OBJECTIVETo study the morphological characteristics and immunophenotype of highly cellular leiomyoma (HCL) of uterus, compared with that of uterine endometrial stromal tumors (EST).

METHODSHE and immuno-stained sections EnVision method from 20 cases of HCL, 21 cases of EST and 1 case of stromomyoma were reviewed. Monoclonal antibodies against h-caldesmon, calponin, CD10, desmin and smooth muscle actin (SMA) were used for immunohistochemistry studies.

RESULTSOn microscopic examination, HCL were densely cellular and composed of cells that ranged from spindle-shaped to round with scanty cytoplasm. A focal fascicular pattern was present in all cases. Blood vessels with large, thick muscular walls were a conspicuous feature of the majority of tumors. Cleft-like spaces were present in 9 tumors and 15 cases exhibited irregular focal extensions into the adjacent myometrium. ESTs were composed of cells that resembled endometrial stromal cells of proliferative endometrium. These cases included a significant component of delicate blood vessels similar to spiral arterioles. All 20 low grade endometrial stromal sarcoma cases had infiltrative growth to adjacent myometrium. Immunoreactivities of HCL for h-caldesmon, calponin, CD10, Desmin and SMA were 80.0% (16/20), 100% (20/20), 0 (0/20), 95.0% (19/20) and 100% (20/20), respectively, whereas the positive rates of EST were 4.7% (1/21), 23.8% (5/21), 66.7% (14/21), 23.8% (5/21) and 19.0% (4/21), respectively (P = 0.001).

CONCLUSIONSHighly cellular leiomyomas have distinct morphologic features. H-caldesmon, calponin, CD10, desmin and SMA are helpful in the differential diagnosis of HCL and EST.

Adult ; Calcium-Binding Proteins ; analysis ; Calmodulin-Binding Proteins ; analysis ; Desmin ; analysis ; Endometrial Neoplasms ; metabolism ; pathology ; Endometrial Stromal Tumors ; metabolism ; pathology ; Female ; Humans ; Immunohistochemistry ; Leiomyoma ; metabolism ; pathology ; Microfilament Proteins ; Middle Aged ; Neprilysin ; analysis ; Uterine Neoplasms ; metabolism ; pathology

OBJECTIVETo test whether in the absence of actin, actin-binding proteins such as caldesmon, calponin, and tropomyosin interact with the myosin of unphosphorylation, Ca2+-dependent phosphorylation (CDP), and Ca2+-independent phosphorylation (CIP) and stimulate myosin Mg2+-ATPase activities.

METHODSMg2+-ATPase activities were measured to evaluate the effects of caldesmon, calponin, and tropomyosin on the myosin in unphosphorylation, CDP by myosin light chain kinase (MLCK), and CIP by MLCK.

RESULTS(1) At different incubation-time, i.e., 5, 10, 20, 40, and 60 minutes, the highest Mg2+-ATPase activity was observed when myosin was in the state of CDP, the medium was CIP of myosin, and the lowest was the unphosphorylated myosin. (2) In the absence of caldesmon, calponin, and tropomyosin, the Mg2+-ATPase activities from high to low were in the following order: CDP, CIP, and unphosphorylated myosin. However, in the presence of caldesmon, calponin, and tropomyosin, the order of relative value of Mg2+-ATPase activities from high to low was unphosphorylated, CIP, and CDP of myosin respectively compared to the corresponding controls.

CONCLUSIONSThe results propose that caldesmon, calponin, and tropomyosin are capable of stimulating Mg2+-ATPase activity of smooth muscle myosin in Ca2+-independent manner, since Ca2+ is not obligating for the stimulating effects of the three proteins. The common characteristic of the three proteins is that when myosin activities are low, their activations are relatively strong and this property might be involved in smooth muscle tension keeping.

