Nevus Sebaceous of the Scalp With Stepwise Progression Into Invasive Syringocystadenocarcinoma Papilliferum

2021 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Hermann Kneitz ◽  
Matthias Goebeler ◽  
Arno Rütten
Keyword(s):  
Nevus Sebaceous

scholarly journals The Role of KRAS Mutations in Cortical Malformation and Epilepsy Surgery: A Novel Report of Nevus Sebaceous Syndrome and Review of the Literature

2021 ◽  
Vol 11 (6) ◽  
pp. 793
Author(s):  
Chiara Pepi ◽  
Luca de Palma ◽  
Marina Trivisano ◽  
Nicola Pietrafusa ◽  
Francesca Romana Lepri ◽  
...  
Keyword(s):  
Epilepsy Surgery ◽  
Focal Cortical Dysplasia ◽  
Hippocampal Sclerosis ◽  
Brain Mri ◽  
Seizure Freedom ◽  
Histopathological Diagnosis ◽  
Cortical Malformation ◽  
Cognitive Improvement ◽  
Cortical Malformations ◽  
Nevus Sebaceous

The rare nevus sebaceous (NS) syndrome (NSS) includes cortical malformations and drug-resistant epilepsy. Somatic RAS-pathway genetic variants are pathogenetic in NS, but not yet described within the brain of patients with NSS. We report on a 5-year-old boy with mild psychomotor delay. A brown-yellow linear skin lesion suggestive of NS in the left temporo-occipital area was evident at birth. Epileptic spasms presented at aged six months. EEG showed continuous left temporo-occipital epileptiform abnormalities. Brain MRI revealed a similarly located diffuse cortical malformation with temporal pole volume reduction and a small hippocampus. We performed a left temporo-occipital resection with histopathological diagnosis of focal cortical dysplasia type Ia in the occipital region and hippocampal sclerosis type 1. Three years after surgery, he is seizure-and drug-free (Engel class Ia) and showed cognitive improvement. Genetic examination of brain and skin specimens revealed the c.35G > T (p.Gly12Val) KRAS somatic missense mutation. Literature review suggests epilepsy surgery in patients with NSS is highly efficacious, with 73% probability of seizure freedom. The few histological analyses reported evidenced disorganized cortex, occasionally with cytomegalic neurons. This is the first reported association of a KRAS genetic variant with cortical malformations associated with epilepsy, and suggests a possible genetic substrate for hippocampal sclerosis.

Tc-99m HMPAO brain SPECT in linear nevus sebaceous syndrome

2003 ◽  
Vol 17 (8) ◽  
pp. 703-706 ◽  
Author(s):  
Abdullah Özkiriş ◽  
Cem Evereklioglu ◽  
Mustafa Kula ◽  
Mehmet Somdaş ◽  
Kuddusi Erkiliç ◽  
...  
Keyword(s):  
Brain Spect ◽  
Nevus Sebaceous

LINEAR NEVUS SEBACEOUS SYNDROME: REPORT OF TWO CASES AND A REVIEW OF THE LITERATURE

PEDIATRICS ◽  
1973 ◽  
Vol 52 (3) ◽  
pp. 382-387
Author(s):  
Frederick H. Lovejoy ◽  
William E. Boyle
Keyword(s):  
Cerebrospinal Fluid ◽  
Neurologic Disease ◽  
Review Of The Literature ◽  
Cutaneous Lesions ◽  
Neurocutaneous Syndromes ◽  
Nevus Sebaceous ◽  
Cerebrospinal Fluid Protein ◽  
Syndrome Report

Two cases of linear nevus sebaceous syndrome are described and a review of the eleven cases now reported in the literature is undertaken. The first patient has retardation, seizures, and classic ectodermal lesions while the second patient manifests typical cutaneous lesions and only an elevated cerebrospinal fluid protein as evidence of neurologic disease. The rationale for defining the syndrome as an entity distinct from other neurocutaneous syndromes is discussed and a pleomorphic presentation of the syndrome is suggested.

scholarly journals Therapeutic effects of CO2 laser therapy of linear nevus sebaceous in the course of the Schimmelpenning-Feuerstein-Mims syndrome

