Sclerosing Rhabdomyosarcoma in Childhood: Case Report and Review of the Literature

2004 ◽  
Vol 7 (4) ◽  
pp. 391-396 ◽  
Author(s):  
Bhumita Vadgama ◽  
Neil James Sebire ◽  
Marian Malone ◽  
Alan Drummond Ramsay

Rhabdomyosarcoma is the most common soft tissue malignancy in children but is rare in adults. The latest World Health Organization classification of soft tissue tumors recognizes embryonal, alveolar, and pleomorphic rhabdomyosarcomas. More recently, a sclerosing variant of rhabdomyosarcoma has been recognized and reported in seven adult patients. We describe a pediatric case of sclerosing rhabdomyosarcoma presenting as a sacral mass in a 3-year-old girl. Morphologically, the tumor showed a prominent sclerosing hyaline matrix and demonstrated pseudovascular and microalveolar architectural foci. Focal positivity was seen with desmin, smooth muscle actin, and myogenin. MyoD1 showed uniform diffuse nuclear staining. Fusion transcripts were not demonstrated by reverse transcriptase-polymerase chain reaction analysis. The histology, immunohistochemistry, and molecular genetics matched those reported in the seven adult cases of sclerosing rhabdomyosarcoma. This is the first case report, to our knowledge, of this rare tumor arising in the pediatric age group, and we compare the features with those reported in adult sclerosing rhabdomyosarcoma.

2014 ◽  
Vol 4 (2) ◽  
pp. 118-123
Author(s):  
Jagdish Vishnu Tupkari ◽  
Tabita Joy Chettiakandy ◽  
Dimple Padawe ◽  
Keshav Kumar ◽  
Manisha Sardar ◽  
...  

ABSTRACT Adult fibrosarcoma (FS), defined by the World Health Organization as ‘malignant neoplasm composed of fibroblasts with variable collagen production and, in classical cases, a ‘herringbone’ architecture’, is a very rare soft tissue sarcoma. The earlier literature revealed that the adult fibrosarcoma was the most commonly occurring lesion, however the incidence of which has declined dramatically over the past several decades. This is due to evolution in the classification of soft tissue tumors, recognition of its subtypes and increased understanding of the many other mesenchymal and nonmesenchymal tumors. Sarcomas are the rare group of malignancies which accounts for less than 1% of all the body sarcomas of which 5 to 15% occurs in head and neck region. It involves deep soft tissues of extremities, trunk, head and neck. Among the head and neck region, intraoral adult fibrosarcomas are very rare without any sex predilection. A case of primary soft tissue FS in posterior mandible of an 13-year-old female patient is documented here, which will be an addition to the cases reported earlier in the literatures. How to cite this article Tupkari JV, Chettiakandy TJ, Padawe D, kumar k, Sardar M, Gupta N. Intraoral Soft Tissue Fibrosarcoma: A Case Report and Review. J Contemp Dent 2014;4(2):118-123.


2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
K. Hamilton ◽  
S. Langley-Hobbs ◽  
C. Warren-Smith ◽  
K. Parsons

This case report details a caudal unilateral traumatic elbow luxation in a 4-year-old male neutered Labrador following a road traffic trauma. This is a highly unusual injury in the dog. The pathogenesis and successful treatment by closed reduction followed by stabilisation with a temporary transarticular external skeletal fixator are discussed. The dog was assessed at 4 weeks and 6 months after surgery. Findings at 6 months after treatment demonstrated a normal gait with no pain or crepitation. A mild amount of soft tissue thickening around the elbow was noted. The range of motion of the elbow was limited to 45 degrees of flexion and 150 degrees of extension. This is the first case of a traumatic caudal luxation of the elbow in a dog described in the English veterinary literature and the first report of successful management of an elbow luxation in a dog by closed reduction and temporary transarticular fixation.


Author(s):  
Khadija S. Tapadar ◽  
Manoj K. Deka ◽  
R. N. Chaubey ◽  
Shah A. Sheikh ◽  
Gargi R. Choudhury ◽  
...  

Background: Soft tissue tumors are defined as mesenchymal proliferations which occur in the extraskeletal non-epithelial tissues of the body, excluding the viscera, coverings of brain and   lymphoreticular system. The objective of this study was to study the histopathological features of soft tissue tumors and to study the occurrence of soft tissue tumors in relation to age, sex and anatomical site.Methods: This study comprised of 89 cases studied over a period of two years. All soft tissue tumors, their gross features, microscopic findings were analysed in detail. Soft tissue tumors were divided into benign and malignant categories and further sub typing were done according to World Health Organization (WHO) classification. The distribution of soft tissue tumors according to the age, sex and site of occurrence was studied.Results: Out of 89 cases of soft tissue tumors, 76 cases were benign, 4 cases belonged to intermediate category and 9 cases were malignant. Adipocytic tumors formed the largest group constituting 39 cases. Vascular tumors were the second commonest (26 cases) followed by peripheral nerve sheath tumors (11 cases). The benign tumors were seen in younger age as compared to malignant tumors. Malignant soft tissue tumors was seen to be more common in male than female and pleomorphic sarcoma and liposarcoma was commonest (3 cases each).Conclusions: Benign tumors were more common than malignant. The most common benign tumors were lipoma followed by hemangioma and schwannoma. The most common malignant tumor was pleomorphic sarcoma. The benign tumors were seen in younger age as compared to malignant tumors.


