scholarly journals Cutaneous Melanocytic Tumor with CRTC1::TRIM11 Fusion: Review of the Literature of a Potentially Novel Entity

Biology ◽  
2021 ◽  
Vol 10 (12) ◽  
pp. 1286
Author(s):  
Ourania Parra ◽  
Konstantinos Linos
Keyword(s):  
S100 Protein ◽  
Review Of The Literature ◽  
Epithelioid Cells ◽  
Melanocytic Tumor ◽  
Comprehensive Literature Review ◽  
The Family ◽  
Initial Resection ◽  
Melanocytic Differentiation ◽  
Hmb 45

“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.

Pneumomediastinum: A Rare Presentation of Inflicted Injuries in Infants

2020 ◽  
Author(s):  
Adam Lee ◽  
Adam Bajinting ◽  
Abby Lunneen ◽  
Colleen M. Fitzpatrick ◽  
Gustavo A. Villalona
Keyword(s):  
Literature Review ◽  
Retrospective Review ◽  
Review Of The Literature ◽  
Rare Presentation ◽  
Spontaneous Pneumomediastinum ◽  
Nonaccidental Trauma ◽  
Comprehensive Literature Review ◽  
Healthy Infants ◽  
History Of

AbstractReports of incidental pneumomediastinum in infants secondary to inflicted trauma are limited. A retrospective review of infants with pneumomediastinum and history of inflicted trauma was performed. A comprehensive literature review was performed. Three infants presented with pneumomediastinum associated with inflicted trauma. Mean age was 4.6 weeks. All patients underwent diagnostic studies, as well as a standardized evaluation for nonaccidental trauma. All patients with pneumomediastinum were resolved at follow-up. Review of the literature identified other cases with similar presentations with related oropharyngeal injuries. Spontaneous pneumomediastinum in previously healthy infants may be associated with inflicted injuries. Clinicians should be aware of the possibility of an oropharyngeal perforation related to this presentation.

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
Keyword(s):  
Clear Cell ◽  
Coagulation Necrosis ◽  
Review Of The Literature ◽  
Epithelioid Cells ◽  
Adult Case ◽  
Pediatric Case ◽  
High Mitotic Activity ◽  
Atypical Mitosis ◽  
Malignant Behavior ◽  
Hmb 45

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.

Malignant Perivascular Epithelioid Cell Tumor Involving the Prostate

2003 ◽  
Vol 127 (2) ◽  
pp. e96-e98 ◽  
Author(s):  
Chin-Chen Pan ◽  
An-Hang Yang ◽  
Hung Chiang
Keyword(s):  
Seminal Vesicle ◽  
Tumor Cells ◽  
S100 Protein ◽  
Epithelioid Cell ◽  
Cell Tumor ◽  
Epithelioid Cells ◽  
Perivascular Epithelioid Cell Tumor ◽  
Perivascular Epithelioid Cell ◽  
Granular Cytoplasm ◽  
Hmb 45

Abstract Perivascular epithelioid cell tumor (PEComa) is a neoplasm chiefly composed of HMB-45–positive epithelioid cells with clear-to-granular cytoplasm and a perivascular distribution. We describe such a tumor involving the prostate and seminal vesicle in a 46-year-old man. The tumor had characteristic histologic features of PEComa. Immunohistochemically, the tumor cells were positive for HMB-45 but negative for epithelial markers, Melan-A, and S100 protein. The tumor behaved in a malignant fashion, and the patient died of the disease 4 years after diagnosis.

Lipomas of the larynx and hypopharynx: a review of the literature with the addition of three new cases

1995 ◽  
Vol 109 (4) ◽  
pp. 353-357 ◽  
Author(s):  
Bruce M. Wenig
Keyword(s):  
Polypoid Lesion ◽  
Pyriform Sinus ◽  
Review Of The Literature ◽  
Complete Excision ◽  
The Third ◽  
Mesenchymal Neoplasms ◽  
One Year ◽  
Initial Resection ◽  
Supraglottic Larynx

AbstractLipomas of the larynx and hypopharynx are uncommon mesenchymal neoplasms. This report discusses the clinical and pathological features of three cases of laryngeal and hypopharyngeal lipomas. Two of the cases occurred in females and one in a male. The ages of the patients were 28, 51 and 51 years respectively. Two of the cases involved the supraglottic larynx (left arytenoid and left vestibular fold); the third involved the pyriform sinus. Symptoms included airway obstruction. dysphagia, throat discomfort, a sensation of excessive secretions in the throat and an increase in snoring. The complaints occurred over periods ranging from several months to one year in duration. Clinically, a polypoid lesion described as yellow in appearance was seen. Histologically. the tumours were composed of mature adipocytes without evidence of pleomorphism, lipoblasts or infiltrative growth. Surgery was the treatment of choice and included simple but complete excision in two of the cases. In these two cases, surgery proved curative with follow-up periods of 11 and seven years, respectively. In one case. the initial tumour was removed in pieces. This lesion recurred 15 years after the initial resection and was totally excised at that time. This patient has been free of tumour for more than five years.

