Mucinous cystadenocarcinoma in the renal pelvis: primary or secondary? Case report and literature review

Tamsin, An; Schillebeeckx, Charlotte; Langenhove, Charlotte Van; Eeckt, Kathy Vander; Ost, David; Wetzels, Kevin

doi:10.1080/00015458.2019.1617515

Cited by 8 publications

(11 citation statements)

References 11 publications

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“…Among the previous cases, most of them originated from the urinary collecting system (pelvic MC) and less from renal parenchyma (parenchymal MC), including our case [3,[10][11][12][13][14][15][16][17][18][19]. There are three theories about glandular metaplastic change, including (1) renal maldevelopment, (2) chronic inflammation (or irritation), and (3) celomic epithelium differentiation [6,20]. Only three MCs and three MCCs were reported in association with maldevelopment [6,8,18].…”

Section: Etiologymentioning

confidence: 98%

“…Its malignant counterpart, mucinous cystadenocarcinoma (MCC), is also a rare entity with only thirteen literature reports. The features defining malignancy are nuclear atypia, invasion of stroma, and multilayers of neoplastic cells [6,7]. Most of the reported cases of these neoplasms were from Japan, followed by India, Mexico, Iran, and Turkey (Table 1) [8,9].…”

Section: Epidemiologymentioning

confidence: 99%

“…There are three theories about glandular metaplastic change, including (1) renal maldevelopment, (2) chronic inflammation (or irritation), and (3) celomic epithelium differentiation [6,20]. Only three MCs and three MCCs were reported in association with maldevelopment [6,8,18]. Some authors suggested that such parenchymal tumors could originate from a sequestrated segment of the renal pelvic epithelium in renal parenchyma in the background of maldevelopment [10,11,18].…”

Section: Etiologymentioning

confidence: 99%

“…The diagnosis of MC was always made after nephrectomy (partial or total) in previous reports [6]. Differential diagnoses of muconephrosis are more frequent than MC.…”

Section: Diagnosis and Differential Diagnosismentioning

confidence: 99%

“…Differential diagnoses of muconephrosis are more frequent than MC. Solid renal cell carcinomas with necrotic or hemorrhagic components, metastatic mucinous cystadenocarcinoma of the appendix, renal papillary adenoma, villous adenoma (arising from urothelial metaplasia), and MCC are other possible diagnoses [6]. It is quite difficult to distinguish an MC from a simple cyst based on clinical history, physical, laboratory, and imaging findings.…”

Section: Diagnosis and Differential Diagnosismentioning

confidence: 99%

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Parenchymal mucinous cystadenoma of the kidney: a case report and literature review

Kalantari

Mottaghi

et al. 2021

Afr J Urol

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Background Mucinous cystadenoma (MC) of the kidney is exceedingly rare. We found 22 similar cases in the literature. These masses are underdiagnosed due to radiologic similarities with simple renal cysts. Case presentation A 66-year-old man with a previous history of hypertension and anxiety was referred to our tertiary clinic with left flank pain. Ultrasound revealed a 60 mm-sized, complex cystic mass with irregular septa in the lower pole of the left kidney (different from last year's sonographic findings of a simple benign cyst with delicate septa). CT scan showed the same results plus calcification. Due to suspected renal cell carcinoma, a radical nephrectomy was performed. Postoperative histopathologic examination revealed a cyst lined by a single layer of columnar mucin-producing cells with small foci of pseudo-stratification, consistent with the MC’s diagnosis. The first follow-up visit showed normal blood pressure without medication and no flank pain and anxiety after a month. Conclusion It is quite challenging to distinguish the primary MC of the kidney from a simple renal cyst based on clinical and imaging findings. The radiologic features of these entities overlap significantly. Thus, complex renal cyst and renal cysts with mural nodules should be followed closely to detect malignancy earlier.

