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1.
Autops. Case Rep ; 11: e2021288, 2021. tab, graf
Article in English | LILACS | ID: biblio-1249005

ABSTRACT

Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare variant of the inflammatory myofibroblastic tumor. It has an aggressive clinical course and a high rate of recurrence. EIMS primarily affects children and young adults. Hereby, we report this entity in a 4-month-old infant who presented with an abdominal mass. Imaging studies revealed a large hypodense mesentery-based lesion involving the right half and mid-region of the abdomen. The mass with an attached segment of the small bowel was excised in toto. Grossly, a large encapsulated tumor was identified arising from the mesentery of the small bowel. The histological examination showed a tumor consisting of epithelioid to spindle cells loosely arranged in a myxoid background with numerous blood vessels and lymphoplasmacytic inflammatory infiltrate. On immunohistochemistry, the tumor cells showed positivity for ALK1 (nuclear), desmin, SMA, CD68, and focal positivity for CD30. A final diagnosis of EIMS of the small intestine was rendered. To the best of our knowledge, this case is the youngest reported case in literature.


Subject(s)
Humans , Female , Infant , Sarcoma , Intestinal Neoplasms/pathology , Immunohistochemistry , Epithelioid Cells/pathology , Anaplastic Lymphoma Kinase , Intestine, Small , Mesentery
2.
Article | IMSEAR | ID: sea-196437

ABSTRACT

Monotypic angiomyolipoma is usually found in the kidneys and is composed predominantly of epithelioid cells which show positivity for melanocyte and smooth muscle markers. It can pose a diagnostic challenge due to a range of differential diagnosis. We report the second case of monotypic angiomyolipoma of nasal cavity and first from India in a 54-year-old male who presented with a nasal polyp. Grossly the tumor was well circumscribed and un-encapsulated. Microscopy showed a large number of epithelioid cells mixed with a few spindle cells, varying sized blood vessels, and focal areas of adipose tissue. Immunohistochemistry was positive for smooth muscle actin (SMA) and human melanoma black (HMB-45) stains. It is important to identify this tumor as it can sometimes be mistaken for malignancy and only needs endoscopic resection.

3.
Autops. Case Rep ; 10(4): e2020190, 2020. graf
Article in English | LILACS | ID: biblio-1131850

ABSTRACT

Epithelioid angiomyolipoma is an uncommon subtype of renal angiomyolipoma associated with potentially malignant behavior and is considered a distinct entity by the World Health Organization classification of renal tumors. We present a case of an epithelioid variant of angiomyolipoma with extension into the renal vein, inferior vena cava reaching up to the right atrium. Pre-operatively, a diagnosis of renal cell carcinoma was considered based on imaging findings. Intra-operatively due to extensive adhesions, surgical resection was not performed and only tissue sampling was performed for histopathology. Microscopic examination revealed short fascicles of spindle cells and perivascular epithelioid cells. A differential diagnosis of renal cell carcinoma with sarcomatoid differentiation was considered. The immunohistochemical profile showed tumor cells that express Melan-A and smooth muscle actin, while they were negative for pan-cytokeratin, PAX8, CK7, CD117 and CD34. Therefore a diagnosis of epithelioid angiomyolipoma was rendered. The presence of intravascular thrombi on radiological investigation and carcinoma-like growth pattern on light microscopy may compound an erroneous diagnosis of renal cell carcinoma. Hence, it is prudent for the urologist to consider differential diagnosis other than renal cell carcinoma when confronted with a renal neoplasm presenting with intravascular thrombi. In these cases, a core biopsy should be planned pre-operatively and diagnosis should be made with aid of appropriate immunohistochemical markers.


