Periosteal Myxoid Leiomyosarcoma Histologically Mimicking Extraskeletal Myxoid Chondrosarcoma: Report of a Case with Histopathological and Cytopathological Comparison with Extraskeletal Myxoid Chondrosarcoma

2021 ◽  
pp. 1-8
Author(s):  
Yoshinao Kikuchi ◽  
Shiori Watabe ◽  
Asako Yamamoto ◽  
Kenji Sato ◽  
Wataru Fujinuma ◽  
...  
Keyword(s):  
Tumor Cells ◽  
Final Diagnosis ◽  
Diagnostic Error ◽  
Wide Resection ◽  
Extraskeletal Myxoid Chondrosarcoma ◽  
Palpable Mass ◽  
Myxoid Chondrosarcoma ◽  
Reticular Pattern ◽  
Biopsy Examination ◽  
The Right

Myxoid leiomyosarcoma (MLS) is a rare variant of leiomyosarcoma, with most cases occurring in the uterus. A case of MLS arising in the periosteal region of the tibia, mimicking extraskeletal myxoid chondrosarcoma (EMC), is described. The evaluation included histological and cytological comparison with EMC. The patient was a 77-year-old man with a palpable mass at the anterior aspect of the right lower leg. After diagnosis by cytopathology and biopsy examination, a wide resection was performed. The resulting cytological smears were composed primarily of spindle-shaped tumor cells in a myxoid and hemorrhagic background. Histologically, the tumor showed abundant myxoid matrix and tumor cells proliferating in a cord-like to reticular pattern, exhibiting a lace-like arrangement that mimicked EMC. Although immunohistochemical findings suggested leiomyosarcoma, a diagnosis of EMC eventually was excluded by the lack of a split signal when assessed for a rearrangement of NR4A3 by chromogenic in situ hybridization. Despite histological similarity to EMC, characteristic cytological findings of EMC such as epithelioid structures with a cord-like pattern and chondroblast-like lacunar structures were not observed in the smears of this patient’s MLS. We propose that cytopathological examination of bone and soft tissue lesions is useful as a diagnostic tool in similar cases. A total diagnostic workup, including clinical, radiographic, cytopathological, histopathological, and molecular findings, is needed to ensure an accurate final diagnosis and to reduce diagnostic error.

scholarly journals Sustained response to imatinib in patient with extraskeletal myxoid chondrosarcoma and novel KIT mutation

2021 ◽  
Vol 14 (8) ◽  
pp. e242039
Author(s):  
Brooke Jennings ◽  
John Rieth ◽  
Travis Snyders ◽  
Mohammed Milhem
Keyword(s):  
Kinase Inhibitor ◽  
Lymph Node Involvement ◽  
Extraskeletal Myxoid Chondrosarcoma ◽  
Palpable Mass ◽  
Kit Mutation ◽  
Myxoid Chondrosarcoma ◽  
Node Involvement ◽  
History Of ◽  
Exon 11 ◽  
The Right

A 55-year-old woman presented with a 3-month history of right groin swelling, discomfort and impaired mobility. On examination, a palpable mass was noted both to the right of midline in the lower abdomen and in the right groin. MRI of the pelvis showed two masses involving the anterior abdominal wall and right groin, as well as lymph node involvement. CT imaging revealed multiple bilateral pulmonary metastases. Pathology demonstrated a myxohayline stroma morphology. Tumour was also notable for NR4A3 gene region rearrangement and mutation in KIT exon 11 at position c.1669 T>G. Based on these findings, she was diagnosed with extraskeletal myxoid chondrosarcoma (EMC). The patient has been on imatinib, a tyrosine kinase inhibitor with activity against KIT, for 3 years with stable disease. Metastatic EMC is generally treated with surgical resection and perioperative radiation therapy with adjuvant chemotherapy and is associated with poor prognosis.

