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Revista Portuguesa de Cardiologia Dec 2018
Topics: Fibroma; Heart Neoplasms; Humans
PubMed: 30545746
DOI: 10.1016/j.repc.2018.11.006 -
In Vivo (Athens, Greece) 2020Low-grade fibromyxoid sarcoma (LGFMS) is a rare sarcoma subtype with a generally indolent pattern of clinical behaviour, but treatments for advanced disease are limited.
BACKGROUND
Low-grade fibromyxoid sarcoma (LGFMS) is a rare sarcoma subtype with a generally indolent pattern of clinical behaviour, but treatments for advanced disease are limited.
PATIENTS AND METHODS
A retrospective search of a prospectively maintained institutional database identified 102 patients treated from December 1994 to August 2018. We evaluated the outcome of patients and the efficacy and safety of non-surgical therapies in LGFMS.
RESULTS
Ninety-four out of 102 (92.2%) underwent primary resection, seven (6.9%) were treated with systemic therapy and one (1.0%) is currently being treated with pre-operative radiotherapy. The RECIST 1.1 response rate to first-line chemotherapy was 0%, and median progression-free survival was 1.84 months (95% confidence intervaI=0.10-3.6 months).
CONCLUSION
Conventional systemic therapy has limited efficacy in advanced LGFMS.
Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Combined Modality Therapy; Female; Fibroma; Fibrosarcoma; Follow-Up Studies; Humans; Male; Middle Aged; Neoplasm Grading; Prospective Studies; Retrospective Studies; Survival Rate
PubMed: 31882484
DOI: 10.21873/invivo.11766 -
Molecular Genetics & Genomic Medicine Sep 2022Ovarian sex cord-stromal tumors (OSCTs) are rare ovarian tumors that can develop from sex cord, stromal cells, or both. OSCTs can be benign or malignant. Bilateral...
BACKGROUND
Ovarian sex cord-stromal tumors (OSCTs) are rare ovarian tumors that can develop from sex cord, stromal cells, or both. OSCTs can be benign or malignant. Bilateral and/or unilateral ovarian fibromas, a type of OSCT of the stromal cells, have been reported in individuals diagnosed with nevoid basal cell carcinoma syndrome (NBCCS). Calcified ovarian fibromas have been reported in 15-25% of individuals diagnosed with NBCCS while 75% of those cases occur bilaterally. The average age at diagnosis of OSCT/ovarian fibromas in patients with NBCSS is in the second to third decade compared with age 50 in the general population. Ovarian tumors are rare in pediatric populations.
METHODS
The patient is a 5-year-old female diagnosed with bilateral ovarian fibromas at age 4. Multigene panel for the patient and subsequent targeted molecular evaluation of parents were completed. Histological evaluations on the surgically resected ovaries were performed for microscopic characterization of fibromas.
RESULTS
Germline testing identified de novo heterozygous novel likely pathogenic variants in PTCH1 gene, exon 12 deletion, and an SMARCA4 splicing variant c.2002-1G > A. Microscopic examination of bilateral tumors was consistent with an ovarian fibroma.
CONCLUSIONS
To our knowledge, this is the first report of bilateral benign ovarian fibroma in a child with a diagnosis of nevoid basal cell carcinoma syndrome (NBCCS) with a potential predisposition to Rhabdoid Tumor Predisposition Syndrome (RTPS).
Topics: Basal Cell Nevus Syndrome; Child; Child, Preschool; DNA Helicases; Female; Fibroma; Germ Cells; Humans; Middle Aged; Nuclear Proteins; Ovarian Neoplasms; Transcription Factors
PubMed: 35775118
DOI: 10.1002/mgg3.2005 -
The Journal of Thoracic and... Oct 2018
Topics: Atrial Appendage; Fibroma; Heart Neoplasms; Humans
PubMed: 29903405
DOI: 10.1016/j.jtcvs.2018.04.114 -
BMC Musculoskeletal Disorders May 2022Neuromuscular choristomas (NMCs), are extremely rare developmental lesions that, have been previously established associated with recurrent fibromatosis after surgery,...
