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Rhabdomyosarcomas (RMS) are among the most common soft-tissue tumors in children. These tumors are derived from mesenchymal tissue with a tendency toward myogenic differentiation that probably originates from immature and highly invasive satellite cells associated with the embryogenesis of skeletal muscle. Some of these tumors are associated with h...
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Rhabdomyosarcomas are among the most common soft-tissue tumors in children. These tumors are derived from mesenchymal tissue with a tendency toward myogenic differentiation that probably originates from immature and highly invasive satellite cells associated with the embryogenesis of skeletal muscle. Some of these tumors are associated with high ra...
Citations
... RMS is a rare, soft tissue malignancy of a mesenchymal origin, suspected to arise from cells of the skeletal muscle lineage [12]. RMS is very uncommon in adults and constitutes only 3% of all soft tissue sarcomas [12]. ...
... RMS is a rare, soft tissue malignancy of a mesenchymal origin, suspected to arise from cells of the skeletal muscle lineage [12]. RMS is very uncommon in adults and constitutes only 3% of all soft tissue sarcomas [12]. The commonest site of metastasis is the lungs followed by bone marrow and lymph nodes, as demonstrated in our case. ...
... Most primary adult RMSs occur in the extremities, and the lower limb mass in our patient was highly suspicious of the primary tumour. Moreover, adult RMS is extremely aggressive with 5year survival rates of approximately 27%, which is lower than survival rates in paediatric populations [12]. ...
Rhabdomyosarcoma (RMS), is a neoplasm of skeletal muscle in origin, is the second most common soft tissue sarcoma encountered in childhood after osteosarcoma. The common sites of involvement are the head and neck region, genitourinary tract, retroperitoneum, and to a lesser extent, the extremities. Site predilections is the oral cavity which are soft palate, maxillary sinus and alveolus, posterior mandibular region, cheek and lip, and sometimes tongue. RMS is highly malignant tumor with extensive local invasions and early hemorrhagic and lymphatic dissemination. We present a case of 25yrs old lady came to our emergency depatment with history of swelling of the right side of the face and protrusion of the right eye.Patient was also pregnant at 38weeks of gestation. C/s was done and later on the lateral rhinotomy with extened weber fergusson was done followed by radiochemotherapy.
... Rhabdomyosarcoma (RMS) constitutes a heterogeneous and aggressive malignant soft tissue neoplasm arises from undifferentiated mesodermal tissue [1][2][3][4]. RMS is primarily in striated muscle but can originate in the soft tissue that does not normally contain striated muscle [1,3,5]. RMS is the most common soft tissue sarcoma among children and adolescents [1,4,6]. ...
... RMS is the most common soft tissue sarcoma among children and adolescents [1,4,6]. This tumor accounts for 5% to 10% of all malignancies in childhood tumors [1,[5][6][7]. All parts of the body can be affected by RMS, but the most affected sites are the head and neck region in 40%, the genitourinary tract in 23%, the extremities in 20% and the other parts in 22% [3, 6,8]. ...
... Non-meningeal locations include the scalp, parotid gland, oral cavity, pharynx, thyroid and parathyroid [8][9][10]. The oropharyngeal cavity is even more rarely reported in the literature [1,5,9,10]. The treatment of RMS must be multidisciplinary and depends on the site. ...
Rhabdomyosarcoma (RMS), a tumor of skeletal muscle origin, is the most common soft tissue sarcoma encountered in childhood and adolescence; it is primarily found in the head and neck region, it is relatively uncommon tumors of the oral cavity. Clinical signs depend on the exact location of the lesion in the oral cavity and its development. Authors reported the case of a 14-year-old patient who presented an oropharyngeal mass causing voice dysfunction, after two surgical operation the patient experimented two 2 recurrences of the lesion. The histopathological examination objectifies an oropharyngeal rhabdomyosarcoma. Immediate postoperative outcome was uneventful with improvement in the voice dysfunction and dysphagia one month after surgery. Complementary treatment (chemotherapy and radiotherapy) was not available and accessible to the patient. Twenty months (20) after surgery, the examination found a recurrence of the tumor with pulmonary metastases and neurological complications. Oropharyngeal rhabdomyosarcomas are rare. Their interest lies in the fact that they often affect children and adolescents. The prognosis remains unfavorable in our context, even for cases accessible to surgery since complementary treatment with chemotherapy and / or radiotherapy does not exist. The prognosis depends on tumor size, location, staging, age of patients and especially the quality of the management.
... Rhabdomyosarcoma (RMS) is the commonest, highly malignant soft tissue sarcoma of childhood and adolescents, which accounts for 3% of all pediatric tumors [4]. Incidence peaks in children aged 1-4 years, lower in children aged 10-14 years and lowest between 15-19 years [5]. RMS is derived from immature striated skeletal muscle; hence, this disease can virtually occur anywhere in the body, though there are distinct clinical patterns according to the age at presentation, the histologic subtype and the site of the tumor. ...
We are presenting a 2-year male child with large abdominal mass extending from right inguino-scrotal region to suprapubic region of size 4.0 × 5.0 cm. Mass causing pressure effect over bilateral kidneys and ureters with bilateral dilated renal pelvis and extending into the right inguinal region and pelvis. Histopathologically and immunocytochemically patient was confirmed as rhabdomyosarcoma of the retroperitoneum. Patient treated with six cycles of infusion chemotherapy with 3-weekly VAC regimen and was having progressive disease because of the aggressive behaviour of the disease and further treated with second line chemotherapy. The present case is a very unusual and rare site of metastatic presentation of the Rhabdomyosarcoma.
