Pheochromocytoma crisis in a patient with newly diagnosed neurofibromatosis type 1

2021 ◽  
Vol 14 (1) ◽  
pp. e237231
Author(s):  
Hisashi Murakami ◽  
Tomohiro Sonoo ◽  
Hideki Hashimoto ◽  
Kensuke Nakamura

Pheochromocytoma occasionally engenders catecholamine-induced hypertension crisis. Pheochromocytoma is clinically identified in 0.1%–5.7% of patients with neurofibromatosis type 1 (NF1), which is 10 times more frequently than in healthy individuals. This report describes a case of newly diagnosed NF1 presenting with pheochromocytoma crisis, with severe electrolyte depletion and deteriorating recurrent ventricular tachycardia storm. Characteristic skin lesions such as café-au-lait macules and neurofibromas contributed to the diagnosis of NF1 and pheochromocytoma. No recurrence of electrolyte depletion was found after the adrenalectomy. Primary care physicians must distinguish the characteristic skin lesions of NF1, such as café-au-lait macules and neurofibromas and recognise the risk for pheochromocytoma.

2010 ◽  
Vol 11 (2) ◽  
pp. 57-63 ◽  
Author(s):  
Ashu Bhardwaj ◽  
Ajay Mahajan

Abstract Aim The purpose of this article is to describe a rare case of neurofibromatosis1 (NF1) of the gingiva and a review of the current literature. Background Neurofibromatosis1 (NF1) of the gingiva is an uncommon cause of gingival enlargement. The disease is clinically characterized by epidermal melanosis, nevi, and flabby skin or bone malformations; in addition, the lesions may undergo malignant transformation. Involvement of the gingiva with or without concurrent skin lesions has been reported only occasionally. Case Description A 40-year-old male patient with a history of NF1 came to us with a chief complaint of enlargement of the gums. Clinical examination revealed diffuse gingival enlargement with no signs of inflammation. The characteristic skin lesions associated with NF1 were also present. A gingival specimen was sent for biopsy. Results Based on the history, clinical, and histological findings, NF1 was established as the cause of the gingival enlargement. Summary NF1 may affect the gingival tissue; considering the neurological complications and malignant potential, NF1 must be diagnosed early and monitored regularly. Clinical Significance The clinician should be aware of clinical and histopathological findings of NF1 considering the fact that the condition has malignant potential. Citation Mahajan A, Dixit J, Bhardwaj A. Gingival Enlargement in Neurofibromatosis Type 1: A Case Report and Literature Review. J Contemp Dent Pract [Internet]. 2010 March; 11(2):057-063. Available from: http://www.thejcdp. com/journal/view/volume11-issue2-mahajan.


2017 ◽  
Vol 1 (1) ◽  
pp. oapoc.0000004
Author(s):  
Andrea Grosso ◽  
Eric J. Sigler ◽  
John Randolph

Retinal astrocytic hamartomas are rare, benign tumors of glial origin. These lesions are often associated with systemic syndromes, including tuberous sclerosis complex and neurofibromatosis type 1, but also may be encountered in otherwise healthy individuals as an acquired lesion. We present the following case to illustrate clinical and imaging characteristics typically seen in astrocytic hamartoma found as “optic nerve incidentaloma” in an otherwise healthy teenager.


2008 ◽  
Author(s):  
Jonathan M. Kurss ◽  
Anna E. Craig ◽  
Jennifer Reiter-Purtill ◽  
Kathryn Vannatta ◽  
Cynthia Gerhardt

2011 ◽  
Vol 42 (01) ◽  
Author(s):  
F. Mainberger ◽  
N. Jung ◽  
M. Zenker ◽  
I. Delvendahl ◽  
U. Wahlländer ◽  
...  

2014 ◽  
Vol 45 (S 01) ◽  
Author(s):  
J. Keppler ◽  
A. Fiedler

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