BACKGROUND
Neuromuscular choristomas (NMCs), are extremely rare developmental lesions that, have been previously established associated with recurrent fibromatosis after surgery, leading to several operations or even amputation. However, reports on the ultrasound imaging features and clinical conditions of NMCs are rare. The purpose of this study is to describe the ultrasound features and clinical analysis of NMCs to provide suggestions to identify the optimal management strategy.
METHODS
From September 2020 to September 2021, 7 patients with a confirmed diagnosis of NMC who underwent ultrasound examination in our department were enrolled in our study. Physical examinations were performed to detect motor deficits, sensory deficits, neuropathic pain, limb undergrowth, muscular atrophy, cavus foot and bone dysplasia. Ultrasound imaging was performed and investigated both in affected nerves and neuromuscular choristomas associated desmoid-type fibromatosis (NMC-DTF). All patients had a definite history and regular follow-up. The clinical course, physical examinations, ultrasound features and pathologic results of NMC patients were analyzed.
RESULTS
Seven patients with an average age of 7.0 ± 7.2 years (range: 2-22 years) were enrolled in our study. The affected nerves included the sciatic nerve (6 cases) and the brachial plexus (1 case). Six patients (85.7%) presented with limb undergrowth, 6 (85.7%) with muscular atrophy, and 5 (71.4%) with cavus foot deformity. Based on ultrasound findings, all the visibly affected nerve segments presented with hypoechoic and fusiform enlargement with intraneural skeletal muscle elements. Five patients (71.4%) had NMC-DTFs at the site of the affected nerve. All NMC-DTFs were shown as hypoechoic solid lesions adjacent to the nerve and were well circumscribed. In the subset of the surgery group, all 5 patients presented with progression to NMC-DTFs at the site of the NMCs. No fibromatosis was detected in the other two nonsurgical patients.
CONCLUSIONS
Understanding the typical ultrasound features and clinically associated conditions would support the early diagnosis of this rare disease. When a potential diagnosis is determined, an invasive procedure such as biopsy or resection might not be a good choice given the frequent occurrence of complications such as aggressive recurrence.
Topics: Adolescent; Child; Choristoma; Fibroma; Fibromatosis, Aggressive; Hamartoma; Humans; Muscle, Skeletal; Muscular Atrophy; Rare Diseases
PubMed: 35581638
DOI: 10.1186/s12891-022-05238-4 -
Medicine Jan 2019Plexiform fibromyxoma (PF) is a rare gastric mesenchymal tumor, with approximately 80 cases reported to date. Gastrointestinal stromal tumor, the most common primary...
BACKGROUND
Plexiform fibromyxoma (PF) is a rare gastric mesenchymal tumor, with approximately 80 cases reported to date. Gastrointestinal stromal tumor, the most common primary mesenchymal tumor of the stomach, shows different biological and clinical characteristics between adult and pediatric patients.
OBJECTIVES
This systematic literature review was conducted to elucidate the pathological and clinical features of pediatric PF compared to adult PF.
METHODS
MEDLINE (1948 to March 2018) and EMBASE (1947 to March 2018) were searched, and all English articles that reported clinical data on PF patients were identified. Two authors independently reviewed the articles and extracted data to assess immunohistochemistry, sex, chief complaint, tumor size, tumor-related mortality, and tumor recurrence and metastasis.
RESULTS
A total of 41 reports with 80 PF patients (of whom 70 were adult PF and 10 were pediatric PF patients) confirmed by histological and immunohistochemical findings were included. Of a total of 80 tumors, 62 (78%) were located in the gastric antrum, 42 (65%) presented with ulceration, and 48 (74%) were resected by partial gastrectomy. Median tumor size of the resected specimen was larger in pediatric PF than in adult PF cases (5.3 cm vs 4.0 cm, P = .036). However, there was no difference between pediatric and adult PFs in immunohistochemical expression, sex predominance, chief complaint, tumor-related mortality, and tumor recurrence and metastasis during the follow-up periods.
CONCLUSION
Other than increased tumor growth in pediatric PFs, PF is a single disease entity with similar pathological features and benign clinical behavior regardless of onset age.
