keyword
https://read.qxmd.com/read/38526831/reticular-myxoid-odontogenic-neoplasm-with-novel-strn-alk-fusion-report-of-2-cases-in-3-year-old-males
#1
JOURNAL ARTICLE
Molly S Rosebush, Molly Housley Smith, Kitrina G Cordell, Nicholas Callahan, Waleed Zaid, Jeffrey Gagan, Justin A Bishop
Odontogenic tumors represent a collection of entities ranging from hamartomas to destructive benign and malignant neoplasms. Occasionally, pathologists encounter gnathic lesions which clearly exhibit an odontogenic origin but do not fit within the confines of established diagnoses. Here, we describe two such odontogenic tumors, both affecting 3-year-old males. Each case presented as a destructive, radiolucent mandibular lesion composed of mesenchymal cells, some with unique multi-lobed nuclei, frequently arranged in a reticular pattern and supported by a myxoid stroma with focal laminations...
March 25, 2024: Head and Neck Pathology
https://read.qxmd.com/read/38492357/endobronchial-solitary-fibrous-tumors-an-enigma-for-diagnosis
#2
Fátima Ramalhosa, Federica Pezzuto, Francesco Fortarezza, Gianluca Canu, Davide Biondini, Eleonora Faccioli, Roberta Polverosi, Chiara Giraudo, Fiorella Calabrese
Solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms constituting less than 2% of all soft tissue tumors. They typically originate in the thoracic cavity, mainly in the pleura, but can also occur in other various sites such as lung parenchyma, pericardium, and bronchus. In this study, a 49-year-old non-smoking female with a history of allergies presented to our pulmonary clinic with a chronic cough. An explorative bronchoscopy revealed an intrabronchial mass in the left superior bronchi, and a 68 Ga-DOTATOC positron emission computed tomography suggested a carcinoid tumor...
March 4, 2024: Pathology, Research and Practice
https://read.qxmd.com/read/38487637/mesenchymal-hepatic-hamartoma-a-rare-case-of-severe-respiratory-distress-in-a-neonate
#3
Kareem Omran, Jayasheela Kannan, Nisha Soares, Sameh Ali, Wissam Jamal Al Tamr
It is critical to consider intra-abdominal pathology in cases of neonatal respiratory distress. Accurate and prompt diagnosis via computed tomography is lifesaving. We have highlighted the effectiveness of rapid surgical intervention as curative.
March 2024: Clinical Case Reports
https://read.qxmd.com/read/38444652/benign-paediatric-liver-tumours-the-radiological-maze-demystified
#4
JOURNAL ARTICLE
Poonam Sherwani, Devasenathipathy Kandasamy, Raju Sharma, Prabudh Goel, Manisha Jana, Nellai Krishnan
UNLABELLED: The trajectory from the clinical identification of a benign liver mass to establishing a precise diagnosis is perplexing and arduous. Related contributory factors are that such lesions are encountered infrequently and that there is a nonavailability of dedicated paediatric radiologists in the developing world. The objective of this study was to review the spectrum of benign liver lesions in children and their typical imaging features. Cross-sectional imaging of all paediatric patients (< 18 years) with liver lesions (single and multiple) performed in the institute from 01 January 2018 to 01 January 2019 as well as those acquired at outside institutions and referred to the institute for management was included...
2024: SA journal of radiology
https://read.qxmd.com/read/38405259/role-of-mesohepatectomy-for-pediatric-centrally-located-liver-tumors
#5
JOURNAL ARTICLE
Shajini Nirmalan, Senthilnathan Ramaswamy, Vivek Shanmugam, Karpaga Vinayagam Natarajan
BACKGROUND: Mesohepatectomy (MH) or central hepatectomy (CH) is a recognized surgical technique for centrally located pediatric liver tumors. This technique of liver resection avoids extended liver resections and thereby helps in the preservation of liver parenchyma. In this article, we aim to analyze our experience and outcome with this technique of liver resection. METHODS: A retrospective analysis of patients who underwent MH from January 2015 to June 2023 at our institute was performed...
