Showing papers on "Mucinous tubular and spindle cell carcinoma published in 2015"
TL;DR: To characterise the clinical, radiological and histological features of mucinous tubular and spindle cell carcinoma (MTSCC), as well as oncological outcomes.
Abstract: Objective
To characterise the clinical, radiological and histological features of mucinous tubular and spindle cell carcinoma (MTSCC), as well as oncological outcomes.
Patients and methods
This is a single institution retrospective analysis of all patients with MTSCC from 2002 to 2011. Patients were excluded if MTSCC could not be confirmed on pathology re-review (four patients). Clinical characteristics, pathology, imaging, and outcomes were reviewed for the 19 included patients.
Results
The median (range) age at diagnosis was 59 (17–71) years with a female predominance (78.9%). On contrast-enhanced computed tomography, MTSCC enhanced less than the cortex during the corticomedullary phase. The mean (range) tumour attenuation was 36 (24–48), 67 (41–133), 89 (49–152), and 76 (52–106) Hounsfield units in the pre-contrast, corticomedullary, nephrographic and excretory phases, respectively. In all, 16 patients were treated with partial (five patients) or radical nephrectomy (11) for pT1 (62.5%), pT2 (31.3%), and pT3a disease (6.3%). One patient underwent active surveillance. Of three patients (13.0%) managed with energy ablation, there was one recurrence that was treated with salvage surgery. One patient (5.3%) had metastatic disease at diagnosis and died from disease 64.7 months later. A patient with a pT2bN0M0 MTSCC with sarcomatoid dedifferentiation developed bone metastases 9.5 months after diagnosis and was alive at 19.0 months. The remainder were free of recurrence or progression.
Conclusion
MTSCC is a rare renal cell carcinoma (RCC) variant. In this largest series to date, MTSCC presented at a broad range of ages and displayed a female predilection. Imaging and pathological features of MTSCC display some overlap with papillary RCC. MTSCC is associated with excellent outcomes overall, but is not universally indolent.
41 citations
TL;DR: The morphologic, genetic, and molecular findings in RCCs previously recognized by the WHO are discussed, and clues to distinction from each other and some of the newer subtypes of RCC are provided.
39 citations
TL;DR: As morphological features and IHC are heterogeneous, TRCC-like renal tumours can only be sub-classified accurately by multi-parameter molecular-genetic analysis.
Abstract: Xp11.2-translocation renal carcinoma (TRCC) is suspected when a renal carcinoma occurs in young patients, patients with a prior history of exposure to chemotherapy and when the neoplasm has morphological features suggestive of that entity. We retrieved 20 renal tumours (from 17,500 archival cases) of which morphology arose suspicion for TRCC. In nine cases, TFE3 translocation was confirmed by fluorescence in situ hybridisation analysis. In 9 of the remaining 11 TRCC-like cases (7 male, 4 female, aged 22-84 years), material was available for further study. The morphological spectrum was diverse. Six tumours showed a mixture of cells with eosinophilic or clear cytoplasm in tubular, acinar and papillary architecture. One case was high grade with epithelioid, spindle cell and sarcomatoid areas. Another showed tubular, solid, and papillary areas and foci containing spindle cells reminiscent of mucinous tubular and spindle cell carcinoma. The third showed dyscohesive nests of large epithelioid and histiocytoid cells in a background of dense lymphoplasmacytic infiltrate. By immunohistochemistry, keratin AE1/AE3 was diffusely positive in three tumours, while CK7 strongly stained one tumour and another focally and weakly. CD10 and Pax8 were expressed by eight, AMACR and vimentin by seven, CA-IX by four and TFE3 and cathepsin K by two tumours. Of the two TFE3-positive tumours, one showed polysomy of chromosome 7 and the other of 17; they were VHL normal and diagnosed as unclassifiable RCC. Of the seven TFE3-negative tumours, three showed polysomy of 7/17 and VHL abnormality and were diagnosed as combined clear cell RCC/papillary RCC. One TFE3-negative tumour with normal 7/17 but LOH 3p (VHL abnormality) was diagnosed as clear cell RCC. One TFE3-negative tumour with polysomy 7/17 but normal VHL was diagnosed as papillary RCC, and two with normal chromosomes 7/17 and VHL gene were considered unclassifiable. As morphological features and IHC are heterogeneous, TRCC-like renal tumours can only be sub-classified accurately by multi-parameter molecular-genetic analysis.
