rhabdomyosarcoma ( rms ) is an aggressive malignant soft tissue tumor that arises from primitive striated muscle cells ( rhabdomyoblasts ) . it can arise from almost anywhere in the body , but is most common in the head and neck region ( orbit and eyelids ) . it is a malignant mesenchymal tumor that frequently involves the urogenital tract and the extremities . the paranasal sinuses are very rarely involved , comprising only 1.5% of the reported head and neck cases . alveolar rms tends to occur in slightly older patients with a mean age of 1520 years . within the head and neck region , embryonal rms is the most common rms , whereas alveolar and pleomorphic tumors are more common in the extremities . clinically positive neck nodes at diagnosis are unusual in head and neck rms , but microscopic involvement is found by an elective neck dissection . the incidence of lymphatic metastases from the head and neck region in rms has been reported to be 7% . the alveolar variant exhibits the greatest propensity for lymph node metastasis ; 33% are associated with positive regional nodes on initial examination and 7585% develop either regional or distant nodal deposits , sometimes during the course of the disease . cervical metastasis in rms of the nose and paranasal sinus was present in 38% of the patients . similarly , alveolar subtype rmss compared with non - alveolar ones exhibited more rhabdomyoblastic cells , alveolar structures , giant , multinucleated cells , mitotic figures , and cytological atypia . inversely , spindle - shaped cells were more frequently seen in non - alveolar rmss . reviewed the cytological findings of rms with histological confirmation and proposed a morphological subtyping based on the most prominent cytologic features . an 18-year - old female presented with complaints of left - sided nasal obstruction , occasional epistaxis , and swellings on both sides of the neck of 2 months duration . on examination , the left nasal passage was found to be filled with a small granular and fleshy tumor , with some purulent discharge from the left side of the nasopharynx . there were bilateral enlarged submandibular lymph nodes that were firm and non - tender , measuring 3 cm  3 cm . fine needle aspiration cytology ( fnac ) was performed on both the cervical lymph nodes . fnac smears revealed a high degree of cellularity and displayed tightly cohesive clusters of homogenous tumor cells , with occasional gland - like spaces as well as single isolated cells with abundant karyorrhectic debris . roundish cell clusters around the connective tissue fragments or vascular cores with central lumen , frequently anastomizing in irregular sheaths , referred to as  alveolar structures ,  were well appreciated [ figure 1 ] . the smears were composed of small round cells with cytonuclear atypia ; few spindle - shaped and plasmacytoid cells with eccentric nuclei were also seen [ figure 2 ] . mitotic figures , binucleated , multinucleated cells , and necrotic background were also well appreciated . the tumor cells exhibited round to oval nuclei with mild irregularity and finely granular chromatin with inconspicuous nucleoli and scanty cytoplasm . most of the criteria used by klijanienko et al . to diagnose and subtype based on these fnac findings , the diagnosis of metastatic deposit of small round cell tumor , probably alveolar rms , was suggested . smear shows alveolar structures composed of roundish cells around a connective septa or vascular structures ( h and e , 400 ) cellular smear made up of small round cells with high nucleocytoplasmic ratio , few plasmacytoid type of cells with a typical multinucleated cell , and few gland - like spaces ( h and e , 400 ) the patient was referred to the department of e.n.t . for further management but , unfortunately , the patient was lost during follow - up . then , after about 6 months , she returned to the department of e.n.t . with a frank fungatating growth involving the nasal vestibule and the upper lip area along with persistent cervical lymphadenopathy . pre - operative baseline parameters and chest radiograph were within normal limits . under general anesthesia , grossly , the excised specimen consisted of the tumor mass with part of the upper lip along with bilateral submandibular lymph nodes . the tumor mass measured 2.5 cm  3 cm , with an irregular elevated surface , and was of firm consistency . the lymph nodes measured 3 cm  3 cm , with a smooth surface and areas of hemorrhage ; the cut surface revealed a homogenous white area . sections from the tumor showed non - cohesive clusters of large anaplastic malignant cells with