scholarly journals Cardiac Epithelioid PEComa: Report of Two Cases and Review of the Literature

2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Huilin Niu ◽  
F. W. Wang ◽  
Paul J. Zhang ◽  
Zhanyong Bing

Cardiac PEComa is very rare. We reported two cases of epithelioid PEComas, one in an adult and one in a 2-year-old child. Both tumors were composed of sheets of epithelioid cells with coagulation necrosis. In addition, the adult case showed marked nuclear atypia and high mitotic activity with atypical mitosis and the pediatric case showed unusual clear cell features. Immunohistochemically, both tumors were positive for HMB-45 and SMA and negative for S100 and cytokeratin. Electron microscopy was performed in the pediatric case and showed premelanosomes. The adult patient developed extensive metastasis indicating malignant behavior. Prior to the two cases, only 5 other cases of cardiac PEComa were reported and the literatures are reviewed.

2004 ◽  
Vol 128 (6) ◽  
pp. 693-696 ◽  
Author(s):  
Sourav Ray ◽  
Zhengbin Lu ◽  
Swaminathan Rajendiran

Abstract We present a unique carcinoma of the pancreas with predominantly clear cell morphology (>95% clear cells). Mucicarmine stain revealed abundant intraluminal and intracytoplasmic mucin. Immunohistochemically, the cells were positive for the epithelial markers cytokeratin 7 and CAM 5.2, and were focally positive for cytokeratin 20. These cells also expressed monoclonal carcinoembryonic antigen. Stains for the neuroendocrine markers synaptophysin and chromogranin were negative, as were stains for vimentin, p53, HMB-45, and CD10. An additional outstanding feature was the presence of dense intraluminal and intracytoplasmic hyaline globules, which were immunohistochemically positive for α1-antitrypsin. Sequencing of the K-ras oncogene revealed a point mutation in codon 12, providing molecular evidence of ductal origin. In the proper morphologic context supported by immunohistochemistry, clear cell carcinoma can be regarded as a rare variant of ductal adenocarcinoma.


2020 ◽  
Author(s):  
leilei shen ◽  
Jixing LIN ◽  
Zhipeng REN ◽  
Bailin WANG ◽  
You Liu ◽  
...  

Abstract Background: Clear cell tumors of the lung (CCTLs) are rare and mostly benign pulmonary neoplasms arising from perivascular epithelioid cells. Only approximately 100 cases have been reported, and half of them were in China. Limited details about CCTLs often cause diagnostic or therapeutic problems. Case presentation: We describe a case of a 28-year-old woman with multiple gradually replicating and enlarging nodules in the left lower lobe. The patient underwent fine-needle aspiration biopsy and was diagnosed with CCTL. A left lower lobectomy and mediastinal lymph node dissection were performed. The gradual changes in size (1.4 cm to 2.8 cm) and quantity (10 to 49) of the CCTLs in this case were the biggest differences from previously reported cases. Conclusions: CCTLs are very uncommon and mostly benign PEComatous tumors with no specific morphologic features. We present a case of CCTL with multiplicity and rapid growth, which may indicate its aggressive nature. The accumulation of similar cases will help clarify the exact nature and improve our understanding of the disease.


Biology ◽  
2021 ◽  
Vol 10 (12) ◽  
pp. 1286
Author(s):  
Ourania Parra ◽  
Konstantinos Linos

“Cutaneous melanocytic tumor with CRTC1::TRIM11 fusion” (CMTCT) is a recently described entity belonging to the family of superficial tumors displaying melanocytic differentiation. Thirteen cases have been reported so far, on the head and neck, extremities, and trunk of adults of all ages (12 cases) and one in an 11-year-old child. Histopathologically, it is a nodular or multilobulated tumor composed of spindle and epithelioid cells arranged in nests, fascicles, or bundles that are surrounded by thin collagenous septa. By immunohistochemistry, the tumor shows variable immunoreactivity for S100-protein, SOX10, and MITF, as well as specific melanocytic markers such as MelanA and HMB-45. The neoplasm’s biologic behavior remains uncertain since the reported cases are limited and the follow-up is short (median 12 months). However, local recurrence and synchronous distant metastasis after 13 years of initial resection has been described in one case. Herein, we present a comprehensive literature review of CMTCT hoping to raise awareness among the dermatopathologists of this potentially novel entity.


2012 ◽  
Vol 126 (3) ◽  
pp. 481-490 ◽  
Author(s):  
Marcela G. del Carmen ◽  
Michael Birrer ◽  
John O. Schorge

1995 ◽  
Vol 53 (4) ◽  
pp. 782-788 ◽  
Author(s):  
Luiz F. Bleggi-Torres ◽  
Lúcia de Noranha ◽  
J. Fillus Neto ◽  
José E. Queiroz Telles ◽  
Luiz E. Madalozzo

The authors present the autopsy findings of two related patients and the biopsy findings of a thrid member of the family. The oldest member was 34 years old at death and on postmortem examination he had haemangioblastomas in the retina, cerebellum, medulla and spinal cord. Other findings were renal cell carcinoma, phaechromocytoma, cysts of kidney and pancreas, hydromyelia and atypical meningiomas. His brother died when 30 years old. The autopsy revealed haemangioblastomas of cerebellum, renal cell carcinoma and a clear cell cystadenoma of epididymus. The third patient was the daughter of the first and presented with headache and dizziness. CT-scan showed a cerebellar haemangioblastoma. Epidemiological considerations on the commonest visceral and CNS lesions and a review of current diagnostic criteria are discussed.


Head & Neck ◽  
1994 ◽  
Vol 16 (3) ◽  
pp. 272-277 ◽  
Author(s):  
M. John Hicks ◽  
Catherine M. Flaitz ◽  
Mark E. K. Wong ◽  
R. Keith McDaniel ◽  
Philip T. Cagle

2013 ◽  
Vol 140 (suppl 1) ◽  
pp. A135-A135
Author(s):  
Yanli Ding ◽  
Weiwei Chen ◽  
Zachary O'Neill ◽  
Maurice Barcos ◽  
Amy Sands ◽  
...  

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