26 citations
TL;DR: Collecting duct carcinoma and renal medullary carcinoma are the most aggressive forms of renal cancer but recent evidence suggests they may respond to targeted therapy so their recognition becomes crucial to the management of these patients.
22 citations
TL;DR: Unenhanced and dynamic MDCT may aid in diagnosis and differentiation of MTSCC and CDC of the kidney and identification of such findings may improve diagnosis of these two rare tumours.
Abstract: Objective:To characterize the multidetector CT (MDCT) imaging characteristics of mucinous tubular and spindle cell carcinoma (MTSCC) and collecting duct carcinoma (CDC) of the kidney.Methods:21 patients with MTSCC and 18 patients with CDC were studied retrospectively. MDCT was undertaken to investigate differences in tumour characteristics.Results:Five patients with MTSCC had calcifications as did nine patients with CDC (p = 0.108). In three patients with MTSCC and four patients with CDC, the tumours had a clear boundary (p = 0.682). No patient with MTSCC had retroperitoneal lymph node metastasis as did five patients with CDC (p = 0.015). 16 patients with MTSCC showed homogeneous enhancement, whereas 11 patients with CDC showed heterogeneous enhancement (p = 0.025). The attenuation value of CDC tumours was greater than that of MTSCC and normal renal parenchyma on an unenhanced CT (p = 0.027). MTSCC and CDC tumour enhancement was less than the normal renal cortex and medulla in all phases (p < 0.001). Tumo...
9 citations
TL;DR: Although rarely encountered in FNA cytology of the kidney, it is believed the cytologic features of this tumor are distinctive and are different from conventional and other subtypes of RCC.
Abstract: Renal mucinous tubular and spindle cell carcinoma (MTSCC) was recently described as a distinct subtype of renal cell carcinoma (RCC) in the 2004 World Health Organization classification of kidney tumors. MTSCC is a rare low grade malignancy with < 100 cases reported in the literature. To the best of our knowledge, there are 5 case reports with a total of 6 patients describing its diagnosis by fine needle aspiration (FNA). All of these cases were diagnosed as conventional RCC on FNA. Subsequent excisions proved them to be MTSCC. We herein report a case in a 67-year-old male. He presented with abdominal pain and was found to have a new colon adenocarcinoma with metastasis to the liver and lungs. The extent of disease made the patient ineligible for surgical excision, and he received chemotherapy. Work-up also revealed a kidney mass which was later biopsied by FNA and core biopsy. The tumor was composed of epithelial and spindled cell components embedded in a myxoid background. It was positive for CK7, AMCAR, vimentin, and epithelial membrane antigen. The tumor was diagnosed as MTSCC. One year later the kidney mass remained stable. However, the patient developed new metastasis to the liver from colonic primary. The kidney mass was not resected. Although rarely encountered in FNA cytology of the kidney, we believe the cytologic features of this tumor are distinctive and are different from conventional and other subtypes of RCC. Therefore, its accurate diagnosis on FNA is possible once pathologists are aware that MTSCC should be considered in the differential diagnosis of kidney tumors.
7 citations
TL;DR: A case of a massive, bilateral MTSCC with histologic findings of heterotopic bone formation, which has not been described before is reported.
Abstract: Renal cell carcinoma (RCC) is the most common kidney malignancy, with many histologic subtypes One of the rare forms of RCC is mucinous tubular and spindle cell carcinoma (MTSCC), which is newly described with limited information on clinical picture and outcome Heterotopic bone formation (osseous metaplasia) is a rare finding within any renal mass Here we report a case of a massive, bilateral MTSCC with histologic findings of heterotopic bone formation, which has not been described before
5 citations
Journal Article•
TL;DR: Since MTSCC-K is a rare low-grade malignancy, with unique histological and immunohistochemical characteristics, it is important for clinicians and pathologists to have a defined awareness of this tumor type in order to decrease the rate of misdiagnosis.
