Composite renal cell carcinoma and angiomyolipoma in a patient with Tuberous sclerosis: A diagnostic dilemma

Tuberous sclerosis (TS) is an autosomal dominant disorder associated with various renal pathologies, including angiomyolipoma (AML). Renal cell carcinoma (RCC) with concomitant AML is rare. We report a case of composite RCC and AML in a young male with TS. This 25-year-old male presented with an abdominal mass. The abdominal computed tomography scan revealed RCC in the left kidney and AML in right kidney. A left radical nephrectomy was performed. Microscopy showed a tumour composed of closely intermingled RCC and AML components. Immunohistochemistry was performed for confirmation. A diagnosis of composite tumour with clear cell RCC and AML was made. Though the coexistence of RCC with AML is rare, and the composite variant is even rarer, one must be aware of this entity to prevent misdiagnosis as well as upstaging of RCC, and also to avoid missing areas of RCC within a large AML, especially in TS patients.

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A case of high-grade mucinous tubular and spindle cell carcinoma

Journal of Surgical Case Reports ◽

10.1093/jscr/rjaa014 ◽

2020 ◽

Vol 2020 (2) ◽

Author(s):

Koujin Miura ◽

Yasushi Adachi ◽

Toshiaki Shirahase ◽

Yoji Nagashima ◽

Kazuki Suemune ◽

...

Keyword(s):

Renal Cell Carcinoma ◽

Cell Carcinoma ◽

Renal Cell ◽

Poor Prognosis ◽

Spindle Cell ◽

Left Kidney ◽

Spindle Cell Carcinoma ◽

Low Grade ◽

High Grade ◽

Tomography Scan

Abstract Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare renal cell carcinoma that initially presents as low-grade renal cell carcinoma. However, cases of MTSCC with high-grade histology and poor prognosis have been reported. Here, we report a case of MTSCC with high-grade histological features and metastasis. A 77-year-old woman consulted a hospital following frequent and painful micturition. Computed tomography scan revealed a tumor of the left kidney. First, chemotherapy was performed, with no effects. Therefore, nephrectomy was subsequently performed. Histologically, the tumor showed the features of MTSCC with sarcomatoid component. Metastasis of the tumor into the lymph node was also observed. Although adjuvant chemotherapy was performed after nephrectomy, metastasis to the lungs and bone and local recurrence was observed. The patient is still alive 2 years after nephrectomy with metastasis and recurrence of the tumor. High-grade MTSCC shows a relatively poor prognosis, specifically MTSCC with metastasis upon nephrectomy.

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Renal Cell Carcinoma in Tuberous Sclerosis Complex

Genes ◽

10.3390/genes12101585 ◽

2021 ◽

Vol 12 (10) ◽

pp. 1585

Author(s):

Elizabeth P. Henske ◽

Kristine M. Cornejo ◽

Chin-Lee Wu

Keyword(s):

Renal Cell Carcinoma ◽

Cell Carcinoma ◽

Tuberous Sclerosis ◽

Tuberous Sclerosis Complex ◽

Renal Cell ◽

Age At Onset ◽

Autosomal Dominant Disorder ◽

Pathologic Features ◽

Female Predominance ◽

Sporadic Counterpart

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder in which renal manifestations are prominent. There are three major renal lesions in TSC: angiomyolipomas, cysts, and renal cell carcinoma (RCC). Major recent advances have revolutionized our understanding of TSC-associated RCC, including two series that together include more than 100 TSC-RCC cases, demonstrating a mean age at onset of about 36 years, tumors in children as young as 7, and a striking 2:1 female predominance. These series also provide the first detailed understanding of the pathologic features of these distinctive tumors, which include chromophobe-like features and eosinophilia, with some of the tumors unclassified. This pathologic heterogeneity is distinctive and reminiscent of the pathologic heterogeneity in Birt–Hogg–Dube-associated RCC, which also includes chromophobe-like tumors. Additional advances include the identification of sporadic counterpart tumors that carry somatic TSC1/TSC2/mTOR mutations. These include unclassified eosinophilic tumors, eosinophilic solid cystic RCC (ESC-RCC), and RCC with leiomyomatous stroma (RCCLMS). A variety of epithelial renal neoplasms have been identified both in patients with tuberous sclerosis complex (TSC) and in the nonsyndromic setting associated with somatic mutations in the TSC1 and TSC2 genes. Interestingly, whether tumors are related to a germline or somatic TSC1/2 mutation, these tumors often display similar morphologic and immunophenotypic features. Finally, recent work has identified molecular links between TSC and BHD-associated tumors, involving the TFEB/TFE3 transcription factors.