Oncocytoma-like Angiomyolipoma

2002 ◽  
Vol 126 (5) ◽  
pp. 610-612 ◽  
Author(s):  
Guido Martignoni ◽  
Maurizio Pea ◽  
Franco Bonetti ◽  
Matteo Brunelli ◽  
John N. Eble
Keyword(s):  
Abdominal Pain ◽  
Breast Carcinoma ◽  
Renal Parenchyma ◽  
Renal Tumors ◽  
Epithelioid Cells ◽  
Epithelial Markers ◽  
Hmb 45

Abstract We report the clinical, pathologic, and immunohistochemical features of an unusual tumor of the kidney composed of densely eosinophilic, polygonal epithelioid cells. The patients were a 56-year-old woman and a 35-year-old man. The renal tumors were discovered during follow-up for breast carcinoma and evaluation for abdominal pain, respectively. The tumors closely resembled oncocytoma in routine sections, but were negative for epithelial markers and positive for HMB-45, a pattern of reactions characteristic of angiomyolipoma. In the woman, a single additional microscopic angiomyolipoma was present in the renal parenchyma at a distance from the main tumor. Both patients are alive without recurrence 7 and 10 years after surgery, respectively. Based on clinical, morphologic, and immunophenotypic features, we conclude that these tumors are oncocytoma-like angiomyolipomas.

Multiple Gastrointestinal Stromal Tumors and Lipomatosis

2008 ◽  
Vol 132 (11) ◽  
pp. 1825-1829
Author(s):  
Xiuli Liu ◽  
Charles Melbern Wilcox ◽  
Laurentia Nodit ◽  
Audrey J. Lazenby
Keyword(s):  
Smooth Muscle ◽  
Gastrointestinal Stromal Tumor ◽  
Young Patient ◽  
Rare Diseases ◽  
Gastrointestinal Stromal Tumors ◽  
S100 Protein ◽  
Genetic Syndromes ◽  
Epithelioid Cells ◽  
Stromal Tumors

Abstract In this report, we describe a 36-year-old man with the synchronous occurrence of multiple gastric gastrointestinal stromal tumors and multiple intestinal lipomas. Multiple, small, and well-circumscribed gastric gastrointestinal stromal tumors (3 mm to 2 cm) were present within the gastrectomy specimen. The gastrointestinal stromal tumors were composed of epithelioid cells that were strongly positive for CD117 but negative for S100 protein or smooth muscle–specific actin. Also, 17 small submucosal lipomas were identified in the duodenal portion of the gastrectomy specimen. Endoscopic follow-up of the patient revealed more than 20 additional lipomas scattered throughout the intestine. Several large intestinal lipomas were resected endoscopically and histologically confirmed. Although both multicentric gastrointestinal stromal tumor and gastrointestinal lipomatosis have been reported in association with a few genetic syndromes, there has been no report, to our knowledge, of their coexistence. We feel that the coexistence of multiple lesions of these 2 rare diseases in this relatively young patient may represent a novel syndrome.

Recent Advances in Treatment of Childhood Obesity

2020 ◽  
Vol 21 (14) ◽  
pp. 1072-1078
Author(s):  
Walter Milano ◽  
Paola Ambrosio ◽  
Francesca Carizzone ◽  
Walter Di Munzio ◽  
Valeria De Biasio ◽  
...  
Keyword(s):  
Public Health ◽  
Physical Activity ◽  
Bariatric Surgery ◽  
Childhood Obesity ◽  
Treatment Strategies ◽  
Lifestyle Changes ◽  
Family Context ◽  
Review Of The Literature ◽  
Biological Factors ◽  
The Family

: Childhood obesity has assumed epidemic proportions and is currently one of the most widespread public health problems. Many are the factors involved in the pathogenesis of excess weight with interactions between genetic, environmental and biological factors and therefore, also the therapeutic approach must be multidisciplinary and multidimensional. In this review of the literature, we report the contiguity of childhood obesity with eating disorders and the importance of involving the family context in order to induce stable lifestyle changes, both in relation to dietary and nutritional habits, but also in increasing physical activity. Finally, among the therapeutic options, although for selected cases, pharmacotherapy and bariatric surgery can be used as treatment strategies.