Abstract: Mucinous tubular and spindle cell carcinoma of the kidney (MTSCC-K) is an unusual renal tumor. It is important to increase the recognition of the clinicopathological features of MTSCC-K and improve its clinical and differential diagnosis. This report described four cases of MTSCC-K with clinical, imaging, and pathological examination and showed that the tumor boundaries of MTSCC-K were clear, and tumor cells arranged into tubules and cord-like beams, between which was lightly stained myxoid stroma. The tumor cells were smaller and cube- or oval-shaped, with single small eosinophilic nucleoli, low-grade nuclei, and little nuclear fission. The myxoid stroma was scattered around lymphocytes and plasma cells. Immunohistochemical markers including CK7, CD117, EMA (epithelial membrane antigen), vimentin, and CK8/18, showed positive expression in tumor cells, but the tumor cells were negative for CD10 and villin. The proliferation index of Ki-67 was 5-10%. Since MTSCC-K is a rare low-grade malignancy, with unique histological and immunohistochemical characteristics, it is important for clinicians and pathologists to have a defined awareness of this tumor type in order to decrease the rate of misdiagnosis.
4 citations
Journal Article•
TL;DR: Analysis of clinicopathologically characterize two cases of kidney MTSpCC microscopically showed multiple elongated tubular branches of tumor cells that are closely arranged in cord-like manner under lightly stained myxoid stroma.
Abstract: Kidney mucinous tubular and spindle cell carcinoma (MTSpCC) is a rare renal low-grade pleomorphic epithelial neoplasm featured by tubular and spindle cells with a relatively indolent behavior. This study was designed to clinicopathologically characterize two cases of kidney MTSpCC. Similar to other tumors, the data showed the diagnosis of MTSpCC relies on histological examination. Tumor cells stained strongly for CK19, CK20, and CK7 within the epithelioid component. Whereas evaluating MTSpCC clinically showed no specific symptoms, analyzing MTSpCC microscopically showed multiple elongated tubular branches of tumor cells that are closely arranged in cord-like manner under lightly stained myxoid stroma. MTSpCC also has the spindle cell area; the single tumor cell is small and nucleus round or oval. Immunohistochemical analysis of cytokeratins, electron microscopy, or genetic tests all improves the diagnosis.
4 citations
TL;DR: A 40-year-old female presenting with right flank pain and well-circumscribed renal mass confined to the lower pole is reported, which consisted of tubules and cords separated by pale mucinous material in some areas, whereas other areas showed dense cellularity without significant mucin.
Abstract: Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare and unusual variant of renal cell carcinoma (RCC). It is important to differentiate this from the other variants particularly papillary RCC since MTSCC is generally low-grade, has low malignant potential and favorable prognosis. We are reporting a 40-year-old female presenting with right flank pain and well-circumscribed renal mass confined to the lower pole. Tumor consisted of tubules and cords separated by pale mucinous material in some areas, whereas other areas showed dense cellularity without significant mucin. The tumor cells were cuboidal or spindle and did not show significant atypical features. The patient underwent nephrectomy and is asymptomatic clinically and radiologically after 2 years.
1 citations
TL;DR: This case highlights the usefulness of (18)FDG-PET/CT in the staging of MTSCC of the kidney and provides 81-89% sensitivity and 83-100% specificity in primary staging of the renal cancers, in diagnosing metastatic RCC, and monitoring the response to therapy.
Abstract: Mucinous tubular and spindle cell carcinoma of the kidney (MTSCC-K) is an extremely rare carcinoma of the kidney, which develops by malignant differentiation of the collecting ducts or the loop of Henle. The tumor can occur at all ages, with 4 times higher prevalence in females than in males. A 62-year-old female patient presented with an asymptomatic mass lesion in the left kidney. The ultrasound revealed a hyperechogenic mass lesion measuring 4 cm in diameter and centrally located in the left kidney. Computed tomography (CT) revealed an isodense mass lesion measuring 4 cm in diameter and posterolaterally located in the left kidney. Magnetic resonance imaging revealed a renal mass of 4 cm in diameter with hypervascular contrast uptake. For primary staging, (18)F-fluorodeoxyglucose-positron-emission tomography ((18)FDG-PET)/CT revealed left renal cell carcinoma (RCC) with a maximum standard uptake value of 6.7. The patient underwent transperitoneal radical nephrectomy. The immunohistochemical examination revealed MTSCC-K. (18)FDG-PET/CT provides 81-89% sensitivity and 83-100% specificity in primary staging of the renal cancers, in diagnosing metastatic RCC, and monitoring the response to therapy. Its use in the staging of MTSCC of the kidney has not been previously described in the literature. This case highlights the usefulness of (18)FDG-PET/CT in the staging of this rare tumor.
TL;DR: The present report aimed to highlight the pathological characteristics of this rare subtype of renal cell tumors and to point out the differential diagnosis – particularly with the papillary renal cell carcinoma, given the different prognosis and therapeutic approach of these two entities.
