Humans ; Child ; Myofibroblasts/pathology* ; Neoplasms/pathology* ; Cell Differentiation

Low-grade myofibroblastic sarcoma (LGMS) is a rare, malignant tumor with myofibroblastic differentiation. Despite it being classified as a distinct entity by the World Health Organization, a few cases were reported in the oral and maxillofacial region. Here, a LGMS developed on the palate of a 73-year-old man who presented with a 1-cm tumor on the posterior border of the palate. Based on the histological and immunohistochemical features, a diagnosis of LGMS was established. The tumor was resected, and no recurrence was observed over 2 years. Although the tongue is the most preferred site for LGMS, it may occur in any region of the oral cavity.
Aged ; Humans ; Male ; Myofibroblasts ; pathology ; Osteosarcoma ; pathology ; surgery ; Palatal Neoplasms ; pathology ; surgery ; Palate, Hard ; pathology

Adult ; Connective Tissue Cells ; Female ; Humans ; Male ; Middle Aged ; Myofibroblasts ; cytology ; Nasal Polyps ; pathology ; Young Adult

Objective To investigate the sequential changes of the number of neutrophils and myofibroblasts during diabetic wound healing, and discuss its application value in wound age estimation. Methods Diabetic DB mice and mice of the same age in the normal control group were selected, a wound healing model was established, wound samples were taken at different time points, while the number of neutrophils and myofibroblasts during diabetic wound healing were determined by immunohistochemical staining technique. Results The number of infiltrated neutrophils in the wounds of control and diabetic groups reached the peak respectively at 12 h and 5 d after injury. Compared with the control group, the number of neutrophils in the diabetic group decreased significantly from 6 h to 1 d after injury, but increased markedly from 5 d to 14 d. From 5 d to 10 d after injury, the average number of neutrophils at high magnification in wounds of the diabetic group was over 30, while that of neutrophils in wounds of the control group was less than 20. Myofibroblasts appeared in wounds from 3 d to 14 d after injury in the control group and from 5 d to 14 d after injury in the diabetic group. The difference in the number of myofibroblasts in wounds between control group and diabetic group from 3 to 7 d after injury had statistical significance. Conclusion In comparison with normal wound healing, the number of neutrophils and myofibroblasts during diabetic wound healing shows different sequential changes. The results of this study can provide reference for wound age estimation of patients with severe diabetes.
Animals ; Diabetes Mellitus, Experimental/pathology* ; Mice ; Myofibroblasts ; Neutrophils ; Wound Healing/physiology*

OBJECTIVETo investigate the clinicopathologic features, immunohistochemic phenotypes and genetic alterations of extrapulmonary inflammatory myofibroblastic tumor (IMT) and the correlation with prognosis.

METHODSThirty cases of IMT with follow-up were analyzed morphologically and immunohistochemically. ALK FISH was also performed to determine the ALK gene status.

RESULTSPatients ranged in age from 12 to 73 years (mean 43.4 years). The male-to-female ratio was 1.0: 1.1. The tumors were located in various anatomical sites including gastrointestinal tract, liver, spleen, kidney, pelvic, retroperitoneum, mediastinum etc. Histologically, the majority of cases were composed of spindled fibroblastic and myofibroblastic cells accompanied by an inflammatory infiltrate of plasma cells, lymphocytes, and eosinophils. Most cases with aggressive behavior had features including prominent nucleoli and edematous myxoid background. Lymphohistiocytic reactions were usually absent. Some cases showed multinucleation, nuclear pleomorphism and mitoses. One case demonstrated epithelioid morphology with round-to-epithelioid cells. The immunohistochemical study showed vimentin, SMA, CK, desmin, and ALK were expressed in 100% (30/30), 70% (21/30), 13% (4/30), 27% (8/30), and 27% (8/30) of IMT, respectively. Diffuse cytoplasmic ALK staining was detected in seven cases. One case (containing round-to-epithelioid cells) demonstrated ALK nuclear membrane staining, coupled with positive reaction for CD30 and negative reaction for LCA. EMA, CD34, CD117 and S-100 protein, and MyoD1 were negative for all cases. Six ALK protein positive cases harbored ALK gene rearrangement, but not the remaining 22 cases. Follow-up data were available in 21 patients. After initial resection, 14 patients were alive with no evidence of disease, while 4 patients were alive with tumor recurrence and 3 patients died of the disease.

CONCLUSIONSMost IMT with aggressive behavior have features including prominent nucleoli, edematous myxoid background, and positive expression of ALK. Lymphohistiocytic reaction is usually absent. ALK may be a potential novel therapeutic target for IMT.

Adolescent ; Adult ; Aged ; Child ; Female ; Fibroblasts ; pathology ; Granuloma, Plasma Cell ; genetics ; pathology ; Humans ; Inflammation ; pathology ; Male ; Middle Aged ; Myofibroblasts ; pathology ; Prognosis ; Receptor Protein-Tyrosine Kinases ; genetics ; Young Adult

OBJECTIVETo explore the role of the fibroblast transdifferentiation into myofibroblasts (MFBs) in the pathogenesis of systemic sclerosis (SSc) and investigate the influence of transforming growth factor β(1) (TGF-β(1)) and blocking of its signal transduction on fibroblast transdifferentiation.

METHODSFibroblasts derived from the skin lesions of SSc patients and normal skin tissue were cultured in vitro. The proportion of MFBs in the fibroblast culture was analyzed qualitatively using immunocytochemistry and quantitatively with ELISA for α-smooth muscle actin (α-SMA). The changes in fibroblast transdifferentiation were observed after addition of TGF-β(1) in the cell culture and after blocking the signal transduction of TGF-β(1).

