mass|
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Diagnostic ultrasound-guided biopsy of the mass revealed tumor arranged in papillae with few foamy histiocytes in the fibrovascular cores. The tumor cells were mildly pleomorphic with hyperchromatic nuclei and inconspicuous nucleoli and a moderate amount of cytoplasm. The sample was immunopositive for PAX-8, AMACR while immunonegative for CK7, WT-1, and calretinin. Based on morphology and immunostain, the possibility of PRCC was suggested. These atypical and unexpected histopathological findings forced us to repeat image-guided biopsy. Again biopsy report confirmed it as PRCC. On repeat IHC, tumor cells were immunopositive for EMA, vimentin, and AMACR.
Considering this as a localized and solitary lesion, surgery planning was done with curative intent. The patient underwent surgery with en bloc removal of the tumor with 12th rib, infiltrated paraspinal muscles, a small part of the densely adhered right kidney, and overlying skin.
Intra-operatively, we found a 5 cm × 5 cm mass infiltrating the erector spinae, superficial part of quadratus lumborum, 12th rib with dense adherence to the right renal capsule [Figure 3]. Wide excision of the tumor was done with en bloc removal of involved structures. Renal parenchyma was removed for better margin assessment [Figure 4]. Under the ICD cover, the exposed right pleura was repaired with prolene. The posterior abdominal wall was strengthened with a Proceed mesh (Prolene + Oxidized regenerated cellulose [ORC] composite mesh). ICD and Abdominal drain were removed on postoperative day 4. The patient had an uneventful postoperative recovery and was discharged on postoperative day 5.
|Figure 3: Intraoperative photograph showing en bloc removal of the tumor with involved muscles and renal parenchyma|
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|Figure 4: Photograph showing tumor bed after en bloc resection of the tumor|
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Histopathology documented a tumor of size 6.5 cm × 4.9 cm × 3 cm with infiltration of rib and adjacent skeletal muscles, although renal parenchyma was free. On microscopy, it showed features of papillary carcinoma RCC. On IHC, it was immunopositive for CK7, AMACR, CD10, and PAX-8 while it was immunonegative for CK20, vimentin, napsin, TTF-1, CDX2, CK19, PSA, Heppar-1, Arginase, and p40 [Figure 5]. Morphology and immunochemistry suggested a renal primary. All the microscopic margins were free of tumor. There was no evidence of papillary adenoma or carcinoma in histopathologically examined en bloc excised renal tissue.
|Figure 5: (A) Low-power photomicrograph showing tumor with papillary pattern and central fibrovascular core. There are scattered lymphomononuclear cells in the stroma. (Handamp; E 100x). (B) High-power photomicrograph showing tumor cells with vesicular chromatin, prominent nucleoli, and moderate to abundant clear cytoplasm. (Handamp; E 200x). (C) Immunostain for PAX-8 showing nuclear positivity in the tumor cells. (D) Immunostain for AMACR showing cytoplasmic positivity in the tumor cells|
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Multiple rounds of discussion in the oncopathology board and Multidisciplinary clinics happened and considering the histopathology report, the same prior pyelolithotomy site, and no other lesion in the right kidney, a diagnosis of PRCC developing in open pyelolithotomy scar was made. In view of margin-free resection, the patient is under observation without any adjuvant therapy. After 6 months of follow-up, there is no evidence of recurrence. The rarity of the case has been discussed with the patient and informed consent was taken for reporting and publication.
| Discussion|| |
RCC arises from the renal tubular epithelium and accounts for >90% of adult renal tumors. The most common histology is clear cell carcinoma (75%) followed by papillary (15%) and chromophobe RCC (5%). The common symptomatology of RCC includes hematuria, flank pain, or flank mass apart from symptoms arising due to metastatic disease (bone pain, adenopathy, or pulmonary symptoms). But the majority of them are diagnosed as an incidental mass on radiology done for other reasons. The common imaging modalities used are CT of the abdomen and pelvis, MRI abdomen (in suspected IVC involvement). The standard of care for a resectable RCC is surgical resection (either partial or radical nephrectomy) whereas, metastatic RCCs usually require systemic therapy in the form of targeted agents or immune checkpoint inhibitors. The estimated 5-year survival rate in RCC ranges from 96% in stage 1 to 23% in stage 4.
Although minimally invasive techniques like ESWL, PCNL, laparoscopic and robotic pyelolithotomy have become standard-of-care in the management of urolithiasis, open pyelolithotomy still plays a major role in selected cases. The development of tumor in the percutaneous renal biopsy tract is well described in the literature., The needle tract seeding of transitional cell carcinoma (TCC) following fine-needle aspiration of the renal tumor may also occur, although rarely. There is a scarcity of literature on malignancy arising in renal tissue present in scar tissue due to which there is no standardized approach to treat these patients. The treatment modality and surgical approach depend on the site of the lesion, the clinical stage of malignancy, comorbid conditions, and the surgeon’s experience. The surgical approach in these cases merits special attention because of uncertain vascular anatomy. CT with intravenous contrast or magnetic resonance angiography may be indicated to precisely identify renal arteries and veins near the mass.
In our case, the patient had the right paravertebral region mass which was initially diagnosed as metastatic PRCC with no obvious radiological evidence of primary tumor in either kidney. In the histopathology report of the en bloc excised tumor, the resected part of the kidney was free of any tumor. Therefore it was concluded that there could have been normal renal tissue spill and seeding might have occurred during right open pyelolithotomy which later on developed malignancy. On review of literature, a similar case was also reported in a 39 years old male patient in whom recurrent waist tumor after percutaneous nephrolithotomy (PCNL) was diagnosed as metastatic adenocarcinoma. The patient underwent local resection of the tumor followed by systemic chemotherapy. The authors concluded that it was a case of PCNL tract seeding with RCC. In our case, since the patient was quite young and the right kidney demonstrated no tumor radiologically, kidney-sparing en bloc tumor resection was planned. The patient did not receive any adjuvant treatment because of margin negative resection. To the best of our knowledge, our case is the first one to report RCC arising in an open pyelolithotomy scar.
| Conclusion|| |
RCC arising at an atypical location and away from the normal kidney is quite rare. These atypical presentations warrant diagnostic challenges and force clinicians to think out of the box. A multidisciplinary team approach, protocol-based assessment, and comprehensive planning usually help in the management of these atypical cases.
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Conflicts of interest
There are no conflicts of interest.
Ethics approval, statement of compliance, and clinical trial transparency statement
The patient provided informed consent for this case to be published. Maximal effort was made to maintain complete anonymity and no identifying information is included in the manuscript. This case report is compliant with all relevant laws and institutional guidelines. This case report was written according to the Declaration of Helsinki.
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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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]