2024: Journal of Indian Association of Pediatric Surgeons
https://read.qxmd.com/read/38398243/androgen-insensitivity-syndrome-with-bilateral-gonadal-sertoli-cell-lesions-sertoli-leydig-cell-tumor-and-paratesticular-leiomyoma-a-case-report-and-first-systematic-literature-review
#6
Apollon I Karseladze, Aleksandra V Asaturova, Irina A Kiseleva, Alina S Badlaeva, Anna V Tregubova, Andrew R Zaretsky, Elena V Uvarova, Magda Zanelli, Andrea Palicelli
Androgen insensitivity syndrome (AIS) is a rare Mendelian disorder caused by mutations of the androgen receptor ( AR ) gene on the long arm of the X chromosome. As a result of the mutation, the receptor becomes resistant to androgens, and hence, karyotypically male patients (46,XY) carry a female phenotype. Their cryptorchid gonads are prone to the development of several types of tumors (germ cell, sex cord stromal, and others). Here, we report a 15-year-old female-looking patient with primary amenorrhea who underwent laparoscopic gonadectomy...
February 6, 2024: Journal of Clinical Medicine
https://read.qxmd.com/read/38389639/unveiling-the-mysteries-of-a-composite-compound-odontoma-insights-from-the-management-of-a-rare-entity
#7
Aakash Gupta, Tarun Kumar Singh, Ashish J Johnson, Rukhsar Showkat
Odontomas are one of the slow-growing odontogenic tumors. They are not a true neoplasm and are considered to be hamartoma. Odontomas consist of four distinct tissues, i.e., enamel, dentin, pulp, and cementum. Odontomas develop from fibroepithelial and undifferentiated mesenchymal cells which are essential for the development of the tooth. These are mostly asymptomatic and are incidentally detected on routine radiographic examination. This case report presents a unique case of a composite compound odontoma in an adult patient with flaring of teeth...
January 2024: Curēus
https://read.qxmd.com/read/38357912/pathogenic-somatic-mutation-of-dicer1-and-clinicopathological-features-in-nasal-chondromesenchymal-hamartomas-a-series-of-nine-cases
#8
JOURNAL ARTICLE
Chunyan Hu, Yifeng Liu, Lan Lin, Cuncun Yuan, Duan Ma, Qiang Huang
Nasal chondromesenchymal hamartoma (NCMH) is a rare benign polypoid mesenchymal tumor arising in the nasal cavity and/or paranasal sinuses. Recognizing these sporadic, rare lesions is crucial, as surgical complete removal of the mass is the common treatment approach. This retrospective study analyzed the demographics, symptoms, and imaging data of 9 patients diagnosed with NCMH between January 2017 and June 2023, possibly representing the largest single-center adult case cohort to date. Diagnostic techniques included nasal endoscopy, CT/MRI scan, immunohistological studies, and morphologic comparisons...
February 15, 2024: American Journal of Surgical Pathology
https://read.qxmd.com/read/38320792/-clinicopathological-analysis-of-adult-hepatic-mesenchymal-hamartoma
#9
JOURNAL ARTICLE
X N Gou, W Xu, Z H Dong, Z B Wang
Objective: To explore the clinicopathological and molecular genetic features of adult hepatic mesenchymal hamartoma (MHL). Methods: A total of five confirmed adult MHL cases diagnosed at the Pathology Department of the First Medical Center of the People's Liberation Army General Hospital between 2009 and 2022 were collected. Histomorphological observation and immunohistochemical staining were conducted. Gene detection was performed by next-generation sequencing. Results: Among the five cases, four were male and one was female, aged 46-67 years, with an average age of 56...
January 20, 2024: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://read.qxmd.com/read/38247726/giant-folliculosebaceous-cystic-hamartoma-of-the-face
#10
Ramona Tasar, Melanie Peckruhn, Jörg Tittelbach
Folliculosebaceous cystic hamartoma (FSCH) is a rare and benign form of cutaneous hamartomas. These skin lesions often lead to clinical and histopathological misdiagnosis due to their similarities to cutaneous lesions with overproduction of clustered sebaceous glands. Clinically, the lesions often present as solitary, skin-colored, pedunculated warts to cauliflower-like, exophytic papules and nodules, usually with a diameter ranging 0.5-1.5 cm that rarely exceed 2 cm in size. Only a small number of giant variants are reported in the literature with a diameter in the range of 5-23 cm...
