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Last update 08 May 2025

Fibromatosis, Gingival, Hereditary

Last update 08 May 2025

Basic Info

Synonyms

Autosomal dominant gingival fibromatosis, Autosomal dominant gingival hyperplasia, FIBROMATOSIS, GINGIVAL, 1

+ [12]

Introduction

Hereditary gingival fibromatosis (HGF) is a rare benign, slowly progressive, non-inflammatory fibrous hyperplasia of the maxillary and mandibular gingivae that generally occurs with the eruption of the permanent (or more rarely the primary) dentition or even at birth. It presents as a localized or generalized, smooth or nodular overgrowth of the gingival tissues of varying severity. It can be isolated, with autosomal dominant inheritance, or as part of a syndrome.

Drugs associated with Fibromatosis, Gingival, Hereditary

Vebreltinib

Target

c-Met

Mechanism

c-Met inhibitors

Active Org.

Apollomics, Inc. (United States) [+2]

Originator Org.

Zhejiang Crownmab Biotech Co. Ltd.

Active Indication

Astrocytoma, IDH-Mutant [+21]

Inactive Indication

Ganglioglioma [+3]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

China

First Approval Date14 Nov 2023

Clinical Trials associated with Fibromatosis, Gingival, Hereditary

ChiCTR2100048862

/ RecruitingNot ApplicableIIT

Genetic characteristics of hereditary gingival fibromatosis

Start Date20 Jul 2021

Sponsor / Collaborator-

NCT03175224

/ RecruitingPhase 2

Phase 1 / 2 Multicenter Study of the Safety, Pharmacokinetics, and Preliminary Efficacy of APL-101 in Subjects With Non-Small Cell Lung Cancer With c-Met EXON 14 Skip Mutations and c-Met Dysregulation Advanced Solid Tumors

To assess:
efficacy of APL-101 as monotherapy for the treatment of NSCLC harboring MET Exon 14 skipping mutations, NSCLC harboring MET amplification, solid tumors harboring MET amplification, solid tumors harboring MET fusion, primary CNS tumors harboring MET alterations, solid tumors harboring wild-type MET with overexpression of HGF and MET
efficacy of APL-101 as an add-on therapy to EGFR inhibitor for the treatment of NSCLC harboring EGFR activating mutations and developed acquired resistance with MET amplification and disease progression after documented CR or PR with 1st line EGFR inhibitors (EGFR-I)

Start Date27 Sep 2017

Sponsor / Collaborator

Apollomics, Inc. (United States)

100 Clinical Results associated with Fibromatosis, Gingival, Hereditary

100 Translational Medicine associated with Fibromatosis, Gingival, Hereditary

0 Patents (Medical) associated with Fibromatosis, Gingival, Hereditary

235

Literatures (Medical) associated with Fibromatosis, Gingival, Hereditary

01 Nov 2024·Translational Cancer Research

Massive gastrointestinal hemorrhage and iodine-125 seed migration due to hepatogastric fistula: a case report and literature review

Article

Author: He, Chengjian ; Ge, Naijian ; Wang, Xiangdong ; Zhou, Jing ; Yang, Yefa ; Zhang, Xiaofeng