scholarly journals Primary Desmoplastic Small Round Cell Tumor of the Submandibular Gland: A Case Report and Literature Review

2021 ◽  
Author(s):  
jiayu zhou ◽  
Qingling Li ◽  
baihua luo ◽  
xiaodan fu ◽  
chunlin ou ◽  
...  
Keyword(s):  
Tumor Cells ◽  
Submandibular Gland ◽  
Tumor Therapy ◽  
Final Diagnosis ◽  
Cell Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
The Right

Abstract Background: Desmoplastic small round cell tumor (DSRCT) is a sporadic, highly malignant tumor with a poor prognosis. The abdomen and pelvis have been reported as the primary localization sites. However, to the best of our knowledge, there are few reports on primary DSRCT in the submandibular gland.Case presentation: We have reported a case of a 26-year-old Chinese man who presented with a mass in the right submandible. Imaging studies showed a hypoechoic mass in the right mandibular region. Intraoperative pathology revealed that the tumor tissue was composed of small round tumor cells and a dense desmoplastic stroma. On immunostaining, the tumor cells showed markers of epithelial, mesenchymal, myogenic, and neural differentiation. The EWSR1 gene rearrangement was detected by fluorescence in situ hybridization. Based on the overall morphological features and immunohistochemical findings, a final diagnosis of DSRCT was made. The patient was treated with comprehensive anti-tumor therapy mainly based on radiotherapy and chemotherapy.Conclusions: DSRCT is a very uncommon disease in which submandibular gland involvement is rare. Considering DSRCT in the differential diagnosis of small round blue cell tumors, even in extraperitoneal locations, is beneficial for a precise diagnosis. The purpose of this report was to describe a cases of DSRCT of submandibular gland and review the literature on the other published cases of DSRCT over the past five years. Full recognition of the clinicopathological features will help to better diagnose this disease.

Metastatic Alveolar Soft Part Sarcoma to the Breast: A Case Report

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S27-S27
Author(s):  
A Cheriyan ◽  
A Bousamra
Keyword(s):  
Differential Diagnosis ◽  
Tumor Cells ◽  
Soft Part ◽  
Left Breast ◽  
Alveolar Soft Part Sarcoma ◽  
Primary Tumors ◽  
Palpable Mass ◽  
Keratin 5 ◽  
Left Base ◽  
The Right

Abstract Introduction/Objective Metastases to the breast are rare (less than 3% of all breast malignancies). In adults, malignant melanoma is the most common type of metastasis followed by carcinomas from various primary sites. Here, we describe a case of metastatic alveolar soft part sarcoma (ASPS) to the breast. Only a handful similar presentations are reported in the literature. Methods Here we describe the case of a 43-year-old female patient with a recent diagnosis of left base of tongue alveolar soft part sarcoma (in an outside hospital), presenting with a palpable mass in her left breast. Sonographic study of the left breast demonstrated a 1.4 cm oval hypoechoic mass with partially microlobulated margins at 1 o’clock. Results Histopathologic examination of the ultrasound guided core biopsy showed a “pink” neoplasm, composed of plump epithelioid tumor cells with an eosinophilic and granular cytoplasm, round to oval nuclei and conspicuous nucleoli. They are arranged in organoid nests separated by sinusoidal spaces. An immunohistochemical study shows the tumor cells to be negative for keratin AE1/AE3, keratin 7, keratin 5/6, GATA-3, Pax-8, estrogen receptor, HER2, p63, S100 and CD117. FISH test for TFE3 (Xp 11.23) is positive. PAS with diastase staining highlights the intracytoplasmic granules. Theses morphologic, clinical and molecular findings confirm the diagnosis of metastatic ASPS. Conclusion The differential diagnosis of “pink” tumors of the breast include primary tumors like secretory carcinoma and apocrine carcinoma and metastatic lesions of renal, thyroid, skin (melanoma), oral cavity and soft tissue. Although extremely rare, in the right clinical context, metastatic ASPS to the breast can occur and should be included in the differential diagnosis.