Intramedullary Teratoma: Case Report and Review of the Literature

1996 ◽  
Vol 82 (6) ◽  
pp. 616-620 ◽  
Author(s):  
Riccardo Caruso ◽  
Mariano Antonelli ◽  
Luigi Cervoni ◽  
Maurizio Salvati
Keyword(s):  
Case Report ◽  
Surgical Removal ◽  
International Literature ◽  
Review Of The Literature ◽  
Personal Case

Aims and Background Intramedullary teratoma is an extremely exceptional tumor (5 cases), although a careful review of international literature has shown it to be more frequent (32 cases) than believed. Methods The authors present a personal case with some unusual aspects. Results Our case is unusual not only because it was diagnosed by MRI (only one case has been reported in the literature) but also because surgical removal of the tumor was apparently total (only 4 other cases have been described), with a long follow-up period (4.5 years) and excellent results, in clinical and neuroradiologic terms.

scholarly journals Angiosarcoma of sigmoid colon with intraperitoneal bleeding: case report and literature review

2011 ◽  
Vol 93 (6) ◽  
pp. e91-e93 ◽  
Author(s):  
Tse-Hua Lo ◽  
Mu-Shiun Tsai ◽  
Tzu-An Chen
Keyword(s):  
Sigmoid Colon ◽  
Alimentary Tract ◽  
Surgical Excision ◽  
Sigmoid Colectomy ◽  
Epithelioid Cells ◽  
Spindle Cells ◽  
Choice Of Treatment ◽  
Intraperitoneal Bleeding ◽  
Immunohistochemical Studies

Primary angiosarcomas arising from the alimentary tract are rare and only a few cases have been reported in the literature. We report a case of an angiosarcoma of the sigmoid colon with intraperitoneal bleeding but not rectal bleeding. A 21-year-old female patient received a laparotomy and a mass lesion over the sigmoid colon was found with active bleeding. A sigmoid colectomy was performed as a curative resection. Grossly, the sigmoid colon contained a kidney shaped, hemorrhagic tumour from the submucosal layer extension to the antimesenteric side. Intraluminally, the mucosa of the colon was intact. Microscopic examination revealed a high grade angiosarcoma composed of fascicles of spindle cells and solid sheets of epithelioid cells. Immunohistochemical stains revealed a positive result for CD31 and the endothelial nature of the malignancy was confirmed. Smooth muscle antigens, desmins, cytokeratins AE1/AE3 and CD117 were all negative. The patient is still alive without evidence of recurrence or metastasis at a three-year follow-up appointment. Owing to the availability of immunohistochemical studies, some atypical sarcomas would now be correctly classified as angiosarcomas. Since no optimal adjuvant treatment is effective, curative surgical excision is still the best choice of treatment.

scholarly journals The Family Psychoeducation Fidelity Scale: Psychometric Properties

2020 ◽  
Vol 47 (6) ◽  
pp. 894-900 ◽  
Author(s):  
I. Joa ◽  
J. O. Johannessen ◽  
K. S. Heiervang ◽  
A. A. Sviland ◽  
H. A. Nordin ◽  
...  
Keyword(s):  
Psychometric Properties ◽  
Internal Consistency ◽  
Interrater Reliability ◽  
Review Process ◽  
Trial Registration ◽  
Family Psychoeducation ◽  
Time Points ◽  
The Family ◽  
Fidelity Scale

Abstract This study examined psychometric properties and feasibility of the Family Psychoeducation (FPE) Fidelity Scale. Fidelity assessors conducted reviews using the FPE fidelity scale four times over 18 months at five sites in Norway. After completing fidelity reviews, assessors rated feasibility of the fidelity review process. The FPE fidelity scale showed excellent interrater reliability (.99), interrater item agreement (88%), and internal consistency (mean = .84 across four time points). By the 18-month follow-up, all five sites increased fidelity and three reached adequate fidelity. Fidelity assessors rated feasibility as excellent. The FPE fidelity scale has good psychometric properties and is feasible for evaluating the implementation of FPE programs. Trial registration ClinicalTrials.gov Identifier: NCT03271242.

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