BackgroundHepatogastric fistula (HGF) is an uncommon occurrence that can be associated with various medical conditions. The primary causes typically involve peptic ulcer disease, infections (such as pyogenic, amoebic or tuberculosis), or iatrogenic factors (like post transarterial chemoembolization or radiotherapy). Massive gastrointestinal hemorrhage following HGF is extremely rare, with iodine-125 (¹²⁵I) seed migration to the stomach through HGF not previously documented. This report explores this unique case and reviews other recent instances of rare gastrointestinal hemorrhage due to HGF.Case DescriptionA 32-year-old man with chronic B viral hepatitis underwent emergency surgery to control bleeding due to hepatocellular carcinoma (HCC) rupture. One month postoperatively, an active residual tumor (44 mm × 33 mm) was found in the caudate lobe of the liver. The patient was admitted for percutaneous microwave coagulation therapy (PMCT) and ¹²⁵I seed implantation sequentially. No postoperative discomfort was observed. Subsequent intrahepatic HCC distant recurrences were successfully managed using PMCT and systemic treatments (molecular targeted drug and checkpoint inhibitor). Twenty months after the initial seed implantation in the caudate lobe, the patient was referred again owing to intrahepatic tumor recurrence in the right lobe and underwent repeat PMCT and ¹²⁵I seed implantation. Two days after the second ¹²⁵I seed implantation, the patient presented with severe upper gastrointestinal bleeding and epigastric pain. The caudate lobe was in communication with the lesser curvature of the stomach, resulting in the formation of the HGF. Subsequently, intermittent massive gastrointestinal hemorrhage occurred, and seed implantation in the caudate lobe migrated to the stomach through the HGF. Endoscopy and imaging confirmed HGF and seed migration to the stomach, and surgery was successfully performed.ConclusionsA thorough clinical medical history and heightened vigilance are essential for diagnosing and managing this rare complication.

21 Aug 2024·Cureus

Idiopathic Gingival Fibromatosis: Report of a Rare Case

Article

Author: Shree Abiraami, N.S. ; Umamaheswari, T N ; Ramalingam, Karthikeyan ; Shree Abiraami, N S ; Pillai, Devika S ; Umamaheswari, T.N.

The progressive overgrowth of the gingiva is the hallmark of idiopathic gingival fibromatosis (IGF). Excess gingival tissue can obscure the crown of a tooth, resulting in spaces between teeth, displacement, retention of primary or permanent teeth, and difficulties with feeding, speaking, and appearance. The diagnosis and management of inherited gingival fibromatosis are the focus of this case report. A 12-year-old girl was referred from the Department of Orthodontics to Oral Medicine as a result of progressive gingival enlargement, which impeded orthodontic treatment for misaligned lower front teeth. The patient underwent a conservative periodontal treatment regimen that encompassed gingivectomy and debridement. The excised gingival tissues were submitted for histopathological examination. Tissue sections stained with hematoxylin and eosin showed connective tissue with dense bundles of collagen fibers and little inflammation. The patient was reviewed after three months, and advised of orthodontic management for further aesthetic correction. The findings indicated that the oral symptoms of gingival fibromatosis are influenced by the severity of the condition and the age at which it begins. Early intervention helps mitigate potential difficulties for younger individuals.

23 Jul 2024·Cureus

Idiopathic Gingival Enlargement: A Case Report

Article

Author: Shraddha Kashinath, Marde ; Kumar Kendole, Rohit

Gingival enlargement (GE) is an increase in the size of the gingiva. It may be due to inflammation caused by extensive plaque accumulation, intake of drugs, systemic conditions like pregnancy and puberty, systemic diseases such as leukemia or Wegener's granulomatosis, hereditary gingival fibromatosis, and neoplastic or false enlargement. Idiopathic GE is the massive increase in the size of the gingiva with an unknown etiology. It may have a hereditary basis, be linked to physical impairment, or begin with eruption of primary or permanent dentition. It is also referred as gingivomatosis, hereditary gingival fibromatosis, elephantiasis gingivae, gigantism of the gingiva, or congenital macrogingivae. The enlarged gingiva compromises oral hygiene maintenance, which secondarily adds to the inflammatory component of enlargement. Altogether, this exaggerates the existing condition. This type of extensively disfigured gingiva affects speech, mastication, and esthetics, causes halitosis, and disturbs the overall well-being of the individual. Surgical removal of the enlarged gingiva along with meticulous non-surgical means of plaque control is expected to provide a satisfactory functional and esthetic outcome. This case report presents a rare case of long-standing massive grade III GE extending up to the occlusal level in a 17-year-old systemically healthy, non-syndromic young female involving both arches, thereby posing a diagnostic dilemma. It was treated by gingivectomy using a conventional technique to facilitate precise incision, lower cost, and faster re-epithelialization. This was followed by gingivoplasty using electrocautery. The postoperative results of three months were satisfactory in terms of function and esthetics with uneventful healing. Further follow-up is ongoing for the same.

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Fibromatosis, Gingival, Hereditary

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