malignant rhabdoid tumors ( mrts ) are highly aggressive neoplasms that most commonly occur in the kidneys of young children . a few cases of primary mrt occurring in extra - renal sites have been reported , particularly in the soft tissues . malignant rhabdoid tumor was first described by beckwith and palmer in 1978 as a rhabdomyosarcomatoid variant of wilms tumor . but due to the absence of ultra - structural or immunohistochemical evidence of myogenic differentiation , the term rhabdoid was later adopted for these neoplasms . malignant rhabdoid tumors usually occur during infancy and the median age is about 11 months . however , mrts have been reported in patients from the age of 3 weeks to 50 years . the course of extra - renal mrt is short and its prognosis is very poor . the 5-year survival without tumor recurrences are less than 50% regardless of the therapy used . a 19-year - old girl presented to our outpatient department with a swelling of the tongue of 1-year duration , which was initially slowly growing but later started progressing rapidly in the past 3 months . she was complaining of inability in the movement of the tongue leading to difficulty in speaking and chewing of food . her general health was fair ( eastern cooperative oncology group performance status was 1 ) . on intra - oral examination , there was a lobulated swelling involving the entire tongue with an intervening ulcer and there was restriction of the tongue movement . multiple bilateral level 1a and 1b nodes of varying sizes were palpable and which were fixed to the underlying structures . the largest node was around 3 cm in size , which was on the left side ( clinical staging was t4bn2cm0 ) . examination of the skin , abdomen , respiratory system , cardio vascular and the central nervous systems were found to be normal . magnetic resonance imaging ( mri ) of the tongue showed an enhancing mass of more than 5  4 cm in its greatest dimension . the mass involved both sides of intrinsic as well as extrinsic muscles with loss of definition of lingual septum [ figure 1a ] . extension of the mass was seen into the right - sided sublingual space with encasement of the neurovascular bundle which was also extending anteriorly to the tip of tongue . magnetic resonance imaging of the neck showed metastatic cervical lymphadenopathy at the level i , ii , iii , iv on the left and level ii on the right [ figure 1b ] . clinically , the diagnosis of the lobulated swelling with an ulcer on the tongue , was that of an infiltrative epithelial malignancy or a minor salivary gland malignancy . limitation of punch biopsy in this case was the presence of a limited intervening area with ulceration on the tongue swelling , which may not be representative of the presence of definite malignant cells . however , punch biopsy from the ulcerative lesion showed solid sheets of moderately pleomorphic tumor cells with round to oval , central to eccentric nuclei and eosinophilic cytoplasm suggestive of undifferentiated malignant neoplasm with features of rhabdoid differentiation [ figure 2a ] . immunohistochemistry ( ihc ) analysis was done to detect the expression of vimentin , desmin , pan cytokeratin ( ck ) and s-100 protein . immunohistochemistry was positive for vimentin [ figure 2b ] but negative for the expression of desmin , pan ck and s-100 . the diagnosis of mrt was established after histopathological examination and ihc analysis . in this case , the patient was not considered for upfront surgery in view of the growth involving the whole of the tongue extending up to the vallecula ; hence , there would be an absence of clear margins for resection . the patient was treated with six cycles of palliative chemotherapy with doxorubicine ( 50 mg / m , d1 ) and ifosphamide ( 1500 mg / m , d1-d5 ) . there was no response to the treatment after completion of chemo - radiotherapy and the patient expired after 3 years from the date of diagnosis . ( a ) magnetic resonance imaging ( mri ) of the tongue showing heterogeneous hyper intensity on t2-weighted ( w ) image . ( b ) magnetic resonance imaging of the neck showing metastatic cervical lymphadenopathy ( a ) photomicrograph showing solid sheets of moderately pleomorphic tumor cells with round to oval and central to eccentric nuclei along with an eosinophilic cytoplasm ( h&e stain , 400 ) . ( b ) photomicrograph showing tumor cells to be positive for vimentin ( ihc stain , 400 ) rhabdoid tumor was first described in the kidneys with a poor prognosis . over the past several years , research have shown that it can also occur in the central nervous system ( brain and spinal cord ) , as well as other locations outside the kidneys such as the liver , muscle , heart , lung , soft tissues , skin , uterus and the thymus . there have been two case reports of mrt of the tongue in the english literature . another case of mrt of the tongue was reported by tomasovic et al . in an 18-year - old female . the cases have been reported from new born to teenagers and adults . in our case , the patient was a 19-year - old female who presented with indurations and swelling of the tongue for a duration of 1 year . the clinical presentation of our case was similar to the case reported by tomasovic et al . in the histology of mrt polygonal and eosinophilic cells with globular hyaline inclusion , open nuclei and prominent nucleoli are the typical findings , but to establish the diagnosis , clinical evaluation and histopathological feature along with ultra - structural and ihc findings are necessary . histopathology of mrt can be heterogeneous , so the differential diagnosis can be poorly differentiated carcinoma , melanoma , anaplastic large - cell lymphoma and rhabdomyosarcoma . in this case , histopathology from the tongue lesion showed sheets of neoplastic cells with abundant eosinophilic cytoplasm and eccentric mildly irregular vesicular nuclei with lobulation . most of the cells showed vimentin immunoreactivity and it was negative for the expressions of desmin and s-100 . to arrive at a definitive diagnosis in this case , we had to take the help of ihc . possibility of adenocarcinoma , anaplastic large - cell lymphoma , melanoma and rhabdomyosarcoma were ruled out by the ihc findings . as pan ck was negative , the diagnosis of adenocarcinomas and other epithelial tumors were excluded , the diagnosis of lymphoma was excluded by vimentin positivity and so , further lymphoma ihc study was not carried out . melanoma was excluded due to s-100 negativity and finally rhabdomyosarcoma diagnosis was excluded based on morphology [ histopathology ( hpe ) ] by the absence of strap cells and cross - striations , which are seen in rhabdomyosarcoma ; furthermore , this case was negative for desmin and ck . extra - renal mrt demonstrates a rapidly progressive evolution , with metastasis occurring in most patients from 2 to 15 months after diagnosis . in some of the reported cases , there were disseminated disease to the lungs , lymph nodes and liver at the time of diagnosis , which are the common sites of metastasis . in our case , there was no response to chemo - radiotherapy in this case , but the patient survived for a considerable period of time , unlike the poor survival of paravertebral rhabdoid tumors seen in infancy as reported by lynch et al . in conclusion , authors would like to add this extremely rare case of mrt of the tongue to the literature . malignant rhabdoid tumor of the tongue poses a therapeutic challenge to the oncologist and no consistently effective regimen has yet been established for the treatment of mrt . although mrts are rare , extra - renal rhabdoid tumor should be considered in the differential diagnosis of tumors of the tongue .
malignant rhabdoid tumors ( mrts ) are highly aggressive neoplasms that most commonly occur in the kidneys of young children . 
 malignant rhabdoid tumor of the tongue is an extremely rare entity and very few have been reported in the literature . 
 the course of extra - renal mrt is short and its prognosis is very poor . a 19-year - old female presented with a progressive swelling and restricted mobility of the tongue for over 3 months duration . 
 we present here a locally advanced case of mrt of the tongue , its diagnosis , management and review of the literature related to it .