Animals ; Ca(2+) Mg(2+)-ATPase ; drug effects ; metabolism ; Calcium ; pharmacology ; Calcium-Binding Proteins ; pharmacology ; Calmodulin-Binding Proteins ; pharmacology ; Chickens ; Microfilament Proteins ; Muscle, Smooth ; enzymology ; Myosins ; metabolism ; Phosphorylation ; Tropomyosin ; pharmacology

Nodular fasciitis is a pseudosarcomatous reactive process composed of fibroblasts and myofibroblasts, and it is most common in the upper extremities. Nodular fasciitis of the external auditory canal is rare. To the best of our knowledge, less than 20 cases have been reported to date. We present a case of nodular fasciitis arising in the cartilaginous part of the external auditory canal. A 19-year-old man complained of an auricular mass with pruritus. Computed tomography showed a 1.7 cm sized soft tissue mass in the right external auditory canal, and total excision was performed. Histologic examination revealed spindle or stellate cells proliferation in a fascicular and storiform pattern. Lymphoid cells and erythrocytes were intermixed with tumor cells. The stroma was myxoid to hyalinized with a few microcysts. The tumor cells were immunoreactive for smooth muscle actin, but not for desmin, caldesmon, CD34, S-100, anaplastic lymphoma kinase, and cytokeratin. The patient has been doing well during the 1 year follow-up period.
Actins ; Calmodulin-Binding Proteins ; Desmin ; Ear ; Ear Canal* ; Erythrocytes ; Fasciitis* ; Fibroblasts ; Follow-Up Studies ; Humans ; Hyalin ; Keratins ; Lymphocytes ; Lymphoma ; Muscle, Smooth ; Myofibroblasts ; Phosphotransferases ; Pruritus ; Upper Extremity ; Young Adult

Adult ; Calmodulin-Binding Proteins ; metabolism ; Desmin ; metabolism ; Diagnosis, Differential ; Female ; Humans ; Leiomyoma ; metabolism ; pathology ; surgery ; Leiomyosarcoma ; pathology ; Spinal Canal ; Spinal Neoplasms ; metabolism ; pathology ; surgery ; Young Adult

OBJECTIVETo investigate the relationship between the polymorphism of alpha-adducin (ADD1) gene and the two phenotypes of constitution in patients with essential hypertension, the Yang-hyperactive (YH) type and phlegm-dampness (PD) type, classified by traditional Chinese medicine (TCM) approach.

METHODSTwo hundred and seven patients differentiated by TCM approach as YH type (113 cases) or PD type (94 cases) were observed, with the systolic blood pressure (SBP), diastolic blood pressure (DBP), body mass index (BMI), fasting blood glucose (FBG), serum creatinine (Cr), uric acid (UA), total cholesterol (TC) and triglycerides (TG) as the criteria of observation. Gly460Trp polymorphism of the ADD1 gene was detected by MALDI-TOF mass spectrometry. Results The levels of BMI, DBP, FBG and UA, etc. in the PD group were significantly higher than those in the YH group respectively. The rate of GG, GT and TT type of ADD1 gene was 29.2%, 41.6% and 29.2% in the YH group, 28.7%, 48.9% and 22.3% in the PD group, showing no significant difference in ADD1 genotype distribution between the two groups, while there was also no difference in the hypertension phenotype distribution among different genotypes (both P > 0.05). For the patients with TT genotype, there were significant differences between the YH group and the PD group in BMI (24.11 +/- 3.04 kg/m2 vs 26.20 +/- 2.30 kg/m2), DBP (96.79 +/- 4.05 mmHg vs 99.56 +/- 3.90 mmHg), FBG (5.01 +/- 0.53 mmol/L vs 5.51 +/- 1.07 mmol/L) and UA level (302.22 +/- 71.95 micromol/L vs 358.25 +/- 88.75 micromol/L, all P < 0.05).

CONCLUSIONThere was no relation between ADD1 gene polymorphism and the TCM genotype of constitution in patients with essential hypertension. However, it is likely that for hypertension patients with TT genotype, those of PD type are more susceptible to cardiovascular disease and have worse prognosis than those of YH type.