moderate to abundant eosinophilic cytoplasm and pleomorphic nuclei with coarse chromatin pattern , mitotic activities , and nuclear vacuolations . cells with abundant dense eosinophilic tapering cytoplasm , suggestive of rhabdomyoblastic differentiation , were seen . the tumor cells were seen shedding from the fibrous septa ( alveolar growth pattern ) . multinucleated giant cells with peripherally arranged nuclei were seen frequently . the histological findings of the lymph nodes showed evidences of infiltration with total effacement of lymph node architecture and focal outstanding sinusoidal infiltration and a prominent alveolar growth pattern , interspersed with occasional typical rhabdomyoblasts . the reticulin - stained section showed a typical criss - cross arrangement of the fibres , which was also evident in the hematoxylin and eosin ( h and e)-stained sections . part of h and e - stained section had a solid or diffuse architecture in which the reticulin stain was helpful . the phosphotungstic acid hematoxylin stain showed striations in the tumor cells pointing toward the origin of the tumor , i.e. from the muscles . immunohistochemistry was carried out , which included leucocyte common antigen ( lca ) , vimentin , s-100 , and cytokeratin , of which vimentin and s-100 were positive . based on the above findings , histopathology of the resected specimen was reported as alveolar rms with a metastatic deposit of the same in the lymph nodes . rms bears a close resemblance to various stages in the embryogenesis of normal skeletal muscle , but its pattern is much more variable , and ranges from poorly differentiated tumors that are very difficult to diagnose without immunohistochemical or electron microscopic examination to a well - differentiated rms . morphologically ,  solid  forms of alveolar rms can also be seen , which might simulate the round cell areas of the embryonal rms . however , a uniform cellular pattern , multinucleate giant cells , and incipient alveolar features , supported by cytogenetic studies , help in differentiating between the two . it is important not to confuse the two as the alveolar type carries a less - favorable prognosis . differential diagnosis of malignant small round cell tumors that includes ewings sarcoma , neuroblastoma , malignant lymphoma , and poorly differentiated carcinoma were considered . in ewings sarcoma , two populations of cells are usually found , large pale cells and smaller dark cells . the cytoplasm is pale blue and contains variable numbers of punched - out vacuoles that correspond to glycogen deposits . variable numbers of pseudorosettes may be seen and mitotic figures are rarely detected ; such features were not present in our case . in neuroblastoma , small clusters of cells are usually separated by a pale blue to light purple fibrillar matrix . malignant lymphoma cells are dominated by a single cell pattern and lymphoglandular bodies . moreover , immunostain for lca was negative in our case , which ruled out lymphoma , and because cytokeratin was negative , poorly differentiated carcinoma was also ruled out . alveolar rms involves tumors that are mostly undifferentiated in nature and therefore readily develop distant metastasis and thus have the worst prognosis . in this case , it is difficult to make a definite diagnosis of small round cell tumors based on cytological findings alone . therefore , it is essential for the cytopathologist to evaluate clinical data and histological and immunohistochemical findings upon encountering these morphological features so that the possibility of this condition is not overlooked .
rhabdomyosarcoma ( rms ) is an aggressive malignant soft tissue tumor that arises from primitive striated muscle cells called rhabdomyoblasts . 
 rms is a rare tumor in adults , and involvement of the sinonasal area is extremely rare , comprising only 1.5% of all reported head and neck rmss . 
 alveolar rms , mainly seen in adults , has the worst prognosis . 
 incidence of lymph node metastases is more common in this type compared with the other forms . 
 fine needle aspiration cytology ( fnac ) has been used extensively in the diagnosis of metastatic malignancies 
 . however , metastatic soft tissue sarcomas are often overlooked , primarily due to the low frequency with which they occur . here , we report a rare case of metastatic alveolar rms in the cervical lymph nodes of an 18-year - old girl that was detected by fnac . after 6 months , 
 the patient came with a huge mass involving the nasal vestibule and the upper lip . 
 histology of both the main mass and the lymph nodes revealed alveolar rms .