December 31, 2023: Dermatopathology (Basel, Switzerland)
https://read.qxmd.com/read/38201471/congenital-tumors-magnetic-resonance-imaging-findings-with-focus-on-rare-tumors
#11
JOURNAL ARTICLE
Piotr Kwasniewicz, Julia Wieczorek-Pastusiak, Anna Romaniuk-Doroszewska, Monika Bekiesinska-Figatowska
Congenital tumors are rare and, owing to this rarity, there is limited information on many of them. A total of 839 fetal and postnatal MRI studies performed in the first 3 months of life were retrospectively reviewed. They were performed with the use of 1.5 T scanners. Seventy-six tumors were diagnosed based on fetal MRI between 20 and 37 gestational weeks, and 27 were found after birth, from 1 day of age to 3 months of life. Teratomas were the most common tumors in our dataset, mainly in the sacrococcygeal region (SCT), followed by cardiac rhabdomyomas and subependymal giant cell astrocytomas (SEGA) associated with TSC, and neuroblastomas...
December 20, 2023: Cancers
https://read.qxmd.com/read/38066766/update-on-the-pathology-of-pediatric-liver-tumors-a-pictorial-review
#12
REVIEW
Priyanka Bhagat, Mukul Vij, Lexmi Priya Raju, Gowripriya Gowrishankar, Jagadeesh Menon, Naresh Shanmugam, Ilankumaran Kaliamoorthy, Ashwin Rammohan, Mohamed Rela
Liver tumors in children are uncommon and show remarkable morphologic heterogeneity. Pediatric tumors may arise from either the epithelial or mesenchymal component of the liver and rarely may also show both lines of differentiation. Both benign and malignant liver tumors have been reported in children. The most common pediatric liver tumors by age are benign hepatic infantile hemangiomas in neonates and infants, malignant hepatoblastoma in infants and toddlers, and malignant hepatocellular carcinoma in teenagers...
November 24, 2023: Diagnostics
https://read.qxmd.com/read/37904415/a-case-report-of-anterior-mediastinal-angiomyolipoma-that-invaded-the-left-thoracic-cavity
#13
JOURNAL ARTICLE
Dong Bai, Yuanzi Liang, Wenting Liu, Yanhan Liu, Zhiqun Wang
RATIONALE: Angiomyolipoma is a mesenchymal tumor composed of blood vessels, smooth muscle, and mature adipose tissue. It is most commonly found in the kidney, and is rare outside the kidney, especially in the mediastinum. Only about 12 cases have been reported worldwide so far. PATIENT CONCERNS: We report a young female patient who had been found with a left thoracic mass for 19 years. In the past 19 years, the patient had no chest pain, dyspnea and other symptoms, but this time she visited the doctor because of cough, and there were no other clinical signs...
October 27, 2023: Medicine (Baltimore)
https://read.qxmd.com/read/37859590/nevus-lipomatosus-superficialis-a-series-of-six-cases
#14
Badr AbdullGaffar, Tasnim Keloth
Nevus lipomatosus still imposes diagnostic, categorization, and etiologic challenges. Even though an intradermal adipose tissue is a histopathologic prerequisite, the lesions are clinically divided into classic multiple forms and a solitary variant, which some consider a separate so-called lipofibroma clinicopathologic entity. This further complicates the true prevalence, classification and etiopathogenesis of nevus lipomatosus. Case reports and series studies have reflected either consistent or variable and sometimes conflicting clinicopathologic findings...
October 20, 2023: Journal of Cutaneous Pathology
https://read.qxmd.com/read/37832361/mesenchymal-hamartoma-versus-hepatoblastoma-a-diagnostic-dilemma
#15
Arkadeep Dhali, Harendra Kumar, Shivangi Sengupta, Jyotirmoy Biswas, Bharat Kumar, Gopal Krishna Dhali
INTRODUCTION AND IMPORTANCE: Pediatric hepatic tumors present diagnostic challenges due to diverse clinical presentations and limited patient communication. Elevated serum alpha-fetoprotein (AFP) levels, often associated with hepatoblastoma, can occur in various hepatic conditions, adding complexity to diagnosis. This report emphasizes the importance of comprehensive assessment and integrated interpretation in such cases. CASE PRESENTATION: We present a rare case of a 1-year-old female child with abdominal swelling and hepatomegaly...
