Epithelioid inflammatory myofibroblastic sarcoma: the youngest case reported

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.

Monotypic angiomyolipoma of the nasal cavity: An extremely rare cause of nasal mass with recurrent epistaxis

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.

Epithelioid angiomyolipoma with tumor thrombus in IVC and right atrium

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.

Atypical epithelioid trophoblastic lesions after a cesarean section with cyst and fistula formation:a clinicopathological analysis of 4 cases / 中华病理学杂志

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.

An Unusual Case Of Myocardial Tuberculosis Alongwith Involvement Of Kidneys

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.

CT features and prognosis of renal epithelioid angiomyolipoma / 中国基层医药

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.

The Significance of Caspase-Cleaved Cytokeratin 18 in Pleural Effusion / 결핵및호흡기질환

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.

A Case of Primary Cutaneous Perivascular Epithelioid Cell Tumor / 대한피부과학회지

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.

Differentiation to renal tubular epithelial-like cells from bone marrow mesenchymal stem cells in vitro / 中华肾脏病杂志

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.

Malignant renal angiomyolipoma with metastases (report of 3 cases) / 中华泌尿外科杂志

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.

Angiomatoid Spitz Nevus

Spitz nevus is a variant of melanocytic nevus which is histopathologically defined as large spindle and/or epithelioid cells. Angiomatoid Spitz nevus is a rare histologic variant of desmoplastic Spitz nevus characterized by prominent vasculature. We present a case of angiomatoid Spitz nevus, celluar type, that has not been reported before. We provide another example to show the remarkable diversity of Spitz nevus.

Renal Angiomyolipoma Partially Containing Epithelioid Component / 대한비뇨기과학회지

Epithelioid renal angiomyolipoma is a recently recognized pathologic variant of angiomyolipoma, which represents a more malignant course than a classical renal angiomyolipoma. We report a case of histologically proven renal angiomyolipoma. The patient presented with the symptoms and signs of spontaneous rupture of the kidney. On surgical exploration, the ruptured kidney with upper pole kidney mass was resected. The enlarged regional lymph nodes were also removed, which showed the same pathology.

Sclerosing Epithelioid Fibrosarcoma of the Kidney / 대한비뇨기과학회지

Sclerosing epithelioid fibrosarcoma(SEF) is an uncommon tumor of the deep soft tissue. It was first described in 1995 by Meis-Kindblom et al. Histologically, this tumor is characterized by uniform, small, round to ovoid epithelioid cells with clear cytoplasm, and the cells are arranged in distinct nests and cords. On immunohistochemistry, the most consistently positive marker is vimentin, although other antigens(cytokeratin, epithelial membrane antigen, S100 protein and neuron specific enolase) have been recorded as being inconsistently positive. We report here on a case of a 46-year-old woman who presented with back pain. The radiologic findings revealed a right renal mass and multiple bone metastases. The patient underwent radical nephrectomy and the pathologic finding was primary SEF of the kidney.

Cellular Blue Nevus: A case report

Cellular blue nevus is an benign variant of blue nevus group, characterized by cellular islands composed of closely aggregated spindle cells with little or no melanin. The other variants include atypical and malignant blue nevus which often had the difficulties in the differential diagnosis from the malignant melanoma. A 4-year-old boy presented with hemispheric scalp nodule, which has been slowly grown since birth, measuring 3.5x3x1.5 cm. The surface showed geographic black pigmentation without hairs, hemorrhage, and ulceration. The excised mass disclosed homogeneoulsy dark black, glistening, and muddy cut surface. Histological examination revealed exuberant melanocytic proliferation with both spindle and dendritic components. Heavily pigment ed spindle cells, melanophages, focal necrosis, and vacuolated epithelioid cells were unusually noted in our case and diagnosed as cellular blue nevus. Electron microscopy disclosed largely mature melanosomes in tumor cells and melanophages. During about 16 months since operation, he has been relatively well with no evidence of disease.