small cell carcinoma is a malignancy that occurs mainly in the lung , and primary lesions in the head and neck are very rare .
extrapulmonary small cell carcinoma ( epscc ) shows a fair response to either chemotherapy or chemoradiotherapy , similar to small cell lung carcinoma ( sclc ) ; however , poor prognosis has been reported due to its high metastatic potential .
irinotecan hydrochloride ( cpt-11 ) , a topoisomerase i inhibitor , has been reported to be effective against sclc . here , we report a rare case of epscc of the tonsil , treated with cpt-11 and cisplatin ( cddp ) , which is the recent treatment protocol for sclc . the present case represents the first report of cpt-11 usage for epscc of the oropharynx .
our case suggests that cpt-11 and cddp may become an effective treatment option for epscc of the oropharynx .
a 65-year - old male presented to our hospital complaining of sore throat for 2 weeks .
he smoked heavily ( 70 cigarettes per day for 40 years ) and was also a long - time alcohol drinker .
physical examination revealed an ulcerated mass in the left palatine tonsil and multiple swollen left neck lymph nodes . computed tomography ( ct )
scans of the head and neck revealed a well - defined , 3.2  3 cm mass in the left tonsil with multiple left cervical lymph node involvement .
hematoxylin and eosin - stained sections revealed nests of monotonous , small , round carcinoma cells with scant cytoplasm and frequent mitotic figures in the subepithelial tissue ( fig .
immunohistochemically , the tumor cells were positive for neural cell adhesion molecule ( cd56 ) , synaptophysin , chromogranin , cytokeratin , and epithelial membrane antigen ; the cells were negative for s-100 protein , hmb-45 , leukocyte common antigen , and thyroid transcription factor-1 ( fig .
these findings indicated small cell carcinoma . to differentiate primary small cell carcinoma from a metastatic sclc ,
in addition , systemic workup , such as of the brain , as well as abdominal ct and fdg - pet showed negative findings for distant metastasis .
the tumor was diagnosed as oropharyngeal cancer , t2n2bm0 ( tnm classification , uicc , 2002 ) . because cardiac echo revealed severe aortic stenosis and moderate aortic regurgitation , the patient received aortic valve replacement before treating the epscc of the tonsil .
the tonsillar mass then grew to 4.6  3.8 cm , and the left neck lymph nodes also progressed ( fig .
one month after the cardiac operation , he underwent radiotherapy of the tonsil and the bilateral cervical area with a dosage of 65.4 gy in 35 fractions .
he was also planned to receive three courses of chemotherapy with a regimen including cpt-11 ( 60 mg / m , days 1 and 15 ) and cddp ( 30 mg / m , days 1 and 15 ) at 4-week intervals .
because severe thrombocytopenia occurred after the first course of chemotherapy , the second and third courses were performed with reduced doses of cpt-11 ( 48 mg / m , days 1 and 15 ) and cddp ( 24 mg / m , days 1 and 15 ) at 3-week intervals .
epscc compromises only 2.54% of all small cell carcinomas and is commonly found in the esophagus , colon , pancreas , uterus , and breast .
it reveals a fair response to either chemotherapy or chemoradiotherapy but exhibits a poor prognosis .
in general , the clinical course of epscc is known to be aggressive because of frequent metastases and recurrences .
systemic disease from the onset of treatment , even when no distant metastasis is recognized at the first medical examination . in the head and neck region ,
the most common sites are the larynx , nose , and paranasal sinuses , and the major salivary glands .
primary small cell carcinoma of the tonsil is rare ; only 11 cases have been reported [ 3 , 4 , 5 , 6 , 7 , 8 , 9 ] .
the prognosis of patients with epscc of the tonsil has been poor , as 6 of those 11 patients died from the disease mainly due to systemic metastasis . when a diagnosis of epscc arising in the head and neck region is made , the patient should be evaluated first to determine whether the disease is a primary or metastatic cancer .
primary sclc is rarely occult because it is aggressive and fast - growing . in our case ,
chest and abdominal ct and fdg - pet revealed no other lesions , and the tonsillar mass was determined to be the primary lesion .
the histopathological aspects of epscc include round , oval- to spindle - shaped small cells with dense chromatin , absence of nucleoli , and inconspicuous cytoplasm .
mitosis , necrosis , apoptosis , and lymphatic , vascular , and perineural invasion are common .
immunohistochemical staining for neuroendocrine markers , including chromogranin a , synaptophysin , and cd56 , is usually positive . in this case ,
histologic features and immunoreactivity for neuroendocrine markers were suggestive of epscc . although the treatment of this tumor has not been defined clearly , it can require surgical excision of the localized tumor , radiation therapy to the primary site , multiple - drug chemotherapy , or a combination of these modalities .
platinum - based chemotherapy , such as the use of etoposide ( vp-16 ) and cddp , has been recognized as the standard regimen for epscc .
recently , cpt-11 combined with cddp was found to be more effective than vp-16 and cddp .
two cases of chemotherapy using cpt-11 and cddp have been reported for epscc arising in the head and neck region : one in the larynx and the other in the nasal cavity [ 10 , 11 ] .
both cases achieved long - term remission . in our case , radiation therapy to primary and locoregional sites followed by immediate chemotherapy with cpt-11/cddp resulted in complete remission of the tumor , although the patient later died of liver metastasis .
in this report , we describe a case of small cell carcinoma of the tonsil treated by combined chemotherapy with cpt-11/cddp after radiotherapy .
this is the first report on the use of combined cpt-11/cddp chemotherapy in small cell carcinoma of the oropharynx .
 we report a rare case of extrapulmonary small cell carcinoma arising in the palatine tonsil treated by combined chemotherapy with irinotecan / cisplatin following irradiation therapy . 
 this chemotherapy regimen was recently found to be effective for small cell lung carcinoma . 
 our case is the first report of combined irinotecan / cisplatin chemotherapy to treat extrapulmonary small cell carcinoma of the oropharynx . 
Introduction
Case Report
Discussion
Conclusion