malignant mesenchymal tumor
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2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Lucas Novaes Teixeira ◽  
Eduardo Zambaldi da Cruz ◽  
Ana Cláudia Garcia Rosa ◽  
Anderson Abdo Rodrigues ◽  
Fabrício Passador-Santos ◽  
...  

Synovial sarcoma (SS) is a rare malignant mesenchymal tumor that mainly occurs in body extremities, being uncommon in the head and neck region. In the present study, we described a case of primary intraosseous SS arising in the mandible of a 22-year-old young male. The patient reported a painful swelling on the left side of the mandible for the last 7 months. Imaging exams showed the presence of an expansive and multilocular radiolucent lesion, extending from the left condyle to the mandibular body. The clinic diagnostic hypotheses were ameloblastoma or malignant neoplasm. Histologically, the lesion was characterized by a proliferation of spindle cells exhibiting vesicular nuclei and evident nucleolus. Neoplastic cells were positive for AE1/AE3, cytokeratin 7, vimentin, CD-99, and TLE-1 and negative for CD-34, S-100, SMA, and HHF-35. A combination of clinical, histologic, and immunohistochemical characteristics supported the diagnosis of SS. The patient was referred for treatment, and preoperative exams did not reveal any other tumor foci in the body of the patient. The final diagnosis was of a primary intraosseous SS of the mandible.


2021 ◽  
Vol 59 (243) ◽  
pp. 1192-1195
Author(s):  
Shiv Raj Shah ◽  
Sujan Regmee ◽  
Dhiresh Kumar Maharjan ◽  
Prabin Bikram Thapa

Dermatofibrosarcoma protuberance represents less than 0.1% of all tumors, treatment of which requires wide local excision (≥5cm) but recurrence is not rare. Here we present a 32-year male presented with a swelling of 15 x 6cm over the left lumbar region for which he underwent excision three years ago, the histopathological examination of the swelling, showed a malignant mesenchymal tumor and Immunohistochemistry features were suggestive of Dermatofibrosarcoma protuberance. After three years of interval, he again presented with complaints of swelling in the previously operated site for nine months and underwent excision of the mass with Split Thickness Skin Graft. Although the tumor was confined to the skin and subcutaneous tissue in the present case, the patient didn’t undergo any adjuvant radiotherapy to avoid a possible relapse that would infiltrate deeper structures for the first time. Being a recurrent tumor, long-term follow-up is strongly recommended.


2021 ◽  
pp. 38-40
Author(s):  
S. Kanimozhi ◽  
P. Pooja sri ◽  
P. Karthika ◽  
M. Sathish Kumar ◽  
A. Mathan Mohan

Rhabdomyosarcoma (RMS) is a rare, aggressive, malignant mesenchymal tumor of skeletal muscle cells. The pleomorphic histological variant of RMS occurs in adults beyond 45years of age and represents the most aggressive subtype with an incidence of approximately 0.44/100,000. The diagnosis of RMS is difcult with the 5year overall survival rate less than 50%. It presents varied clinical and biological behavior and requires individualized management. The common region of metastasis includes lymph nodes, lungs and bone marrow. Here we report a case of pleomorphic rhabdomyosarcoma in 58year old female. The patient reported with a swelling in the neck region and had a history of surgical treatment for ovarian tumor. Histopathology ndings revealed metastatic undifferentiated carcinoma. The case was positive for immunohistochemistry markers and their ndings are diagnosed as pleomorphic rhabdomyosarcoma. This is a rare case of RMS which had metastasized to head and neck and this article emphasizes the importance of IHC in accurate and clear diagnosis of RMS.


2021 ◽  
pp. JCO.20.03060
Author(s):  
Jack F. Shern ◽  
Joanna Selfe ◽  
Elisa Izquierdo ◽  
Rajesh Patidar ◽  
Hsien-Chao Chou ◽  
...  

