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Archives of Pathology & Laboratory... May 2020Succinate dehydrogenase (SDH)-deficient gastrointestinal stromal tumor (GIST) is a subset of wild-type GIST that constitutes approximately 10% of gastric GISTs.... (Review)
Review
Succinate dehydrogenase (SDH)-deficient gastrointestinal stromal tumor (GIST) is a subset of wild-type GIST that constitutes approximately 10% of gastric GISTs. SDH-mutated GISTs lack mutations in the proto-oncogene receptor tyrosine kinase (also known as KIT, c-KIT, or CD117) or platelet-derived growth factor receptor α (PDGFR-α). These tumors have female predilection, affect children and young adults, and have a spectrum of behavior from indolent to progressive. These tumors have characteristic morphologic features including multinodular architecture, multiple tumors, lymphovascular involvement, and occasional lymph node metastasis. They can be seen in patients with Carney triad or Carney-Stratakis syndrome. Although a mutation in any one of the SDH subunits can be pathogenic, deficiency of a single subunit leads to loss of detectable SDH subunit B by immunohistochemistry, enabling a convenient, tissue-based screening method. The prognosis and the clinical course of these tumors is different from that of KIT- or PDGFR-α-mutated GISTs. Surgical management is considered the main line of treatment. SDH-mutated GISTs do not respond well to the common targeted therapy, with no objective tumor response to imatinib. The role of the pathologist in diagnosing these cases is imperative in management and subsequent follow-up.
Topics: Chondroma; Electron Transport Complex II; Gastrointestinal Stromal Tumors; Humans; Immunohistochemistry; Leiomyosarcoma; Lung Neoplasms; Membrane Proteins; Mutation; Paraganglioma; Paraganglioma, Extra-Adrenal; Prognosis; Proto-Oncogene Mas; Stomach Neoplasms; Succinate Dehydrogenase
PubMed: 31169996
DOI: 10.5858/arpa.2018-0370-RS -
Indian Journal of Otolaryngology and... Dec 2022Mass lesions of the larynx are one of the most common clinical entity which we come across in routine otorhinolaryngology and head neck practice with varied...
Mass lesions of the larynx are one of the most common clinical entity which we come across in routine otorhinolaryngology and head neck practice with varied symptomatology. Among all the mass lesions of the larynx, Epithelial neoplasms constitute up to 97%. Mesenchymal tumours of the larynx constitute only 0.3-1.0% of all the laryngeal tumors. Abundance of cartilage structures in the larynx made it a spot for mesenchymal tumors [chondromas and chandrosacrcomas]. The spectrum of mesenchymal neoplasms can vary from chondromas, chondroblastoma to chondrosarcoma. Here we want to share our experience of a mesenchymal tumour of the larynx. This case is reported for the rarity and ambiguity in diagnosis. Though these are slow-growing tumours with an early presentation, in our case, the patient had a supportive tracheostomy without definitive treatment for more than 2 years. We managed this patient by excising the mass by lateral pharyngotomy with the preservation of larynx followed by successful Decannulation in 20 days.
PubMed: 36742506
DOI: 10.1007/s12070-020-02308-8 -
Atencion Primaria 2019
Topics: Adult; Chondroma; Diaphyses; Female; Humans; Humerus; Shoulder Pain
PubMed: 30686678
DOI: 10.1016/j.aprim.2018.12.003 -
Respiratory Care Dec 2014Endobronchial chondromas are rare benign tumors. They may be part of the Carney triad; however, isolated endobronchial chondromas can occur in the larynx, trachea, and...
Endobronchial chondromas are rare benign tumors. They may be part of the Carney triad; however, isolated endobronchial chondromas can occur in the larynx, trachea, and bronchi. We report a case of an endobronchial chondroma in a 61-y-old man with an incidental 5-mm endobronchial polypoid lesion at the proximal left main bronchus that was later found to be an endobronchial chondroma. Flexible bronchoscopy demonstrated a pedunculated, vascularized, pink mass. An excisional biopsy revealed a well-circumscribed lesion with nodular areas of mature cartilage and myxoid tissue confirmatory of an endobronchial chondroma. We reviewed a total of 23 cases of isolated endobronchial chondromas reported in the medical literature. Despite their rarity, endobronchial chondromas should be considered in the differential diagnosis of endobronchial tumors. Prompt recognition and resection of endobronchial chondromas is important to prevent airway obstruction and its associated complications.