Adult ; Aged ; Calmodulin-Binding Proteins ; genetics ; Diagnosis, Differential ; Female ; Humans ; Hypertension ; diagnosis ; genetics ; Male ; Medicine, Chinese Traditional ; Middle Aged ; Phenotype ; Polymorphism, Genetic

Objective: To investigate the clinicopathological features, immunophenotype, diagnosis and differential diagnosis of SRF-rearranged cellular perivascular myoid tumor. Methods: Two cases of SRF-rearranged cellular perivascular myoid tumor diagnosed in the Department of Pathology, Fudan University Shanghai Cancer Center from October 2021 to March 2022 were collected. Immunohistochemical staining, fluorescence in-situ hybridization (FISH) and next-generation sequencing (NGS) were performed, and the literature was reviewed. Results: Case 1, a 3-month-old boy presented with a painless tumor of the scalp, measuring about 2 cm in diameter. Case 2, a 3-year-old girl complained with a painless tumor of the knee, measuring approximately 1.5 cm in diameter. Microscopically, the tumor had a clear boundary and showed multinodular growth. The tumor was mainly composed of spindle cells arranged in long intersecting fascicles associated with thin, slit-like or branching ectatic vessels, focally forming hemangiopericytoma-like appearance. The tumor cells were abundant, but there was no obvious atypia. Mitotic figures (3-4/10 HPF) were noted. H-caldesmon and SMA were positive in both cases. Case 1 showed diffuse and strong positivity for Desmin, and focally for CKpan. Ki-67 proliferation index was 20% and 30%, respectively. FISH displayed NCOA2 gene translocation in case 1 and the RELA gene translocation in case 2. NGS detected the SRF-NCOA2 gene fusion in case 1 and the SRF-RELA gene fusion in case 2. Both patients underwent local excisions. During the follow-up of 5-14 months, case 1 had no local recurrence, while case 2 developed local recurrence 1 year post operatively. Conclusions: SRF-rearranged cellular perivascular myoid tumor is a novel variant of perivascular cell tumor, which tends to occur in children and adolescents. The tumor forms a broad morphologic spectrum ranging from a pericytic pattern to a myoid pattern, and include hybrid tumors with a mixture of pericytic and myoid patterns. Due to its diffuse hypercellularity and increased mitotic figures and smooth muscle-like immunophenotype, the tumor is easy to be misdiagnosed as myogenic sarcomas. The tumor usually pursues a benign clinical course and rare cases may locally recur.
Child, Preschool ; Female ; Humans ; Infant ; Male ; Biomarkers, Tumor/analysis* ; Calmodulin-Binding Proteins ; China ; Hemangiopericytoma/pathology* ; Sarcoma/pathology* ; Soft Tissue Neoplasms/pathology*

Extraskeletal Ewing's sarcoma (EES) is a branch of neuroectodermal tumor (PNET), which is very rare soft tissue sarcoma. We report a case of EES/PNET arising is the lung of a 67-yr-old man. Computed tomography, bone scintigraphy, and positron emission tomography confirmed the mass to have a primary pulmonary origin. The mass showed positive reactivity in the Periodic Acid Schiff (PAS) stain and MIC-2 immunoreactivity in immunohistochemical stain. Fluorescence in situ hybridization (FISH) was performed, which revealed an EWSR1 (Ewing sarcoma breakpoint region 1) 22q12 rearrangement. The diagnosis was confirmed both pathologically and genetically. The mass lesion was resected, and the patient is currently undergoing chemotherapy.
Aged ; Calmodulin-Binding Proteins/genetics ; Chromosome Breakage ; Chromosomes, Human, Pair 22/genetics ; Diagnosis, Differential ; Humans ; Immunohistochemistry ; In Situ Hybridization, Fluorescence ; Lung Neoplasms/*diagnosis/genetics/metabolism/pathology ; Male ; Neuroectodermal Tumors, Primitive, ; RNA-Binding Proteins/genetics ; Sarcoma, Ewing's/*diagnosis/genetics/metabolism/pathology

