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Chinese Medical Journal Oct 2019
Topics: Adolescent; Femur; Humans; Hypophosphatemia; Male; Mesenchymoma; Neoplasms, Connective Tissue; Osteomalacia; Paraneoplastic Syndromes; Soft Tissue Neoplasms
PubMed: 31567385
DOI: 10.1097/CM9.0000000000000458 -
Frontiers in Oncology 2019Ectopic ovaries are a rare occurrence. A 33-year-old woman presented to our unit for evaluation of a 2-year history of sporadic abdominal pain that was becoming sharp...
Ectopic ovaries are a rare occurrence. A 33-year-old woman presented to our unit for evaluation of a 2-year history of sporadic abdominal pain that was becoming sharp and frequent. Computed tomography (CT) suggested a gastrointestinal tract mesenchymoma. An abdominal laparotomy was performed and the tumor was excised for pathologic evaluation. A rapid frozen section pathologic examination showed a solitary fibrous tumor (SFT). The final pathology report was an ectopic ovary with corpora lutea bleeding. Ectopic ovaries are benign and the present case is the first report involving an ectopic ovary mimicking a gastrointestinal stromal tumor (GIST). The patient recovered well after surgery. Maldevelopment of the genital tract can lead to ectopic ovaries and surgery is a good management choice. The present case provides a possible differential diagnosis for GISTs.
PubMed: 31334114
DOI: 10.3389/fonc.2019.00580 -
Medicine Jul 2019Tumor-induced osteomalacia causing by phosphaturic mesenchymal tumor of the foot is exceedingly rare, thus may bring great challenges to the timely and proper diagnosis...
Tumor-induced osteomalacia causing by phosphaturic mesenchymal tumor of the foot is exceedingly rare, thus may bring great challenges to the timely and proper diagnosis and treatment of clinicians. The only definitive management is removal of the phosphaturic mesenchymal tumor completely. The objective of this article is to report 2 unusual cases with tumor-induced osteomalacia causing by phosphaturic mesenchymal tumor of the foot.We describe 2 patients with phosphaturic mesenchymal tumor involving the foot who were successfully treated with tumor resection. On presentation to our institution, the patients both had signs of severe osteomalacia, and the patients' most outstanding complaints were diffuse bone pain, general weakness, and disabled walking. A 53-year-old female underwent surgical excision of pathogenic tumor on the sole of left foot. A 62-year-old female underwent complete excision of pathogenic tumor of right plantar. The patients showed appropriate destruction of the tumor, adequate pain relief, and the elevated blood phosphorus levels compared with the previous status.Surgical resection is the most effective treatment option for patients with tumor-induced osteomalacia who can undergo appropriate surgical treatment. This represents a safe and reasonable approach to sustainably relieve pain and other symptoms with tumor-induced osteomalacia in the foot.
Topics: Female; Follow-Up Studies; Foot; Humans; Magnetic Resonance Imaging; Mesenchymoma; Middle Aged; Neoplasms, Connective Tissue; Osteomalacia; Paraneoplastic Syndromes; Soft Tissue Neoplasms
PubMed: 31277164
DOI: 10.1097/MD.0000000000016296 -
Ultrasound in Obstetrics & Gynecology :... Feb 2020
Topics: Adult; Female; Humans; Imaging, Three-Dimensional; Medical Illustration; Mesenchymoma; Placenta Diseases; Pregnancy; Pregnancy Complications, Neoplastic; Ultrasonography, Prenatal; Umbilical Cord
PubMed: 31115104
DOI: 10.1002/uog.20349 -
Medicina 2018The condition of immunosuppressed increases the risk of cancer in kidney transplant patients, as compared to the general population. The best survival of inmunosupressed...
The condition of immunosuppressed increases the risk of cancer in kidney transplant patients, as compared to the general population. The best survival of inmunosupressed patients in recent years has turned both neoplasms and cardiovascular diseases into the main causes of morbidity and mortality. We present the case of a renal transplanted patient who developed an unusual form of mesenchymal tumor such as the aggressive angiomyxoma, four years after the implant and requiring wide surgical resection.
Topics: Abdominal Neoplasms; Adult; Humans; Immunocompetence; Immunosuppressive Agents; Kidney Transplantation; Magnetic Resonance Spectroscopy; Male; Mesenchymoma; Myxoma; Risk Factors
PubMed: 30504112
DOI: No ID Found -
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 -
Modern Pathology : An Official Journal... Feb 2019Information on the heterogeneity of phosphaturic mesenchymal tumor, a rare entity associated with tumor-induced osteomalacia, is limited. In this retrospective analysis... (Review)
Review
Phosphaturic mesenchymal tumor with an admixture of epithelial and mesenchymal elements in the jaws: clinicopathological and immunohistochemical analysis of 22 cases with literature review.
