-
Journal of Zoo and Wildlife Medicine :... Jun 2018A captive-born adult female Nilgiri langur ( Semnopithecus johnii) developed an edematous swelling of the left thigh and a firm mass around the right ankle joint....
A captive-born adult female Nilgiri langur ( Semnopithecus johnii) developed an edematous swelling of the left thigh and a firm mass around the right ankle joint. The animal also suffered from lethargy and anorexia and was euthanized because of poor general condition. Necropsy revealed that the skeletal muscle of the left thigh had been replaced by a multilocular cystic mass containing numerous sand-grain-sized whitish structures. Small cysts were also present in the lung and the myocardium. The mass of the right ankle joint was histologically consistent with a myxosarcoma. In contrast, the cystic masses from the left thigh, the lung, and the myocardium represented metacestode tissue with evidence of numerous larval cestodes consistent with cysticerci. Cysticerci showed morphological characteristics of Cysticercus longicollis, the larval form of Taenia crassiceps, which was confirmed by genetic analysis. This is the first documented case of a Taenia crassiceps cysticercosis in an Old World monkey species.
Topics: Animals; Animals, Zoo; Colobinae; Cysticercosis; Cysticercus; Female; Germany; Monkey Diseases
PubMed: 29900783
DOI: 10.1638/2017-0156.1 -
Annals of Surgical Oncology Dec 2014Attenuated endogenous protein levels of cyclin-dependent kinase 2 associated protein 1 (p12(CDK2AP1)) and its active homodimer p25(CDK2AP1) were found in...
Clinical aggressiveness of myxofibrosarcomas associates with down-regulation of p12CDK2AP1: prognostic implication of a putative tumor suppressor that induces cell cycle arrest and apoptosis via mitochondrial pathway.
BACKGROUND
Attenuated endogenous protein levels of cyclin-dependent kinase 2 associated protein 1 (p12(CDK2AP1)) and its active homodimer p25(CDK2AP1) were found in myxofibrosarcoma-derived cell lines. Clinical and biological significances of this putative tumor suppressor in myxofibrosarcoma were studied.
METHODS
Plasmids carrying the CDK2AP1 gene and small hairpin RNA interference (shRNAi) targeting CDK2AP1 were transfected into NMFH-1 and/or OH931 cells to evaluate the effects on the CDK2, active caspase 3 (CASP3), cleaved-CASP8 and -CASP9 levels, cell cycle regulation, and/or apoptotic responses. Immunostaining of p12(CDK2AP1) was interpretable in 102 primary myxofibrosarcomas and correlated with clinicopathological variables, CDK2, Ki-67 and active CASP3 protein levels, and disease-specific survival.
RESULTS
Exogenous expression of p12(CDK2AP1) in NMFH-1 and OH931 cells significantly induced G0/G1 cell cycle arrest and down-regulated CDK2 protein level. In NMFH-1 cells, these aspects were reversed by shRNAi targeting CDK2AP1 gene. Increased active CASP3 and cleaved-CASP9, but not -CASP8, were detected after CDK2AP1 overexpression, suggesting the cellular apoptosis were induced through the mitochondrial pathway. Immunostains of p12(CDK2AP1) were aberrantly decreased in 56.9 % of cases; positively and negatively correlated with protein levels of CDK2 (p = 0.023), Ki-67 (p = 0.001) and active CASP3 (p < 0.001), respectively. Following by high histological grades, p12(CDK2AP1) down-regulation was predictive of worse disease-specific survival in univariate (p = 0.003) and multivariate (p = 0.004) analyses.
CONCLUSIONS
Through down-regulation of CDK2, high p12(CDK2AP1) level induced cell cycle arrest and the mitochondrial-dependent apoptotic pathway. Low p12(CDK2AP1) level represents a poor prognostic factor in patients with myxofibrosarcoma.
Topics: Apoptosis; Caspase 3; Caspase 8; Caspase 9; Cell Cycle Checkpoints; Cell Line, Tumor; Cell Survival; Chromobox Protein Homolog 5; Cyclin-Dependent Kinase 2; Female; Fibrosarcoma; Humans; Ki-67 Antigen; Male; Middle Aged; Mitochondria; Mitotic Index; Myxosarcoma; Plasmids; RNA, Messenger; RNA, Small Interfering; Survival Rate; Transfection; Tumor Suppressor Proteins
PubMed: 24889487
DOI: 10.1245/s10434-014-3825-0 -
Annals of Diagnostic Pathology Jun 2015We describe 23 cases of high-grade myxoinflammatory fibroblastic sarcoma (MIFS). The patients were 15 women and 8 men, with the age ranging at the time of diagnosis from...