Primary tumors of the great vessels are rare. Most encountered cases are sarcomas which most commonly develop in the aorta, pulmonary artery, and inferior vena cava. We experienced an intimal sarcoma arising in the left common iliac artery in a 68-year-old male, who suffered from claudication in his left lower extremity for a year and was diagnosed as arteriosclerosis obliterans, clinically. Bypass surgery was performed on the obstructive lesion. Grossly, the vascular lumen was filled with dark hemorrhagic materials. Microscopically, the lesion showed proliferation of anaplastic spindle cells with a marked nuclear atypia, arranged haphazardly. There were numerous mitotic figures. Foci of cholesterol clefts were also found in the intima. Immunohistochemically, the tumor cells were positive for vimentin, smooth muscle actin, and cytokeratin in certain areas. Stains for CD34, desmin, myosin heavy chain, caldesmon, and S-100 protein were negative. A pathologic diagnosis was made as intimal sarcoma with myofibroblastic differentiation.
Actins ; Aged ; Aorta ; Arteriosclerosis Obliterans ; Calmodulin-Binding Proteins ; Cholesterol ; Coloring Agents ; Desmin ; Humans ; Iliac Artery ; Keratins ; Lower Extremity ; Male ; Muscle, Smooth ; Myofibroblasts ; Myosin Heavy Chains ; Pulmonary Artery ; S100 Proteins ; Sarcoma ; Vena Cava, Inferior ; Vimentin

Perivascular epithelioid cell tumor (PEComa) is a rare family of related mesenchymal neoplasms that include angiomyolipoma, lymphangiomyomatosis and clear cell 'sugar' tumor of the lung. Although this type of tumor has been described in the literature in organs such as kidney, lung, uterus and urinary bladder, there are few reports of gastrointestinal tract-related tumor. We report here on a case of PEComa arising in the transverse colon. This occurred in a 41-year-old male who had no history of tuberous sclerosis complex. Histopathologically, the tumor consisted of nests or sheets of epithelioid cells with eosinophilic cytoplasm. The tumor cells were positive for HMB-45, vimentin and caldesmon, but they were negative for S-100 protein, cytokeratin and CD117, according to immunohistochemical staining. Careful follow up is warranted because the biological behavior of PEComa has not yet been documented. We present here a case of colonic PEComa that was confirmed by immunohistochemical staining and the histopathologic findings, and we include a review of the literature.
Adult ; Angiomyolipoma ; Calmodulin-Binding Proteins ; Colon* ; Colon, Transverse ; Cytoplasm ; Eosinophils ; Epithelioid Cells* ; Humans ; Keratins ; Kidney ; Lung ; Lymphangioleiomyomatosis ; Male ; Perivascular Epithelioid Cell Neoplasms ; S100 Proteins ; Tuberous Sclerosis ; Urinary Bladder ; Uterus ; Vimentin

Caldesmon (CaD), one of microfilament-associated proteins, plays a key role in microfilament assembly in mitosis. We have investigated the effects of overexpression of the high molecular weight isoform of CaD (h-CaD) on the physiology of vascular smooth muscle cells (VSMCs). Rat aortic VSMCs were stably transfected with plasmids carrying a full length human h-CaD cDNA under control of cytomegalovirus promoter. The majority of the overexpressed h-CaD appears to be localized predominantly on cytoskeleton structures as determined by detergent lysis. The overexpression of h-CaD, however, does not decrease the level of endogenous low molecular weight isoform of CaD. h-CaD overexpressing VSMCs (h-CaD/VSMCs) show a decreased growth rate than that of vector-only transfected cells when determined by (3H)thymidine uptake and cell counting after fetal bovine serum (FBS) stimulation. h-CaD/VSMCs were smaller than vector-transfected cells by 18% in cell diameter. These data suggest that overexpression of h-CaD can inhibit the poliferation and the cell volume of VSMCs stimulated by growth factors and that the gene therapy with h-CaD may be helpful to prevent the conditions associated with hypertrophy and/or hyperplasia of VSMCs after arterial injuries.
Actin Cytoskeleton ; Animals ; Calmodulin-Binding Proteins* ; Cell Count ; Cell Size ; Cytomegalovirus ; Cytoskeleton ; Detergents ; DNA* ; DNA, Complementary ; Genetic Therapy ; Humans ; Hyperplasia ; Hypertrophy ; Intercellular Signaling Peptides and Proteins ; Mitosis ; Molecular Weight ; Muscle, Smooth, Vascular* ; Physiology ; Plasmids ; Rats* ; Transfection*