Perivascular Epithelioid Cell Neoplasms
Epithelioid Cells
Angiomyolipoma
Adenomyosis
Neoplasms, Plasma Cell
Neoplasms, Connective and Soft Tissue
Round Ligament
Tuberous Sclerosis
Epithelial Cells
Clear cell "sugar" tumor of the lung: a well-enhanced mass with an early washout pattern on dynamic contrast-enhanced computed tomography. (1/32)
(+info)Perivascular epithelioid cell tumor (PEComa) of abdominal cavity from falciform ligament: a case report. (2/32)
(+info)Malignant perivascular epithelioid cell tumour of the round ligament mimics leiomyoma on computed tomography. (3/32)
We report a case of a 45-year-old woman who had a palpable mass in the left lower quadrant of the abdomen. Computed tomography (CT) showed a circumscribed mass in the left round ligament of the uterus. The mass had heterogeneous density and enhancement accompanied by mottled calcification, which was initially identified as a leiomyoma. A histopathological examination revealed a malignant perivascular epithelioid cell tumour (PEComa), which is a rare soft tissue tumour. This case demonstrates that the appearance of malignant PEComa on the CT can mimic leiomyoma, which is the most common tumour of the round ligament. To the best of our knowledge, this is the first report of a CT appearance of this round ligament tumour. The radiological features and differential diagnosis are discussed. (+info)Pancreatic PEComa (sugar tumor): MDCT and EUS features. (4/32)
CONTEXT: PEComas (tumors showing perivascular epithelioid cell differentiation) of the pancreas are exceedingly rare. CASE REPORT: We herein report on a 60-year-old female who noticed a bulge in her right upper quadrant while exercising. Subsequent multidetector-row CT scan showed a 3.5 cm well-defined, encapsulated, hypovascular, solid tumor in the body of the pancreas. Endoscopic ultrasound demonstrated a mixed hypo- and hyper-echoic, well-defined, heterogeneous tumor. CONCLUSIONS: Although three pancreatic PEComas (sugar tumors) have been described previously, to the best of our knowledge, this is the first report of a pancreatic PEComa with illustration of its multidetector-row CT and endoscopic ultrasound features in the radiological literature. (+info)Perivascular epithelioid cell tumor (PEComa) of the liver diagnosed by contrast-enhanced ultrasonography. (5/32)
Perivascular epithelioid cell (PEC) is a unique cell which expresses both myogenic and melanocytic markers, and forms PEComa. A 36-year-old woman presented with a 35 mm-diameter liver tumor. MRI showed poor fat component in the tumor. Contrast-enhanced ultrasonography using the newly developed enhancing reagent, Sonazoid, clearly demonstrated early-phase enhancement of the tumor and rapid drainage of the reagent to veins, suggesting a PEComa. Lateral segmentectomy of the liver was performed. Histologically, epithelioid tumor cells around the vessels were immunostained with both HMB-45 and alpha-smooth muscle actin, confirming the diagnosis of PEComa. No recurrence has been found for 18 months following the operation. (+info)Clinical activity of mTOR inhibition with sirolimus in malignant perivascular epithelioid cell tumors: targeting the pathogenic activation of mTORC1 in tumors. (6/32)
(+info)A perivascular epithelioid cell tumor of the stomach: an unsuspected diagnosis. (7/32)
Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasia and currently well recognized as a distinct entity with characteristic morphological, immunohistochemical and molecular findings. We report a case of PEComa arising in the antrum of a 71-year-old female with melena. The tumor, located predominantly in the submucosa as a well delimited nodule, measured 3.0 cm in diameter and was completely resected, with no evidence of the disease elsewhere. Histologically, it was composed predominantly of eosinophilic epithelioid cells arranged in small nests commonly related to variably sized vessels, with abundant extracellular material, moderate nuclear variation and discrete mitotic activity. No necrosis, angiolymphatic invasion or perineural infiltration was seen. Tumor cells were uniformly positive for vimentin, smooth muscle actin, desmin and melan A. Although unusual, PEComa should be considered in the differential diagnosis of gastric neoplasia with characteristic epithelioid and oncocytic features and prominent vasculature. (+info)Perivascular epithelioid cell tumor (PEComa) of the uterus with aggressive behavior at presentation. (8/32)
Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor composed of histologically and immunohistochemically distinctive perivascular epithelioid cells (PECs). Both benign and malignant tumors have been identified, but the criteria for diagnosis of malignancy have not been fully established due to the rarity of the tumor. We report on a case of uterine PEComa in a 33-year old woman with lymph node metastasis at presentation. The tumor had the characteristic histologic features of PEComa with cytologic atypia, mitotic activity of 2/10 high power field (HPF), and necrosis; it exhibited immunopositivity for HMB-45, calponin and desmin and was negative for melan-A. The patient received neoadjuvant chemotherapy, debulking surgery and adjuvant chemotherapy. No evidence of recurrence or metastasis was apparent 8 months after surgery. (+info)Perivascular Epithelioid Cell Neoplasms (PEComas) are a rare group of mesenchymal tumors that demonstrate unique clinical and pathological features. These neoplasms are characterized by the proliferation of perivascular epithelioid cells (PECs), which are distinctive cells with an epithelioid appearance and a close association with blood vessel walls.
