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Head and Neck Pathology Mar 2016Several benign and malignant mesenchymal and meningothelial lesions may preferentially affect or extend into the sinonasal tract. Glomangiopericytoma (GPC, formerly... (Review)
Review
Several benign and malignant mesenchymal and meningothelial lesions may preferentially affect or extend into the sinonasal tract. Glomangiopericytoma (GPC, formerly sinonasal-type hemangiopericytoma) is a specific tumor with a predilection to the sinonasal tract. Sinonasal tract polyps with stromal atypia (antrochoanal polyp) demonstrate unique histologic findings in the sinonasal tract. Juvenile nasopharyngeal angiofibroma (JNA) arises from specialized tissue in this location. Meningioma may develop as direct extension from its intracranial counterpart or as an ectopic tumor. Selected benign mesenchymal tumors may arise in the sinonasal tract and pose a unique differential diagnostic consideration, such as solitary fibrous tumor and GPC or lobular capillary hemangioma and JNA. Although benign and malignant vascular, fibrous, fatty, skeletal muscle, and nerve sheath tumors may occur in this location, this paper focuses on a highly select group of rare benign sinonasal tract tumors with their clinicopathological and molecular findings, and differential diagnosis.
Topics: Brain Neoplasms; Humans; Meningioma; Mesenchymoma; Paranasal Sinus Neoplasms
PubMed: 26830398
DOI: 10.1007/s12105-016-0697-6 -
Genes, Chromosomes & Cancer Feb 2019Pediatric soft tissue tumors are relatively rare and show significant overlap in morphology and immunoprofile, often posing diagnostic and management challenges. Thus,... (Review)
Review
Pediatric soft tissue tumors are relatively rare and show significant overlap in morphology and immunoprofile, often posing diagnostic and management challenges. Thus, their classification remains often subjective or lumped under "unclassified categories," as a number of lesions lack objective and reproducible criteria in diagnosis. Although in a subset of cases immunohistochemistry has been proved useful to identify a specific line of differentiation, most tumors lack a readily defined histogenesis, being characterized by a rather non-specific immunoprofile. Furthermore, tumors with an ambiguous diagnosis are difficult to grade and their risk of malignancy or clinical management remains uncertain. Advances in molecular genetics, including the more wide application of next generation sequencing in routine clinical practice, have improved diagnosis and refined classification based on objective molecular markers. Importantly, some soft tissue tumors in children are characterized by recurrent gene fusions involving either growth factors (eg, PDGFB) or protein kinases (eg, ALK, ROS, NTRK, BRAF), which have paved the way for new targeted treatments that block the respective upregulated downstream pathways. However, the majority of gene fusions or mutations detected in soft tissue tumors result in an abnormal function of transcription factors or chromatin remodeling. The present review focuses on the latest genetic discoveries in the spectrum of both benign and malignant pediatric soft tissue neoplasia. These genetic abnormalities promise to provide relevant insight for their proper classification, prognosis, and treatment. The entities discussed herein are grouped either based on their shared genetic mechanism or based on their presumed line of differentiation.
Topics: Biomarkers, Tumor; Child; Humans; Mesenchymoma; Oncogene Proteins, Fusion; Soft Tissue Neoplasms
PubMed: 30187985
DOI: 10.1002/gcc.22681 -
Journal of Pathology and Translational... Jan 2021Fibrocartilaginous mesenchymoma is a rare bone tumor, with fewer than 35 cases reported in the literature since 1984. This tumor usually occurs in the long bones of...
