Clinics and genetic background of hereditary gingival fibromatosis

Hereditary gingival fibromatosis (HGF) is a rare condition characterized by slowly progressive overgrowth of the gingiva. The severity of overgrowth may differ from mild causing phonetic and masticatory issues...
Source: Orphanet Journal of Rare Diseases - Category: Internal Medicine Authors: Tags: Review Source Type: research

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Desmoid tumor is a rare disease, which is histologically characterized by local invasion, monoclonality, and fibroblast proliferation; and clinically characterized by a variable and often unpredictable course. The treatment of desmoid tumor is mainly surgical resection, but the recurrence rate is high. In recent years, a variety of treatment methods, including endocrine therapy, surgery, radiotherapy, chemotherapy, non-steroidal anti-inflammatory drugs, targeted drugs, interferon and more, have been used and achieved certain curative effects. In addition, in view of the inertia characteristics of desmoid tumor, observation...
Source: Frontiers in Oncology - Category: Cancer & Oncology Source Type: research
ConclusionWe experienced successful treatment for an inoperable case of desmoid-type fibromatosis with hydronephrosis. Moderate-dose radiation therapy alone is an effective and feasible approach for the management of hydronephrosis associated with desmoid-type fibromatosis.
Source: Journal of Medical Case Reports - Category: General Medicine Source Type: research
ConclusionsDesmoplastic fibroblastoma of the mediastinum is an extremely rare disease. Preoperative diagnosis is difficult. Early surgical resection is suitable for diagnosis and treatment planning.
Source: Journal of Medical Case Reports - Category: General Medicine Source Type: research
Ann Ital Chir. 2021 Sep 10;92:S2239253X21035064.ABSTRACTMandible can be affected by a great variety of neoformations, like aneurysmal bone cyst, odontogenic myxoma, CGCG (Central Giant Cell Granuloma), GCT (giant cell tumor), sarcoma, ameloblastoma, lymphoma, ossifyng fibroma, odontogenic mixoma, granuloma, arteriovenous malformations and Schwannoma. Occasionally is not possible to find clinical or radiological distinctive findings so is usefull to perform additional exams, think about rare disease and perform an explorative surgical treatment which can be adapted to the intraoperatory findings. This attitude may help to r...
Source: Annali Italiani di Chirurgia - Category: Surgery Authors: Source Type: research
ConclusionsA good trend was found in the questionnaire survey. It will be further necessary to disseminate clinical practice guidelines to physicians more widely, and to have them understand and implement the most up-to-date medical practice strategies for this rare disease.
Source: Japanese Journal of Clinical Oncology - Category: Cancer & Oncology Source Type: research
We report 3 rare disease entities, presenting with multifocal osteolytic lesions in the wrist. They all presented with similar clinical manifestations, and the final diagnoses were made via pathological evaluation. Compared with tenosynovial giant cell tumor and calcifying aponeurotic fibroma, rheumatoid arthritis had the poorest outcome.
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
Authors: Zhang Z, Shi J, Yang T, Liu T, Zhang K Abstract Aggressive fibromatosis or desmoid tumor is a rare disease resulting from fibroblasts which do not metastasize. However, desmoid tumors belong to low-grade malignant tumors since they have high potential to infiltrate surrounding tissues, causing high local recurrence rates and may affect surrounding organs, threatening life quality and expectancy. Although surgery, watch and wait, radiotherapy, chemotherapy, high intensity focused ultrasound, ablation techniques or several agents have all been frequently investigated for the treatment of this type of disease...
Source: Oncology Letters - Category: Cancer & Oncology Tags: Oncol Lett Source Type: research
Conclusions : We should consider desmoid-type fibromatosis when we find a large abdominal mass, but it may be difficult to diagnose based only on imaging findings. Immunohistochemical examination of the specimen may make the diagnosis. J. Med. Invest. 67 : 375-377, August, 2020. PMID: 33148921 [PubMed - in process]
Source: Journal of Medical Investigation - Category: General Medicine Tags: J Med Invest Source Type: research
This study aimed to report the long-term clinical course of benign fibro-osseous lesions (BFOLs) in the paranasal sinuses, including clinical and radiologic features. Methods: Radiologically confirmed BFOLs except osteoma cases were reviewed retrospectively between 1994 and 2016. We compared demographic data between the surgery and observation groups. Reason for image study, radiographic features, histopathology, and clinical course with serial image scans were analyzed. Results: A total of 183 subjects were selected from a thorough review of head and neck radiologic tests (n= 606,068) in a tertiary referral hospit...
Source: Clinical and Experimental Otorhinolaryngology - Category: ENT & OMF Tags: Clin Exp Otorhinolaryngol Source Type: research
CONCLUSIONS: In conclusion, cardiac tumor is a rare but nonneglectable reason for arrhythmia, and surgical resection is the optimal procedure, with satisfactory results. PMID: 32364911 [PubMed - as supplied by publisher]
Source: The Heart Surgery Forum - Category: Cardiovascular & Thoracic Surgery Authors: Tags: Heart Surg Forum Source Type: research
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