scholarly journals Primary Desmoplastic Small Round Cell Tumor of the Mandible: A Case Report and Literature Review

2021 ◽  
Author(s):  
jiayu zhou ◽  
Qingling Li ◽  
Baihua Luo ◽  
Xiaodan Fu ◽  
Chunlin Ou ◽  
...  
Keyword(s):  
Tumor Cells ◽  
Tumor Therapy ◽  
Final Diagnosis ◽  
Cell Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Precise Diagnosis ◽  
The Right

Abstract Background: Desmoplastic small round cell tumor (DSRCT) is a sporadic, highly malignant tumor with a poor prognosis. The abdomen and pelvis have been reported as the primary localization sites. However, to the best of our knowledge, there are few reports on primary DSRCT in the mandible.Case presentation: We have reported a case of a 26-year-old Chinese man who presented with a mass in the right mandible. Imaging studies showed a hypoechoic mass in the right mandibular region. Intraoperative pathology revealed that the tumor tissue was composed of small round tumor cells and a dense desmoplastic stroma. On immunostaining, the tumor cells showed markers of epithelial, mesenchymal, myogenic, and neural differentiation. The EWSR1 gene rearrangement was detected by fluorescence in situ hybridization. Based on the overall morphological features and immunohistochemical findings, a final diagnosis of DSRCT was made. The patient was treated with comprehensive anti-tumor therapy mainly based on radiotherapy and chemotherapy.Conclusions: DSRCT is a very uncommon disease in which mandibular involvement is rare. Considering DSRCT in the differential diagnosis of small round blue cell tumors, even in extraperitoneal locations, is beneficial for a precise diagnosis. Full recognition of the clinicopathological features will help to better diagnose this disease.

scholarly journals Salivary Gland Myoepithelioma with Focal Capsular Invasion

2009 ◽  
Vol 88 (7) ◽  
pp. 1005-1009 ◽  
Author(s):  
Banu Dogan Gun ◽  
Sukru Oguz Ozdamar ◽  
Burak Bahadir ◽  
Lokman Uzun
Keyword(s):  
Salivary Gland ◽  
Tumor Cells ◽  
Smooth Muscle Actin ◽  
Minor Component ◽  
Final Diagnosis ◽  
Capsular Invasion ◽  
Spindle Cells ◽  
Painless Mass ◽  
The Right ◽  
A Minor

Salivary gland tumors that display myoepithelial differentiation exclusively or predominantly are relatively uncommon, and the assessment of malignancy in a myoepithelial tumor can be difficult. We report a case of parotid gland myoepithelioma composed predominantly of spindle cells with focal capsular invasion. The patient was a 65-year-old woman who presented with a painless mass in the right preauricular region. Histologically, the tumor had a solid and multinodular growth pattern and was predominantly made up of spindle cells with a minor component of epithelioid cells with moderate cellular atypia. Focal regions of tumor cells infiltrated the capsule with tongue-like processes, hut tumor infiltration into the adjacent parotid tissue was absent. The tumor cells showed strong cytoplasmic immunoexpression of vimentin, pankeratin, S-100 protein, and smooth-muscle actin. Immunostains with glial fibrillary acidic protein, melanoma marker, epithelial membrane antigen, and carcinoembryonic antigen were negative. Expression of p53 was observed focally in the nuclei of the tumor cells. A final diagnosis of salivary gland myoepithelioma with focal capsular invasion was made, and the case was regarded as a myoepithelial tumor of uncertain malignant potential. In this report, we discuss the histologic criteria required to diagnose malignancy in salivary gland myoepithelial tumors.