Subject(s)
Humans , Female , Adult , Epithelioid Cells/pathology , Angiomyolipoma/pathology , Kidney Neoplasms/pathology , Carcinoma, Renal Cell , Diagnosis, Differential
4.
Chinese Journal of Pathology ; (12): 112-115, 2019.
Article in Chinese | WPRIM | ID: wpr-810448

ABSTRACT

Objective@#To elucidate the clinicopathologic characteristics of atypical epithelioid trophoblastic lesions with cyst and fistula formation after cesarean section.@*Methods@#The clinical and pathological data of 4 cases of post-cesarean atypical epithelioid trophoblastic lesions with cyst and fistula formation diagnosed at Women′s Hospital, School of Medicine, Zhejiang University during April 2007 to June 2018 were evaluated by hematoxylin and eosin stain and EnVision two-step immunohistochemical staining technique.@*Results@#The age of the 4 patients ranged from 32 to 41 years, with a mean age of 36.5 years. Three patients recieved cystectomy and one underwent subtotal hysterectomy. Histologically, the lesions were well circumscribed and consisted of uniform cells of medium size, irregularly enlarged with hyperchromatic nuclei and 1 to 2 inconspicuous nucleoli embedded in abundant hyalinized matrix with fibrinoid material in the center. The cells exhibited immunohistochemical feature of chorionic-type intermediate trophoblastic cells (CK18+, p63+ and CD146-). All patients were alive without recurrence during follow-up of 1 to 40 months (mean=22 months).@*Conclusion@#Atypical epithelioid trophoblastic lesion with cyst and fistula formation after cesarean section has unique histological features, and its biological behavior and prognosis are still unclear, which need further exploration.

5.
Article | IMSEAR | ID: sea-184785

ABSTRACT

Pulmonary tuberculosis is one of the commonest infectious disease encountered in clinical practice and more so in the developing countries. Despite of effectiveness of treatment,it is still a major cause of death. Tuberculosis can effect almost every organ of the body with maximum cases in the lungs accounting to 80%[1]. It is seen that extrapulmonary site have increased morbidity and mortality. Cardiovascular involvement is rare about 1-2%[2] and that too seen usually in the pericardium.The involvement of myocardium is even rarer. Here is a case of autopsy finding in 47 years old female showing myocardial involvement due to tuberculosis. Tubercular findings were also found in the bilateral kidneys.

6.
Article in Chinese | WPRIM | ID: wpr-807822

ABSTRACT

Objective@#To analyze the computed tomography(CT) features and clinical outcomes of renal epithelioid angiomyolipoma(EAML).@*Methods@#From January 2004 to June 2015, 17 patients with EAML in the Second People′s Hospital of Lianyungang and Nanjing Drum Tower Hospital were included in the study.All patients underwent CT examination.The patients' general data, imaging characteristics and pathologic features were determined by chart review.@*Results@#Nine patients underwent radical nephrectomy, and 8 patients underwent partial nephrectomy.The mean maximal tumor diameter was 6.1 cm (ranged 1.2-12.5 cm). The fat component of one lesion was detected by CT.On unenhanced CT, the intratumoral attenuations were hyperattenuating in 9 patients, isoattenuating in 1 patient and hypoattenuating in 7 patients.The contrast enhancement degree was mild in 1 patient, moderate in 6 patients and marked in 10 patients.The contrast enhancement pattern was homogeneous in 8 lesions and heterogeneous in 9 lesions.All patients were positive for melanoma(12 cases were positive for HMB-45, 3 cases were positive for melan A, and 2 cases were positive for both). The mean follow-up period was 28.5 months (ranged 2-126 months), and 15 patients were alive with no evidence of disease at the time of the last follow-up, 1 patient exhibited local recurrence and lung metastases, and another 1 patient developed distant metastasis.@*Conclusion@#Renal EAML has a range of imaging appearances.Our data suggested that the majority of the tumors (size 10 cm) were solid and had a tendency to be hyperattenuating on unenhanced CT images.Hemorrhaging or necrosis was observed in tumors with sizes≥10cm with heterogeneous enhancement.In contrast to classic AML, which is benign, EAML is potentially malignant and exhibits aggressive clinical features, including local recurrence and distant metastasis.