Topics: Biopsy; Bronchial Neoplasms; Bronchoscopy; Chondroma; Humans; Incidental Findings; Male; Middle Aged; Radiography
PubMed: 25233382
DOI: 10.4187/respcare.02673 -
European Annals of Otorhinolaryngology,... May 2021
Topics: Chondroma; Humans; Laryngeal Neoplasms; Larynx
PubMed: 32811795
DOI: 10.1016/j.anorl.2020.08.003 -
Proceedings of the Royal Society of... May 1946
PubMed: 19993310
DOI: No ID Found -
Archives of Pathology & Laboratory... Jan 2020Cartilaginous tumors represent one of the most common tumors of bone. Management of these tumors includes observation, curettage, and surgical excision or resection,... (Review)
Review
CONTEXT.—
Cartilaginous tumors represent one of the most common tumors of bone. Management of these tumors includes observation, curettage, and surgical excision or resection, depending on their locations and whether they are benign or malignant. They can be diagnostically challenging, particularly in small biopsies. In rare cases, benign tumors may undergo malignant transformation.
OBJECTIVE.—
To review common cartilaginous tumors, including in patients with multiple hereditary exostosis, Ollier disease, and Maffucci syndrome, and to discuss problems in the interpretation of well-differentiated cartilaginous neoplasms of bone. Additionally, the concept of atypical cartilaginous tumor/chondrosarcoma grade 1 will be discussed and its use clarified.
DATA SOURCES.—
PubMed (US National Library of Medicine, Bethesda, Maryland) literature review, case review of archival cases at the Massachusetts General Hospital, and personal experience of the authors.
CONCLUSIONS.—
This review has examined primary well-differentiated cartilaginous lesions of bone, including their differential diagnosis and approach to management. Because of the frequent overlap in histologic features, particularly between low-grade chondrosarcoma and enchondroma, evaluation of well-differentiated cartilaginous lesions should be undertaken in conjunction with thorough review of the imaging studies.
Topics: Bone Neoplasms; Chondroblastoma; Chondroma; Chondrosarcoma; Diagnosis, Differential; Humans
PubMed: 31877083
DOI: 10.5858/arpa.2019-0441-RA -
Indian Journal of Otolaryngology and... Jul 1997Chondroma of thyroid cartilage is a rare condition. Two cases of laryngeal chondroma diagnosed clinically and confirmed histopathologically are presented. Surgical...
Chondroma of thyroid cartilage is a rare condition. Two cases of laryngeal chondroma diagnosed clinically and confirmed histopathologically are presented. Surgical excision was undertaken in both the cases.
PubMed: 23119313
DOI: 10.1007/BF02991295 -
The Journal of Thoracic and... May 2020
Topics: Adult; Aorta, Thoracic; Chondroma; Female; Humans; Margins of Excision; Neoplasm Invasiveness; Neoplasm Recurrence, Local; Reoperation; Treatment Outcome; Vascular Neoplasms
PubMed: 31174859
DOI: 10.1016/j.jtcvs.2019.04.011 -
Journal of Neurosurgery. Case Lessons Feb 2021Chondromas are benign cartilaginous tumors that are rarely seen in the brain.
BACKGROUND
Chondromas are benign cartilaginous tumors that are rarely seen in the brain.
OBSERVATIONS
A 58-year-old woman had undergone routine brain imaging after a motor vehicle accident and was incidentally found to have a right falcine lesion. Contrast magnetic resonance imaging showed a mostly nonenhancing mass with discontinuous rim enhancement. She was taken to the operating room and pathology revealed a chondroma.
LESSONS
Falcine intracranial chondromas are rare and typically misdiagnosed as meningiomas. Chondromas should be in the differential for patients presenting with nonenhancing falcine lesions.
PubMed: 36045938
DOI: 10.3171/CASE20124