We describe 23 cases of high-grade myxoinflammatory fibroblastic sarcoma (MIFS). The patients were 15 women and 8 men, with the age ranging at the time of diagnosis from 39 to 93 years (mean, 64.3 years; median, 66 years). Follow-up was available for 18 patients, of whom 9 developed metastatic disease; 7 of these died. Most tumors showed a predilection for the soft tissues of the extremities, with 14 cases involving the lower limb and 5 the upper extremity. However, in both sites, the acral parts were affected in only 1 case each. Of the 4 remaining tumors, 2 were found in axilla, 1 was found in sacral area, and 1 developed in the scar on the breast, 14 years after previous excision of a mammary carcinoma and subsequent local irradiation. The tumor size ranged from 1.3 cm to as much as 30 cm in the largest dimension with a mean size of 8.3 cm. Histologically, the tumors were characterized by occurrence of 3 types of characteristic cells, including (1) lipoblast-like cells with an ample, distended, mucin-filled cytoplasm compartmentalized by a variable number of intracytoplasmic septa, thus remotely resembling soccer balls; (2) large, polygonal, bizarre ganglion-like cells similar to those seen in the Hodgkin disease, also called Reed-Sternberg-like cells. Within an ample, deeply eosinophilic cytoplasm, there was an oval nucleus with vesicular chromatin and a large, inclusion-like nucleolus. Binucleated, multinucleated, or more pleomorphic forms of these cells were also present; (3) cells with emperipolesis of variable sizes, ranging from very inconspicuous neoplastic cells containing only one to a few engulfed cells to conspicuous large ones having many inflammatory cells, usually polymorphonuclear leukocytes admixed with various numbers of some lymphoid cells, within the cytoplasm. Quite often, we found elements that combined the histologic features of all the above 3 characteristic tumor cell types. In 2 tumors, we found an additional undifferentiated spindle cell sarcoma component, whereas in another tumor, a chondrosarcomatous moiety was evident. For comparison, we studied 10 cases of pleomorphic hyalinizing angiectatic tumor (PHAT) of soft tissues. Based on the identification of morphological changes typical for MIFS within most of the cases of PHAT, we suggest that most cases of PHAT represent examples of MIFS merely having hyaline ectatic vessels.
Topics: Adult; Aged; Aged, 80 and over; Chondrosarcoma; Cyclin D1; Female; Fibrosarcoma; Follow-Up Studies; Humans; Hyalin; Immunohistochemistry; Inflammation; Male; Middle Aged; Myxosarcoma; Neoplasm Recurrence, Local; Soft Tissue Neoplasms
PubMed: 25886867
DOI: 10.1016/j.anndiagpath.2015.03.012 -
Histopathology Aug 2020
Topics: Aged; Diagnosis, Differential; Female; Fibroma; Fibrosarcoma; Humans; Male; Middle Aged; Myxosarcoma; Neoplasm Grading; Retrospective Studies; Soft Tissue Neoplasms
PubMed: 32416614
DOI: 10.1111/his.14142 -
Medicine Mar 2021Myxofibrosarcoma (MFS) is a locally aggressive tumor and has the potential to be fatal because of distant metastasis. Immunotherapy targeting either programmed cell...
INTRODUCTION
Myxofibrosarcoma (MFS) is a locally aggressive tumor and has the potential to be fatal because of distant metastasis. Immunotherapy targeting either programmed cell death protein 1 (PD-1) or programmed death ligand 1 (PD-L1) has recently shown a curative effect on multiple cancers including melanoma, non-small cell lung cancer, and renal cell carcinoma. Although the immunotherapy has been applied in sarcoma, there is little information about the efficiency to treat metastatic MFS.
PATIENT CONCERNS
A 42-year-old male presented to the clinic with a mass in the left thigh. Mass resection and ligament replacement surgery were performed.
DIAGNOSES
The patient was diagnosed as high-grade MFS (federation nationale des centres de lutte contre le cancer, Grade 3) with pulmonary metastasis.