Fibrocartilaginous mesenchymoma is a rare bone tumor, with fewer than 35 cases reported in the literature since 1984. This tumor usually occurs in the long bones of children and adolescents. In the current case, the tumor affected a rib. A 17-year-old boy presented with a mass in the right fifth rib. Radiologic findings revealed an osteolytic mass with cortical destruction and calcification; en bloc resection was performed. The tumor showed three distinct histologic features: bland spindle cell proliferation, benign cartilage nodules, and epiphyseal plate-like enchondral ossification. The pathologic diagnosis was fibrocartilaginous mesenchymoma. The patient remains free of disease 1 year after the surgery. Pathological diagnosis of fibrocartilaginous mesenchymoma can be challenging, especially when the tumor occurs in an unusual site. When any fibro-osseous lesion with a cartilaginous component is encountered, the possibility of fibrocartilaginous mesenchymoma should be considered because of its locally aggressive behavior.
PubMed: 33260287
DOI: 10.4132/jptm.2020.10.08 -
Medicine Oct 2018Phosphaturic mesenchymal tumor mixed connective tissue type (PMT/MCT) is the most common type (up to 90%) of phosphaturic mesenchymal tumor (PMT), a rare... (Meta-Analysis)
Meta-Analysis Review
INTRODUCTION
Phosphaturic mesenchymal tumor mixed connective tissue type (PMT/MCT) is the most common type (up to 90%) of phosphaturic mesenchymal tumor (PMT), a rare clinicopathologic entity. Besides overproduction of fibroblast growth factor 23 (FGF23), there is a big variation of immunohistochemical characteristic across types of PMT, which makes it difficult to obtain an early diagnosis of PMT/MCT. As a benign tumor, PMT/MCT usually happens in subcutaneous tissues and leads to nonhealing of wound. A complete excision of PMT/MCT facilitates wound healing.
CONCLUSIONS
Review of the existing evidence indicates that early diagnosis of PMT/MCT is critically important when treating PMT/MCT wound. Hence standardization of early diagnosis for PMT/MCT is mandated.
Topics: Biomarkers, Tumor; Diagnosis, Differential; Early Detection of Cancer; Fibroblast Growth Factor-23; Fibroblast Growth Factors; Humans; Hypophosphatemia, Familial; Mesenchymoma; Mixed Connective Tissue Disease; Soft Tissue Neoplasms; Wounds and Injuries
PubMed: 30290606
DOI: 10.1097/MD.0000000000012507 -
Computational and Mathematical Methods... 2022The aim of this study was to explore the application of computed tomography (CT) images in the diagnosis of gastric tumor under the intelligent reconstruction algorithm...
The aim of this study was to explore the application of computed tomography (CT) images in the diagnosis of gastric tumor under the intelligent reconstruction algorithm (IRA). 120 patients with gastric cancer were selected and all the patients underwent CT scanning, and CT images were analyzed based on the Feldkamp-Davis-Kress algorithm (FDK algorithm) to evaluate the imaging features of gastric lesions. According to biopsy or surgical pathology, the detection rate of CT images was calculated. The results showed that there were three pathological types of benign tumors (polyps, leiomyomas, and mesenchymomas) and three pathological types of malignant tumors (mesenchymomas, adenomas, and lymphomas). In addition, the detection rates of CT scans were different, reaching 94.2% on different orientations of the stomach, 90.7% of benign tumors, and 90.9% of malignant tumors, so the detection rate of different orientations was relatively high. CT images based on the FDK IRA could realize a high detection rate in diagnosis, accurately locate the lesion, and display the characteristics of the lesion and the metastasis of surrounding tissues; there were significant differences between benign and malignant gastric tumors in CT images, and the detection effect was obvious, which is worthy of clinical application and promotion.
Topics: Algorithms; Humans; Mesenchymoma; Phantoms, Imaging; Stomach Neoplasms; Tomography, X-Ray Computed
PubMed: 35799664
DOI: 10.1155/2022/8179766 -
International Journal of Surgery Case... 2018Benign Osseous metaplasia of the breast is rare, with only a few cases reported in the literature. Here we present a case of benign osseous metaplasia of the breast...
INTRODUCTION
Benign Osseous metaplasia of the breast is rare, with only a few cases reported in the literature. Here we present a case of benign osseous metaplasia of the breast presenting as a breast lump.