... RMS, which was first described by Webner in 1854, is a malignant soft tissue tumor of skeletal muscle origin. 8 RMS commonly affected areas are the extremities, genitourinary tract, retroperitonium, and head-and-neck region. In the head-and-neck region, RMS usually occurs at the orbit and the oral cavity areas, such as maxillary or mandibular alveolar ridge, 9,10 palate, 11 buccal mucosa, 5 and tongue. ...
Background: Oral cancer is one of the sixth most common cancers in human, and oral squamous cell carcinoma (OSCC) is the most common type of oral cancer. The clinical appearance of OSCC may resemble other types of oral cancer such as rhabdomyosarcoma (RMS). The definitive diagnosis is crucial as the management and prognosis of both lesions are quite different. Case Report: A 42-year-old male presented with a unhealed painful ulcer of the posterior right lateral border of the tongue for about 4 months. Restricted mobility of the tongue and positive submandibular lymph nodes were detected. Wide excision with regional neck dissection was performed. Discussion: Pleomorphic RMS (PRMS) was identifed by immunohistochemistry of positive desmin and smooth muscle actin. The clinical presentation of the lesion is exactly similar to OSCC. PRMS is a rare type of high-grade sarcoma of the oral cavity. It usually occurs in the deep of lower extremities of adult on the six decade. Conclusion: Although the management of OSCC and RMS is almost alike, the ability of metastasis is salient compared to OSCC. Therefore, the definitive diagnosis should be careful due to its characteristic.
... The most frequent site of involvement in the oral cavity is the tongue, followed by soft palate, hard palate, and buccal mucosa. [3] The majority of RMS occurs randomly, without any risk factors, although a small proportion of these tumors are associated with genetic mutations. [4] There are three types of RMS on the basis of histopathology such as alveolar (20%), embryonal (60%), and pleomorphic (20%). ...
... 18 and cytokeratin may also be positive . Thus, only vimentin is present in the cytoplasm of the most primitive cells, and desmin 2,5,11 and actin are acquired by developing rhabdomyoblasts . Several histochemical stains were key in arriving at this conclusion 19 . ...
... Desmin is an intermediate filament protein present in smooth muscle cells, striated muscle cells and myocardium. A positive reaction for 20 vimentin confirms a mesenchymal derived tumor and desmin 11 indicates muscular differentiation . These both histochemical stains were positive in present case. ...
An embryonal rhabdomyosarcoma (ERMS) is a most common , primitive, malignant, soft tissue sarcoma in children under 15 yrs of age. It is considered to result from malignant transformation of primitive mesenchymal cells. Although it has a predominance for head and neck region , it is found less often in oral cavity. Here we report a case of ERMS of mandible in a 13 year old young girl. Clinical and pathologic findings are described , which were confirmed by histochemical and immunohistochemical stains. Three cycles of chemotherapy resulted in over 50% reduction in size of the mass. However, increase in size was noted subsequent to completion of therapy. In nine months, the patient developed multiple metastases, radionuclide bone scan showed increased tracer uptake on left side of mandible in angle, ramus region, and right pubis region. Patient died despite chemotherapy .
... The most frequent site of involvement in the oral cavity is the tongue, followed by soft palate, hard palate, and buccal mucosa. [3] The majority of RMS occurs randomly, without any risk factors, although a small proportion of these tumors are associated with genetic mutations. [4] There are three types of RMS on the basis of histopathology such as alveolar (20%), embryonal (60%), and pleomorphic (20%). ...
... Rhabdomyosarcoma (RMS) is an aggressive malignant neoplasm of mesenchymal origin, with a penchant for persistent aggressive growth and a potential for metastasis. [1][2][3] It is a fairly common pediatric soft-tissue malignancy, representing 6% of all malignancies in children younger than 15 years. [4,5] However, it is less commonly seen among adults, representing <1% of all solid malignancies among them. ...
... [6,10,11] Orofacial RMSs have a potential to result in grotesque facial distortions, causing grave esthetic complications. [3,12] Patients often present with varying clinical features, including pain, tooth mobility, spontaneous bleeding, and trismus. [12,13] The clinical features observed is generally dependent on factors such as the site and stage of the tumor. ...
... [19] This may be attributed to improved understanding of the disease and advent of multimodal treatment methods. [1,3,5,20] Treatment has evolved to become multimodal in nature, involving surgery, chemotherapy, and radiotherapy in different combinations. [20,21] Despite this, mortality rates remain significantly higher in developing countries than in developed ccij_1_18R2 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 countries. ...
... Patients with RMS may present signs and symptoms such as pain, paresthesia, loss of teeth and trismus as a result of factors such as advanced tumor stage, infiltrative growth and tumor location. [10] Pain, proptosis, diplopia, strabismus, decreased hearing, nasal obstruction, dysphagia, cervical lymphadenopathy are other signs and symptoms. [11] In the present case pain, dysphagia and cervical lymphadenopathy are reported. ...
... Patients with RMS may present signs and symptoms such as pain, paresthesia, loss of teeth and trismus as a result of factors such as advanced tumor stage, infiltrative growth and tumor location. [10] Pain, proptosis, diplopia, strabismus, decreased hearing, nasal obstruction, dysphagia, cervical lymphadenopathy are other signs and symptoms. [11] In the present case pain, dysphagia and cervical lymphadenopathy are reported. ...
Rhabdomyosarcoma (RMS), a malignant neoplasm of skeletal muscle origin, is the most common soft tissue sarcoma seen in childhood and adolescence. The most frequent site is the head and neck accounting for 40% of all cases and other involved sites are genitourinary tract, retroperitoneum, and to a lesser extent, the extremities. RMS is relatively uncommon in the oral cavity and the involvement of the jaws is extremely rare. Here, we report a case of 50-year-old female with oral RMS involving maxillary alveolar region with clinical, radiological, histopathological and immunohistochemical findings.
