|Year : 2018 | Volume
| Issue : 1 | Page : 64-66
Urothelial carcinoma with extensive glandular differentiation of renal pelvis: An unusual case
Anitha Chakravarthy1, H L Kishan Prasad1, T Rajeev2, Jayaprakash K Shetty1
1 Department of Pathology, K.S. Hegde Medical Academy, Mangalore, Karnataka, India
2 Department of Urosurgery, K.S. Hegde Medical Academy, Mangalore, Karnataka, India
|Date of Web Publication||1-Mar-2018|
Dr. H L Kishan Prasad
Department of Pathology, K.S. Hegde Medical Academy, Mangalore, Karnataka
Source of Support: None, Conflict of Interest: None
Malignancies in the renal pelvis are rare and few cases have been described in the literature till date. They present in various morphologies which cause diagnosis dilemma due to their close resemblance to other benign and malignant lesions. Histopathology has a major role in diagnostics since recognition of the morphological variant is important for prognostic and treatment stratification. We report a case of urothelial carcinoma of the renal pelvis with extensive glandular differentiation in a 48-year-old male.
Keywords: Glandular differentiation, mucin pool, renal pelvis, urothelial cell carcinoma
|How to cite this article:|
Chakravarthy A, Prasad H L, Rajeev T, Shetty JK. Urothelial carcinoma with extensive glandular differentiation of renal pelvis: An unusual case. Int J Health Allied Sci 2018;7:64-6
|How to cite this URL:|
Chakravarthy A, Prasad H L, Rajeev T, Shetty JK. Urothelial carcinoma with extensive glandular differentiation of renal pelvis: An unusual case. Int J Health Allied Sci [serial online] 2018 [cited 2021 Jan 19];7:64-6. Available from: https://www.ijhas.in/text.asp?2018/7/1/64/226252
| Introduction|| |
The most common urinary tract malignancy occurs in the urinary bladder, which is lined by a specialized form of transitional epithelium called urothelium. They are heterogeneous group of malignancies presenting with variable natural history. A small proportion of malignancies occurs in the renal pelvis (<7%) of which urothelial carcinoma is the most common. Here, we report a case of a 48-year-old male, who presented with abdomen pain and hematuria. Diagnosis of transitional cell carcinoma was made on computerized tomography scan. Histopathology confirmed the diagnosis of urothelial carcinoma with extensive glandular differentiation of the renal pelvis. This case is presented due to its diagnostic challenge and rarity.
| Case Report|| |
A 48-year-old male patient presented to the urology department with complaints of hematuria and pain in the left iliac region. General clinical examination was normal. Hematological finding showed hemoglobin 11.4 g%. Biochemical parameters were normal. Ultrasonography of the abdomen revealed a large semilunar calculus measuring 5 cm × 5 cm with hydronephrosis in the left kidney. Contrast-enhanced CT scan of the abdomen and pelvis showed gross hydroureteronephrosis of the left kidney with a heterogeneous soft-tissue mass measuring 5 cm × 4.5 cm in the posterior wall of the pelvis. The mass showed extension into the pelviureteric junction and proximal ureter causing luminal obstruction. There was evidence of ballooning of the calyces with thinning of the parenchyma and a large impacted calculus measuring 5.6 cm × 3.6 cm × 3.7 cm. Diagnosis of transitional cell carcinoma was suspected and left radical nephrectomy was performed.
Grossly, the left radical nephrectomy specimen measured 15 cm × 9 cm × 5 cm with attached ureter measuring 7 cm in length. Outer surface appeared enlarged and cystic with few mucoid areas. There was dilatation of pelvicalyceal system, with a proliferative friable growth measuring 6 cm × 5 cm. The tumor occupied the whole of renal pelvis with extension into the proximal ureter [Figure 1].
|Figure 1: Contrast-enhanced computerized tomography scan of the abdomen showing a heterogeneous soft-tissue mass in the posterior wall of the pelvis; gross appearance showing a proliferative friable growth occupying the whole of renal pelvis with extension into the proximal ureter with mucoid areas|
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Microscopically, the proliferative growth showed tumor cells with vacuolated cytoplasm and pleomorphic hyperchromatic nucleus arranged in papillae, tubules, and solid nests. Areas showed tumor necrosis, hemorrhage, and pools of extracellular mucin. The tumor cells revealed extension into the pelvicalyceal wall with sparing of the distal ureter and peripelvic fat. Urothelium showed multilayering epithelium with significant pleomorphism and hyperchromasia. Cortex and medulla showed chronic pyelonephritis. Perinephric fat, distal ureter, adrenal gland, Gerota's fascia, and lymph nodes remained free from the tumor. Histopathological diagnosis of infiltrating urothelial carcinoma with extensive glandular differentiation of the left renal pelvis was made [Figure 2].