2013 ◽  
Vol 5 ◽  
pp. 320-323 ◽  
Author(s):  
Magdalena Kiedrowicz ◽  
Anna Kacalak-Rzepka ◽  
Andrzej Królicki ◽  
Romuald Maleszka ◽  
Stanisława Bielecka-Grzela
Keyword(s):  
Laser Therapy ◽  
Co2 Laser ◽  
Therapeutic Effects ◽  
Nevus Sebaceous

scholarly journals Simultaneous Presentation of Trichilemmal Carcinoma and Syringocystadenoma Papilliferum within a Nevus Sebaceous

2018 ◽  
Vol 30 (3) ◽  
pp. 368
Author(s):  
Seung Hyun Chun ◽  
Bo Young Kim ◽  
Ji Hyun Park ◽  
Il-Hwan Kim ◽  
Hwa Jung Ryu
Keyword(s):  
Simultaneous Presentation ◽  
Syringocystadenoma Papilliferum ◽  
Nevus Sebaceous

scholarly journals A unexpected growth arising within nevus sebaceous of Jadassohn

2016 ◽  
Vol 22 (1) ◽  
Author(s):  
SA Chan ◽  
R Hejmadi ◽  
K Webster ◽  
MR Kaur
Keyword(s):  
Nevus Sebaceous

scholarly journals Trichoscopy: The best auxiliary tool in the evaluation of nevus sebaceous

2016 ◽  
Vol 8 (1) ◽  
pp. 5 ◽  
Author(s):  
BalachandraS Ankad ◽  
SavithaL Beergouder ◽  
Vijay Domble
Keyword(s):  
Nevus Sebaceous

Simultaneous Occurrence of two Squamous Cell Carcinomas within a Nevus Sebaceous of Jadassohn in an 11-Year-Old Girl

2009 ◽  
Vol 26 (2) ◽  
pp. 236-237 ◽  
Author(s):  
HICHEM BELHADJALI ◽  
ADNENE MOUSSA ◽  
SAMIRA YAHIA ◽  
LEILA NJIM ◽  
ABDELFATTAH ZAKHAMA ◽  
...  
Keyword(s):  
Squamous Cell ◽  
Squamous Cell Carcinomas ◽  
Simultaneous Occurrence ◽  
Nevus Sebaceous

CD10, TDAG51, CK20, AR, INSM1, and Nestin Expression in the Differential Diagnosis of Trichoblastoma and Basal Cell Carcinoma

2018 ◽  
Vol 27 (1) ◽  
pp. 19-27 ◽  
Author(s):  
Cem Leblebici ◽  
Buket Bambul Sığırcı ◽  
Canan Kelten Talu ◽  
Sevim Baykal Koca ◽  
Gülben Erdem Huq
Keyword(s):  
Differential Diagnosis ◽  
Basal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Basal Cell ◽  
The Other ◽  
Cytokeratin 20 ◽  
Nestin Expression ◽  
The Difference ◽  
Nevus Sebaceous ◽  
Sensitive Marker

Background. Trichoblastoma (TB) and basal cell carcinoma (BCC) are 2 different neoplasms composed of basaloid cells and have overlapping histopathological features. We compared the immunoexpression of CD10, T-cell death-associated gene 51 (TDAG51), cytokeratin 20 (CK20), androgen receptor (AR), insulinoma-associated protein 1 (INSM1), and nestin for the differential diagnosis of these tumors. Materials and Methods. We assessed a total of 27 BCC and 27 TB cases, including 4 TB lesions in nevus sebaceous and 3 malignant TB lesions for CD10, TDAG51, CK20, AR, INSM1, and nestin expression. Results. Staining for CK20, TDAG51, INSM1, and stromal CD10 was significantly more common in TB cases than in BCC cases ( P < .001). Epithelial CD10 and AR staining was significantly more common in BCC cases than in TB cases ( P < .001). The difference between the groups for nestin staining was not significant ( P > .05). Stromal CD10 staining was the most sensitive marker (96.3%) and INSM1 the least sensitive (55.6%) marker for TB. TDAG51 showed 100% specificity for TB. A larger number of CK20 positive cells was found in the cases associated with nevus sebaceous than in the other TBs. Conclusion. All the selected markers except nestin were useful for the differential diagnosis between TB and BCC. CD10 and TDAG51 were more useful than the other markers. The use of CK20 could be preferred in nevus sebaceous lesions. INSM1 was less effective in highlighting Merkel cells within the lesion than CK20.

Sign in / Sign up

Export Citation Format

Share Document