Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 6  |  Issue : 2  |  Page : 137-140

Aggressive rhabdomyosarcoma of palate: A case report and literature review


1 Departments of Oral and Maxillofacial Surgery, King George's Medical University, Lucknow, Uttar Pradesh, India
2 Department of Oral Pathology, King George's Medical University, Lucknow, Uttar Pradesh, India
3 Faculty of Dental Sciences, IMS, BHU, Varanasi, Uttar Pradesh, India

Date of Web Publication16-Oct-2014

Correspondence Address:
Shalini Gupta
Department of Oral Pathology, King George's Medical University, Lucknow, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-8844.143062

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  Abstract 

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma encountered in childhood and adolescence. The common sites of occurrence are the head and neck region, genitourinary tract, retroperitonium and the extremities. Orbit, paranasal sinuses, cheek, and the neck are the common affected site in head and neck region. RMS is relatively uncommon in the oral cavity, and the involvement of the jaws is extremely rare. Multidisciplinary approaches surgery, chemotherapy and radiotherapy, are use for treatment. Here, we report an aggressive form of RMS at palate of a 9 months old female child which did not respond to chemotherapy.

Keywords: Rhabdomyosarcoma, malignant, tumors


How to cite this article:
Ram H, Gupta S, Mohammad S, Chandra A. Aggressive rhabdomyosarcoma of palate: A case report and literature review . J Orofac Sci 2014;6:137-40

How to cite this URL:
Ram H, Gupta S, Mohammad S, Chandra A. Aggressive rhabdomyosarcoma of palate: A case report and literature review . J Orofac Sci [serial online] 2014 [cited 2019 Aug 24];6:137-40. Available from: http://www.jofs.in/text.asp?2014/6/2/137/143062


  Introduction Top


Rhabdomyosarcoma (RMS) is a malignant soft tissue neoplasm of striated muscle cell called rhabdomyoblast. [1] It accounts for 6% of all malignancies in children under 15 years of age. [2] It may occur anywhere in the body but most commonly affected areas are the head and neck region, genitourinary tract, retroperitonium and extremities. [3] Histologically RMS are classified into four subtypes: Embryonal, alveolar, botryoid and pleomorphic types. The embryonal subtype is most common in children, while the pleomorphic type is exclusively found in adults. [4] Multidisciplinary approaches including surgery, chemo and radiation therapy are used in the treatment of RMS. [5]


  Case report Top


A 9 months old female child was brought by her parents to the Department of Oral and Maxillofacial Surgery with the complaint of fast growing swelling on the left side of the palate since 1 month. On extra oral examination a swelling was observed on the left side of the palate involving face measuring about 7 cm × 6 cm in size, extending from the angle of mouth to the temporal region and from tragus of the ear to the medial side of the eye. Overlying skin was normal in colour and texture except skin of the orbital region which was slightly reddish. On palpation swelling it was soft to firm in consistency and non-tender. Two submandibular lymph nodes were palpable, measuring 1.5 cm × 1.5 cm and 1 cm × 1 cm respectively. On intra oral examination a soft to firm swelling was seen on the palate measuring 4 cm × 3 cm crossing midline [Figure 1].
Figure 1: Patient showing lesion at palate and its extension

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Computed tomography of the face revealed a left-sided large fairly well defined hyperdense enhancing lesion in the region of left temporal fossa, infratemporal region, ipsilateral nasal and oral cavities and with extension into the orbit and brain. Destruction of the sphenoid bone, temporal squama, posterolateral wall of maxillary sinus, pterygoid plate and posterolateral wall of the orbital bones and palate was noted [Figure 2] and [Figure 3].
Figure 2: Computed tomography scan (axial view), showing lesion crossing mid line

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Figure 3: Computed tomography scan (coronal view), showing lesion extending in brain

