A 22 y.o. woman presents with a chief complaint of "blue things seem to be neon colored and too bright"

She was seen in clinic and sent to the ED for MRI when the ophthalmologist noted temporal field cuts.

there appears to be a mass in the frontal lobe in the midline

and the mass extends down into the sinuses

The initial differential included meningioma, esthesioneuroblastoma, lymphoma, rhabdomyosarcoma, and embryonal tumor.

 A biopsy was done by ENT showing our patient had a low/intermediate grade spindle cell sinonasal sarcoma/meningioma. This dual nature of some sinonasal tumors has been described as showing dual differentiation with both myogenic and neural elements. On pathologic assessment they contain both S-100 and actin.  On the hematoxylin-eosin stain there is a proliferation of uniform spindle cells in fascicles. The differential here still includes  biphenotypic sinonasal sarcoma (most likely), solitary fibrous tumor, meningioma and synovial sarcoma. The way they are pathologically differentiated is with immunophenotyping .  The  biphenotypic sinonasal sarcomas tend to be low grade but half have become locally invasive.

While biphenotypic sinonasal sarcoma is rare  with the largest series containing only 95 individuals, other midline tumors also exist.

Lethal midline granuloma- this was the old term for an aggressive tumor which destroys the nose and other structures of the respiratory passages. Thiese are NK/T cell lymphomas. The previous mortality was 100% because of perforation into the blood vessel and brain leading to sepsis. Currently, the strategy for treatment is radiation followed by chemotherapy.  The tumors are often originate in the palate and are found in patients of  East Asian descent.

lymphoma aka “lethal midline granuloma”

Squamous cell Carcinoma,

Adenocarcinoma

Adenoid cystic carcinoma

Melanoma

Esthesioneuroblastoma-grows in the nerve cells of the nose and affect the sense of smell.

Our patient is having surgery today and depending on the path will be receiving radiation.

Chitguppi C, Koszewski I, Collura K, et al. Biphenotypic sinonasal sarcoma-case report and reviw of clinicopathological features and diagnostic modalities. J Neurol Surg B Skull Base 2019 Feb;80:51-58.

Mendenhall W, Olivier K, Lynch J, et al. Lethal midline granuloma-nasal natural killer/T cell lymphoma. Am J Clin Oncol 2006;29:202-6. 

Mallya V, Singh A, Pahwa M. Lethal midline granuloma. Indian Dermatol Online J. 2013 Jan-Mar;4(1):37-39.