7.
Article in English | WPRIM | ID: wpr-15358

ABSTRACT

BACKGROUND: Apoptosis plays a role in the development of pleural effusion. Caspase-cleaved cytokeratin 18, a marker for epithelial cell apoptosis, was evaluated in pleural effusion. METHODS: A total of 79 patients with pleural effusion were enrolled. The underlying causes were lung cancer (n=24), parapneumonic effusion (n=15), tuberculous effusion (n=28), and transudates (n=12). The levels of M30, an epitope of caspase-cleaved cytokeratin 18, were measured in blood and pleural fluids using enzyme-linked immunosorbent assay along with routine cellular and biochemical parameters. The expression of M30 was evaluated in the pleural tissues using immunohistochemistry for M30. RESULTS: The M30 levels in pleural fluid were significantly higher in patients with tuberculosis (2,632.1+/-1,467.3 U/mL) than in patients with lung cancer (956.5+/-618.5 U/mL), parapneumonic effusion (689.9+/-413.6 U/mL), and transudates (273.6+/-144.5 U/mL; all p<0.01). The serum levels were not significantly different among the disease groups. Based on receiver operating characteristics analysis, the area under the curve of M30 for differentiating tuberculous pleural effusion from all other effusions was 0.93. In the immunohistochemical analysis of M30, all pathologic types of cancer cells showed moderate to high expression, and the epithelioid cells in granulomas showed high expression in tuberculous pleural tissues. CONCLUSION: Caspase-cleaved cytokeratin 18 was most prominently observed in tuberculous pleural effusion and showed utility as a clinical marker. The main source of M30 was found to be the epithelioid cells of granulomas in tuberculous pleural tissues.


Subject(s)
Humans , Apoptosis , Biomarkers , Cytoskeleton , Enzyme-Linked Immunosorbent Assay , Epithelial Cells , Epithelioid Cells , Exudates and Transudates , Granuloma , Immunohistochemistry , Keratin-18 , Keratins , Lung Neoplasms , Pleural Effusion , ROC Curve , Tuberculosis , Tuberculosis, Pleural
8.
Article in English | WPRIM | ID: wpr-106449

ABSTRACT

Perivascular epithelioid cell tumors (PEComas) are a rare, recently described group of mesenchymal tumors, which is composed of distinctive perivascular epithelioid cells. Primary cutaneous PEComas are composed of focally nested or trabecularly arranged groups of epithelioid cells with clear to eosinophilic cytoplasm and round to oval nuclei with obvious nucleoli. Here, we describe the case of a 27-year-old Korean man who was presented with a solitary, cutaneous nodule on his left popliteal fossa. Physical examination revealed a solitary, 1.2x1.0 cm-sized erythematous protruding nodule on his left popliteal fossa. Immunohistochemical evaluation showed that the epithelioid tumor cells were positive for HMB-45 and desmin. These clinical and histological findings were consistent with a diagnosis of primary cutaneous PEComa.


Subject(s)
Adult , Humans , Cytoplasm , Desmin , Eosinophils , Epithelioid Cells , Perivascular Epithelioid Cell Neoplasms , Physical Examination , Skin
9.
Chinese Journal of Nephrology ; (12): 476-480, 2009.
Article in Chinese | WPRIM | ID: wpr-380807