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Histopathological finding revealed, fibrous connective tissue covered by squamous epithelium and infiltrated by tumour. The tumour was composed of proliferated small round or ovoid cells disposed of diffusely. The cells enclosed round or ovoid hyperchromatic nuclei and light stained cytoplasm. Moderate anisocytosis was also seen [Figure 4]. Immuno-histochemistry was positive for myogenin, vimentin, desmin [Figure 5], [Figure 6], [Figure 7].
Figure 4: Sheet of round (mesenchymal) cells in Hematoxylin and Eosin stain (10×)

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Figure 5: Sheet of round (mesenchymal) cells with nuclear Myogenin stain (10×)

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Figure 6: Sheet of round (mesenchymal) cells with cytoplasmic Vimentin stain (10×)

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Figure 7: Sheet of round (mesenchymal) cells with cytoplasmic Desmin stain (10×)

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On the basis of above findings, chemotherapy comprising of vincristin, dactinomycin with cyclophosphanide was given. But However, it did not respond to chemotherapy and patient died after second cycle of therapy.


  Discussion Top


RMS accounts for only 2-5% tumors in adults but approximately 60% tumors in children. [6] The incidence of RMS is highest in children aged 1-4 years, lower in children aged 10-14 years and lowest in those aged 15-19 years. [7],[8] Males are affected 1.5 times more often than females. [9] Reported tumour seen in the female child of only 9 months.

Orbital RNS are most common in the head and neck region followed by nose, paranasal sinuses, mouth, pharynx and cheek. [10] The most common site of involvement in the oral cavity is the tongue followed by the soft palate, hard palate, and buccal mucosa. [11] Though orbital RNS are most common but our patient had RNS at hard palate and soft palate both, which is very rare.

Clinical manifestations of RMS vary from a small cutaneous nodule on the face to an extensive fast-growing facial swelling, which may be painless or occasionally associated with pain, trismus, paresthesia, facial palsy, and nasal discharge. [2],[12]

Horn and Enter line first classified 4-four histological subtypes of RMS as embryonal, alveolar, botryoid and pleomorphic. [13] Diagnosis of RMS is confirmed by detection of cross striations characteristic of skeletal muscle under light or electron microscope. Stainig for actin, desmin, Z-band protein and Myo-D1 confirm the diagnosis. Molecular and genetic markers are also used to differentiate the various subtypes. [14] In our case IHC was positive for Vimentin, desmin, myogenin and negative for CD 45 (LCA) and Pancytokeratin. Alveolar RMS has been reported to have a poor prognosis and to be associated with a greater frequency of disseminated metastases, [9] whereas pleomorphic types has the best prognosis. [13]

Different treatment modalities e.g. Surgery, chemotherapy and radiotherapy, are used for the treatment of RMS. Selection of treatment depend on site, size, location and extent of the disease. In the head and neck region surgery is performed if lesion is not involving any vital structure. Chemoradiation is done if lesion is not respectable. However, in some cases combination therapy is performed.

In general, most patients have an advanced disease even at the stage of initial presentation because RMSs are known to show rapid growth and the patients generally tend to delay medical consultation. [7],[8] In our case, when the patient presented to us there was metastasis in the brain, so there was only option of chemotherapy only. Chemo therapy comprising of vincristin, dactinomycin with cyclophosphanide was given. Two cycles of therapy was given but patient did not respond to chemotherapy and died after second cycle of treatment.

Alveolar RMS has been reported to have a poor prognosis and have a greater tendency of lymphatic metastases. [9]

The overall survival rate for pediatric RMS is 71%, if combined modality therapy - e.g. chemotherapy, radiation therapy and surgery are used. [15] Orbital RMS commonly metastasizes through hematogenous route. It usually spread to lungs, liver and bones. [16] regional lymph node metastases occurs in the advanced stage because orbit is relatively devoid of lymphatic tissue. [17]

Prognosis of RMS is relatively poor compared to that of other oral soft tissue malignant lesions [18],[19],[20],[21] and depends on the clinical staging and the anatomic site of the tumor, [2],[18],[19],[20] however early diagnosis, and accurate treatment planning may improve survival rate.