PEComas can occur in various organs, such as the kidney, liver, lung, pancreas, and gastrointestinal tract, but they most commonly involve the uterus. The World Health Organization (WHO) recognizes three main types of PEComas: epithelioid angiomyolipoma, clear cell "sugar" tumor, and lymphangioleiomyomatosis (LAM).
PEComas exhibit a wide range of clinical behaviors, from benign to malignant. Malignant PEComas typically display features such as infiltrative growth, high cellularity, nuclear atypia, increased mitotic activity, and necrosis. The pathogenesis of PEComas is not well understood, but recent studies suggest that they may be related to the TSC1 or TSC2 gene mutations, which are also associated with tuberous sclerosis complex (TSC), a genetic disorder characterized by benign tumors in multiple organs.
Diagnosis of PEComas is based on histopathological examination and immunohistochemical staining. The typical immunophenotype of PECs includes positivity for both melanocytic markers (such as HMB-45 and Melan-A) and smooth muscle markers (such as actin and desmin).
Treatment options for PEComas depend on the tumor's location, size, and clinical behavior. Surgical resection is the primary treatment modality for localized, symptomatic, or malignant PEComas. In some cases, systemic therapy with mammalian target of rapamycin (mTOR) inhibitors may be considered, particularly in metastatic or recurrent tumors.
Epithelioid cells are a type of cell that can be found in certain types of tissue in the body, including connective tissue and some organs. These cells have a characteristic appearance under a microscope, with an enlarged, oval or round shape and a pale, abundant cytoplasm. They may also have a nucleus that is centrally located and has a uniform, rounded shape.
Epithelioid cells are often seen in the context of inflammation or disease, particularly in relation to granulomatous disorders such as sarcoidosis and tuberculosis. In these conditions, epithelioid cells can form clusters known as granulomas, which are a hallmark of the diseases. The exact function of epithelioid cells is not fully understood, but they are thought to play a role in the immune response and may help to contain and eliminate foreign substances or pathogens from the body.
Angiomyolipoma is a type of benign tumor that occurs most commonly in the kidney. It is composed of blood vessels (angio-), smooth muscle cells (myo-), and fat cells (lipo-). Angiomyolipomas are usually associated with the genetic disorder tuberous sclerosis complex, but they can also occur spontaneously or as a result of other genetic conditions.
These tumors can vary in size and may cause symptoms such as pain, blood in the urine, or a palpable mass in the abdomen if they grow large enough. In some cases, angiomyolipomas may also be at risk for rupture and bleeding, particularly if they are larger than 4 cm in size.
Treatment options for angiomyolipomas include surveillance with imaging tests, medication to reduce the risk of bleeding, or surgical removal of the tumor. The choice of treatment depends on factors such as the size and location of the tumor, the presence of symptoms, and the patient's overall health.
Adenomyosis is a medical condition that affects the uterus (womb). It occurs when the tissue that normally lines the uterus (endometrium) grows into the muscular wall of the uterus. This causes the uterine wall to thicken and can result in heavy, prolonged menstrual periods, as well as pain during menstruation, sexual intercourse, or pelvic examinations.
In some cases, adenomyosis may cause no symptoms at all, while in others it can lead to significant discomfort and impact a woman's quality of life. The exact causes of adenomyosis are not fully understood, but it is thought to be related to hormonal factors, inflammation, and previous uterine trauma or surgery.
Adenomyosis is typically diagnosed through imaging tests such as ultrasound or MRI, although in some cases a biopsy may be necessary to confirm the diagnosis. Treatment options for adenomyosis include medication to manage pain and reduce heavy menstrual bleeding, hormonal therapies to help regulate menstrual cycles, and in severe cases, surgery to remove all or part of the uterus (hysterectomy).
Melanoma-specific antigens are proteins or other molecules that are present on melanoma cells but not normally found on healthy cells in the body. These antigens can be recognized by the immune system as foreign and trigger an immune response, making them potential targets for immunotherapy treatments for melanoma.