CASE PRESENTATION
38-year-old previously well woman presented with a one-year history of bilateral breast pain and a left-sided breast lump. Ultrasound and mammography suggested calcified fibroadenoma. An ultrasound-guided true cut biopsy revealed fibrous tissue containing foci of adenosis in the presence of a myoepithelial cell layer. Excision biopsy was performed, and histopathological examination showed bone matrix deposition occupying most of the nodule with peripheral hyalinized tissue but no evidence of malignancy. A diagnosis of benign osseous metaplasia of the breast was made, and the patient recovered well without recurrence after lump excision.
DISCUSSION
Only a few cases of osseous metaplasia are reported in the literature. Most reported cases are malignant, such as in fibrosarcoma, malignant mesenchymoma, osteoid sarcoma, osteogenic sarcoma, and osteochondrosarcoma.Very few cases of osseous sarcoma are reported in benign lesions such as fibroadenoma, pleomorphic adenoma, benign mesenchymoma, phyllodes tumor, and amyloid tumor of the breast. Joshi et al. first reported a case of benign osseous metaplasia of the breast presenting as breast lump in an HIV-positive patient [18]. We, therefore, consider this case to be the second case report of benign osseous metaplasia of the breast presenting as a breast lump, but the patient had no chronic illness.
CONCLUSION
A breast lump can be the first presentation of benign osseous metaplasia.
PubMed: 29482081
DOI: 10.1016/j.ijscr.2018.02.025 -
World Journal of Gastroenterology Jun 2010Plexiform angiomyxoid myofibroblastic tumor of the stomach is a unique mesenchymal tumor that we first described in 2007. The tumor is very rare, and to date, only 18... (Review)
Review
Plexiform angiomyxoid myofibroblastic tumor of the stomach is a unique mesenchymal tumor that we first described in 2007. The tumor is very rare, and to date, only 18 cases confirmed by immunohistochemistry have been reported in the literature. The patients' ages ranged from 7 to 75 years (mean, 43 years), and the male-to-female ratio was approximately 1:1. Representative clinical symptoms are ulceration, associated upper gastrointestinal bleeding (hematemesis), and anemia. The tumors are located at the antrum in all cases, and grossly, the tumor is whitish to brownish or reddish, and forms a lobulated submucosal or transmural mass. Microscopically, the tumor is characterized by a plexiform growth pattern, the proliferation of cytologically bland spindle cells, and a myxoid stroma that is rich in small vessels and positive for Alcian blue stain. Immunohistochemically, the tumor cells are positive for alpha-smooth muscle actin and negative for KIT and CD34. Differential diagnoses include gastrointestinal stromal tumor and other mesenchymal tumors of the gastrointestinal tract. Some authors proposed that this tumor should be designated as "plexiform fibromyxoma", but this designation might cause confusion. The tumor is probably benign and thus far, neither recurrence nor metastasis has been reported.
Topics: Adolescent; Adult; Aged; Child; DNA Mutational Analysis; Diagnosis, Differential; Female; Fibroma; Gastrointestinal Stromal Tumors; Humans; Immunohistochemistry; Male; Mesenchymoma; Middle Aged; Stomach Neoplasms; Young Adult
PubMed: 20556828
DOI: 10.3748/wjg.v16.i23.2835 -
Eplasty 2017Lipomas are very common benign tumors located in any part of the body in which fat is normally present, but lipomas containing both osseous and cartilaginous elements...
Lipomas are very common benign tumors located in any part of the body in which fat is normally present, but lipomas containing both osseous and cartilaginous elements are rare. A case of osteochondrolipoma in a 72-year-old man is reported. The tumor in the mental region was 2×1.5×1.5 cm. After resection of the tumor, there has been no recurrence during the 6-month postoperative follow-up. Histological examination confirmed the definitive diagnosis. Osteochondrolipoma is an extremely unusual lesion that should be kept in mind in the differential diagnosis of soft-tissue tumors.
