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Journal of Clinical Pathology Feb 2001Gastrointestinal stromal tumours (GISTs), initially presumed to be of "true" smooth muscle origin, encompass a heterogeneous, and as yet incompletely understood, group... (Review)
Review
Gastrointestinal stromal tumours (GISTs), initially presumed to be of "true" smooth muscle origin, encompass a heterogeneous, and as yet incompletely understood, group of mesenchymal tumours with respect to their origin, cellular differentiation, and prognosis. Cellular morphology ranges from predominantly spindle shaped to epithelioid in character, whereas differentiation pathways, as determined primarily by immunohistochemistry and ultrastructure, can vary from indeterminate to myoid and/or neural. Recent work has indicated that the interstitial cells of Cajal, a complex cellular network postulated to act as pacemaker cells of the gastrointestinal tract, which exhibit both myoid and neural features, could be candidates for tumour histogenesis. This would provide a plausible and attractive explanation for the variable differentiation pathways identified in the GIST category to date. Nevertheless, the occasional but undisputed location of GISTs outside the gastrointestinal tract (omentum, peritoneum, and retroperitoneum) might mitigate against such an origin, and their histogenesis remains open to debate. The c-kit proto-oncogene, encoding a growth factor receptor with tyrosine kinase activity, has been postulated to play an important role in tumorigenesis because "gain of function" mutations in this gene, localised to chromosome 4q11-21, are being increasingly identified in hereditary and sporadic cases. Monoclonal and polyclonal antibodies directed at the c-kit gene product expressed on the cell surface (CD117/c-kit) appear to be increasingly helpful in resolving the histopathological differential diagnosis between GISTs and true gastrointestinal smooth muscle neoplasms, schwannomas, and other far less frequently occurring mesenchymal tumours at this site. Although tumours with a clinically benign course appear to be more common than their malignant counterparts, no specific histological criteria have as yet been identified to enable an unambiguous prediction of biological behaviour. Increasing tumour size and mitotic activity favour aggressive tumour behaviour, whereas the prognostic value of germline and somatic mutations within the c-kit proto-oncogene remains to be elucidated further. It is the aim of this synopsis to highlight the relevant fundamental and diagnostic developments with respect to this complex group of neoplasms.
Topics: Biomarkers, Tumor; Diagnosis, Differential; Gastrointestinal Neoplasms; Humans; Mesenchymoma; Neoplasm Proteins; Proto-Oncogene Mas; Proto-Oncogene Proteins c-kit
PubMed: 11215292
DOI: 10.1136/jcp.54.2.96 -
Acta Medica Portuguesa 2002The so-called pulmonary hamartomas occur in 0.3% of the general population, with a higher frequency in male gender and in the sixth decade of life. Usually they are...
The so-called pulmonary hamartomas occur in 0.3% of the general population, with a higher frequency in male gender and in the sixth decade of life. Usually they are single lesions peripherally located in the lung, are formed by mature lobular cartilage and less frequently show adipous and fibromixoid tissue, also with calcified foci. These tumors should be called benign mesenchymomas, due to studies that showed cromossomic alterations and genetic translocations, whose malignant potential has not been recognized.
Topics: Adult; Aged; Female; Hamartoma; Humans; Lung Diseases; Male; Middle Aged; Prognosis
PubMed: 12379992
DOI: No ID Found -
BMC Musculoskeletal Disorders Aug 2021A phosphaturic mesenchymal tumor (PMT) is classified into four histological subtypes: mixed connective tissue, osteoblast-like, non-ossifying fibroma-like, and ossifying... (Review)
Review
BACKGROUND
A phosphaturic mesenchymal tumor (PMT) is classified into four histological subtypes: mixed connective tissue, osteoblast-like, non-ossifying fibroma-like, and ossifying fibroma-like. The ossifying fibroma-like subtype being extremely rare. Most PMTs are benign, with a minimal number becoming malignant after recurrence. In this study, we report a case of recurrence and malignant transformation of PMT-ossifying fibroma-like subtype in the left hip bone.
CASE PRESENTATION
Here, we report the clinical manifestations, histology, pathological features, and treatment of a 57-year-old Chinese woman with a recurrent and malignant ossifying fibroma-like subtype PMT of the left iliac bone. The tumor was first discovered 3 years ago when the patient underwent surgery to remove the tumor. Precisely 2 years and 6 months after the operation, the pain in the left hip reappeared. After 6 months, the patient went to our hospital for treatment. After the tumor resection, the postoperative symptoms improved significantly, and the serum alkaline phosphatase level returned to normal. Based on clinical manifestations, evaluation of serum biochemical indicators, X-ray examination, computerized tomography scan of the pelvis, and histopathological examination of the two operations, the patient was finally diagnosed with a recurring and malignant transformation of the left iliac bone phosphaturic mesenchymal tumor-ossifying fibroma-like subtype. No tumor recurrence was found during the follow-up 15 months after the operation.