PURPOSE Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood. Despite aggressive therapy, the 5-year survival rate for patients with metastatic or recurrent disease remains poor, and beyond PAX-FOXO1 fusion status, no genomic markers are available for risk stratification. We present an international consortium study designed to determine the incidence of driver mutations and their association with clinical outcome. PATIENTS AND METHODS Tumor samples collected from patients enrolled on Children's Oncology Group trials (1998-2017) and UK patients enrolled on malignant mesenchymal tumor and RMS2005 (1995-2016) trials were subjected to custom-capture sequencing. Mutations, indels, gene deletions, and amplifications were identified, and survival analysis was performed. RESULTS DNA from 641 patients was suitable for analyses. A median of one mutation was found per tumor. In FOXO1 fusion-negative cases, mutation of any RAS pathway member was found in > 50% of cases, and 21% had no putative driver mutation identified. BCOR (15%), NF1 (15%), and TP53 (13%) mutations were found at a higher incidence than previously reported and TP53 mutations were associated with worse outcomes in both fusion-negative and FOXO1 fusion-positive cases. Interestingly, mutations in RAS isoforms predominated in infants < 1 year (64% of cases). Mutation of MYOD1 was associated with histologic patterns beyond those previously described, older age, head and neck primary site, and a dismal survival. Finally, we provide a searchable companion database ( ClinOmics ), containing all genomic variants, and clinical annotation including survival data. CONCLUSION This is the largest genomic characterization of clinically annotated rhabdomyosarcoma tumors to date and provides prognostic genetic features that refine risk stratification and will be incorporated into prospective trials.


Author(s):  
Alan Alexander ◽  
Kyle Hunter ◽  
Michael Rubin ◽  
Ambarish P. Bhat

AbstractExtraosseous Ewing’s sarcoma (EES), first described in 1969, is a malignant mesenchymal tumor just like its intraosseous counterpart. Although Ewing’s sarcomas are common bone tumors in young children, EESs are rarer and more commonly found in older children/adults, often carrying a poorer prognosis. We discuss the multimodality imaging features of EES and the differential diagnosis of an aggressive appearing mass in proximity to skeletal structures, with pathologic correlates. This review highlights the need to recognize the variability of radiologic findings in EES such as the presence of hemorrhage, rich vascularity, and cystic or necrotic regions and its imaging similarity to other neoplasms that are closely related pathologically.


2021 ◽  
Vol 8 (5) ◽  
pp. 1614
Author(s):  
Vikash C. Tiwari ◽  
Vaibhav Mudhale ◽  
Kulkarni S. Sharang ◽  
Jineshwar Nyamagoud ◽  
Malluru Srividya

Retroperitoneal liposarcoma is a rare malignant mesenchymal tumor with an incidence of 2.5 per million individuals. It is usually asymptomatic until its large enough to compress the surrounding organs, so its early diagnosis is difficult. In current case a 41 year old male patient with no significant past medical history presented to emergency department with sudden onset of abdominal pain in the left lumbar region. On examination, the abdomen was distended, guarding and rigidity present with tenderness to palpation in the left lumbar region, and no mass was palpated. Abdominal USG revealed a large heterogeneous mass in the left lumbar region. As the origin of the mass was uncertain, the patient was evaluated with CECT of the abdomen which revealed a retroperitoneal mass highly s/o liposarcoma. Given the suspicion of a liposarcoma, the patient underwent an abdominal exploration and there was a retroperitoneal mass of 12x8 cm adherent to the left colon. Wide resection of this mass was done along with left colectomy and the specimen was sent for examination. The histopathology study determined lipomatous tumor well differentiated retroperitoneal liposarcoma. Retroperitoneal liposarcoma is a malignant tumor whose treatment is fundamentally surgical. These tumors tend to be resistant to radiotherapy and/or chemotherapy. Among the most important prognostic factors related to survival is surgery with non-affected margins.