There are two main types of melanoma-specific antigens: tumor-specific antigens (TSAs) and tumor-associated antigens (TAAs). TSAs are unique to cancer cells and are not found on normal cells, while TAAs are overexpressed or mutated versions of proteins that are also present in normal cells.
Examples of melanoma-specific antigens include Melan-A/MART-1, gp100, and tyrosinase. These antigens have been studied extensively as targets for cancer vaccines, adoptive cell therapy, and other immunotherapy approaches to treat melanoma.
Plasma cell neoplasms are a type of cancer that originates from plasma cells, which are a type of white blood cell found in the bone marrow. These cells are responsible for producing antibodies to help fight off infections. When plasma cells become cancerous and multiply out of control, they can form a tumor called a plasmacytoma.
There are two main types of plasma cell neoplasms: solitary plasmacytoma and multiple myeloma. Solitary plasmacytoma is a localized tumor that typically forms in the bone, while multiple myeloma is a systemic disease that affects multiple bones and can cause a variety of symptoms such as bone pain, fatigue, and anemia.
Plasma cell neoplasms are diagnosed through a combination of tests, including blood tests, imaging studies, and bone marrow biopsy. Treatment options depend on the stage and extent of the disease, but may include radiation therapy, chemotherapy, and stem cell transplantation.
Neoplasms of connective and soft tissue are abnormal growths or tumors that develop in the body's supportive tissues, such as cartilage, tendons, ligaments, fascia, and fat. These neoplasms can be benign (non-cancerous) or malignant (cancerous).
Benign connective and soft tissue neoplasms include:
- Lipomas: slow-growing, fatty tumors that develop under the skin.
- Fibromas: firm, benign tumors that develop in connective tissue such as tendons or ligaments.
- Nevi (plural of nevus): benign growths made up of cells called melanocytes, which produce pigment.
Malignant connective and soft tissue neoplasms include:
- Sarcomas: a type of cancer that develops in the body's supportive tissues such as muscle, bone, fat, cartilage, or blood vessels. There are many different types of sarcomas, including liposarcoma (fatty tissue), rhabdomyosarcoma (muscle), and osteosarcoma (bone).
- Desmoid tumors: a rare type of benign tumor that can become aggressive and invade surrounding tissues. While not considered cancerous, desmoid tumors can cause significant morbidity due to their tendency to grow and infiltrate nearby structures.
Connective and soft tissue neoplasms can present with various symptoms depending on their location and size. Treatment options include surgery, radiation therapy, chemotherapy, or a combination of these modalities. Regular follow-up care is essential to monitor for recurrence or metastasis (spread) of the tumor.
The round ligament is a cord-like structure in the female pelvis that extends from the uterus to the labia majora. It is one of the major ligaments that support the uterus and helps to maintain its position within the pelvis. The round ligament is composed of fibrous tissue and smooth muscle, and it plays a role in maintaining the tone and shape of the uterus.
During pregnancy, the round ligament can become stretched and thickened as the uterus grows and expands. This can sometimes cause discomfort or pain, particularly on one side of the pelvis. In some cases, the round ligament may also contribute to the development of certain gynecological conditions, such as uterine prolapse or urinary incontinence.
It is important for healthcare providers to consider the round ligament when evaluating and treating female reproductive health issues, as it can have a significant impact on the function and positioning of the uterus and other pelvic organs.
Tuberous Sclerosis Complex (TSC) is a rare genetic disorder that causes non-cancerous (benign) tumors to grow in many parts of the body. These tumors can affect the brain, skin, heart, kidneys, eyes, and lungs. The signs and symptoms of TSC can vary widely, depending on where the tumors develop and how severely a person is affected.
The condition is caused by mutations in either the TSC1 or TSC2 gene, which regulate a protein that helps control cell growth and division. When these genes are mutated, the protein is not produced correctly, leading to excessive cell growth and the development of tumors.
TSC is typically diagnosed based on clinical symptoms, medical imaging, and genetic testing. Treatment for TSC often involves a multidisciplinary approach, with specialists in neurology, dermatology, cardiology, nephrology, pulmonology, and ophthalmology working together to manage the various symptoms of the condition. Medications, surgery, and other therapies may be used to help control seizures, developmental delays, skin abnormalities, and other complications of TSC.
Epithelial cells are types of cells that cover the outer surfaces of the body, line the inner surfaces of organs and glands, and form the lining of blood vessels and body cavities. They provide a protective barrier against the external environment, regulate the movement of materials between the internal and external environments, and are involved in the sense of touch, temperature, and pain. Epithelial cells can be squamous (flat and thin), cuboidal (square-shaped and of equal height), or columnar (tall and narrow) in shape and are classified based on their location and function.