Abstract: Mucinous tubular and spindle cell carcinoma (MTSCC) of the kidney, a rare renal cell carcinoma, is a low grade malignant tumor with histogenesis still under debate. We report the case of a 60-years-old female, asymptomatic, with no significant personal or family history, diagnosed on routine ultrasound examination with a unilateral non-homogenous mass in the right kidney. On gross examination the tumor was solid, well circumscribed, and limited to the kidney parenchyma. Microscopically, the tumor was composed from eosinophilic small cuboidal cell arranged in small, tightly packed, parallel tubular structures, myxoid stroma and mucinous areas (PAS and Alcian Blue positive). Therefore, the differential diagnosis between MTSCC and papillary renal cell carcinoma was compulsory. The immunohistochemistry assays confirmed MTSCC as the final diagnosis, based on the intense positive reaction for CK7 and negative reaction for CD10. The present report aimed to highlight the pathological characteristics of this rare subtype of renal cell tumors and to point out the differential diagnosis – particularly with the papillary renal cell carcinoma, given the different prognosis and therapeutic approach of these two entities.
Journal Article•
TL;DR: A case of MTSCC is reported in a female aged 54years who presented with a long standing vague loin pain and with the preoperative diagnosis of RCC followed by nephrectomy showed a completely different histology of a rare tumour with tubules, spindle cells and mucinous stroma consistent with M TSCC, a low grade tumours with good prognosis.
Abstract: Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare and recently described histologic variant of renal cell carcinoma (RCC). Usually considered as a tumor of low malignant potential it is important to appreciate the characteristic histologic features to arrive at the correct diagnosis. We report a case of MTSCC in a female aged 54years who presented with a long standing vague loin pain. Diagnostic work-up showed a renal mass suggestive of RCC. With the preoperative diagnosis of RCC followed by nephrectomy showed a completely different histology of a rare tumour with tubules, spindle cells and mucinous stroma consistent with MTSCC, a low grade tumour with good prognosis.
01 Jan 2015
TL;DR: It was shown that the tumor boundaries of MTSCC-K were clear, and tumor cells arranged into tubules and cord-like beams, between which was lightly stained myxoid stroma, and the proliferation index of Ki-67 was 5-10%.
Abstract: Mucinous tubular and spindle cell carcinoma of the kidney (MTSCC-K) is an unusual renal tumor. It is im- portant to increase the recognition of the clinicopathological features of MTSCC-K and improve its clinical and differ- ential diagnosis. This report described four cases of MTSCC-K with clinical, imaging, and pathological examination and showed that the tumor boundaries of MTSCC-K were clear, and tumor cells arranged into tubules and cord-like beams, between which was lightly stained myxoid stroma. The tumor cells were smaller and cube- or oval-shaped, with single small eosinophilic nucleoli, low-grade nuclei, and little nuclear fission. The myxoid stroma was scattered around lymphocytes and plasma cells. Immunohistochemical markers including CK7, CD117, EMA (epithelial mem- brane antigen), vimentin, and CK8/18, showed positive expression in tumor cells, but the tumor cells were negative for CD10 and villin. The proliferation index of Ki-67 was 5-10%. Since MTSCC-K is a rare low-grade malignancy, with unique histological and immunohistochemical characteristics, it is important for clinicians and pathologists to have a defined awareness of this tumor type in order to decrease the rate of misdiagnosis.
TL;DR: A case of a 50-year-old female that revealed a renal mass on an abdominal echography, incidentally discovered during a hepatic hemangioma follow-up, and after pathological evaluation the final diagnosis of MTSCC was made.
Abstract: Mucinous tubular and spindle cell carcinoma (MTSCC) of the kidney is a rare renal cancer type, recently recognized by the World Health Organization (WHO) in 2004, with indolent behavior. We present a case of a 50-year-old female that revealed a renal mass on an abdominal echography, incidentally discovered during a hepatic hemangioma follow-up. The patient underwent partial nephrectomy, and after pathological evaluation the final diagnosis of MTSCC was made. MSTCC is a special renal cancer type, with low degree of malignancy and a favorable prognosis. Surgical resection is the treatment of choice. It’s important to be aware of this entity in order to differentiate MTSCC with renal cancers of less favorable prognosis and higher degree of malignancy like papillary renal cell carcinoma.