ABSTRACT

Objective To investigate the differentiation of rat bone marrow mesenchymal stem cells (MSCs) to renal tubular epithelial-like cells under different conditions. Methods MSCs were obtained from rat marrow. MSCs were isolated by gradient density centrifugation and plastic adherence and then purified. Surface markers were identified with flow cytometry after amplification in vitro. The purified MSCs of the third passage were cultured respectively as follows: (1) control group: DMEM medium with fetal bovine serum(FBS). (2) all-trans retinoic acid (ATRA) group: DMEM medium with FBS, ATRA and ischemic reperfusion-injured kidney tissue homogenate. (3)combination group: DMEM medium with FBS, ATRA, ischemic reperfusion-injured kidney tissue homogenate, epidermal growth factor (EGF) and bone morphogenetic protein 7 (BMP-7). After 7 days, the MSCs were collected for alkaline phosphatase (AKP) staining, cytokeratin-18 and E-cadherin immunocytochemical analysis. Results The positive rates of the third passage MSCs in CD44, CD90 and CD29 were 97.8%±0.9%, 96.8%±1.4% and 97.6%±2.4%,respectively, but in CD11b/c and CD34 were only 13.2%±0.6% and 1.2%±0.5%. The MSCs in control group were spindle. The MSCs in ATRA group were round and elliptic. The MSCs in combination group became cobblestone-like cells after 7 days. AKP staining showed that tubular epithelial-like cells from MSCs in control group were negative, some above cells in ATRA group were positive and number of above cells increased in combination group. Compared with negative control group, the ratios of cytokeratin-18 positive cells in ATRA group and combination group were respectively increassed by 29.47%±1.08% and 47.52%±2.13% (all P<0.05), the ratios of E-cadherin positive cells in ATRA group and combination group were respectively increased by 14.88%±2.46% and 36.15%±1.13% (all P<0.05). Conclusion MSCs may differentiate by renal tubular epithelial-like cells under the induction of ischemic reperfusion-injured kidney tissue homogenate and ATRA in vitro, which are further differentiated under the combined induction of EGF and BMP-7.

10.
Chinese Journal of Urology ; (12): 744-747, 2008.
Article in Chinese | WPRIM | ID: wpr-397767

ABSTRACT

Objective To study the diagnosis and treatment of malignant renal angiomyolipoma with metastases. Methods Three cases of advanced malignant renal angiomyolipoma with metasta-ses were retrospectively reviewed. Case 1 was a 55-year-old woman presenting with recurrent low-grade fever and aching pain in left flank. Ultrasound showed solid mass in the left kidney. Left radical nephrectomy was then performed. The right pulmonary lobectomy of the inferior lobe and wedge ex-section of superior lobe was performed 7 years later because of multiple pulmonary metastases. Case 2 was a 37-year-old woman. Left nephrectomy was performed because Ultrasound and CT showed left kidney solid mass. Six years later, multi-site metastases were found in liver and retroperitoneum and mestastasis tumors were resected. At 10 years after the primary diagnosis, CT showed multi-metasta-ses in liver and retroperitoneum. The retroperitoneal masses were resected and liver lesions were trea-ted by radiofrequency ablation. Case 3 was a 34-year-old man presenting with swelling pain in right flank. CT scan showed a lesion in the right kidney and right radical nephrectomy was performed. Four months after the surgery, MRI revealed multiple liver and retroperitoneal nodules. All the 3 cases had not been diagnosed with tuberous sclerosis and did not accept chemotherapy. Results The cut sur-face of the lesions was red-brown and yellow and the texture was tender. Under microscopic examina-tion, the tumors of case 1 and case 3 were composed of sheets or nests of large polygonal epithelioid cells. It revealed that occasionally clear cytoplasm with abundant eosinophilic, prominent nucleoli, and multinucleated and markedly pleomorphic form. Necrosis was presented as well. Large areas of case 2 tumor were made up of spindle smooth-muscle cells, adipose tissue, thick-wall blood vessels and some areas merged with a proliferation of epithelioid which was consistent with typical angiomyolipoma. Im-munohistochemical study showed that the epithelioid cells and spindle smooth-muscle cells were posi-tive for VM, HMB45, Melan-A and negative for S100, CK. Case 1 and case 3 were diagnosed with malignant epithelioid angiomyolipoma, while case 2 was diagnosed with malignant classic angiomyoli-poma and epithelioid in part of the tumor. Case 1 was well alive. Case 2 was alive with tumor 12 years after the diagnosis. And case 3 was missed in the follow-up 3 months after metastasis resection. Conclusions Malignant renal angiomyolipoma is a rare disease. The diagnosis depends on histopatho-logic, immunohistochemieal study and clinical follow-up. Radical resection of the primary, recurrent and metastatic tumors is the main therapy. It needs more research to clarify if metastasis has any effect on prognosis.

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