Sarcomas of the Oral and Maxillofacial Region: Analysis of 26 Cases with Emphasis on Diagnostic Challenges

Kumar, Priya; Surya, Varun; Urs, Aadithya B; Augustine, Jeyaseelan; Mohanty, Sujata; Gupta, Sunita

doi:10.1007/s12253-018-0510-9

Cited by 11 publications

(19 citation statements)

References 49 publications

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“…Multimodal therapy has been used in the treatment of RMS, and these treatments contribute to locoregional control (LCR) of the disease, especially in those where the surgical approach is restricted due to the extension and impairment of anatomic structures [ 1 , 3 ]. In this case report, a favorable clinical response to LCR was shown, based on neoadjuvant QT+ followed by concomitant RT+.…”

Section: Discussionmentioning

confidence: 99%

“…Sarcomas of the Head and Neck (SHN) are defined as malignant neoplasms derived from mesenchymal cells, whose origin is from bone, cartilage, muscle, vascular, adipose, and neurogenic tissues, with rapid growth, aggressive and infiltrative behavior, and the capacity for high locoregional recurrence [ 1 , 2 ].…”

Section: Introductionmentioning

confidence: 99%

“…When they occur, establishment of their initial diagnosis and their proper management are challenging tasks, due to their location and the anatomical structures involved. Among the most common sarcomas, especially in the maxillofacial region, the most representative are rhabdomyosarcoma (RMS) and osteosarcoma (OS) of the gnathic bones [ 1 , 2 ].…”

Section: Introductionmentioning

confidence: 99%

“…RMS of gnathic bones belong to the group of nonparameningeal entities and are mostly of the embryonic subtype (approximately 60% of the total reported cases) [ 3 ]. The diagnosis and classification of the lesion are established by immunohistochemistry assessment (IHC) exams and biomarkers; however, part of the cases remains unclassified, making their management difficult [ 1 ]. Among the sensitive and specific markers for RMS are Myogenin and MyoD1, both markers detected by IHC of initial muscle cell differentiation, which has a sensitivity of up to 100% [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

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An Atypical Presentation of Sinonasal Tract Alveolar Rhabdomyosarcoma in a Young Male Patient Submitted to Multimodality Treatment

Bernaola-Paredes

Favareto

Filho

et al. 2021

Case Reports in Oncological Medicine

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Rhabdomyosarcoma (RMS), a malignant tumor derived from the neoplastic proliferation of striated skeletal muscle cells, is the most common pediatric soft tissue sarcoma. Its treatment is mainly based on neoadjuvant chemotherapy (QT+), surgical resection, and adjuvant radiotherapy (RT+). RT+ has shown satisfactory results for locoregional control of the disease, in spite of promoting local side effects. The present case report was aimed at describing the clinical and therapeutic characteristics and the management of complications resulting from multimodal therapy in a patient with an atypical presentation of RMS in the sinonasal tract. A 20-year-old Afro-descendant man complained of an expansive tumor lesion, with left eye proptosis that reduced visual acuity and caused severe regional pain. Imaging analysis showed an extensive and infiltrative lesion in the periorbital region, sinonasal tract, left maxilla, and orbital roof. According to the histopathological analysis, the diagnosis was established corresponding to parameningeal alveolar RMS that was unresectable. Treatment was initiated with three cycles of QT+ which showed partial response and later RT+. After completing half of the RT+ sessions, the patient showed a complete response with reduction in tumor volume and improvement in pain and local conditions. Side effects such as alopecia and dermatological changes induced by radiation were observed. Moreover, painful erythematous areas were observed in the region of the hard and soft palate, uvula, and oropharynx, compatible with Grade 2 mucositis lesions. After the cytological swab test, some of them were diagnosed as herpes simplex lesions; thickening and decrease in salivary flow were also found. A local drug therapy approach was instituted, and photobiomodulation was performed to manage oral complications. RT+ was shown to be effective in locoregional control of the disease; however, the early management of its undesirable effects on the surrounding tissues was required.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

An Atypical Presentation of Sinonasal Tract Alveolar Rhabdomyosarcoma in a Young Male Patient Submitted to Multimodality Treatment

Bernaola-Paredes

Favareto

Filho

et al. 2021

Case Reports in Oncological Medicine

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“…They comprise of < 1% of adult malignancies and 15-20% of pediatric malignancies [3][4]. Maxillofacial sarcomas comprise 5-10% of all sarcoma cases [5][6][7][8][9][10][11][12] and < 1% of all malignant maxillofacial tumors [5,10,[13][14][15][16]. Over 70 subtypes of sarcomas were desribed so far [3,17].…”

Section: Epidemiologymentioning

confidence: 99%