Information on the heterogeneity of phosphaturic mesenchymal tumor, a rare entity associated with tumor-induced osteomalacia, is limited. In this retrospective analysis of 222 phosphaturic mesenchymal tumors, 22 cases exhibited mixed mesenchymal and epithelial elements, which we propose to term "phosphaturic mesenchymal tumor, mixed epithelial, and connective tissue type." Phosphaturic mesenchymal tumor of the mixed epithelial and connective tissue type showed a distinctive and significant male predominance (male:female = 2.67:1), with most patients diagnosed at <40 years old. Moreover, all tumors were mainly located in the alveolar bone with focal invasion into surrounding soft tissue and oral mucosa, which could be detected preoperatively by oral examination. The mesenchymal component, composed of spindled cells resembling fibroblasts or myofibroblasts arranged in a storiform or fascicular pattern, exhibited a less prominent vasculature and lower cellularity than the typical phosphaturic mesenchymal tumor (mixed connective tissue type). The epithelial component was typically haphazardly and diffusely distributed throughout the tumor, forming small, irregular nests resembling odontogenic epithelial nests. All cases were immunoreactive for fibroblast growth factor-23, somatostatin receptor 2A, and NSE in both components. Mostly also demonstrated positive staining for CD99 (21/22, 96%), CD56 (16/22, 73%), Bcl-2 (21/22, 96%), and D2-40 (19/22, 86%) in one or both components. S100 was positive in both components in one of seven cases. Interestingly, immunoreactivity was typically stronger and more diffuse in the epithelial than in the paired mesenchymal components. The mesenchymal component was also diffusely positive for CD68 (17/17, 100%) and showed variable focal staining for SMA (15/22, 68%) and CD34 (9/19, 47 %). These results indicate that phosphaturic mesenchymal tumor of the mixed epithelial and connective tissue type has distinctive clinicopathological characteristics and a polyimmunophenotypic profile.
Topics: Adolescent; Adult; Aged; Female; Humans; Immunohistochemistry; Jaw Neoplasms; Male; Mesenchymoma; Middle Aged; Neoplasms, Connective Tissue; Osteomalacia; Paraneoplastic Syndromes; Retrospective Studies
PubMed: 30206408
DOI: 10.1038/s41379-018-0100-0 -
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 -
Polish Archives of Internal Medicine Sep 2018
Topics: Adult; Antineoplastic Agents, Hormonal; Humans; Male; Mesenchymoma; Neoplasms, Connective Tissue; Octreotide; Osteomalacia; Paraneoplastic Syndromes; Receptors, Somatostatin; Somatostatin; Treatment Outcome
PubMed: 30141426
DOI: 10.20452/pamw.4318 -
European Annals of Otorhinolaryngology,... Oct 2018Oncogenic osteomalacia is a very rare disease usually caused by a phosphaturic mesenchymal tumor, particularly the "mixed connective tissue type", secreting FGF-23... (Review)
Review
INTRODUCTION
Oncogenic osteomalacia is a very rare disease usually caused by a phosphaturic mesenchymal tumor, particularly the "mixed connective tissue type", secreting FGF-23 hormone.
OBJECTIVE
The authors report a case of ethmoid tumor associated with oncogenic osteomalacia and discuss management based on a review of the literature.
CASE SUMMARY
A 41-year-old woman with multiple fractures causing major disability was diagnosed with early-onset osteoporosis. CT scan followed by MRI, performed due to the concomitant presence of nasal obstruction, showed a right ethmoid tumor in contact with the dura mater and periorbital tissues, but with no signs of invasion. Endoscopic resection was performed with reconstruction of the defect of the cribriform plate by a nasoseptal flap. Nasal and bone symptoms subsequently resolved. Histological examination revealed a phosphaturic mesenchymal tumor.
DISCUSSION
Twelve cases of mesenchymal tumor of the ethmoid sinus associated with oncogenic osteomalacia have been reported to date. FGF-23 assay and whole-body MRI with STIR sequence are useful for the diagnosis. A very favorable outcome is observed after surgical treatment in the majority of cases.
Topics: Adult; Ethmoid Sinus; Female; Fibroblast Growth Factor-23; Fibroblast Growth Factors; Humans; Mesenchymoma; Osteomalacia; Paranasal Sinus Neoplasms
PubMed: 30026073
DOI: 10.1016/j.anorl.2018.07.001