CONCLUSIONS
This case increases the awareness of a rare malignant subtype of PMT and provides a valuable reference for the diagnosis of this disease.
Topics: Female; Fibroma; Fibroma, Ossifying; Humans; Mesenchymoma; Middle Aged; Neoplasm Recurrence, Local; Tomography, X-Ray Computed
PubMed: 34376178
DOI: 10.1186/s12891-021-04558-1 -
International Journal of Oral Science Feb 2018Prompted by a unique case of an ectomesenchymal chondromyxoid tumor (ECT) of the palate in a 54-year-old female, we reviewed the English and German literature on this... (Review)
Review
Prompted by a unique case of an ectomesenchymal chondromyxoid tumor (ECT) of the palate in a 54-year-old female, we reviewed the English and German literature on this entity until the end of 2016 using PubMed. The search produced 74 lingual cases with a nearly equal sex distribution and a mean age of 39.3 years, and two extra-lingual cases sharing histological and immunohistological features including nodular growth, round, fusiform or spindle-shaped cellular architecture, and chondromyxoid stroma. Immunophenotyping showed the majority of cases to be positive for glial fibrillary acidic protein (GFAP), S-100 protein, glycoprotein CD57, pancytokeratin (AE1/AE3), and smooth muscle actin (SMA); in isolated cases there was molecular-genetic rearrangement or gain of Ewing sarcoma breakpoint region 1 (EWSR1) but no rearrangement of pleomorphic adenoma gene 1 (PLAG1). At present, ectomesenchymal cells that migrate from the neural crest are considered to play a pivotal role in tumor origin. All cases had a benign course, although there were three recurrences. Because of the rarity of this tumor and the need for differential diagnostic differentiation from myoepithelioma and pleomorphic adenoma, both oral surgeons and pathologists should be aware of this entity.
Topics: Biomarkers, Tumor; Chondroma; Diagnosis, Differential; Female; Humans; Immunophenotyping; Mesenchymoma; Middle Aged; Myoepithelioma; Palatal Neoplasms
PubMed: 29491357
DOI: 10.1038/s41368-017-0003-9 -
Journal of Cancer Research and... 2018Benign mesenchymomas, exceptionally rare tumors, composed of two or more nonepithelial mesenchymal elements are not usually found together in a tumor. We report herein a...
Benign mesenchymomas, exceptionally rare tumors, composed of two or more nonepithelial mesenchymal elements are not usually found together in a tumor. We report herein a rare case of benign mesenchymoma in the lower end of tibia in a 36-year-old female showing fibrous, fatty, and osseous elements.
Topics: Adult; Bone Neoplasms; Female; Humans; Mesenchymoma; Prognosis; Rare Diseases
PubMed: 29893345
DOI: 10.4103/0973-1482.172578 -
Case Reports in Otolaryngology 2015Pediatric head and neck masses are commonly congenital in origin or of infectious etiology. We present a rare case of benign mesenchymoma in close proximity to the...
Pediatric head and neck masses are commonly congenital in origin or of infectious etiology. We present a rare case of benign mesenchymoma in close proximity to the submandibular gland in an otherwise asymptomatic child. Computerized tomography (CT) scan of the head and neck area revealed a benign lesion, which was later determined to be a benign mesenchymoma on histopathology. The child did well after surgery without any reported recurrence. We discuss the salient features of a benign mesenchymoma in a child and also discuss relevant imaging and management.
PubMed: 26064744
DOI: 10.1155/2015/131469 -
International Orthopaedics 2000We report a case of a large cartilage and bone containing mesenchymoma of the thigh and popliteal fossa in a 56-year-old man. Mesenchymomas are rare tumors with a...
We report a case of a large cartilage and bone containing mesenchymoma of the thigh and popliteal fossa in a 56-year-old man. Mesenchymomas are rare tumors with a histologically benign pattern. They may be associated with morbidity as a result of local infiltrative growth.
Topics: Humans; Male; Mesenchymoma; Middle Aged; Soft Tissue Neoplasms; Thigh
PubMed: 10894384
DOI: 10.1007/s002640000126 -
Mesenchymoma of the lung (so called hamartoma): a review of 154 parenchymal and endobronchial cases.Thorax Oct 1987In a series of 154 patients (116 male and 38 female) with so called pulmonary hamartoma the peak incidence was in the sixth decade, with only three patients less than 20... (Review)
Review
In a series of 154 patients (116 male and 38 female) with so called pulmonary hamartoma the peak incidence was in the sixth decade, with only three patients less than 20 years of age. Sequential radiographs showed that in 55 patients the tumour first appeared in adult life and that in 53 it progressively increased in size. The age incidence and progressive growth leads to the conclusion that the tumour is a benign neoplasm rather than a hamartoma, consisting of various connective tissues intersected by clefts lined by respiratory epithelium. The epithelial elements are regarded as entrapped non-neoplastic inclusions and the tumour as a purely mesenchymal neoplasm: the name mesenchymoma therefore seems the most appropriate. There were two recurrences after simple enucleation, 10 and 12 years later. A total of 142 tumours were parenchymal, and only 12 were endobronchial. All lobes were affected but there was a slight preponderance in the left upper lobe. Four patients had two (synchronous) mesenchymomas. There was an associated bronchial carcinoma in 11 patients, synchronous in six and metachronous in five.
