Standardized treatment protocols are hindered by the rarity of the PCas. However, literature concludes that optimal debulking is mandatory, whereas the efficacy of adjuvant chemotherapy remains to be elucidated.

Objectives/Hypothesis: Stenosis of the middle meatus antrostomy (MMA) represents a major cause of recurrent disease following endoscopic sinus surgery (ESS). Various strategies have been developed to prevent the occurrence of MMA stenosis. The aim of the present study was to evaluate the effects of spray cryotherapy (SC) on nasal wound healing following ESS. Methods: This is a prospective within-subject, randomized, and controlled trial. Twenty-six patients submitted to bilateral ESS with chronic rhinosinusitis without polyps were included. Following surgery, patients were randomized to receive SC on one side and saline contralaterally. Outcomes were represented by MMA diameter and area, histology of nasal mucosa, and nasal symptoms. Variables were assessed at 3 and 12 months postoperatively. Results: The MMA size in the SC group at 3 and 12 months (area-0.578 ± 0.1025 cm 2 , diameter-0.645 ± 0.1024 cm; 0.605 ± 0.1891 cm 2 , 0.624 ± 0.0961 cm, respectively) was significantly larger (p = 0.000) than in the control group. Histology established that cell infiltration, goblet cells, edema, and epithelial hyperplasia were prominent and persistent in the control side compared to the SC side. Nasal obstruction and discharge were significantly improved in the SC group compared to the control group. Conclusion: SC is a promising therapy following ESS, since it precludes MMA stenosis and decreases inflammation, edema, and goblet cell hyperplasia.

Pheochromocytoma is a rare neuroendocrine tumor that arises from the chromaffin cells of the sympathetic nervous system. Over one third of pheochromocytomas are associated with germline mutations. We describe a 3 year-old girl with an inherited right adrenal malignant pheochromocytoma, with the mother diagnosed with von Hippel-Lindau syndrome. Genetic tests revealed the presence of the VHL c 244 C>G (p. Arg 82 Gly) heterozygote mutation in the mother, as well as in the child. After 6 months from the complete resection of the tumor, the patient is without any clinical symptoms, with normal blood pressure, normal ophthalmoscopy, no tumor markers and no evidence of tumor on cerebral or abdominal MRI. Lifelong complex follow-up is needed, as it is known that at a later age VHL mutation may cause retinal angiomas, cerebellar and spinal hemangioblastomas, relapsed pheocromocytoma, pancreatic and renal cysts, clear cell renal cell carcinoma and endolymphatic sac tumors.

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