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Section: Etiologymentioning

confidence: 98%

Section: Epidemiologymentioning

confidence: 99%

Section: Etiologymentioning

confidence: 99%

“…The diagnosis of MC was always made after nephrectomy (partial or total) in previous reports [6]. Differential diagnoses of muconephrosis are more frequent than MC.…”

Section: Diagnosis and Differential Diagnosismentioning

confidence: 99%

Section: Diagnosis and Differential Diagnosismentioning

confidence: 99%

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Parenchymal mucinous cystadenoma of the kidney: a case report and literature review

Kalantari

Mottaghi

et al. 2021

Afr J Urol

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Mucinous Borderline Tumors of the Pelvicalyceal System: Report of Two Tumors With Molecular Findings and Review of Literature

Paralkar,

Akbari,

Kandukuri

et al. 2024

Int J Surg Pathol

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Mucinous borderline tumors of the pelvicalyceal system are exceedingly rare tumors with very little information regarding their clinicopathological characteristics and molecular underpinnings in the published literature. Herein, we report two mucinous borderline tumors and review the literature pertaining to these tumors. The patients were women who presented with flank pain without any history of nephrolithiasis. Abdominal computed tomography (CT) showed multiloculated cystic lesions without mural nodules involving the kidney (Bosniak category III). The nephrectomy specimens showed multiloculated cysts filled with mucoid material, without any solid areas. On microscopic examination, the cystic spaces were lined predominantly by a single layer of mucinous epithelium with variable foci of epithelial stratification. Extensive sampling of the tumors did not show any evidence of invasion. The tumor cells were positive for keratin 7, keratin 20, and CDX2 and were negative for urothelial markers. Molecular analysis performed on one of the tumors showed loss of function mutation of SMAD4 p.S32fs. The presence of SMAD4 alterations alludes to the potential role of the SMAD4-TGFß pathway in the pathogenesis of this tumor. On clinical follow-up, both patients are well with no evidence of disease. Our case report expands on the clinical characteristics of these rare tumors and provides insight into the previously unreported molecular landscape of this tumor.

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Mucinous adenocarcinoma in kidneys with developmental anomalies - a report of two cases

Gali,

Chawla,

Surag

et al. 2024

BMC Urol

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Background Primary mucinous adenocarcinomas of the kidney are rare and pose a challenge for preoperative diagnosis. The histogenesis of these tumours remains largely unknown, with three proposed theories: chronic irritation, differentiation of celomic epithelium, and kidney maldevelopment. Here, we present two cases of renal mucinous adenocarcinoma in patients with developmental renal anomalies, specifically a duplex collecting system and a horseshoe kidney. Case Presentation First, A 50-year-old male presented with loin pain and jelly-like discharge in urine with a duplex collecting system and gross hydronephrosis of the upper moiety on imaging. The patient underwent upper polar nephrectomy with controlled drainage of 1.5 L of mucinous fluid. Histopathology was suggestive of mucinous borderline cystic neoplasm with invasive microcarcinoma. The patient presented one year later, with a hydronephrotic lower moiety of the left kidney and a rectus abdominis mass. Fine needle aspiration biopsy of the mass revealed papillary adenocarcinoma with histological resemblance to the renal pelvis lesion, establishing it as a metastasis from the primary renal malignancy. Second, A 53-year-old male who had undergone right laparoscopic cyst deroofing for a symptomatic renal cyst, whose postoperative histology revealed findings consistent with mucinous adenocarcinoma, presented with flank pain and palpable retroperitoneal mass. Imaging revealed a horseshoe kidney morphology with a large multilobulated hypodense non-enhancing cystic lesion arising from the right kidney. Cyst excision with right open radical nephrectomy was performed. Gross examination revealed multiple cystic spaces replacing renal parenchyma, filled with gelatinous material. Microscopy was suggestive of recurrent mucinous adenocarcinoma. Conclusions Renal mucinous cystadenocarcinomas can be associated with anomalous kidneys. Definitive diagnosis relies on histopathology, and these tumours are recognized for their aggressive nature. Complete resection is the preferred treatment, but further studies are needed to assess the efficacy of adjuvant treatment, given the poor prognosis and high likelihood of recurrence. Clinical trial number Not applicable.

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Mucinous cystadenocarcinoma in the renal pelvis: primary or secondary? Case report and literature review

Cited by 8 publications

References 11 publications

Parenchymal mucinous cystadenoma of the kidney: a case report and literature review

Parenchymal mucinous cystadenoma of the kidney: a case report and literature review

Mucinous Borderline Tumors of the Pelvicalyceal System: Report of Two Tumors With Molecular Findings and Review of Literature

Mucinous adenocarcinoma in kidneys with developmental anomalies - a report of two cases

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