INTERVENTIONS
In the past few years, he was treated with surgery, chemoradiotherapy, and Anlotinib (an angiogenesis inhibitor), but the metastatic lesion continued to progress. About 40% to 50% of tumor cells in his pulmonary tissues were showed positive PD-L1 expression and his tumor mutational burden was 215Muts. Thus, he received Camrelizumab (PD-1 inhibitor).
OUTCOMES
Six months after the initiating immunotherapy of Camrelizumab, the size of pulmonary lesions showed marked shrinkage, indicating a partial response. After a follow-up of 18 months, the patient remained in good condition without progressive disease.
CONCLUSION
This case described here demonstrated that immunotherapy of PD-1 inhibitor is a promising treatment option for refractory MFS with PD-L1 positive or tumor mutational burden -high, which could contribute to effective tumor response.
Topics: Adult; Antibodies, Monoclonal, Humanized; Antineoplastic Agents; Dissection; Humans; Immunotherapy; Lung Neoplasms; Male; Myxosarcoma; Neoplasm Grading; Neoplasm Staging; Programmed Cell Death 1 Receptor; Progression-Free Survival; Soft Tissue Neoplasms; Thigh; Treatment Outcome
PubMed: 33761725
DOI: 10.1097/MD.0000000000025262 -
Veterinary Clinical Pathology Jun 2016
Topics: Animals; Dog Diseases; Dogs; Male; Myxosarcoma; Perineum
PubMed: 27093459
DOI: 10.1111/vcp.12341 -
Journal of Comparative Pathology 2015Two adult koalas (Phascolarctos cinereus) were presented for necropsy examination without any clinical history. A diffuse, severe gelatinous effusion was found in the...
Two adult koalas (Phascolarctos cinereus) were presented for necropsy examination without any clinical history. A diffuse, severe gelatinous effusion was found in the abdominal cavity, while numerous 1-3 mm diameter, round, well-demarcated, multifocal to coalescing, raised, firm nodules containing a clear gelatinous fluid were scattered on the surface of the parietal peritoneum, diaphragm, liver, gastrointestinal tract and mesentery. Microscopically, the nodular lesions consisted of spindle-shaped to stellate neoplastic cells, with scant eosinophilic cytoplasm and moderate anisocytosis and anisokaryosis. The neoplastic cells were admixed with and surrounded by abundant acellular pale eosinophilic material that was stained by Alcian blue and only weakly by the periodic acid-Schiff reaction. Serosal proliferations are described rarely in koalas and to the authors' knowledge this is the first reported case of serosal myxosarcoma. The significance and pathogenesis of this condition in the koala population is unknown.
Topics: Animals; Female; Male; Myxosarcoma; Phascolarctidae; Serous Membrane
PubMed: 25728815
DOI: 10.1016/j.jcpa.2015.01.004 -
Skeletal Radiology Feb 2024Sclerosing epithelioid fibrosarcoma (SEF) occurring as a primary bone tumor is exceptionally uncommon. Even more rare are cases of SEF that show morphologic overlap with...
Sclerosing epithelioid fibrosarcoma (SEF) occurring as a primary bone tumor is exceptionally uncommon. Even more rare are cases of SEF that show morphologic overlap with low-grade fibromyxoid sarcoma (LGFMS). Such hybrid lesions arising within the bone have only rarely been reported in the literature. Due to their variegated histomorphology and non-specific radiologic features, these tumors may pose diagnostic difficulties. Herein we describe three molecularly confirmed primary bone cases of sclerosing epithelioid fibrosarcoma that demonstrated prominent areas showing the features of LGFMS and with areas resembling so-called hyalinizing spindle cell tumor with giant rosettes (HSCTGR). Two patients were female and one was male aged 26, 47, and 16, respectively. The tumors occurred in the femoral head, clavicle, and temporal bone. Imaging studies demonstrated relatively well-circumscribed radiolucent bone lesions with enhancement on MRI. Cortical breakthrough and soft tissue extension were present in one case. Histologically the tumors all demonstrated hyalinized areas with SEF-like morphology as well as spindled and myxoid areas with LGFMS-like morphology. Two cases demonstrated focal areas with rosette-like architecture as seen in HSCTGR. The tumors were all positive for MUC4 by immunohistochemistry and cytogenetics, fluorescence in-situ hybridization, and next-generation sequencing studies identified EWSR1 gene rearrangements confirming the diagnosis in all three cases.Hybrid SEF is exceedingly rare as a primary bone tumor and can be difficult to distinguish from other low-grade spindled and epithelioid lesions of bone. MUC4 positivity and identification of underlying EWSR1 gene rearrangements help support this diagnosis and exclude other tumor types.