PubMed: 29238440
DOI: No ID Found -
ORL; Journal For Oto-rhino-laryngology... 2017Benign mesenchymal sinonasal neoplasms (BMSN) are rare and histologically heterogeneous. Differential diagnosis, appropriate management, and outcome are still a matter... (Comparative Study)
Comparative Study
PURPOSE
Benign mesenchymal sinonasal neoplasms (BMSN) are rare and histologically heterogeneous. Differential diagnosis, appropriate management, and outcome are still a matter of debate. The aim of this study is to provide evidence for further refinement of assessment and treatment in the future.
PROCEDURES
We retrospectively reviewed data on 93 patients with neuroradiologically verified BMSN treated at our university reference center during the past 22 years.
RESULTS
The most frequent BMSN recorded in our cohort was osteoma of the frontal sinus. Only one-third of the patients affected were symptomatic at initial presentation. The 2 other common fibro-osseous tumor entities, fibrous dysplasia and ossifying fibroma, were confirmed in 12 and 6 patients, respectively. Patients with soft tissue tumor entities such as hemangioma, glomangiopericytoma, angiofibroma, and hamartoma were all symptomatic and underwent surgical resection.
CONCLUSION
Understanding and recognizing the spectrum of appearances of benign mesenchymal sinonasal tumors will improve patient assessment and clinical management. The pathognomonic neuroradiological signs of a particular tumor entity should be actively sought as the neuroradiological features may be the diagnostic clues. Computed tomography and magnetic resonance imaging play complementary roles in identifying the morphological details and locoregional staging of benign mesenchymal sinonasal tumors.
Topics: Adult; Aged; Biopsy, Needle; Cohort Studies; Female; Fibroma, Ossifying; Humans; Immunohistochemistry; Magnetic Resonance Imaging; Male; Mesenchymoma; Middle Aged; Neoplasm Invasiveness; Neoplasm Staging; Osteoma; Paranasal Sinus Neoplasms; Prognosis; Rare Diseases; Retrospective Studies; Switzerland; Tomography, X-Ray Computed
PubMed: 28391267
DOI: 10.1159/000468945 -
Head and Neck Pathology Dec 2022Nasal chondromesenchymal hamartoma (NCMH) is a very rare, benign sinonasal tract tumor commonly affecting infants. In this paper, in addition to presenting a systematic... (Review)
Review
Nasal chondromesenchymal hamartoma (NCMH) is a very rare, benign sinonasal tract tumor commonly affecting infants. In this paper, in addition to presenting a systematic review of the literature on NCMH, we also report an unusual case of NCMH in an adolescent patient. A systematic review conducted following the PRISMA guidelines. PubMed, EMBASE and manual search through references of relevant publication were utilised to gather all published case-reports of NCMH. Data collected from each case-report for patient demographics, site and size of NCMH, clinical presentation, co-morbidities, diagnostic methods, treatment options and follow-up methods. The systemic review collected sixty-two case-reports of NCMH (including our case) affecting 42 men and 21 women (2:1 male to female ratio). Mean average age was 5.1 years (age range: 1 day to 70 years). The anatomical sites of the tumor were: nasal cavity (n = 17), paranasal sinuses (n = 30), orbital region (n = 17), and the base of the skull (n = 16). The reported clinical manifestations were nasal obstruction or congestion (n = 29), nasal mass (n = 27), epistaxis (n = 6), orbital symptoms (n = 14). NCMH is a very rare cause of nasal masses in infants and toddlers. Our case and previous case reports confirm that NCMH can mimic other benign and malignant tumors, therefore we should be vigilant for rare pathologies that lead to nasal masses. Recently the link between DIECR1 mutation with NCMH has been established, so NCMH should be considered in any patient with nasal or orbital symptoms with a history of DICER1-related tumor spectrum.
Topics: Female; Male; Humans; Adolescent; Child, Preschool; Infant; Neoplasms; Ribonuclease III; DEAD-box RNA Helicases
PubMed: 35507301
DOI: 10.1007/s12105-022-01452-7