2016 ◽  
Vol 9 (1) ◽  
pp. 85-89
Author(s):  
Svetlana A. Mateva ◽  
Margarita R. Nikolova ◽  
Alexandar V. Valkov ◽  
Margarita R. Nikolova

Summary Liposarcoma is one of the most common soft tissue sarcomas in adults with a relative incidence amongst other sarcomas ranging from 9.8% to 16%. It usually locates in the limbs and retroperitoneum. Primary liposarcomas of the larynx and hypopharynx are rare, comprising less than 20% of all head and neck liposarcomas. According to World Health Organization, these tumors are divided into four histologic types, and well-differentiated liposarcoma is the most common one. It is a tumor of low-grade malignancy that may recur locally, but does not metastasize. We present a case of laryngopharyngeal well- differentiated liposarcoma in an old patient with two previous removals. We also discuss recently published cases with this unusual location of liposarcoma.


2019 ◽  
Vol 7 ◽  
pp. 2050313X1982891
Author(s):  
Miguel Angel Moyón Constante ◽  
Fernando Xavier Moyón Constante ◽  
Jorge Fernando Tufiño ◽  
Andres Cárdenas Patiño ◽  
Gabriel Alejandro Molina ◽  
...  

Mixed adenoneuroendocrine carcinomas are rare tumors that contain both an exocrine and an endocrine component. Since the latest classification by the World Health Organization and with the aid of immunostaining, more mixed adenoneuroendocrine carcinomas are now identified and diagnosed. Nonetheless, our knowledge of these tumors is still limited, notably concerning gastric variants, as the cases reported in the literature are very limited. The clinical and surgical treatment, including the chemotherapy schemes, the prognosis, and recurrence still represent challenges for the medical teams. We present the case of a 62-year-old woman. After an upper endoscopy revealed multiple polyps and a low-grade neuroendocrine tumor, a D2 radical gastrectomy was performed. A low output esophageal anastomotic leak was discovered in the postoperative period and successfully managed. Pathology revealed a gastric mixed adenoneuroendocrine carcinoma, the first case of this kind reported in Ecuador. Patient is doing well and under constant surveillance up until her 13th postoperative month.


2012 ◽  
Vol 126 (7) ◽  
pp. 743-746 ◽  
Author(s):  
N Aslam ◽  
Z-U-S Qazi ◽  
A H Ahmad ◽  
R U Khan

AbstractObjective:We describe the first reported case of a malignant glomus tumour of the larynx.Method:Case report and review of the world literature concerning malignant glomus tumours.Results:A 37-year-old man presented with progressive hoarseness and dyspnoea. A smooth, right-sided laryngeal mass was found on flexible nasolaryngoscopy. Initial biopsy of this lesion was reported as a true ‘glomus tumour’, which is a benign lesion. Wide local excision was performed. The final histology of this specimen showed it to be a ‘malignant glomus tumour’. Immunohistochemistry was positive for smooth muscle actin, collagen type IV, vimentin and cluster of differentiation 34 glycoprotein. Total laryngectomy was then performed as a definitive curative procedure.Conclusion:Glomus tumour is rarely found in visceral locations. This tumour and its malignant counterpart are rare differential diagnoses of laryngeal masses.


2021 ◽  
Author(s):  
Chujie Bai ◽  
Lu Zhang ◽  
Yaohui Wang ◽  
Taiyan Guo ◽  
Xia You ◽  
...  

Abstract Undifferentiated high-grade pleomorphic sarcoma (UPS) is a rare soft tissue sarcoma (STS) of mesenchymal origin, particularly the extremities and retroperitoneum, meanwhile it has been reported in almost all parts of the body. UPS is highly invasive and has a poor prognosis due to its clinical manifestations of painless mass and deep tumor site, which are usually found at an advanced stage. Patients with UPS tend to have a lower 5-year survival rate than patients with other types of STS. Recently, NTRK fusions were detected in many cancer types, such as thyroid cancer, colorectal cancer, non-small cell lung cancer, soft tissue tumors, uterine sarcomas, and melanomas. However, the mutation frequency of NTRK fusion in all cancers is only 0.1-1%. Targeted therapy with NTRK inhibitors, such as Larotrectinib and Entrectinib, leads to a response in most patients with NTRK1/2/3 gene fusion-positive tumors. Herein, we present a 68-years old man diagnosed with stage IIIA (T2N0M0G3) UPS. Next-generation sequencing (NGS) revealed a novel TMTC2-NTRK3 fusion. The NTRK3 positivity was also detected by immunohistochemistry (IHC) and fluorescence in situ hybridization (FISH). He had a response to Larotrectinib. This report broadens the spectrum of NTRK fusions in UPS and highlights a new target for treatment.


2013 ◽  
Vol 58 (No. 12) ◽  
pp. 621-627 ◽  
Author(s):  
JS Ko ◽  
HJ Kim ◽  
YM Choi ◽  
JW Kim ◽  
C. Park ◽  
...  

Malignant fibrous histiocytomas (MFHs), newly named as ‘undifferentiated pleomorphic sarcomas’ in 2002 by the World Health Organization, generally show an ambiguous origin. They have been described to fibroblastic or histiocytic in origin, while storiform-pleomorphic variants share a similar morphologic pattern with other sarcomas. For this reason additional diagnostic methods including immunology, ultrastructure analysis and molecular approaches are necessary for a more accurate diagnosis. We report three cases of MFH in individual dogs which presented histological characteristics of pleomorphic sarcomatoid tumours. Microscopic investigation, immunohistochemistry (vimentin, CD68, desmin, α-smooth muscle actin, and S-100) and electron microscopy were performed for diagnosis. One case was diagnosed as storiform-pleomorphic type and two were diagnosed as giant cell type MFHs. The present study demonstrates concepts of MFHs based on newly described categories and suggests useful diagnostic approaches for uncertain sarcomatoid soft tissue tumours in canines.


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