sinonasal teratocarcinosarcoma ( sntcs ) is a very unusual and aggressive neoplasm first characterized as a separate entity by heffner and hyams in 1984.1 it is currently defined by the world health organization as  a complex malignant sinonasal neoplasm combining features of teratoma and carcinosarcoma  that typically includes benign and malignant epithelial , mesenchymal , and neuroepithelial elements including immature tissue with blastomatous features.2 unlike other malignant mixed germ cell tumors ( gcts ) , sntcs lacks embryonal carcinoma , yolk sac tumor , choriocarcinoma , and seminoma components .
based on an inadequate sample , this heterogenous tumor can be misdiagnosed as olfactory neuroblastoma , squamous cell carcinoma , undifferentiated carcinoma , adenocarcinoma , malignant salivary gland - type tumors , or adenosquamous carcinoma.2 a recent comprehensive review of the literature by wei et al revealed  <  60 cases reported in the english literature.3 the primary treatment in 82% of the cases reviewed was surgery , with or without adjuvant radiation therapy ( rt ) .
there were high rates of recurrence and metastasis overall , although no clear prognostic factors were identified .
overall , 1-year and 3-year survival rates were 36% and 25% , respectively , for those patients with reported outcomes.3 of all the reported cases of sntcs , only one has shown any hormone secretion : antidiuretic hormone , causing hyponatremia.4 in that case , sodium levels were a reliable marker of tumor control and were an early postoperative indicator of tumor recurrence . to date , no sntcs with elevated gct markers has been reported .
we present a case in which a highly radiosensitive sntcs was associated with elevated -hcg .
a 22-year - old african american woman presented to the emergency department at  16 weeks of gestation with a 2- to 3-month history of worsening headaches and a 3-week history of increasing somnolence , nausea , vomiting , weight loss , and personality changes .
a magnetic resonance imaging ( mri ) of her head revealed an 11-cm right frontal mass with a 7-cm cystic component causing significant compression of the right frontal and temporal lobes , mild herniation of the right uncus , and mild hydrocephalus .
the tumor extended through the anterior cranial base to the nasal cavity and paranasal sinuses ( fig .
noncontrast magnetic resonance imaging of the head showing a large cystic mass in the paranasal sinuses and frontal lobe . on day 2 of admission , she had rapid mental status decline and underwent an emergent decompressive craniotomy .
the tumor was extra - axial , and the cyst expressed a dark fluid  similar to crankcase oil .  the small biopsy specimen taken during this procedure revealed neoplastic cells with squamous and basaloid differentiation and necrosis , leading to the impression of basaloid squamous cell carcinoma .
she subsequently underwent a combined endoscopic transnasal and cranial resection of the entire anterior cranial fossa and nasal cavity mass on day 28 of admission . the surgical specimen consisted of a 9.0    8.5    3.5-cm aggregate of tan - brown soft tissue and bony fragments .
histologically , the tumor consisted of a heterogeneous mixture of malignant epithelial , mesenchymal , and primitive neuroepithelial elements .
tumor infiltration of brain tissue was observed . the epithelial component included squamous and basaloid carcinoma features ( fig .
the mesenchymal component included areas of hypercellular smooth muscle , ranging from mildly anaplastic to overtly malignant , with focal areas of osteoid deposition ( fig .
3 ) . the neuroepithelial component included neuroblastoma - like and primitive neuroectodermal tumor - like areas ( fig .
immunohistochemical staining revealed expression of vimentin , pancytokeratin , and 34e12 , with focal positivity for cd99 and synaptophysin among the heterogeneous tumor components .
( a ) stromal smooth muscle with mitoses ( hematoxylin and eosin [ h&e ] ) . ( b ) osteoid
( lower right ) admixed with epithelial component ( upper left ) ( h&e ) . 