IMMUNOTHERAPY FOR NSCLC: THE RIGHT TREATMENT FOR THE RIGHT PERSON

2019 ◽  
Vol 65 (1) ◽  
pp. 27-41
Author(s):  
Yelena Artamonova
Keyword(s):  
Life Expectancy ◽  
Tumor Cells ◽  
Advanced Nsclc ◽  
Objective Response ◽  
Combination Group ◽  
Line Treatment ◽  
Phase 3 ◽  
Median Response ◽  
The Right ◽  
High Level

Lung cancer is the leading cause of mortality from malignant tumors all over the world. Since most patients at the time of diagnosis already have stage III-IV of the disease, the search for new effective treatment strategies for advanced NSCLC is the most important problem of modern oncology. The results of the study of the anti-PD1 monoclonal antibody pembrolizumab were a real breakthrough in the treatment of NSCLC. In the KEYN0TE-001 study, the expression of PD-L1 on tumor cells was validated as a predictive biomarker of the drug's efficiency. Pembrolizumab demonstrated the possibility of achieving long-term objective responses, and a 4-year 0S with all histological types in the subgroup of pre-treated patients with PD-L1 expression> 50% was 24.8% and 15.6% in the PD-L1> 1% group. In a phase 2/3 randomized study KEYN0TE-10 in the 2nd line treatment of NSCLC with PD-L1 expression > 1% pembrolizumab significantly increased life expectancy compared to docetaxel and confirmed the possibility of longterm duration of objective responses, even after cessation of treatment. Then the focus of research shifted to the 1st line of treatment. About 30% of patients with NSCLC have a high level of PD-L1 expression on tumor cells and demonstrate the most impressive response to pembrolizumab therapy. A randomized phase 3 study KEYN0TE-024 compared the effectiveness of pembrolizumab monotherapy with a standard platinum combination in patients with advanced NSCLC with a high level of PD-L1 expression without EGFR mutations or ALK translocation. Compared with the platinum doublet the administration of pembrolizumab significantly increased all estimated parameters, including the median of progression-free survival (mPFS was 10.3 months versus 6 months; HR = 0.50; 95% CI 0.37-0.68, p < 0.001), the objective response rate (ORR 44.8% versus 27.8%), duration of response (in the pembrolizumab arm the median was not reached, in the chemotherapy (CT) group - 6.3 months). Despite the approved crossover, the use of pembrolizumab in the 1st line of treatment more than doubled the life expectancy of NSCLC patients with high PD-L1 expression as compared to CT: the median overall survival (OS) was 30.0 months versus 14.2 months (HR = 0.63, p = 0.002), 1-year OS 70.3% versus 54.8%; 2-year OS - 51.5% versus 34.5%. The remaining population to study were untreated patients with any level of PD-L1 expression. A randomized phase 3 study KEYNOTE-189 evaluated the effectiveness of adding pembrolizumab to the platinum combination in the 1st line treatment of non-squamous NSCLC without EGFR and ALK mutations with any PD-L1 expression. The addition of pembrolizumab to the standard 1st line CT significantly increased all estimated efficacy indicators including OS, PFS and ORR. After a median follow-up of 10.5 months the median OS in the pembrolizumab combination group was not reached and in CT group was 11.3 months. The estimated 12-months survival was 69.2% and 49.4% respectively (HR = 0.49; 95% CI 0.38-0,64; p <0.001). The median PFS was 8.8 months versus 4.9 months, alive 1 year without progression 34.1% and 17.3% of patients respectively (HR = 0.52; p <0.001). The ORR in the group with pembrolizumab reached 47.6% versus 18.9% in CT group, moreover the tumor regressions were much longer. Finally a randomized 3-phase study KEYN0TE-407 evaluated the effectiveness of adding pembrolizumab to 1st-line CT of NSCLC with squamous histology with any PD-L1 expression. As the first analysis showed, the addition of permboli-zumab significantly increased OS of patients with squamous NSCLC, median OS 15.9 months versus 11.3 months in the groups of pembrolizumab + CT and placebo + CT respectively (HR = 0.64; 95% CI 0,49-0.95; p = 0.0006), median PFS 6.4 months and 4.8 months respectively (HR = 0.56; 95% CI 0.450.70; p <0, 0001) and OrR 57.9% versus 38.4%, the median response duration 7.7 months versus 4.8 months. Thus, the convincing advantages of using pembrolizumab in 1st line therapy were demonstrated in 3 randomized phase 3 studies: in monotherapy of NSCLC of any histological subtype with high PD-L1 expression, and in combination with CT in squamous and non-squamous hystologies regardless of the level of PD-L1 expression.

