Aggressive macroprolactinomas represent 0.4 to 4% of pituitary adenomas, they are more frequent in men, the positive diagnosis is easy, the evaluation of aggressiveness is essentially based on MRI and The treatment with dopaminergic agonist in first intention is now well established and should not be deferred apart from neurological emergency situations.
Primary sinonasal neuroendocrine carcinoma (SNEC) is a rare aggressive sinonasal malignancy which typically occurs in the ethmoidal bone or sinuses, with a slight male preponderance. No risk factors have been identified. With a very few reported cases. Most patients present in advanced stages due to the lack of significant symptoms. Nomenclature has been ambiguous, but SNECs can be classified as well-, moderately- or poorly differentiated. Its treatment is not well established. We report the case of a large cell neuroendocrine carcinoma (LCNEC) in a 34 years old patient presented the occurrence of a right epistaxis. The beginning of the symptomatology goes back to 1 year before, with right nasal obstruction of progressive onset, associated with right epistaxis and bloody rhinorrhea with right chronic eye watering. Nasosinusal MRI shows a right nasal mass enhanced strongly after injection of gadolinium, extension to contralateral ethmoidal cells with orbital bone lysis and cribriform plate of the Ethmoid bone. The Histopathologic analysis of the mass biopsy showed a poorly differentiated carcinomatous tumor proliferation with positive neuroendocrine markers. The multidisciplinary decision taken was the treatment by chemotherapy before surgery.
Pituitary adenomas (PA) are benign well-differentiated tumors, with monoclonal development from the anterior pituitary gland, which can result in serious complications, in particular endocrine, metabolic and visual. Mixed ACTH and prolactin- secreting pituitary adenomas (PA) represent a rare association. We report a rare case of a patient who has Cushings disease with macroadenomaand the immunohistochemistry surprisinglyrevealed a co-expression of ACTH and PRL, with a silent prolactin lineage. She was a 41 years old female, admitted with aCushing syndrome ACTH-dependent of 73.63pg/ml, complicated with diabetes, dyslipidemia and hypertension. The hypothalamic-pituitary MRI revealed an 11.4x7 mm minimally invasivemacroadenoma,and thevisual fieldwas slightly reduced in both eyes. Her PRL level was normal. She had undergone transsphenoidal removal of the tumor. The immunohistochemical study showed90% expression of ACTHantibodies and 10% of PRL antibodies, the KI67 proliferation index was at 5%. The outcomes showedregression of clinical syndrome with persistent biological cortisolic activity, and the MRI performed 8 months later was normal. Immunohistochemistry remains the key element in diagnosing pituitary tumors and orientating their management. Mixed adenomas, in particularACTH co-secreting adenomas are difficult to manage because of their higher risk of recurrence and their secretory features.
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