( b ) primitive neuroectodermal tumor  like area ; hematoxylin and eosin ; synaptophysin ( inset ) .
postoperative rt was recommended after surgery but was delayed for 5 months due to a complicated hospital course and family concerns about her pregnancy .
the radiation planning mri showed some residual soft tissue thickening and gyral enhancement in the floor of the craniotomy site but no gross tumor .
after delivery of a healthy infant , she underwent a 2-month course of rt to the surgical bed to a total dose of 60 gy .
the follow - up mris showed no evidence of recurrent disease for 18 months post - rt .
two years ( 27 months ) after finishing her postoperative rt , she was admitted for evaluation through the emergency department following an alleged sexual assault by a family member .
her quantitative serum -human chorionic gonadotropin ( hcg ) was elevated ( 60 u / l ; normal nonpregnant range : 03 ) but did not rise as would be expected for pregnancy .
mri of the head showed a recurrent intracranial mass in the right frontal lobe ( fig .
she was deemed a high surgical risk , so reirradiation of the tumor was recommended .
radiation was delayed by 45 days , and the planning mri showed significant interval growth of the tumor with further elevation of -hcg to 130 u / l ( fig .
( a ) magnetic resonance imaging ( mri ) showing frontal lobe recurrence 27 months after radiation therapy .
( b ) mri showing tumor growth 45 days later , before reirradiation . ( c )
mri showing complete response of frontal lobe mass ( and new posterior fossa disease ) 1  month after reirradiation .
because of clinical concern over the origin of the serum -hcg elevation , the previous tumor tissue was examined with additional immunohistochemical staining for smooth muscle actin and -hcg expression .
the patient received 48  gy ( of a planned 50 gy ) of rt to the recurrent mass in 24 fractions .
she missed many appointments but finished most of her prescribed course in a 6-week period with no acute morbidity . computed tomography scan after the 10th fraction showed significant tumor response , and the treatment was replanned to account for the smaller tumor volume .
her serum -hcg level dropped from a peak of 130 u / l , measured at the start of rt , to 6 u / l ( almost within normal limits ) at the end of treatment ( fig .
7 ) , 
 serum -human chorionic gonadotropin levels before and after radiation therapy ( rt ) for recurrence in frontal lobe .
one month later she was admitted to the hospital again for increasing ataxia and falls .
5c ) but revealed at least five intracranial masses in the posterior fossa , outside of the prior rt fields . serum -hcg level was in the normal range .
the decision was made to palliate with 30  gy of rt to the posterior fossa in 10 fractions .
by definition , sntcs do not contain areas of embryonal carcinoma , choriocarcinoma , yolk -sac tumor , or seminoma .
they are hypothesized to arise from primitive pluripotent olfactory epithelial cells rather than germ cells .
thus they are traditionally believed to be incapable of producing gct markers including -hcg or -fetoprotein , although they have rarely been noted to produce pituitary hormones.4 these tumors comprise varying combinations of epithelial , mesenchymal , and neuroectodermal components exhibiting varying maturation
. the mesenchymal component may appear along a continuum from benign to malignant and can include mature components such as cartilage , bone , and striated or smooth muscle .
malignant sarcomatous transformation is also documented.1 
 5 
 6 
 7 
  in this case , smooth muscle actin highlighted an anaplastic smooth muscle component in this heterogeneous tumor .
there are rare reports of pure leiomyosarcomas secreting -hcg in the literature.8 
 9 
 10 in the absence of the gct components just mentioned , the neoplastic smooth muscle component of the tumor with expression of -hcg was the likely source for the elevated serum -hcg levels . because this patient was pregnant on initial presentation , any tumor - related -hcg production at that time
however , on recurrence , tumor production of -hcg was confirmed with elevation of serum -hcg , expression of -hcg on the smooth muscle component of the tumor tissue , and correlation of serum -hcg levels with changes in tumor size seen on imaging .
this patient 's recurrent tumor initially showed a complete response to rt by both serum marker and imaging criteria .
subsequent tumor growth outside of the rt field was not associated with elevation of serum -hcg .
sntcs is a rare and unusual neoplasm that lacks embryonal carcinoma , yolk sac tumor , choriocarcinoma , and seminoma components .
the source of -hcg production was traced to a neoplastic smooth muscle component of the tumor .
serum levels of this hormone were a reliable tumor marker during the patient 's first recurrence and subsequent rt .
 
 background and importance  sinonasal teratocarcinosarcoma ( sntcs ) is an unusual and aggressive neoplasm characterized by the combination of teratoma and carcinosarcoma features that is often misdiagnosed based on a biopsy sample . 
 approximately 60 cases have been reported in the english literature , but none have been associated with serum tumor markers . 
 we report a case of sntcs with elevation of serum -human chorionic gonadotropin ( hcg ) level . 
 
 clinical presentation  the patient was a 22-year - old pregnant woman at diagnosis , and her -hcg level was appropriately elevated . 
 her initial treatment was a surgical resection followed by radiation therapy ( rt ) . 
 two years later , the patient had a pregnancy work - up after a sexual assault . her serum -hcg was elevated , but a nongravid uterus was seen on ultrasound . magnetic resonance imaging ( mri ) of the head revealed an intracranial tumor recurrence in the right frontal lobe . during salvage rt , -hcg decreased rapidly to normal levels , with a complete tumor response on mri . 
 retrospective examination of the initial surgical specimen showed expression of -hcg associated with the anaplastic mesenchymal component . 
 
 conclusion  this is the first reported case of a -hcg secreting sntcs . the source of -hcg production was traced to a neoplastic smooth muscle component of the tumor . 