Indian Journal of Pathology and Microbiology
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  Table of Contents    
CASE REPORT  
Year : 2011  |  Volume : 54  |  Issue : 1  |  Page : 136-137
Embryonal rhabdomyosarcoma of the adult soft palate


1 Department of ENT, Altnagelvin Area Hospital, Londonderry, BT47 6SB, United Kingdom
2 Department of Pathology, Antrim Area Hospital, Antrim, BT41 2RL, United Kingdom

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Date of Web Publication7-Mar-2011
 

   Abstract 

Embryonal rhabdomyosarcoma is the most common soft tissue sarcoma in children. We report a rare case of embryonal rhabdomyosarcoma of the soft palate in a 32-year-old Caucasian female. Detailed histology of the tumor is described. Positive staining with desmin, myogenin and myoD1 confirmed the tumor to be embryonal rhabdomyosarcoma. A genetic association between rhabdomyosarcoma, polycystic ovary syndrome and the FEM1A gene on the human chromosome is speculated upon.

Keywords: FEM1A protein, polycystic ovary disease, rhabdomyosarcoma, soft palate

How to cite this article:
Bhutoria S, ONeil C. Embryonal rhabdomyosarcoma of the adult soft palate. Indian J Pathol Microbiol 2011;54:136-7

How to cite this URL:
Bhutoria S, ONeil C. Embryonal rhabdomyosarcoma of the adult soft palate. Indian J Pathol Microbiol [serial online] 2011 [cited 2014 Apr 21];54:136-7. Available from: http://www.ijpmonline.org/text.asp?2011/54/1/136/77367



   Introduction Top


Rhabdomyosarcoma is the most common soft tissue sarcoma in children. The occurrence of rhabdomyosarcoma is rare in adults. It is rarer to find it in the soft palate. [1] A literature search was undertaken using PubMed. The search did not reveal a published case of embryonal rhabdomyosarcoma of the soft palate in an adult in the English language literature.


   Case Report Top


A 32-year-old Caucasian female presented with a 2-month history of a mass on the soft palate with associated pain, dysphagia and dysphonia. She had a past history of polycystic ovary syndrome. She was a smoker of 18 pack-years and had penicillin allergy.

Examination revealed a 0.5 cm × 0.5 cm firm mass on the right side of the soft palate. Magnetic resonance imaging (MRI) scan of the head and neck [Figure 1] and [Figure 2] revealed fluid and mucosal thickening of right maxillary sinus. Computerized tomography scan of the chest and abdomen revealed bilateral ovarian cysts. The lesion was excised under general anesthesia after an informed consent was obtained from the patient. Postoperatively, a small palatal fistula developed. Histopathology of the excised lesion revealed the presence of embryonal rhabdomyosarcoma. This was a biphasic tumor with small round cell areas and spindled areas [Figure 3]. In addition, there were areas with clear cytoplasm. There was no dysplasia within the overlying squamous epithelium. There was no cambium layer. Melanoma was excluded by immunohistochemistry (IHC). There were occasional areas with cells having abundant eosinophilic cytoplasm, imparting a rhabdoid appearance [Figure 4]. Positive staining with desmin [Figure 5], myogenin and myoD1 confirmed the tumor as a rhabdomyosarcoma. Morphologically, it belonged to the embryonal subcategory.
Figure 1: MRI scan: Sagittal view at level of pathology in soft palate

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Figure 2: MRI scan: Transverse view at level of pathology in soft palate

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Figure 3: Spindled areas (H and E, ×200)

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Figure 4: Rhabdoid appearance (H and E, ×600)

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Figure 5: Desmin stain (IHC, ×200)

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Further excision biopsies around the previous excision site proved negative. The patient is currently undergoing chemotherapy with ifosfamide, vincristine and actinomycin D regime and is scheduled to have radiotherapy following the chemotherapy.


   Discussion Top


Annual incidence of rhabdomyosarcoma, mostly in children, is around 60 in the UK and 350 in the USA. [2] Orbital tumors are the most common in the head and neck region. Other sites are the nose, paranasal sinuses, mouth, pharynx and cheek. [3] Rhabdomyosarcomas were classified histologically into embryonal, alveolar, botryoid and pleomorphic subtypes. The embryonal subtype is the most common in children, while the pure pleomorphic subtype is exclusive to adults. [2] Diagnosis of rhabdomyosarcoma is by detection of cross striations characteristic of skeletal muscle under light or electron microscopy. Staining for actin, desmin, Z-band protein and Myo-D1 confirm the diagnosis. Molecular and genetic markers are also used to differentiate the various subtypes. [4]

FEM1A gene was evaluated as a candidate for involvement in rhabdomyosarcoma and it was shown that FEM1A was downregulated in eight different human rhabdomyosarcoma cell lines, including those derived from embryonal and alveolar rhabdomyosarcoma. [5] Interestingly, based on a pilot study on Caucasian polycystic ovary syndrome patients, it was suggested that FEM1A should be considered a candidate gene for polycystic ovary syndrome. [6]

Current treatment of rhabdomyosarcoma uses a multidisciplinary team approach and involves surgery, chemotherapy and radiation therapy.

In conclusion, embryonal rhabdomyosarcoma of the adult soft palate is rare. Further research into molecular and genetic markers, such as the FEM1A gene, needs to be carried out. Histopathology proved useful in diagnosing embryonal rhabdomyosarcoma and formulating treatment plans.

 
   References Top

1.Martin GE, Alexander WA. A Case of Rhabdomyosarcoma of the soft palate. J Laryngol Otol 1924;39:312-21.   Back to cited text no. 1
    
2.El-Ghazali AM, McLaren KM. Embryonal rhabdomyosarcoma of adult nasopharynx. J Laryngol Otol 2005;119:639-42  Back to cited text no. 2
    
3.Parham DM, Ellison DA. Rhabdomyosarcomas in adults and children: An update. Arch Pathol Lab Med 2006;130:1454-65.   Back to cited text no. 3
[PUBMED]  [FULLTEXT]  
4.Merlino G, Helman LJ. Rhabdomyosarcoma-working out the pathways. Oncogene 1999;18:5340-8.  Back to cited text no. 4
[PUBMED]  [FULLTEXT]  
5.Ventura-Holman T, Hahn H, Subauste JS, Maher JF. The FEM1A gene is downregulated in Rhabdomyosarcoma. Tumour Biol 2005;26:294-9.  Back to cited text no. 5
[PUBMED]    
6.Maher JF, Hines RS, Futterweit W, Crawford S, Lu D, Shen P, et al. FEM1A is a candidate gene for polycystic ovary syndrome. Gynecol Endocrinol 2005;21:330-5.  Back to cited text no. 6
[PUBMED]  [FULLTEXT]  

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Correspondence Address:
Samar Bhutoria
c/o ENT Secretaries, Altnagelvin Area Hospital, Londonderry, BT47 6SB
United Kingdom
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DOI: 10.4103/0377-4929.77367

PMID: 21393897

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    Figures

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



 

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    Abstract
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