Topics: Humans; Male; Female; Biomarkers, Tumor; Fibrosarcoma; Myxosarcoma; Immunohistochemistry; Bone Neoplasms; Soft Tissue Neoplasms
PubMed: 37524934
DOI: 10.1007/s00256-023-04412-6 -
Journal of Surgical Oncology Jul 2016The clinical course of soft tissue myxofibrosarcoma is characterized by a high incidence of recurrences and there is no agreement on how to identify patients at major...
BACKGROUND AND OBJECTIVES
The clinical course of soft tissue myxofibrosarcoma is characterized by a high incidence of recurrences and there is no agreement on how to identify patients at major risk. An epithelioid histological variant has been described, with a possible worse prognosis. We reviewed our series to identify prognostic factors and assess clinical significance of the epithelioid variant.
METHODS
We examined the clinico-pathological features of a series of 75 patients affected by soft tissue myxofibrosarcoma at a mean follow-up of 63 months (range 17-132).
RESULTS
Disease specific survival and local recurrence free survival were, respectively, 84.8% and 76.8% at 5 years. Seven patients (8.6%) presented with the epithelioid variant with a survival of 62.5%. High grade and epithelioid morphology were negative prognostic factors for patient survival, high grade, and inadequate surgical margins for local recurrence. Radiotherapy had a local protective effect in high grade tumors.
CONCLUSIONS
Our experience confirms the difficulties in obtaining wide margins in myxofibrosarcoma and the high rate of recurrence. Local recurrences did not significantly affect survival and a limb-sparing approach can be chosen also in recurrences. Patients affected by the epithelioid variant showed a worse prognosis. Chemotherapy should be considered as adjuvant treatment in this subtype. J. Surg. Oncol. 2016;114:50-55. © 2016 Wiley Periodicals, Inc.
Topics: Adult; Aged; Aged, 80 and over; Antineoplastic Agents; Chemotherapy, Adjuvant; Epithelioid Cells; Female; Fibrosarcoma; Follow-Up Studies; Humans; Limb Salvage; Male; Margins of Excision; Middle Aged; Myxosarcoma; Neoadjuvant Therapy; Neoplasm Grading; Neoplasm Recurrence, Local; Prognosis; Radiotherapy, Adjuvant; Soft Tissue Neoplasms; Survival Analysis; Treatment Outcome
PubMed: 27076198
DOI: 10.1002/jso.24250 -
Head & Neck Dec 2016Extirpation of tumors arising in the pterygopalatine fossa is challenging because of its anatomic complexity.
BACKGROUND
Extirpation of tumors arising in the pterygopalatine fossa is challenging because of its anatomic complexity.
METHODS AND RESULTS
A 67-year-old man was referred to our department with a diagnosis of a tumor in his left pterygoid fossa. An incisional biopsy through the canine fossa was diagnosed as myxofibrosarcoma. The upper part of the maxilla was swung laterally to remove the tumor while the hard plate was preserved. The defect was reconstructed using rectus abdominis musculocutaneous free and ipsilateral temporal. The postoperative course was uneventful, without facial palsy or mastication disorders.
CONCLUSION
Our experience with this case suggests that the modified partial maxillary swing approach with preservation of the hard palate and orbital floor in combination with infratemporal and cervical approaches is useful for lesions in the pterygoid process without causing severe complications. © 2016 Wiley Periodicals, Inc. Head Neck 38: E2519-E2522, 2016.
Topics: Aged; Biopsy, Needle; Follow-Up Studies; Head and Neck Neoplasms; Humans; Imaging, Three-Dimensional; Immunohistochemistry; Male; Maxilla; Myxosarcoma; Neoplasm Invasiveness; Neoplasm Staging; Pterygopalatine Fossa; Rare Diseases; Plastic Surgery Procedures; Surgical Flaps; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 27501198
DOI: 10.1002/hed.24558