Topics: Adolescent; Aged; Female; Hamartoma; Humans; Lung Neoplasms; Male; Mesenchymoma; Middle Aged
PubMed: 3321538
DOI: 10.1136/thx.42.10.790 -
Clinics (Sao Paulo, Brazil) Apr 2017Esophageal leiomyoma is the most common benign tumor of the esophagus, and it originates from mesenchymal tissue. This study analyzed the clinicopathological...
OBJECTIVES:
Esophageal leiomyoma is the most common benign tumor of the esophagus, and it originates from mesenchymal tissue. This study analyzed the clinicopathological characteristics of esophageal leiomyoma and aimed to evaluate the role of endoscopic ultrasonography in the diagnosis and treatment selection for these lesions.
METHODS:
Two hundred and twenty-five patients who had suspected esophageal leiomyomas in endoscopic ultrasonography were enrolled at the Endoscopy Center of The First Affiliated Hospital, Zhejiang University from January 1st, 2009 to May 31th, 2015. The main outcomes included the demographic and morphological characteristics, symptoms, comparisons of diagnosis and treatment methods, adverse events, and prognosis.
RESULTS:
One hundred and sixty-seven patients were diagnosed as having an esophageal leiomyoma by pathological examination. The mean patient age was 50.57±9.983 years. In total, 62.9% of the lesions originated from the muscularis mucosa, and the others originated from the muscularis propria. The median distance to the incisors was 30±12 cm. The median diameter was 0.72±0.99 cm. As determined by endoscopic ultrasonography, most existing leiomyomas were homogeneous, endophytic, and spherical. The leiomyomas from the muscularis mucosa were smaller than those from the muscularis propria and much closer to the incisors (p<0.05). SMA (smooth muscle antibody) (97.2%) and desmin (94.5%) were positive in the majority of patients. In terms of treatments, patients preferred endoscopic therapies, which led to less adverse events (e.g., intraoperative bleeding, local infection, pleural effusion) than surgical operations (p<0.05). The superficial leiomyomas presented less adverse events and better recovery (p<0.05) than deep leiomyomas.
CONCLUSION:
Endoscopic ultrasonography has demonstrated high accuracy in the diagnosis of esophageal leiomyomas and provides great support in selecting treatments; however, EUS cannot completely avoid misdiagnosis, so combining it with other examinations may be a good strategy to solve this problem.
Topics: Adult; Aged; Data Accuracy; Desmin; Endoscopic Mucosal Resection; Endosonography; Esophageal Neoplasms; Female; Humans; Leiomyoma; Male; Mesenchymoma; Middle Aged; Muscle, Smooth; Retrospective Studies; Tomography
PubMed: 28492717
DOI: 10.6061/clinics/2017(04)01 -
International Journal of Cancer Nov 2001Mouse coding region determinant-binding (mCRD-BP) and human IGF-II mRNA-binding 1 (hIMP-1) proteins are orthologous mRNA-binding proteins that recognize c-myc and IGF-II...
Mouse coding region determinant-binding (mCRD-BP) and human IGF-II mRNA-binding 1 (hIMP-1) proteins are orthologous mRNA-binding proteins that recognize c-myc and IGF-II mRNA, respectively, and regulate their expression posttranscriptionally. Here, we confirm that human CRD-BP/IMP-1 binds to c-myc mRNA and that it is predominantly expressed in fetal tissues. Moreover, hCRD-BP/IMP-1 expression was detected in cell lines of neoplastic origin and in selected primary tumors. In a series of 33 malignant and 10 benign mesenchymal tumors, 73% and 40%, respectively, were found to express hCRD-BP/IMP-1. In particular, expression was significant in 14 Ewing's sarcomas, all of which were positive. The data suggest that hCRD-BP/IMP-1 plays a role in abnormal cell proliferation in mesenchymal tumors.
Topics: Amino Acid Motifs; Animals; Base Sequence; Cell Division; Chromosome Mapping; DNA, Complementary; Humans; Mesenchymoma; Mice; Molecular Sequence Data; Protein Binding; Proto-Oncogene Proteins c-myc; RNA; RNA, Messenger; RNA-Binding Proteins; Reverse Transcriptase Polymerase Chain Reaction; Sarcoma, Ewing; Tissue Distribution; Tumor Cells, Cultured
PubMed: 11745432
DOI: 10.1002/ijc.1512