|Figure 2: Microscopy showing tumor cells with vacuolated cytoplasm and pleomorphic hyperchromatic nucleus arranged in papillae, tubules, and solid nests. Areas showing tumor necrosis, hemorrhage, and pools of extracellular mucin|
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Two months after the surgery, cystoscopy with left ureterectomy was performed. Histopathology suggested residual urothelial carcinoma in the proximal ureter with metastatic deposits in the periureteric and left iliac lymph nodes. The patient was advised to undergo six cycles of radiotherapy followed by chemotherapy. However, the therapy was halted due to the patient's personal constraints.
| Discussion|| |
Neoplasms in the renal pelvis are uncommon constituting only 7% of renal tumors, of which 80% are malignant. The attributable risk factors include tobacco smoking, caffeine intake, analgesic abuse, and occupational exposure to petrochemicals and tar. Majority of the tumors are transitional cell carcinoma which are high grade. The urothelium undergoes various differentiation due to metaplastic changes. According to the World Health Organization (WHO), the recent classification of the histologic variants consisted of squamous, glandular, microcystic, nested, micropapillary, clear cell, lymphoepithelioma, plasmacytoid, giant cell, and sarcomatoid variants. The recent WHO classification has replaced the term extensive glandular differentiation to infiltrating urothelial carcinomas with divergent differentiation. The classification helps to differentiate the morphology and the biological behavior of the tumor. They include mucinous and enteric type. The mucinous type shows abundant extravasated mucin and neoplastic cells with signet ring cell changes. The enteric type resembles its colonic counterpart. They arise from gene mutation which include FGFR-3, TP-53, RB-1, PIK3CA, and HRAS. Extensive glandular differentiation of the renal pelvis accounts for very small number of cases reported so far, the incidence of which is unknown. The most common symptoms are hematuria and flank pain. Extensive glandular urothelial carcinoma consists of true glandular spaces. They show enteric or tubular glands with mucin production. The presence of mucin in the cytoplasm is seen in normal urothelium in 60%–65% of cases of urothelial neoplasm. This feature alone does not support the diagnosis of urothelial carcinoma. Patients with only glandular differentiation can be diagnosed as adenocarcinoma., Glands have single-layered tall columnar epithelium. Mucin production is enormously increased such that occasional signet ring cells may be seen floating in the mucin pool., Tumors with urothelial and glandular component with the above-mentioned features can be described as urothelial carcinoma with glandular differentiation., In our case, microscopy showed multilayering of urothelium with pools of extracellular mucin amid the tumor cells.
The differential diagnoses include benign and malignant lesions. The various malignant lesions are adenocarcinoma, microcystic/nested variants of urothelial carcinoma, paraganglioma, and metastatic adenocarcinoma. The various benign conditions of the bladder are cystitis cystica, cystitis glandularis, von Brunn's nests, and inverted papilloma., The presence of combined urothelial and glandular component helps in differentiating benign lesions of the bladder. Malignant differential diagnosis differs in morphology and anatomic site. Metastatic adenocarcinoma can present with a primary in a different anatomical location. Microcystic carcinoma does not have a true glandular component.,
There are various stains which help to differentiate extensive glandular differentiation from conventional urothelial and other malignant counterparts. Apomucin stains positive in extensive glandular differentiation while negative in conventional urothelial carcinoma. Carcinoembryonic antigen staining highlights squamous and glandular differentiation. Glandular differentiation in urothelial carcinoma is positive for CK7 staining and negative for villin, CDX2, and CK20 which helps to differentiate metastatic adenocarcinoma which stains negative for CD7 and positive for the others.
Urothelial carcinoma presents with different morphologies which is the most important aspect in diagnosis. Histopathological diagnosis lies in recognition of invasion because the management is different for noninvasive and invasive carcinomas., Aggressive urothelial carcinomas can metastasize to prostate, ureter, seminal vesicle, rectum, vagina, and retroperitoneal cavity. The prognosis is very poor with a life expectancy of 3 years following a metastatic disease. The management varies depending on the morphology of the variants. For a pure form of glandular disease, resection followed by radiation therapy and chemotherapy is followed. However, there is no evidence suggestive of therapeutic recommendations in the literature. The chemotherapy was stopped in this patient due to family constraints, and hence, follow-up of the case was not possible.
| Conclusion|| |
Primary urothelial carcinomas of the renal pelvis present with a spectrum of histologic features similar to the presentation in the urinary bladder. Majority of them are high-grade neoplasms and present in advanced stage of the disease. The importance of diagnosing the various morphologic variants is a challenge to the histopathologist to avoid confusion with other differential conditions. This diagnosis was a challenge to a pathologist due to the various morphological mimickers of the primary tumors of the renal pelvis.
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Conflicts of interest
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[Figure 1], [Figure 2]