Multiple Abdominal Cysts in a Patient with Gorlin-Goltz Syndrome: a Case Report

Abstract

A rare case of symptomatic mesenteric cysts in a patient with Gorlin-Goltz syndrome, associated with various neoplasms, is presented. The patient, known with Gorlin-Goltz syndrome, consulted with increasingly severe abdominal pain and large abdominal cysts. At surgery, the cysts were excised and the postoperative course was uneventful. In conclusion, this case reminds clinicians to always maintain a wide differential diagnosis when dealing with patients known with Gorlin-Goltz syndrome.

A guideline for the clinical management of basal cell naevus syndrome (Gorlin–Goltz syndrome)*

“…The presence of (lympho)mesenteric cysts is a minor diagnostic criterion with an unknown specific frequency 14 . Only a few case reports have been published, but these types of cysts are probably under‐reported in patients with BCNS 78,79 . (Lympho)mesenteric cysts are benign, intra‐abdominal tumours which are usually asymptomatic and often an occasional finding, although cases with abdominal pain have been reported 80 .…”

“…The cysts can be seen on ultrasound examination, MRI and CT, but for definitive diagnosis, histological examination is necessary 80 . Surgical excision of cysts has been performed, but it is unknown whether this leads to a decrease in abdominal complaints 78,80 .

Physicians should screen for (lympho)mesenteric cysts using ultrasound examination in patients with BCNS who report unexplained abdominal pain.

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“… 14 Only a few case reports have been published, but these types of cysts are probably under‐reported in patients with BCNS. 78 , 79 (Lympho)mesenteric cysts are benign, intra‐abdominal tumours which are usually asymptomatic and often an occasional finding, although cases with abdominal pain have been reported. 80 The cysts can be seen on ultrasound examination, MRI and CT, but for definitive diagnosis, histological examination is necessary.…”

“… 80 Surgical excision of cysts has been performed, but it is unknown whether this leads to a decrease in abdominal complaints. 78 , 80

Physicians should screen for (lympho)mesenteric cysts using ultrasound examination in patients with BCNS who report unexplained abdominal pain. (GEC, very low; GSoR, strong)

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Radiological evaluation of odontogenic keratocysts in patients with nevoid basal cell carcinoma syndrome: A review

“…Nevoid Basal Cell Carcinoma Syndrome (NBCCS) is an autosomal dominant syndrome that has different various expressions in each patient ( Curatolo et al, 2013 , Daneswari and Reddy, 2013 , Fini et al, 2013 , Grundig et al, 2013 , Haenen et al, 2013 , Mohan et al, 2013 , Pol et al, 2013 , Rambocas and Murphy, 2013 , Saulite et al, 2013 , Budincevic et al, 2014 , De Craene et al, 2014 , Friedrich, 2014 , Inani and Mernissi, 2014 , Keceli et al, 2014 , Khan et al, 2014 , Kulkarni et al, 2014 , Larsen et al, 2014 , Mehta et al, 2014 , Mufaddel et al, 2014 , Patankar et al, 2014 , Shephard and Coleman, 2014 , Tarnoki et al, 2014 , Abreu et al, 2015 , Anchlia et al, 2015 , Chandran et al, 2015 , da Silva Pierro et al, 2015 , Galati et al, 2015 , Ganguly et al, 2015 , Grechi et al, 2015 , Hajalioghli et al, 2015 , Lata et al, 2015 , Lazaridou et al, 2015 , Majdoub et al, 2015 , Manjima et al, 2015 , Ojevwe et al, 2015 , Pickrell et al, 2015 , Ramesh et al, 2015 , Hubacek et al, 2016 , Khaliq et al, 2016 , Ozcan et al, 2016 , Ponti et al, 2016 , Ribeiro et al, 2016 , Scalise et al, 2016 , Tandon et al, 2016 , Thomas et al, 2016 , Virgone et al, 2016 , Casari et al, 2017 , da Paz Oliveira et al, 2017 , Mendes-Abreu et al, 2017 , Mendes-Bastos et al, 2017 , Nilesh et al, 2017 , Pennisi et al, 2017 , Sereflican et al, 2017 , Trento et al, 2017 , …”