the diffuse sclerosing variant of papillary thyroid carcinoma ( dsv - ptc ) , first described by vickery et al . in 1985 , is a histological variant of ptc . it is characterized by diffuse involvement of one or both thyroid lobules with prominent squamous metaplasia , numerous psammoma bodies , and dense sclerosis in intratumoral areas together with a background of lymphocytic infiltration . extensive lymphovascular invasion indicates frequent metastasis . because of the early spread of dsv - ptc dsv - ptc is a major subtype of ptc in young patients , with a higher female predominance ( male : female ratio , 1:7.5 ) . however , the frequency of dsv - ptc is 0.35.5% among ptc [ 4 , 5 , 6 ] . we herein report a 15-year - old female patient with estrogen receptor ( er)- and progesterone receptor ( pgr)-positive dsv - ptc with paratracheal lymph node metastasis in whom recurrence in the right cervical lymph nodes was diagnosed 7 years after the first operation . a 15-year - old female patient with no medical or family history of thyroid tumors consulted an otolaryngological clinic for evaluation of an anterior neck swelling . a thyroid neoplasm was suspected , and she was referred to kansai medical university takii hospital . diffuse enlargement of the right thyroid gland with many small calcifications was detected by ultrasonography ( fig . however , lymph node swelling of the right neck was detected 7 years after the operation and reexcision was performed . during the treatment course , her serum t3 , t4 , and tsh levels were within the reference ranges and autoantibodies were not detected . on macroscopic examination , the tumor , which had been located in the right lobe , was 22 mm along its major axis without formation of a dominant mass . the tumor showed a papillary growth pattern with distinct fibrosis , severe lymphocyte infiltration ( fig . 3c ) ; all characteristics of dsv - ptc were frequently seen . in high - power fields , many of the tumor cell nuclei exhibited a ground - glass appearance , frequent grooves , and pseudoinclusions ( fig . tumor invasion extended outside the capsule and reached the mesenchymal tissue , and lymph node metastasis was notable . 4a , b ) , and the cells were positive for epithelial membrane antigen , cytokeratin 7 , thyroglobulin , e - cadherin , and -catenin . reduced membranous staining and increased cytoplasmic staining of e - cadherin were notable ( fig . however , the tumor cells were negative for cytokeratin 20 , bcl-2 , c - kit , wilms tumor antigen 1 , cd56 , chromogranin a , synaptophysin , and androgen receptor . the ki-67 and p53 indices were both < a 15-year - old female patient with no medical or family history of thyroid tumors consulted an otolaryngological clinic for evaluation of an anterior neck swelling . a thyroid neoplasm was suspected , and she was referred to kansai medical university takii hospital . diffuse enlargement of the right thyroid gland with many small calcifications was detected by ultrasonography ( fig . however , lymph node swelling of the right neck was detected 7 years after the operation and reexcision was performed . during the treatment course , her serum t3 , t4 , and tsh levels were within the reference ranges and autoantibodies were not detected . on macroscopic examination , the tumor , which had been located in the right lobe , was 22 mm along its major axis without formation of a dominant mass . the tumor showed a papillary growth pattern with distinct fibrosis , severe lymphocyte infiltration ( fig . 3c ) ; all characteristics of dsv - ptc were frequently seen . in high - power fields , many of the tumor cell nuclei exhibited a ground - glass appearance , frequent grooves , and pseudoinclusions ( fig . tumor invasion extended outside the capsule and reached the mesenchymal tissue , and lymph node metastasis was notable . 4a , b ) , and the cells were positive for epithelial membrane antigen , cytokeratin 7 , thyroglobulin , e - cadherin , and -catenin . reduced membranous staining and increased cytoplasmic staining of e - cadherin were notable ( fig . however , the tumor cells were negative for cytokeratin 20 , bcl-2 , c - kit , wilms tumor antigen 1 , cd56 , chromogranin a , synaptophysin , and androgen receptor . dsv - ptc is a subtype of ptc and is characterized by diffuse enlargement of the thyroid gland and numerous scattered microcalcifications ( a so - called  snowstorm appearance ) on ultrasonography . histologically , dsv - ptc is characterized by papillary growth of atypical cells , similar to conventional ptc   with prominent fibrosis , numerous psammoma bodies , frequent squamous metaplasia , and dense infiltration of lymphocytes [ 2 , 3 , 8 , 9 ] . in addition , dsv - ptc shows a strong tendency for invasion and metastasis , and metastatic lesions have been frequently reported in lymph nodes , lungs , bones , and brain [ 10 , 11 ] . although lymph node metastasis was seen , multifocal tumor emboli in lymphatic channels at the primary site were unremarkable . immunohistochemical studies have been performed on dsv - ptc [ 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 ] . immunohistochemically , p63 expression in foci of squamous metaplasia is the hallmark for distinguishing between dsv - ptc and conventional ptc [ 3 , 12 ] . ptc cells are positive for thyroid transcription factor-1 , thyroglobulin , and -catenin [ 12 , 18 ] . in addition , positive localization of e - cadherin is decreased in the cell membrane and relocation to the cytoplasm occurs . therefore , it is easy to conclude that the decrement in the membrane level of e - cadherin may promote increased potency of invasion and/or metastasis of dsv - ptc . because dsv - ptc is characterized by aggressive growth , it has been thought to be associated with a poor prognosis . in contrast , although the recurrence - free survival rate of dsv - ptc is lower than that of non - dsv - ptc , there is no difference in the overall survival rate [ 3 , 19 , 20 , 21 ] . the reason for the dissociation between the clinical course and survival should be further investigated . dense accumulations of s-100 protein - positive dendritic / langerhans cells , usually in areas of lymphocytic infiltration   and between tumor cells , correlate with a benign clinical course . thyroid cancer nuclei are sex hormone receptor positive [ 14 , 15 , 16 ] , and er and pgr expression is demonstrated mainly in differentiated thyroid cancer nuclei . however , overexpression of er and pgr in thyroid cancer suggests their role in carcinogenesis . in small t1-differentiated thyroid cancer , er positivity and androgen receptor expression are associated with a more aggressive phenotype . because the present case was er and pgr positive and androgen receptor negative with accumulation of s-100 protein - positive dendritic / langerhans cells , a prognostic prediction based on these markers was complex . more reliable markers , which are necessary in order to determine prognosis , must be investigated . in the present case , although the macis ( metastases , age , completeness of resection , invasion , size ) score   was 7.85 ( table 2 ) , the 20-year survival rate after surgery was estimated as 56% . strict follow - up is desired in such cases . in the present tumor , strong infiltration of lymphocytes with a germinal center although approximately 30% of patients with dsv - ptc have positive antithyroid antibodies as part of hashimoto 's thyroiditis , autoimmune antibodies were not detected in the present case . interestingly , hrthle cells are reportedly absent in dsv - ptc , including in the present case [ 2 , 12 ] . taken together , diagnosis of dsv - ptc seems to be possible using ultrasonography and histological investigation . in conclusion , we herein reported a case of er- and pgr - positive dsv - ptc . although survival remains unpredictable , further studies are necessary to identify good markers with which to predict the prognosis of dsv - ptc . this paper is the first to report er- and pgr - positive dsv - ptc . the authors declare no potential conflicts of interests with respect to the authorship and/or publication of this article .