A 53-year-old African-American male presented to his primary care physician with two months of nasal congestion and recurrent epistaxis. There was no significant past medical or family history. Saline nasal spray and a short course of azithromycin and prednisone were prescribed. However, symptoms continued to progress over the following three months and he was referred to an otolaryngologist. At this time a small nasal mass was noted. Biopsy subsequently revealed a heterologous malignant neoplasm with a neuroendocrine component consistent with Sinonasal Teratocarcinosarcoma (SNTCS). Unfortunately, the patient was unable to be contacted and was lost to follow-up until he presented to the emergency department two months later with complaints of lethargy, anorexia and 40-pound weight loss. Additional symptoms included daily epistaxis, loss of vision in the left eye and odynophagia. Vital signs were within normal limits. BMI was 17.3. Physical exam revealed a cachectic and unkempt individual. A large necrotic mass was seen in the nasal cavity with penetration through the hard palate. Bulging of the right nasal bridge was noted. There was significant proptosis of the left eye with complete loss of all left extraocular movement. Diffuse cervical and clavicular lymphadenopathy was evident. Laboratory was significant for hypercalcemia. MRI of the brain showed extension of the mass into the base of the skull. Given the extent of disease, the patient was deemed inoperable and not a candidate for chemoradiation therapy given his poor functional status. He was transitioned to in-patient hospice. Calcium normalized with the administration of calcitonin, fluids and Zoledronate. Pain was controlled with hydromorphone. He declined PEG tube placement and passed away two weeks thereafter.
Discussion:
SNTCS is a rare and highly aggressive entity with less than 100 reported cases 1. The diagnosis of SNTCS is based on the presence of heterologous histology with components from all three germ layers 2. The most complete literature review to date shows a mean age of diagnosis between 55-60 years with the most common presenting symptoms being nasal obstruction (75.6%) and epistaxis (62.8%) 3,4. There is a consistent 7-8:1 male predominance 3,5. The diagnosis carries a poor prognosis with 3 and 5 year survival rates of 30% and 20% respectively 6. There are no definitive management guidelines given the rarity with which SNTCS occurs. Nevertheless, the majority (59.5%) of cases are treated with surgery and adjuvant radiation therapy 3,7.
This case highlights the aggressive nature of SNTCS and the importance of including SNTCS in the differential diagnosis of any individual with recurrent epistaxis given this malignancy’s aggressive nature and poor prognosis.
Conclusions:
SNTCS is a rare but deadly malignancy that must be considered in the differential diagnosis of any individual who presents with recurrent and unexplained epistaxis.