Topics: Adult; Child; Diagnosis, Differential; Female; Fibroma; Gastrectomy; Gastrointestinal Stromal Tumors; Humans; Immunohistochemistry; Male; Middle Aged; Stomach; Stomach Neoplasms
PubMed: 30653169
DOI: 10.1097/MD.0000000000014186 -
Medicina 2020
Topics: Breast Neoplasms; Fibroma; Fibromatosis, Aggressive; Humans
PubMed: 33048808
DOI: No ID Found -
Endocrine Regulations Apr 2020Fibromatosis is a rare tumor with a prevalence of 2-4 per million per year. Its occur-rence in the thyroid is very rare and to the best of our knowledge only five cases...
Fibromatosis is a rare tumor with a prevalence of 2-4 per million per year. Its occur-rence in the thyroid is very rare and to the best of our knowledge only five cases have been reported in the literature.Subject and Results. In this case report, we present a thirty-seven-year-old man without any significant past medical history, who presented with an anterior neck swelling and occasional cough for four months before admission. Sonographic-guided fine needle aspiration cytology studies reported a follicular neoplasm. Microscopic and immunohistochemical evaluation of the thyroidectomy specimen showed thyroid fibromatosis. The patient had no symptoms after surgery and no signs of recurrence after 2 years of follow-up.
Topics: Adenocarcinoma, Follicular; Adult; Fibroma; Humans; Male; Thyroid Neoplasms
PubMed: 32597156
DOI: 10.2478/enr-2020-0016 -
Ugeskrift For Laeger Sep 2014Elastofibroma dorsi (ED) is a benign, slow-growing soft tissue pseudo-tumor and is regarded as rare. The aetiology is unclear and ED is often associated with only...
Elastofibroma dorsi (ED) is a benign, slow-growing soft tissue pseudo-tumor and is regarded as rare. The aetiology is unclear and ED is often associated with only distinct symptoms. This case report describes a 55-year-old woman with bilateral ED, who underwent surgical treatment with great result. Some prevalence studies suggest that ED is more common than previously assumed, and on suspicion of a low diagnostic rate the importance of sufficient primary examination is therefore emphasized, especially since surgical treatment of symptomatic ED is associated with fine results in case of symptoms, and low complication rate.
Topics: Female; Fibroma; Humans; Magnetic Resonance Imaging; Middle Aged; Scapula; Soft Tissue Neoplasms
PubMed: 25294509
DOI: No ID Found -
Head and Neck Pathology Sep 2021Gingival growths, barring a few are mostly reactive and seldom exhibit significant true neoplastic potential. The common etiology is local irritation from dental...
Gingival growths, barring a few are mostly reactive and seldom exhibit significant true neoplastic potential. The common etiology is local irritation from dental plaque/calculus, trauma as well as medication-related overgrowth. Such lesions are easily distinguishable and categorized into diagnoses such as pyogenic granuloma, peripheral ossifying fibroma, etc. We present a previously undescribed, but commonly encountered, reactive gingival growth with unique histologic features and suggest the diagnostic term "gingival fibroma." An IRB approved retrospective review of the University of Florida Oral Pathology Biopsy Service encompassing years 2010-2019, was performed to select cases. Demographics, clinical data, and microscopic diagnoses were recorded and analyzed. Four board-certified oral and maxillofacial pathologists agreed upon and established the diagnostic criteria. These are: a prominent fibromyxoid stroma, variable cellularity, a whorled or storiform pattern of arrangement of the cellular elements, lack of significant inflammation or vascularity, and complete absence of calcification, and/or odontogenic islands. A total of 60 cases met all criteria and were included in the study. Age range in years was 14-87 with the mean at 45.11 years. A striking female predilection (90%) was noted. Approximately 62% of cases were reported on the maxillary gingiva, followed by 38.3% in the mandibular gingiva. Majority, 66.7% were in the anterior incisor region followed by 11.7% in the canine/first premolar areas. All lesions were submitted as excisional biopsy, and 4 cases recurred within 2-3 years of excision. In all cases, lesional tissue appeared to extend to the surgical base of the specimen. We present 60 cases of a histologically unique entity occurring exclusively on the gingiva and introduce the diagnostic term "Gingival Fibroma" for these lesions. Further studies with adequate clinical follow-up may help understand the exact clinical behavior of these lesions.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Female; Fibroma; Gingival Neoplasms; Humans; Male; Middle Aged; Retrospective Studies; Young Adult
PubMed: 33686583
DOI: 10.1007/s12105-021-01315-7