Intergroup rhabdomyosarcoma study (IRS) clinical grouping is very useful for treatment planning of RMS. Prognosis of RMS depends on clinical group, histology of tumour site of tumour and age of the patient. The IRS classification includes four groups; based on tumour resectibility [20],[22] Group 1 includes localized disease, completely resectable. Group 2 includes gross resection with evidence of regional spread. Group 3 includes incomplete resection with gross residual disease. Finally, Group 4 includes distant metastatic disease present at diagnosis. Reported case was in Group 3 which includes that lesion is not completely resectable.


  Conclusion Top


Although soft tissue sarcomas like RMS appear frequently in the oral cavity but its palatal location is rare and should, therefore be highlighted. Dental practitioners frequently encounter cases of children with swelling on the palate and so RMS must also be considered in the differential diagnosis. The use of chemotherapy in the treatment of RMS has shown wonderful results about with regard to the literature but could not prove beneficial in our case and unfortunately the patient did not survive.



 
  References Top

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Durães GV, Jham BC, Mesquita ATM. Oral embryonal rhabdomyosarcoma in a child: A case report with immunohistochemical analysis. Oral Oncol Extra 2006;42:105-8.  Back to cited text no. 3
    
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El Ghazali AM, McLaren KM. Emroyonal rhabdomyosarcoma of adult nasopharynx. J laryngol Otol 2005;119:639-42.  Back to cited text no. 4
    
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Bhutoria S, Oneil C. Emroyonal rhabdomyosarcoma of the adult soft palate. Indian J Pathol and Microbiol 2011;54:136-7.  Back to cited text no. 5
    
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Gordon Nunez MA, Piva MR, Dos Anjos ED. Orofacial rhabdomyosarcoma: Report of a case and review of the literature. Med Oral Patol Oral Cir Bucal 2008;13:765-74.  Back to cited text no. 8
    
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Wendy Kar Yee Ng. Embryonal Rhabdomyosarcoma in a Young Boy. Mcgill J Med 2007;10:16-19.  Back to cited text no. 9
    
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12.
Wiss K, Solomon AR, Raimer SS. Rhabdomyosarcoma presenting as a cutaneous nodule Arch Dermatol 1988;124:1687-1777.  Back to cited text no. 12
    
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Volpe NJ, Jakobiec FA. Pediatric Orbital tumours. Int Ophthalmol Clin 1992;32:210-22.  Back to cited text no. 13
    
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Merlino G, Helman LJ. Rhabdomyosarcoma Working out the pathways. Oncogene 1990;18:5340-8.  Back to cited text no. 14
    
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McCarville MB, Spunt SL, Pappo AS. Rhabdomyosarcoma in Pediatric Patients. American Journal of Roentgenology 2001; 176:1563-69.  Back to cited text no. 16
    
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Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumours and simulating lesions: The 2002 Mongomery Lecture, part 1. Ophthalmology 2004;111:997-1008.  Back to cited text no. 17
    
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Shields JA, Shields CL. Rhabdomyosarcoma: Review for the ophthalmologist. Surv Ophthalmol 2003;48:39-57.  Back to cited text no. 18
    
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Pappo AS, Shapiro DN, Crist WM, Maurer HM. Biology and therapy of pediatric rhabdomyosarcoma. J Clin Oncol 1995; 13:2123-39.  Back to cited text no. 19
    
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Peters E, Cohen M, Altini M. Rhabdomyosarcoma of the oral and paraoral region. Cancer 1989;63:963-9.  Back to cited text no. 20
    
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Yamaguchi S, Nagasawa H, Suzuki T. Sarcomas of the oral and maxillofacial region: A review of 32 cases in 25 years. Clin Oral Investig 2004;8:52-7.  Back to cited text no. 21
    
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Pappo AS, Shapiro DN, Crist WM. Biology and therapy of pediatric rhabdomyosarcoma. Ciln Oncol 1995;13:2123-39.  Back to cited text no. 22
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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