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Primary Leiomyosarcoma of the Kidney

Pathology Research International ◽

10.4061/2010/652398 ◽

2010 ◽

Vol 2010 ◽

pp. 1-4 ◽

Cited By ~ 5

Author(s):

Kusuma Venkatesh ◽

Monika Lamba Saini ◽

S. R. Niveditha ◽

Chaithra Krishnagiri ◽

Sudarshan Babu

Keyword(s):

Renal Cell Carcinoma ◽

Smooth Muscle ◽

Cell Carcinoma ◽

Renal Cell ◽

Spindle Cell ◽

Smooth Muscle Actin ◽

Abdominal Mass ◽

Left Kidney ◽

Spindle Cells ◽

Primary Leiomyosarcoma

Primary leiomyosarcoma of the kidney is a rare tumor with an aggressive behaviour. A 55-year-old woman presented with a left sided abdominal mass in our outpatient department. Radiologic investigations revealed the mass to be renal in origin with colonic adhesions for which radical nephrectomy and hemicolectomy were done. The tumor completely appeared to replace the left kidney and had a whorled character focally on cut section. Microscopically, spindle cells having malignant features with cigar shaped nuclei were seen. The smooth muscle origin of the cells was confirmed by immunohistochemical positivity for smooth muscle actin. Sarcomatoid variant of the renal cell carcinoma was ruled out as the tumor was negative for cytokeratin. Tumors with spindle cell morphology in the kidney should not always be taken for a sarcomatoid variant of renal cell carcinoma and should be investigated thoroughly.

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Collecting Duct Carcinoma: A Rare Entity

Qanun Medika - Medical Journal Faculty of Medicine Muhammadiyah Surabaya ◽

10.30651/jqm.v4i1.3083 ◽

2020 ◽

Vol 4 (1) ◽

pp. 129

Author(s):

Ferdinant Martinus Djawa ◽

Anny Setijo Rahaju

Keyword(s):

Renal Cell Carcinoma ◽

Cell Carcinoma ◽

Renal Cell ◽

Histopathological Examination ◽

Collecting Duct ◽

Abdominal Mass ◽

Left Kidney ◽

Renal Tumors ◽

Duct Carcinoma ◽

Collecting Duct Carcinoma

ABSTRACTCollecting duct carcinoma is a rare and highly aggressive subtype of renal cell carcinoma. The incidence rate is less than 1-2% of all renal tumors and usually, affect middle-aged adult, commonly in men. We reported a 76-year-old man complains of an intermittent painless gross hematuria, abdominal mass and left flank pain for approximately three months. The CT abdomen showed a slightly enhancing solid mass in the left kidney and para-aorta lymphadenopathy. Cut surfaces of the kidney showed a solid-cystic and ill-defined greyish-white tumor. Microscopically, tumor formed solid sheets and tubulopapillary structures lined by neoplastic cells, hobnailing nuclei, abnormal mitotic, and a desmoplastic stroma with lymphoplasmacytic infiltration, and the immunochemical profile were PAX8 (+) /p63 (-). Based on these findings, the diagnosis was a collecting duct carcinoma. This tumor arising from the collecting duct of Bellini in the renal medulla, accounts for less than 1-2% of all renal masses and important to be distinguished from other tumors due to differences in prognosis and therapeutic. Histopathological examination is needed to establish the diagnosis. A case of collecting duct carcinoma that occurred in a 76-year-old man has been reported. A definitive diagnosis can only be done with a detailed histopathological examination for patient management benefits.Keywords : renal cell carcinoma, collecting duct carcinoma, urothelial carcinoma, PAX8, p63

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Editorial Comment from Dr Kondo to Clinical utility of head computed tomography scan during systemic therapy for metastatic renal cell carcinoma

International Journal of Urology ◽

10.1111/iju.14520 ◽

2021 ◽

Author(s):

Tsunenori Kondo

Keyword(s):

Computed Tomography ◽

Renal Cell Carcinoma ◽

Editorial Comment ◽

Cell Carcinoma ◽

Systemic Therapy ◽

Renal Cell ◽

Clinical Utility ◽

Computed Tomography Scan ◽

Head Computed Tomography ◽

Tomography Scan

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Residents' teaching files. Papillary renal cell carcinoma: diagnostic dilemma of a cystic renal mass.