2021 ◽  
Vol 12 ◽  
pp. 17
Author(s):  
Daniel B Murray ◽  
Jack Horan ◽  
Alan Beausang ◽  
Mohammed Ben Husien

Background: Ewing’s sarcoma (ES) is a malignant mesenchymal tumor, most often found in the long bones, and usually affecting children and adolescents in the second decade of life. ES of the spine is a clinical rarity. Case Description: A 45-year-old male presented with a 3-month history of lower back pain which acutely worsened in conjunction with urinary retention. The magnetic resonance imaging revealed a mass extending from L5 to S2 with additional extension through the left S2-3 neural foramen. The metastatic workup was negative. At surgery, the lesion was both intradural and extradural. Following complete surgical resection, the patient was later treated with radiation and chemotherapy. Conclusion: Here, we report an adult male who acutely presented with low back pain attributable to primary intradural/extradural sacral ES.


2021 ◽  
pp. 1-1
Author(s):  
Snezana Knezevic ◽  
Biljana Sreckovic ◽  
Jelena Vulovic ◽  
Marijana Jandric-Kocic

Soft tissue sarcomas are heterogeneous group of neoplasms making up to 1% of all malignant tumors in the adult population. The tumor generally appears on the extremities near large joints of middle-aged patients, especially in the popliteal fossa. Our patient presented in February 2014 due to a slowly enlarging, darker-colored swelling in the left popliteal fossa. Upon physical examination, a somewhat nodular, immobile, tender subcutaneous mass was observed. There was no locoregional lymphadenopathy. Patohistological findings showed a high-grade primary malignant mesenchymal tumor, biphasic synovial sarcoma type. The patient underwent surgery with wide surgical excision, followed by radiotherapy treatment. Magnetic resonance imagining follow up after one year revealed tumor recurrence. Neurovascular bundle involvement was detected, but without adjacent bone and muscular invasion and above-the-knee partial amputation of the left leg was performed. The intervention resulted in a remission of the neoplastic process and the patient was scheduled for regular check-ups. Broad surgical resection of the tumor with negative margins was the primary treatment in this case. Mutilating operations are necessary when anatomical structures around the tumor do not allow complete reintervention.


2020 ◽  
Vol 7 (4) ◽  
pp. 145
Author(s):  
Joong-Hyun Song ◽  
Do-Hyeon Yu ◽  
Dong-In Jung

A 2-year-old female Mongrel dog weighing 3.12 kg presented with a 2-month history of progressive exophthalmos of the left eye and periorbital swelling. Fine-needle aspiration cytology of the affected tissue revealed atypical cells of suspected malignant mesenchymal tumor origin. Computed tomography and magnetic resonance imaging revealed an ill-demarcated soft tissue mass in the left retrobulbar space extending into the nasal cavity and into the frontal lobes of the brain with destruction of the adjacent cribriform plate and the basisphenoid bone. Histopathological features of the tumor were consistent with the diagnosis of undifferentiated sarcoma. The tumor cells were immunoreactive for vimentin, smooth muscle actin, and desmin and negative for S100. These findings were mostly consistent with leiomyosarcoma arising from the smooth muscle on the retrobulbar tissues. Primary retrobulbar leiomyosarcoma is an extremely rare tumor in dogs. To expand our knowledge of retrobulbar leiomyosarcoma in dogs, we have described its clinical, diagnostic imaging, histopathological, and immunohistochemical characteristics in a dog.


2020 ◽  
Vol 2 ◽  
pp. 73-76
Author(s):  
Birma Ram ◽  
Pankaj Sharma ◽  
Manoj Gopinath ◽  
Shamresh Kumar Singh

Extraskeletal osteosarcoma is a rare malignant mesenchymal tumor which typically affects elderly patients and the commonly affected sites are extremities, retroperitoneum, mesentery, and trunk. Extraskeletal osteosarcoma arising from colon or retroperitoneum can manifest as a large mass with features of intestinal or urinary tract obstruction. Local recurrence after excision and distant metastasis are common, so regular follow-up and surveillance of such patients are advisable after excision of the primary tumor.


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