Systemic and Craniospinal Rosai Dorfman Disease with Intraparenchymal, Intramedullary and Leptomeningeal Disease

Rosai Dorfman disease is a rare histiocytic disorder of over-production of non-Langerhans histiocytes, which typically manifests with massive lymphadenopathy and sinonasal involvement. We report a rare case of systemic and disseminated craniospinal Rosai-Dorfman disease with intraparenchymal and leptomeningeal involvement, but no sinus or dural-based disease. The diagnosis was established by biopsy of a hypothalamic mass. Additionally, UCSF500 Next Generation Sequencing demonstrated a solitary pathogenic alteration affecting the BRAF oncogene, which supports the morphologic and immunohistochemical diagnosis of Rosai-Dorfman disease.

Frontal Sinus Schwannoma: Case Report and Literature Review

Whilst schwannomas are mostly developed in the head neck area, the sinonasal involvement is extremely rare, especially those of the frontal sinus. We report a patient with sinonasal schwannoma, invading both frontal sinuses, anterior cranial fossa, the left anterior ethmoidal sinus, and the left nasal cavity. Radiologic imaging showed an isodensed, T2 isointense, T1 hypointense, with heterogeneous enhanced postcontrast mass with bone destruction. The patient underwent resection of the tumor via two approaches, external one and endonasal one. The histological diagnosis was consistent with a schwannoma. The follow-up was marked by the presence of diplopia without any signs of meningitis or cerebrospinal fluid rhinorrhoea.

Sarcoidosis Causing Complete Nasal Obstruction

Our case demonstrates the rare presentation of sinonasal sarcoidosis causing severe nasal obstruction. While the patient had a remote history of pulmonary sarcoidosis, she was in remission and had no prior history of sinonasal involvement. Sarcoidosis should be considered in a patient with nasal obstruction especially when there is a history of systemic sarcoid disease.

Sinonasal inflammatory myofibroblastic tumour: a rare entity with diagnostic challenges

Inflammatory myofibroblastic tumour is a rare entity causing sinonasal involvement with variable behaviour. Mimicking various benign and malignant lesions, accurate diagnosis is often clinched on histopathology complemented with appropriate immunohistochemistry markers. Surgical resection is the main treatment modality with other forms of therapy reserved for unresectable lesions. We highlight a case of dual involvement of the sinonasal region and nasal bones along with the diagnostic and treatment challenges encountered. As the nasal bones were involved, surgical resection with negative margins required cosmetic reconstruction in the same sitting. A costochondral graft helped in achieving cosmetic pleasing results with no recurrence on follow-up.