Radiographics ◽

10.1148/radiographics.17.4.9225396 ◽

1997 ◽

Vol 17 (4) ◽

pp. 993-998 ◽

Cited By ~ 5

Author(s):

S C Roberts ◽

A B Winick ◽

M R Santi

Keyword(s):

Renal Cell Carcinoma ◽

Cell Carcinoma ◽

Renal Cell ◽

Renal Mass ◽

Papillary Renal Cell Carcinoma ◽

Diagnostic Dilemma ◽

Teaching Files

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Tubulocystic renal cell carcinoma: Report of a rare case

Canadian Urological Association Journal ◽

10.5489/cuaj.2935 ◽

2015 ◽

Vol 9 (9-10) ◽

pp. 654 ◽

Cited By ~ 2

Author(s):

Aanchal Kakkar ◽

Mehar C. Sharma ◽

Manpreet Uppal ◽

Sunil Chumber

Keyword(s):

Renal Cell Carcinoma ◽

Cell Carcinoma ◽

Renal Cell ◽

Histopathological Examination ◽

Cystic Neoplasm ◽

Diagnostic Challenge ◽

Elderly Male ◽

Diagnostic Dilemma ◽

Cystic Neoplasms ◽

Atypical Cells

Cystic neoplasms of the kidney are rare, and present a unique diagnostic challenge. We report the case of an elderly male who presented with a large cystic neoplasm, which was a diagnostic dilemma clinically and radiologically. Histopathological examination showed a tumour composed of variably sized tubules lined by atypical cells having large round nuclei with prominent nucleoli. Hobnailing was seen at places. Tumour cells were immunopositive for pancytokeratin, vimentin, CD10, CK19 and AMACR, confirming a diagnosis of tubulocystic renal cell carcinoma (TC-RCC).

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Synchronous Esophageal and Renal Malignancies with Unexpected Site of Metastasis: A Case Report

IIUM Medical Journal Malaysia ◽

10.31436/imjm.v18i2.674 ◽

2020 ◽

Vol 18 (2) ◽

Author(s):

Sharhanin Bahrudin

Keyword(s):

Renal Cell Carcinoma ◽

Case Report ◽

Cell Carcinoma ◽

Renal Cell ◽

Obstructive Uropathy ◽

Left Kidney ◽

Gastroesophageal Junction ◽

Wall Thickening ◽

Solid Organ ◽

Synchronous Malignancies

Introduction: Esophageal cancer is the fifth most common cancer in Asia. Synchronous malignancies with an esophageal malignancies is not uncommon however synchronous esophageal malignancy and renal cell carcinoma is rare. Case Report: A 70 years old non smoker man with comorbid of hypertension and diabetes mellitus presented with multiple episode of hemoptysis and pleuritic chest pain within one day duration. He also complaint of worsening dysphagia to solid food for 1 month duration with constitutional symptoms. Otherwise he does not have any other symptoms. Clinically he was pale and cachexic, otherwise all systemic examination was unremarkable. His blood investigation reveal low hemoglobin level of 9.9g/L with normal coagulation and renal profile. He underwent Oesophagoduodenoscopy which showed a tumor narrowing the lumen of the lower esophagus. Biopsy of the tumor revealed esophageal adenocarcinoma. CT thorax, abdomen and pelvis showed smooth circumferential wall thickening of the distal part of the esophagus near the gastroesophageal junction. Incidentally, there were heterogenous enhancing lobulated mass seen at the lower pole of left kidney with no obstructive uropathy features. Otherwise, others solid organ were normal. A left renal mass biopsy performed revealed renal cell carcinoma. An oncologist opinion was gathered for initiation of palliative chemotherapy however he developed symptomatic malignant pleural effusion which need a drainage. HRCT Thorax revealed multiple bilateral intrapulmonary nodule. He also complaint of a new right upper eyelid mass that increasing in size with contact bleeding however does not affect his visual. An assessment from an ophthalmologist and CT orbital performed consistent with features of a metastasis. Conclusion: Synchronous malignancies with an esophageal malignancies is not uncommon. Most common site reported is head and neck followed by lung malignancies. However synchronous esophageal malignancies and renal cell carcinoma is rare. Esophageal malignancies often have distant metastasis to the liver, lung and bone however rare to the eyelid.

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