Abstract :
Introduction: Rhabdoid tumor is relatively rare highly malignant tumor in adult older than 40 years, therefore treatment regimens often throwing from pediatric age group. It is characterized by extremely aggressive behavior, rapid metastasis to other organ, low survival rate and no targeted therapy. So, early diagnosis is necessary for better treatment and reduce mortality outcome.
Case Report: A 42 years male suffering from a right side cheek mass of 5x3cm associated with tongue displacement to left side and difficulty in swallowing. After complete removal of lesion for histopathological examination, revealed grossly the tumor is well defined gray-white, round, lobulated 5x3cm in size having infiltrating borders. Cut surface shows gray-white to gray-brown, solid areas. Microscopically H&E stained section revealed, sheets of tightly packed large, discohesive, polygonal cells having abundant eosinophilic cytoplasm with eccentrically placed round nuclei showing distinct prominent vesicular nucleoli separated by thin fibrous septae with blood vessels. These cells lie in myxoid background. These tumor cells have deeply acidophilic having finely granular abundant cytoplasm with small, peripherally placed nuclei and abundant intra-cytoplasmic vacuoles along with nuclear pleomorphic, no mitotic figure seen.
Discussion: Histologically, the adult type tumor proliferating as sheet of tightly packed large, discohesive, polygonal cells having abundant eosinophilic cytoplasm with eccentrically placed round nuclei showing distinct prominent vesicular nucleoli separated by thin fibrous septae with blood vessels lie in fibromyxoid background. These tumor cells have deeply acidophilic, finely granular cytoplasm with small, peripherally placed nuclei with occasional intra-cytoplasmic vacuoles along with cross striations, no mitotic figures seen.
Conclusion: Study concludes that adult type Rhabdoid tumors have extremely aggressive behavior, rapid metastasis spread to other organs, low survival rate and no targeted therapy. Usually males older than 40years of age are affected. It is crucial to make a rapid correct diagnosis and early treatment may improve the outcome.
Keyword :
H & E – Hematoxylin and Eosin, CD99- Cluster of differentiation 99, CD 56- Cluster of differentiation 56, SALL 4- Spalt Like sal-like