Transferrin-Conjugated Nanocarriers as Active-Targeted Drug Delivery Platforms for Cancer Therapy

2017 ◽  
Vol 23 (3) ◽  
pp. 454-466 ◽  
Author(s):  
Daniele R. Nogueira-Librelotto ◽  
Cristiane F. Codevilla ◽  
Ammad Farooqi ◽  
Clarice M. B. Rolim
Keyword(s):  
Drug Delivery ◽  
Cancer Therapy ◽  
Tumor Cells ◽  
Therapeutic Benefit ◽  
Active Targeting ◽  
Future Research ◽  
Passive Targeting ◽  
The Right ◽  
Review Current ◽  
Target Effects

A lot of effort has been devoted to achieving active targeting for cancer therapy in order to reach the right cells. Hence, increasingly it is being realized that active-targeted nanocarriers notably reduce off-target effects, mainly because of targeted localization in tumors and active cellular uptake. In this context, by taking advantage of the overexpression of transferrin receptors on the surface of tumor cells, transferrin-conjugated nanodevices have been designed, in hope that the biomarker grafting would help to maximize the therapeutic benefit and to minimize the side effects. Notably, active targeting nanoparticles have shown improved therapeutic performances in different tumor models as compared to their passive targeting counterparts. In this review, current development of nano-based devices conjugated with transferrin for active tumor-targeting drug delivery are highlighted and discussed. The main objective of this review is to provide a summary of the vast types of nanomaterials that have been used to deliver different chemotherapeutics into tumor cells, and to ultimately evaluate the progression on the strategies for cancer therapy in view of the future research.

scholarly journals A First Case Report of Orbital Extra-Adrenal Paraganglioma in Cat

2021 ◽  
Vol 8 (5) ◽  
pp. 86
Author(s):  
Leonardo Leonardi ◽  
Raluca Ioana Rizac ◽  
Ilaria Pettinari ◽  
Luca Mechelli ◽  
Carlo De Feo
Keyword(s):  
Parasympathetic Nervous System ◽  
Neuron Specific Enolase ◽  
Final Diagnosis ◽  
Neuroendocrine Cells ◽  
Neuroendocrine Neoplasm ◽  
Glial Fibrillary Acid Protein ◽  
Veterinary Clinic ◽  
First Case ◽  
The Right ◽  
Extra Adrenal Paraganglioma

Paraganglioma is a rare neuroendocrine neoplasm originating from paraganglia and consisting of neuroendocrine cells of the sympathetic and parasympathetic nervous system. Extra-adrenal paraganglioma occurs with a low incidence in both humans and animals. This report presents the first case of paraganglioma in a cat with orbital primary location. An 18-year-old spayed female European domestic shorthair cat of 3.60 kg body weight was evaluated in a private veterinary clinic in Perugia, Italy, for a pronounced exophthalmos of the right eye. The cat underwent surgery for the enucleation of the right eye and of the mass. The biopsy samples of the removed tissue were fixed in 10% buffered neutral formalin for histological and immunohistochemical evaluations. Therefore, specific markers were used for immunohistochemical investigations, such as anti-neuron specific enolase (NSE), anti-synaptophysin, anti-glial fibrillary acid protein, anti-cytokeratin and anti-chromogranin. The results of these investigations allowed establishing the final diagnosis of ocular extra-adrenal paraganglioma of the cat.

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