RESULTSThe fibroblasts isolated from SSc patients and control subjects showed a similar morphology. The mean number of cells positive for α-SMA in SSc group was significantly higher than that in the control group (P<0.01). As culture time extended, α-SMA levels of the two groups both increased gradually (P<0.01), but the increments were significantly greater in SSc group than in the control group at 24, 48, and 72 h (P<0.05 all). Addition of TGF-β(1) resulted in significantly increased α-SMA levels in both groups (P<0.05), and SSc group showed significantly higher α-SMA levels than the control group at 24, 48, and 72 h (P<0.01). In the presence of TGF-β(1), blocking of Smads, ERK/MAPK, and p38MAPK pathways, but not JNK/MAPK pathway, caused an obvious decrease in α-SMA levels in the fibroblasts in both groups.

CONCLUSIONThe fibroblasts in the skin lesion of SSc patients have strong potential of transdifferentiation into MFBs, and TGF-β(1) can promote this transdifferentiation process possibly involving Smads, and ERK/MAPK, and p38MAPK signalling pathways.

Actins ; metabolism ; Adult ; Cell Transdifferentiation ; physiology ; Cells, Cultured ; Female ; Fibroblasts ; pathology ; Humans ; Male ; Myofibroblasts ; pathology ; Scleroderma, Systemic ; pathology ; Signal Transduction ; Skin ; pathology ; Transforming Growth Factor beta1 ; metabolism

AIMTo observe the expression of a-smooth muscle actin(a-SMA) in primary cultural fibroblasts of rats.

METHODS12 female Wistar rats were randomly assigned into two groups, the normal group and the model group. The model group was filled with bleomycin A2 (5 mg/kg) once into the trachea. The normal group was filled with equal saline into the trachea. The rats were sacrificed under drugged state at 28 days of feeding, then Hematoxylin-Eosin staining and electron microscopy were used to evaluate the foundation of the model. The isolated fibroblasts from the rats were cultured in vitro. Flow cytometry was used in the test to observe the expression of alpha-SMA in fibroblast in vitro in rats.

RESULTSThe formation of fibroblast foci was observed in the model group by optical microscope. The ultrastructure in pulmonary tissue of the model group rats were changed and proliferated myofibroblasts with filaments were found in the alveolar septa by electron microscopy. The expression of alpha-SMA was positive in the normal and model group. There was no difference between the two groups in the rates of positive cells (P > 0.05).

CONCLUSIONBoth the normal and model groups had the phenotype conversion in lung fibroblasts in vitro.

Actins ; metabolism ; Animals ; Bleomycin ; Cells, Cultured ; Female ; Fibroblasts ; metabolism ; pathology ; Lung ; pathology ; Myofibroblasts ; pathology ; Pulmonary Fibrosis ; chemically induced ; pathology ; Rats ; Rats, Wistar

A Mullerian adenosarcoma is an extremely rare tumor characterized by a stromal component of usually low-grade malignancy and by a benign glandular epithelial component. A Mullerian adenosarcoma occurs mainly in the uterus, but also in extrauterine locations. Extrauterine Mullerian adenosarcomas are thought to arise from endometriotic deposits. A 36-year-old female presented to Daegu Catholic University Medical Center with a symptom of loose stool for several months. The imaging studies revealed a rectal mass, so she underwent a laparoscopic low anterior resection. Although extemporary pathology revealed an inflammatory myofibroblastic tumor, the final histologic diagnosis was a Mullerian adenosarcoma arising from rectal endometriosis. To our knowledge, except a concomitant rectal villotubular adenoma, cases of Mullerian adenosarcomas arising the rectal wall are rare. An adenosarcoma arising from endometriosis should be considered in the differential diagnosis of a pelvic mass, even one appearing in rectal wall, because ectopic endometrial tissue exists everywhere.
Academic Medical Centers ; Adenoma ; Adenosarcoma* ; Adult ; Daegu ; Diagnosis ; Diagnosis, Differential ; Endometriosis* ; Female ; Humans ; Myofibroblasts ; Pathology ; Rectal Neoplasms ; Uterus

Plexiform angiomyxoid myofibroblastic tumor (PAMT) is a recently described mesenchymal tumor of the stomach. We report the first case of PAMT in Korea. A 52-yr-old man underwent esophagogastroduodenoscopy due to dyspepsia for 2 yr. There was a submucosal mass with small mucosal ulceration in the gastric antrum. The tumor measured 3.5 x 2.3 cm in size and showed multinodular plexiform growth pattern of bland-looking spindle cells separated by an abundant myxoid or fibromyxoid matrix rich in small thin-walled blood vessels. The tumor cells were negative for CD117 (c-KIT), CD34 and S-100 protein, but diffusely positive for smooth muscle actin consistent with predominant myofibroblastic differentiation. The patient is doing well without recurrence or metastasis for 5 months after surgery. Although there have been limited follow-up data, PAMT is regarded as a benign gastric neoplasm with histological and immunohistochemical charateristics distinguished from gastrointestinal stromal tumor and other mesenchymal tumors of the stomach.
Dyspepsia/diagnosis ; Endoscopy, Digestive System ; Humans ; Male ; Middle Aged ; Myofibroblasts ; *Myxoma/diagnosis/pathology/surgery ; Pyloric Antrum/pathology ; *Stomach Neoplasms/diagnosis/pathology/surgery

Animals ; Autophagy ; Biomarkers, Tumor ; metabolism ; Caveolin 1 ; deficiency ; metabolism ; Drug Delivery Systems ; Humans ; Myofibroblasts ; pathology ; Neoplasms ; metabolism ; pathology ; Prognosis ; Stromal Cells ; metabolism ; Tumor Microenvironment