October 10, 2023: International Journal of Surgery Case Reports
https://read.qxmd.com/read/37829956/an-adult-with-undifferentiated-embryonal-sarcoma-of-the-liver-a-case-report-of-a-rare-encounter
#16
Rajmohan Rammohan, Melvin Joy, Sai Greeshma Magam, Achal Patel, Sai Reshma Magam, Dilman Natt, Jiten Desai, Susan Bunting, Paul Mustacchia
Undifferentiated embryonal sarcoma of the liver (UESL) is a rare, aggressive tumor mainly found in children but can also appear in adults. Its diagnosis in adults remains a conundrum; it is often identified late due to its non-specific symptoms and resemblance to benign lesions. A comprehensive treatment regimen involving surgical intervention, chemotherapy, and possibly radiation significantly boosts survival rates. Imaging often yields inconclusive outcomes, further complicating the diagnostic process. Here, we report the case of a 28-year-old female diagnosed with UESL, emphasizing the need for timely intervention...
September 2023: Curēus
https://read.qxmd.com/read/37828892/-a-case-of-pediatric-tracheal-inflammatory-myofibroblastic-tumor
#17
JOURNAL ARTICLE
Shichao Qin, Dongmin Wei, Chenyang Xu, Tongdong Su, Dapeng Lei
<b/>Inflammatory myofibroblastic tumor is a rare tumor of mesenchymal origin. A case of intratracheal inflammatory myofibroblastic tumor in a male child was reported. The clinical characteristics, diagnosis, treatment and prognosis of the disease were reviewed based on the literature, and a differential diagnosis between inflammatory myofibroblastic tumor and hamartoma was performed to ultimately confirm the nature of the tumor in the child.
October 2023: Journal of Clinical Otorhinolaryngology, Head, and Neck Surgery
https://read.qxmd.com/read/37817291/multiple-rhabdomyomatous-mesenchymal-hamartomas-in-a-patient-with-mosaic-barber-say-syndrome
#18
Aniza Giacaman, Oriol Corral-Magaña, Carlos Saus Sarrias, Guillermo González-López, Víctor José Asensio Landa, Ana Martín-Santiago
Barber-Say syndrome (BSS) is a rare congenital ectodermal dysplasia with few cases reported in the literature. We describe a 9-year-old boy with congenital generalized hypertrichosis and multiple rhabdomyomatous mesenchymal hamartomas (RMHs) on his nose and periocular region. Next-generation sequencing, performed in DNA from a blood sample, and RMH tissue, revealed a pathogenic variant in the TWIST2 gene, which was not detected in a salivary sample of the patient, nor in his parents. Therefore, we consider this variant as de novo mosaicism...
October 10, 2023: Pediatric Dermatology
https://read.qxmd.com/read/37817244/large-mesenchymal-cystic-and-chondroid-pulmonary-hamartoma-mimicking-lung-cancer-case-report
#19
JOURNAL ARTICLE
Seha Ahn, Heejin Lee, Joon Kyu Kang, In Sub Kim, Youngkyu Moon, Jung Suk Choi, Si Young Choi
Pulmonary hamartoma is the most commonly resected benign neoplasm of lung. The mesenchymal cystic subtype is a rare and often bilaterally occurring variant composed of multiple cysts and nodules. Herein, we present an asymptomatic 70-year-old woman with a large and mostly cystic growth of right hilar region. Computed tomography of the chest and fluorodeoxyglucose positron emission tomography/computed tomography imaging traced its origins to right middle lobe. Overall features suggested primary lung cancer or perhaps other cystic lung disease...
October 10, 2023: Journal of Cardiothoracic Surgery
https://read.qxmd.com/read/37775408/outcomes-of-primary-tumor-excision-do-not-differ-from-non-resection-methods-in-pediatric-mesenchymal-hamartoma-of-the-liver-a-rapid-systematic-review
#20
JOURNAL ARTICLE
Justin Bauzon, Mary Froehlich, Noam Dadon, Desiree Morris, Shirong Chang
BACKGROUND: To conduct a rapid systematic review comparing the outcomes of primary tumor resection versus non-resection on patients with hepatic mesenchymal hamartoma. METHODS: We searched the Ovid MEDLINE, EMBASE, Scopus, PubMed, Web of Science, and Google Scholar databases from January 1, 2000 to March 31, 2022. Studies that described cases of hepatic mesenchymal hamartoma, including management and outcomes, were included. RESULTS: 62 articles met inclusion criteria with 95 cases in total...
August 22, 2023: Journal of Pediatric Surgery
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